JBR 2011-1

Page 1

Wetteren 1

1

P 702083

volume 94 Page 1-58

January-February

Bimonthly

2011

diagnostic and interventional imaging, related imaging sciences, and continuing education

Organe de la societe royale belge de radiologie (srbr) orgaan van de koninklijke belgische vereniging voor radiologie (kbvr) Project couv-2011.indd 1

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01-JBR-contents-11-1(dr)_Opmaak 1 16/02/11 11:19 Pagina 1

JBR-BTR ♦ 94/1 ♦ 2011 Journal Belge de ♦ Belgisch Tijdschrift voor ♦ RADIOLOGIE

Founded in 1907 A bimonthly journal devoted to diagnostic and interventional imaging, related imaging sciences, and continuing education Contents The value of proton MR-spectroscopy in the differentiation of brain tumours from non-neoplastic brain lesions. H. Aydın, S. Sipahiog˘lu, N. Aydın Oktay, E. Altın, V. Kızılgöz, B. Hekimoglu . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Pleuropulmonary blastoma presenting as a complicated pleural effusion. J. O’Brien, D. Rea, R. Hayes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Primary pelvic hydatid cyst with sciatic compression. F. Nouira, T. Chouikh, A. Charieg, S. Ghorbel, S. Jlidi, B. Chaouachi . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Uterus didelphys with obstructed hemivagina and renal agenesis: MRI findings. A. Talebian Yazdi, K. De Smet, C. Ernst, B. Desprechins, J. de Mey . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Acute adrenal insufficiency due to bilateral adrenal hemorrhage. X. Zhu, I.C. van der Schaaf, J.A. van der Valk, A.K. Bartelink, M. Nix . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Hepatic alveolar echinococcosis: a diagnostic challenge. O. Yapici, S.M. Erturk, M. Ulusay, A. Ozel, A. Halefoglu, Z. Karpat, M. Basak . . . . . . . . . . . . . . . . . . . . . . . . . . . . Wernicke’s encephalopathy: a case report and MRI findings. S. Yucebilgin, T. Cirpan, C.Y. Sanhal, E. Ozan, T. Acar, S. Ozsener . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Diffuse “vertebra-within-vertebra” appearance at the adult age due to biphosphonate (pamidronate) administration during early adolescence. B. Coulier . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Incidental radiological finding of a renal tumour leading to the diagnosis of Birt-Hogg-Dube syndrome. M. Schreuer, M. Lemmerling, W. Pauwels, D. Dewilde, C. Heyse, K.L. Verstraete . . . . . . . . . . . . . . . . . . . . . . . .

3 13 15 18 21 23 26

28 31

LETTER TO THE EDITOR R. D’Hauwe, E. Lerut, L. De Wever, R. Oyen, F. Claus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

34

IMAGES IN CLINICAL RADIOLOGY Pedunculated pleural lipoma. L. Cardinale, F. Avogliero, F. Solitro, C. Fava . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Denim Sandblasters’ Pneumoconiosis. M. Apaydin, M. Varer, S. Ayik . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Dilated cisterna chyli. F.M. Vanhoenacker, M. Eyselbergs, C. Petre . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Iatrogenic facial subcutaneous emphysema after endodontic treatment. J. Coulier, F.C. Deprez . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . The ‘torus palatinus’: a common but relatively unknown entity. C. Boulet, M. De Maeseneer, T. Buisseret, M. Shahabpour, J. De Mey . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . An unusual pelvic mass: bladder lymphoma. M. Alsinnawi, M. Quinlan, A. Brady, N. Khan . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . The European day of Radiology (EDOR): an initiative of the ESR . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . News from the Universities: Wetenschappelijke Prijs em Prof. Dr A.L. Baert . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Book presentation: De eerste wereldoorlog in Belgïe – Radiologie in “Trench coat” / La grande guerre de 1914-1918 – La radiologie belge monte au front. R. Van Tiggelen . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Forthcoming courses and meetings . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Classified services . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Grants of the RBRS . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Instructions to Authors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Subscribers information . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Advertising index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

37 38 39 40 41 42 43 44 45 54 55 56 ii cii ciii

The terms used for indexation of subjects were developed by the Radiological Society of North America (RSNA) over a period of years. Their use here is by permission of the RSNA. The terms may not be used in any other index, print or electronic, except by specific permission of RSNA. ◆◆ Indexed in Index Medicus and in Zentralblatt Radiologie. Evaluated for Medline User, EMBASE and CANCERNET. Abstracted in Excerpta Medica Journals. ◆


EDOR (Radiation)_Opmaak 1 9/02/11 09:52 Pagina 1

JBR–BTR, 2011, 94: 1-2.

CELEBRATE THE POWDER OF IMAGING: THE EUROPEAN DAY OF RADIOLOGY

Radiation exposure in medicine: what you need to know Imaging is an indispensable tool in modern medicine, yet very few patients know just how important it is. From cancer detection and therapy to diagnosing stroke or serious trauma in time, radiologists contribute to saving lives by covering every field of medicine. To raise public awareness, the European Society of Radiology will launch the European Day of Radiology on February 10, marking the anniversary of x-ray discoverer Wilhelm Conrad Röntgen's death. Most European national societies have joined this initiative, including the Royal Belgian Radiological Society, which has chosen to address the sensitive topic of radiation exposure. Over the last decade, technical advances in computed tomography (CT) have been considerable. CT examinations, which use x-rays and a computer to provide 3-dimensional and slice images of the inside of the body, are now very fast and accurate. For instance, CT can now acquire excellent images of the entire body in less than 20 seconds. In patients suffering polytrauma, this enables the quick overview of possibly life-threatening pathologies and increases chances of survival. CT has also improved the detection of many cancers, significantly extending survival time. Moreover, many abdominal diseases are now easily diagnosed and assessed with CT, preventing unnecessary surgery in many cases. Finally, CT has become the standard imaging modality in diagnosing pulmonary embolism and is also gradually replacing cardiac catheterisation for some heart diseases. All these benefits have translated into a massive rise in CT examinations worldwide over the past twenty years. As a result, the population’s cumulative exposure to ionising radiation, a substance suspected of causing cancer in humans, has grown – and it may continue to grow in the future. Ionising radiation is an integral part of all imaging methods

that use x-rays, such as CT or radiography. The dose provided by a CT examination depends on the region on which it focuses. A chest CT generates on average 7 mSv (millisievert – a derived unit of dose equivalent that attempts to reflect the biological effects of radiation), head CT, 2 mSv, and abdomen/ pelvic CT, 10 mSv. The annual exposure for an average person is about 3.6 mSv, 50 percent of which comes from natural sources of radiation contained in water, food, some materials and the atmosphere. The remaining 50 percent results from exposure to artificial radiation sources, such as industrial sources like smoke detectors, a small fraction from nuclear weapons tests, and medical sources – 60% of which are induced by CT examinations. However, it remains very difficult to assess the cancer risk induced by a CT examination, as the majority of estimates so far are based on scientific studies of atomic bomb survivors in Japan. Every patient has a different sensitivity to radiation exposure, but some factors are particularly significant. Children and young patients are more sensitive than adults, for example. “Fortunately, the number of CT examinations performed in children is very low, especially when scanning the body. Belgian radiologists always consider all alternatives to CT prior to performing such examinations on children”, said Doctor Denis Tack, radiologist at the Clinique Louis Caty, Baudour Hospital. Females are also at slightly higher risk, depending of the type of CT scan (e.g. CT of the thorax). Finally, the type of CT scan has a direct influence on the exposure level. Whenever possible, one performs examinations with non-ionising modalities such as magnetic resonance imaging (MRI), which uses magnetic fields to produce images of the inside of the body, and ultrasound (US). US, which uses sound

pressure and frequencies, is widely used in prenatal and paediatric imaging. Over recent years, numerous successful efforts have been made to reduce the CT radiation dose. CT manufacturers have developed CT scanners which are more doseefficient and many radiologists have optimised their CT protocols in order to reduce the radiation dose. “The approach is to act on the number of performed CT examinations and on the dose per examination”,Tack said. The high number of examinations has been addressed by introducing prescription criteria to the entire Belgian medical community in November 2011. “The effects of such guidelines are however not known and almost impossible to predict as there is no clear publication reporting the potential benefit of such an approach applied to an entire country yet”,Tack explained. Radiologists follow directives to ensure patient safety. The ALARA principle (As Low As Reasonably Achievable), which guarantees that the best examination is carried out using the lowest possible dose of radiation, is practised by every radiologist all around the world. Regarding the dose per CT examination, one can potentially reduce the collective dose by 30% if all scanners are correctly optimised. Surveys are being performed in the EU to measure inadequate practice at least once every five years. Surveys show that there are significant differences between the lowest and the highest doses per acquisition. It is suspected that the proportion of radiologists able to optimise their CT machines is low. In order to stimulate optimisation (reduction of the dose per examination), the frequency of surveys of CT examinations, conducted by the FANC (Federal Agency on Nuclear Control), will be increased to once a year in Belgium. Results from surveys could serve as an objective of optimisation for those who have not optimised


EDOR (Radiation)_Opmaak 1 9/02/11 09:52 Pagina 2

2

their CT acquisition parameters yet, with a system of awards for those who optimise and penalties for those who don't. The benefits of CT are undeniable, but nevertheless every examination has to be justified by a clinical indication. In principle, the radiologist has to weigh the potential benefits against the risks and then decide if the ordered examination is appropriate or if a non-ionising modality would be more appropriate to answer the clinical question. “In adults, talking only about risks is not satisfactory. One has to take into account the benefit. For example, the risk of dying of pulmonary embolism without adequate treatment is at least 50 to 500 times higher than the radiation burden”, said Tack. Educating referring doctors and radiologists could help reduce the

JBR–BTR, 2011, 94 (1)

number of examinations and the dose from CT even further. Doctors should be taught to take guidelines into account and be allowed to work within a reasonable timeframe. One could also reduce CT doses by enabling radiologists to change a prescription to a more appropriate test or into a no-test strategy. In addition, one should help the medical community not to fear malpractice lawsuits, Tack believes. “Defensive medicine, to avoid any possible malpractice lawsuit, plays a very important role in prescription when it comes to radiology”, he said. Patients throughout the world remain poorly informed about radiation exposure, so contact with the radiologist is key. They should first be informed that imaging tests may not solve their problems. “They usually believe CT and MR will see almost everything”, added Tack.

Patients should bear in mind that CT examinations are not to be repeated at a different radiological institution, as the ordering physician might not know about CT studies conducted shortly before the consultation. Finally, frequent follow-up studies using CT could be avoided. In addition, if CT is the only diagnostic method to answer the question of the follow-up study, a low dose protocol with a reduced scan range should be used. Depending on the medical question, diagnostic studies without ionising radiation such as MRI or US can be frequently used. Contact: Royal Belgian Radiological Society Dr. B. Desprechins President RBRS


aydin-hekimoglu-_Opmaak 1 9/02/11 10:10 Pagina 3

JBR–BTR, 2010, 94: 3-12.

THE VALUE OF PROTON MR-SPECTROSCOPY IN THE DIFFERENTIATION OF BRAIN TUMOURS FROM NON-NEOPLASTIC BRAIN LESIONS H. Aydın, S. Sipahiog˘lu, N. Aydın Oktay, E. Altın, V. Kızılgöz, B. Hekimoglu1 Purpose: Our aim was to evaluate the efficacy of Proton-MR Spectroscopy for the differentiation of cranial masses from non-neoplastic brain disorders. Material and method: 33 patients with intracranial mass lesions, 29 patients with non-neoplastic brain lesions: Ischemic-demyelinating-metabolic-benign cystic mass group; As a whole 62 patients: 30 males and 32 females were included in this study. Results: In brain tumours, average Cho/NAA ratio 2.84-NAA/Cr ratio was 0.97, Cho/Cr ratio 2.42 and Cho/MI ratio was 3.51. In non-neoplastic group; NAA/Cr ratio was extremely higher than tumour group, the other ratios were far lower than cranial mass lesions. Average Cho/NAA ratio: 0.50 ± 0.15, Cho/Cr ratio: 1.05 ± 0.14, Cho/MI ratio: 1.07 ± 0.73. Conclusion: Higher Cho/NAA and Cho/MI ratios with lower NAA/Cr ratio were most likely to be malignant. Additional lipid and lactate peaks were generally seen in malignant group. Key-words: Brain, diseases – Brain neoplasms, MR – Magnetic resonance (MR), spectroscopy.

Non-invasive and accurate differentiation between neoplastic and non-neoplastic brain lesions is important for determining the correct treatment plan and in some cases, may avoid the necessity of biopsy (1-3). Conventional MR imaging is a useful tool in the evaluation of tumoral and non-tumoral brain lesions but not really sufficient for diagnosing all conditions (2-4). Proton magnetic resonance spectroscopy [H-MRS]; A non-invasive technique, has been helpful in understanding the pathophysiology of different pathologic processes (16). It has been used to observe metabolite changes for different intracranial abnormalities such as tumours, stroke, tuberculomas, multiple sclerosis (MS) and metabolic-inherited brain disorders, epilepsy and traumatic injuries (1-3, 6). H-MRS provides biochemical information from tissues by reflecting the alterations of metabolites in the spectra, has proved to be useful for evaluating brain lesions especially the differentiation of tumours and non-neoplastic lesions (1, 2, 4). Several types of non-neoplastic brain disorders (infectious-demyelinating lesions etc.) can be potentially misdiagnosed as brain tumours, MR- Spectroscopy may improve the diagnosis of unknown brain lesions (2-4, 7). H-MRS provides information related to the

neuronal integrity, cell proliferation or degradation, energy metabolism and necrotic transformation of brain or neoplastic tissues (5, 6, 8, 9). Particularly H-MRS is added to the routine brain MRI in order to solve diagnostic problems such as differentiation of neoplastic and non-neoplastic lesions, low and high grade tumours, ischemia from low grade gliomas or discriminating the metastases from primary brain tumours and abcesses (3, 5, 6, 9). Various spectroscopic methods have been used to study tumour biology, grade gliomas, plan treatment and etc (3, 5, 6, 8). In this study, we aimed to test the strength of H-MRS in the discrimination of tumoral masses from nonneoplastic brain lesions. Furthermore, we also wanted to check if MRS can distinguish among the types of cerebral neoplasms. Material and method 33 patients with intracranial mass lesions confirmed by cranial MRI were selected for proton-MRSpectroscopy; 17 males-16 females, age range from 9 to 85, mean age 49 ± 2, patients for non-neoplastic brain lesions suggested by cranial MRI were selected for H-MRS; 13 males16 females with age range 17-80, mean age 48 ± 2. Totally 62 patients; 30 males and 32 females were

From: 1. Dıs¸kapı Yıldırım Beyazıt Research Hospital, Radiology Department, Ankara, Turkey. Address for correspondence: Dr H. Aydin, Dıs¸kapı Yıldırım Beyazıt Research Hospital, Radiology Department, Ankara, Turkey. E-mail: dr.hasanaydin@hotmail.com, serdar_sipahioglu@yahoo.com

included in this retrospective study. Informed consent was obtained from all the patients before the study. The stratification of patients into tumoral or non-neoplastic group depends upon the following brain MRI items; For neoplastic group, Centrally or peripherally strong enhancing mass lesions with surrounding discrete vasogenic edema in cerebral or cerebellar hemispheres. For the non-neoplastic group; Plaque or nodular lesions mostly situated at pericallosalperiventricular white matterthalamus and basal ganglia, nonenhanced lesions without obvious mass effect with restricted diffusion, extra-axial non-enhancing cystic masses with or without restriction in the Diffusion Weighted MRI and focal nodular or conglomerated white matter lesions. We have variety of non-neoplastic brain disorders that include ischemic-demyelinatingmetabolic (Wilson’s disease) and benign mass lesions. Ischemic group consisted of acute and subacute enfarcts , chronic ischemic areas or encephalomalasic regions were excluded. The demyelinating lesions were; MS and Acute disseminated ensephalomyelitis (ADEM), all the active or inactive plaques whether new or old, were included in this research. Diagnosis of all nonneoplastic brain disorders were confirmed by brain MRI, clinical and laboratoy findings. We had intracranial tumours that include high grade glial tumours (multiform glioblastoma-anaplastic astrocytomas), low-grade glial tumours (gliomatosis serebri-serebelli, gangliogliomas), meningiomas and metastasis. Except for a metastasis case and two menengiomas, diagnosis of all brain neoplasms


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4

were confirmed pathologically either by biopsy or open-surgery. Patient with cranial metastases was still suffering from a known primary neoplasm and has also metastatic infiltrations in his lungs and liver so biopsy was not needed. The diagnosis of extra-axial menengiomas was based upon the MRI and clinical findings as the patients refused the biopsy. All the MRI and Multi-voxel spectroscopic analysis were carried out with an 8-channel 1.5 T MR scanner (Philips Achieva, Philips Medical systems-Netherlands) by using a standart head and neck array coil. Multivoxel spectroscopic technique (MVS) was taken into account for all lesions in the study; in contrary we didn’t perform single-voxel spectroscopy in this research. The MRS was performed by using point-resolved spectroscopy (PRESS) with a volume of interest (VOI), 1*1*0.5 cm3 standard voxel sizes for the MVS and presaturation bands placed around the VOI. Depending upon the tumour and the lesion size, approximately 5-10 cm3 tumor area on the multivoxel imaging was harbored with the volume made up of such Standard voxels. We have positioned the possible voxel within the solid tumoural or lesional area avoiding areas of cysts, normal appearing brain parenchyma, scalp or skull base contamination (6-8). Automatic shimming of the linear x,y,z channels was used to optimize field homogeneity, water resonance and water suppression pulses were optimized for the consistent water saturation. Data analysis Proton spectrum was recorded in axial plane with TR; 1500 ms,TE; 26 and 144 ms, FOV; 24 x 24 cm, 0.5 cm slice thickness, 256 x 256 matrix and 24 x 24 phase encoding. Duration of scan for both TE acquisitions was about 5 min. Time domain data were multiplied with a Gaussian function of 90 (Centre 0, halfwidth 256 ms), 2D Fourier transformed phase and base-line corrected, quantified by means of frequency domain curve fitting with the assumption of a Gaussian line shape, spectral analysis and all post-processing were carried out by using a software of Philips Achieva Netherland workshop. 0-4.35 ppm is analysed and metabolite signals and the data were processed as follows; N-Acetyl aspartate (NAA) at 2 ppm, creatine (Cr) at 3-3.1 ppm, Phosphocreatine (Cr2) at 3.8-3.9 ppm, Choline (Cho) at

JBR–BTR, 2011, 94 (1)

3.2 ppm, lipids (Lip) at 0.9-1.3 ppm, lactate (Lac) at 1.3-1.4 ppm , glutamate and glutamine (Glx) at 2.45 ppm, glycine and or myo-inositol (Gly-MI) at 3.6-3.75 ppm (3, 4, 6, 7). Standard, optimum and sufficient base-line correction for metabolites were also performed. Two doublets inverted owing to phase modulation due to J coupling were defined, Lac at 1.4 ppm and alanine (Ala) at 1.5 ppm. At TE 144 ms, Lac can be differentiated from lipids with a narrow bandwidth comparable with the peaks of other metabolites and shows an inverted J-coupled double peak at 1.4 ppm (3, 4, 6). Tumour and lesion metabolite signal intensities were quantified, normalized by expressing the peak area intensities of the metabolites especially NAACho-Cr as ratios of normal brain parenchymal values to intratumoral metabolites (NAA\Cr, NAA \ Cho), Lip and Lac which were not detectable in normal brain, were normalized using the contralateral reference spectrum as an internal standart (1, 3, 8). For instance, we compared the lesional NAA to the lesional Cr, had the lesional NAA\Cr ratio and this was identical for all metabolites and ratios. Contralateral reference voxel was placed just symmetric to the center of the original brain lesion, however for the midline lesions; The normal reference spectra and metabolite ratios were obtained from the healthy volunteers who had no cerebral or cerebellar abnormalities. 3 healthy volunteers were included in this study. All analyses were performed by using a software program (SPSS for Windows, SPSS, Chicago-Illinois). Significance of differences between various cranial masses and non-neoplastic lesion groups (Ischemicdemyelinating-metabolic and benign cystic mass lesions) for brain metabolites and metabolite ratios were tested with one-tailed parametric variance analysis test, Pearson chi-square test and difference test among mass groups, the sensitivity and the specificity of H-MRS for all neoplastic and non-neoplastic group, were tested by chi-square cross table test, p < 0.05 were considered to be statistically significant differences. Results In both cranial neoplastic mass lesions (Glial tumours-metastasismenengiomas) and non neoplastic brain disorders, including ischemic-

demyelinating (MS-ADEM)-metabolic (Wilson syndrome), benign cystic mass group (arachnoid-epidermoid cysts-cavernoma); we have the age and the gender of patients, lesion localizations, NAA/Cr, Cho/NAA, Cho/Cr, Cho/MI ratios, biopsy results and other increased metabolite peaks (Table I). 30 patients with space-occupying brain masses underwent biopsy or open surgery, only the metastasis and meningiomas had no histopathologic confirmation. In one patient, there was no mass profile in MRSI but the histopathology was anaplastic astrocytoma, except for this case H-MRS proved all the mass lesions (32/33). The statistical analysis and measurement of metabolite peaks were performed in 32 patients. The diagnosis of other non-neoplastic lesions were proved by MRI, by clinical routes and by biochemical laboratory results. The spectra from the contralateral brain or from the healthy volunteers revealed a consistent pattern of the four major peaks of NAA, Cr, Cho and MI, no lactate or lipid resonances were visible in these cases. The average NAA/Cr, Cho/NAA, Cho/Cr, Cho/MI ratios were 1.46 ± 0.13, 0.56 ± -0.22, 0.80 ± -0.07, 0.41 ± 0.09. These ratios were assumed to be the cut-off values for the differentiation between malignant and nonneoplastic brain lesions. We performed two acquisitions in this research, TE: 26 msec and 144 msec. Cho/MI ratio was obtained at short TE acquisition, all the other ratios were calculated at long TE application. The metabolites or the ratios of them were assumed to be increased or decreased in a voxel, only if the measurements in the mentioned pixel had been normalized with reference to the contralateral normal appearing pixel, this was calculated for each metabolite with regard to the healthy opposite side reference point. According to H-MRS; We had 9 low-grade (Grade 1-2) gliomas, all low grade gliomas showed increased Cho and reduced NAA resonances, as an average Cho/NAA ratio; 1.66 ± 0.35, all low-grade gliomas in this study showed increased Cho and moderately decreased Cr, as an average Cho/Cr ratio; 2.25 ± 0.63. Low-grade glial tumours also showed moderately increased MI, with an average Cho/MI ratio; 2.55 ± 0.86 which was precisely elevated (Table II, Fig. 1). According to Cho/Cr ratio by using one-tailed parametrical variance analysis test, there was a significant


Highgrade Glial tm

Corpus Callozum

Left Frontal

4. Ventricule

Cerebellum

Left Parietal

Cerebellar Vermis

Left Occipito-Parietal

Bil.Centrumsemiovale

Right Post. Parietal

Left Frontoparieta

Right Temporal

Right Cerebellar

Left Occipito-Temporal Low grade Glial tm

Left Temporal

E.B 43 F

Y.Y 43 F

E.K. 22 M

L.S 40 M

B.U. 9 M

M.P.48 F

H.C. 50 F

H.B. 43 M

C.A. 44 M

I.K. 78 M

A.D. 69 M

A.D. 34 F

D.D. 38 F

G.D. 64 F

Highgrade Glial tm

GBM

Low grade Glial tm

Low grade Glial tm

Low grade Glial tm

Low grade Glial tm

Highgrade Glial tm

GBM

Low grade Glial tm

Biopsi Results

GBM

Low grade Glial tm Low grade Glial tm

Left Frontotemporal

Right Frontotemporal

M.S. 50 F

Highgrade Glial tm Low grade Glial tm GBM

No mass effect. Low grade Glial tm GBM

Left Pontobulbar

Right Mezencephalon

Left Frontoparietal

Right Temporal

Corpus Callozum

Right Temporal

Right Frontoparietal

Left Frontoparietal

Corpus Callozum

Left Pontobulbar

B.T. 42 M

R.Ö 43 M

I.S¸.24 M

V.K. 52 M

S.T 81 F

N.Y. 28 F

M.G. 43 M

A.A. 60 M

A.Ö. 33 F

NEOPLASTIC MASS GROUP

Left Frontal Low grade Glial tm

S¸.Ü. 52 F

GBM

Left Temporo-oksipital

Left Temporo-oksipital

H.B. 85 F

Ö.D. 38 F

Highgrade Glial tm

Menengioma

GBM

Highgrade Glial tm

Highgrade Glial tm

Highgrade Glial tm

GBM

Highgrade Glial tm

Low grade Glial tm

GBM

GBM

Extra-axial, no biopsy.

GBM

Highgrade Glial tm

Low grade Glial tm

Highgrade Glial tm

GBM

Metastasis

Anaplastik Astrositom

Highgrade Glial tm

S.K. 39 F

Highgrade Glial tm

Left Frontoparietal

Left Occipitotemporal

M.H 42 F

S.K. 36 M

Highgrade Glial tm

GBM

Low grade Glial tm

Right Medial Temporal Low grade Glial tm

H.C. 46 M

M.B. 58 M Highgrade Glial tm

Highgrade Glial tm

GBM

Highgrade Glial tm

GBM

Low grade Glial tm

Highgrade Glial tm

Highgrade Glial tm

Highgrade Glial tm

Menengioma Extra- axial, no biopsy

Highgrade Glial tm

GBM

Highgrade Glial tm

Low grade Glial tm

Low grade Glial tm

Highgrade Glial tm

Highgrade Glial tm

Highgrade Glial tm

MRS RESULTS

Right Frontal

T.K. 34 M

Table I. — List of patients.

Name-age-gender Lesion localization

63-

62-

61-

60-

59-

58-

57-

56-

55-

54-

53-

52-

51-

50-

49-

48-

47-

46-

45-

44-

43-

42-

41-

40-

39-

38-

37-

36-

35-

İschemia

İschemia

İschemia

İschemia

İschemia

İschemia

İschemia

İschemia

İschemia

İschemia

İschemia

İschemia

İschemia

Ischemia

İschemia

İschemia

MRS RESULTS

Right Occipital

Right Frontal

Cerebellum

Right paramedian

Right post. parietal

Thalamus-Midbrain

Bilat.periventricular

Bilat.periventricular

Bilat.periventricular

Bilat.periventricular

Bilat.periventricular

Right occipital

Neuroepithelial cyst, MRS negative

Arachnoid cyst, MRS negative.

Cavernoma.

epidermoid cyst

Arachnoid cyst, MRS negative.

Metabolic disease

Demyelinating lesions

Demyelinating lesions.

Demyelinating lesions.

Demyelinating lesions.

Demyelinating lesions

İschemia

Right thalamus,basal ganglia. İschemia

Left Frontal

Right Parietal

Left Fronto-parietal

Left Temporo-occipital

Right Occipito-temporal

Right Occipital

Right Temporo-occipital

Bilat.periventricular

Right Occipito-temporal

Right Occipital

Right fronto-parietal

Left parieto-occipital

Left Thalamus

Left occipital

Right Fronto-parietal

Left temporal

NON-NEOPLASTIC MASS GROUP.

H.Ö. 30 F

C.E 72 M

C.Ö 65 F

M.D 45 F

G.N 47 F

R.Ö 32 M

V.T 31 M

H.B 30 M

H.G 38 M

E.B 32 F

S.T 53 F

A 36y,M

Z.T.67 F

B.D. 44M

A 50 F

H.U 23 F

M.D 17 M

M.K. 56 M

H.B. 53 F

S. 58 F

M.Ö. 31 M

S.A. 60 M

C. . 29 M

S.A. 36 F

M.E. 52 F

F.Y. 24 F

N.A. 44 F

K.Y. 37 M

E.E 80 F

Name-age-gender Lesion localization

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JBR–BTR, 2011, 94 (1)

Table II. — Ratios in different brain lesions. Measurement

Mass groups

Patient

Mean value

Cho/MI Ratio

Low grade glial tm High grade glial tm GBM Menengioma Metastasis Sum

9 15 5 2 1 32

2,55 3,46 6,12 3,46 3,96 3,61

NAA/Cr ratio

Patient

Low grade glial tm High grade glial tm GBM Menengioma Metastasis Sum

9 15 5 2 1 32

Mean value 1,24 0,88 1,08 0,74 0,84 1,00

Measurement

Mass groups

Patient

Mean value

Cho/NAA ratio

Low grade glial tm High grade glial tm GBM Menengioma Metastasis Sum

9 15 5 2 1 32

1,66 3,31 4,39 1,96 2,88 2,91

Patient

Mean value

9 15 5 2 1 32

2.25 2,24 4,39 1,39 2,42 2,46

Measurement

Cho/Cre Ratio

Mass groups Low grade glial tm High grade glial tm GBM Menengioma Metastasis Sum

statistically difference between lowgrade tumours and the other cranial mass groups (p < 0.05). With parametrical variance analysis test for Cho/NAA and Cho/MI ratios, there was no statistical difference between low-grade tumours and the other brain neoplasms (p > 0.05). In lowgrade group, the average NAA/Cr ratio was about 1.24 ± 0.43 and by using difference test among mass groups, there wasn’t any statistical difference between low-grade tumours and the other brain neoplasms (p > 0.05) (Table II). We had 15 high-grade glial neoplasms (Grade 3 astrocytomas and anaplastic astrocytomas ); They presented increased Cho and highly reduced NAA resonances, as an average Cho/NAA ratio; 3.31 ± 1.04 (Table II), increased Cho, moderately decreased Cr and decreased MI, as an average Cho/Cr ratio; 2.24 ± 0.99

Standart deviation 0.86 3,19 5,37 2,59

F

P

0.873

0,512

Standart deviation 0,43 0,42 0,43 0,33 . 0,40

F

P

1,066

0,401

Standart deviation 0,35 3,04 1,54 0,33 2,04

F

P

1,126

0,372

Standart deviation 0,86 0,99 1,58 0,41 . 0.96

F

P

4.810

0.003*

3,20

and Cho/MI ratio; 3.46 ± 1.19 (Table II – Fig. 2). According to Cho/NAA and Cho/MI ratios; There was no statistical differences between high grade glial tumours and the other brain tumour groups (p > 0.05). According to Cho/Cr ratio by using parametrical variance analysis test; There was a significant statistically difference between high grade astrocytomas and the other cranial space occupying mass lesions (p < 0.05). In high-grade gliomas, the average NAA/Cr ratio was about 0.88 ± 0.48 and by using difference test among mass groups, there was no statistically difference between high grade glial tumours and the other brain neoplasms (p > 0.05) (Table II). We had in this report 5 glioblastome multiforme (GBM) cases (Grade 4 ). Although they were also high-grade neoplasms, we had organized them in a private group as

the GBM masses due to their extremely high metabolite ratios; They presented precisely increased Cho and reduced NAA, MI and Cr resonances , as an average Cho/NAA ratio; 4.39 ± 1.54, Cho/Cr ratio; 4.39 ± 1.58, Cho/MI ratio; 6.12 ± 3.37 (Table II, Fig. 3). According to Cho/NAA and Cho/MI ratios, there wasn’t any statistical difference between GBM group and the other cranial brain tumour groups (p > 0.05), according to Cho/Cr ratio by using a tailed parametrical variance analysis test, there was a significant statistically differences between GBM and the other cranial neoplasm groups (p < 0.05). In GBM group; The average NAA/Cr ratio was about 1.08 ± 0.43 and by using difference test among mass groups, there was no statistical differences between GBM and the other brain neoplasms (p > 0.05) (Table II).


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PROTON MR-SPECTROSCOPY FOR DIFFERENTIATING BRAIN TUMORS — AYDIN et al

Fig. 1. — Metabolite peaks in low grade glial tumour

Fig. 2. — Metabolite peaks in high grade glial tumour

Menengiomas represented increased Cho, slightly decreased Cr, reduced NAA and MI resonances; As an average Cho/NAA ratio 1.39 ±

0.41, Cho/Cr ratio; 1.66 ± 0.33, Cho/MI ratio; 3.46 ± 1.59 (Table II). According to Cho/NAA and Cho/MI ratios; There was no statistical differ-

7

ence between meningiomas and the other brain mass lesions (p > 0.05), According to Cho/Cr ratio by using parametrical variance analysis test, there was a significant statistical difference between meningiomas and all the other cranial mass groups (p < 0.05). For meningiomas, average NAA/Cr ratio was about 0.74 ± 0.38 and by using difference test among mass groups, there was no statistical difference between meningiomas and the other brain neoplasms (p > 0.05) (Table II). For metastasis; Increased Cho, decreased Cr, highly reduced NAA and MI resonances, Cho/NAA ratio; 2.88, Cho/Cr ratio; 2.42, Cho/MI ratio; 3.96 were taken (Table II). According to Cho/NAA and Cho/MI ratios. There was no statistical difference between metastasis and the other brain tumour groups (p > 0.05), according to Cho/Cr ratio by using parametrical variance analysis test, there was a significant statistically difference between metastasis and the other brain neoplasms (p < 0.05). In this group, the average NAA/Cr ratio was about 0.84 and by using difference test among mass groups, there was no statistical difference between metastasis and the other brain neoplasms (p > 0.05) (Table II). As a whole for 33 brain mass lesions; Average Cho/NAA ratio was 2.84 ± 2.35, NAA/Cr ratio was 0.97 ± 0.42, Cho/Cr ratio was 2.42 ± 1.28 and Cho/MI ratio about 3.51 ± 3.21 (Table III). The sensitivity of MRSI was 97% and the specificity of HMRS was 87.9%, calculated by chisquare cross table test. We had 18 cases in ischemic group; Average NAA/Cr ratio was 1.96 ± 0.80 , average Cho/NAA ratio was; 0.40 ± 0.23, average Cho/Cr ratio; 0.58 ± 0.37 and the average Cho / MI ratio was about 1.37 ± 1.23 (Fig. 4), 5 cases in demyelinating group; Average NAA/Cr ratio was 2.72 ± 0.92, Cho/NAA ratio ; 0.59 ± 0.17, Cho/Cr ratio; 1.75 ± 1.05 and the average Cho / MI ratio was about 0.78 ± 0.30 (Fig. 5), one case for metabolic disease group ; Average NAA/Cr ratio was 2.23, Cho/NAA ratio; 0.89, Cho/Cr ratio; 0.68 and the average Cho/ MI ratio was about 1.05, 5 cases in benign cystic mass group; The average NAA/Cr ratio was 2.69 ± 1.51, Cho/NAA ratio; 0.34 ± 0.15, Cho/Cr ratio; 1.04 ± 0.51 and the average Cho/MI ratio was about 1.10 ± 0.76 (Table IV). With variance analysis test for Cho/Cr and Cho/NAA ratios; There was a significant statistical difference through the all non-neoplastic brain disorder


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JBR–BTR, 2011, 94 (1)

Fig. 3. — Metabolite peaks in GBM.

groups (p < 0.05), according to NAA/Cr and Cho / MI ratios; There was no statistical differences between non-neoplastic brain lesions (p > 0.05) (Table IV). In ischemic and demyelinating group; There was no NAA depression and no Cho peak rise, Cr peak was almost normal in ischemic group and decreased in demyelinating group. In only one case of ischemia, we had increased MI and Glx. In metabolic group; There was no NAA depression, slightly increased Cho peak and the Cr peak was slightly decreased, MI was also increased. In benign cystic mass group, we had reliably increased Lip peaks but in 3 cases, H-MRS was inaccurate for showing the details of these lesions, didn’t provide adequate information for the diagnosis. The sensitivity of H-MRS was about 40% and 27% specificity for this group. When we make a comparison between cranial masses and all the

other non-neoplastic brain disorders; Cho/Cr- Cho / MI and Cho/NAA ratios were far more higher in brain masses than the other nonneoplastic groups but at the same time, NAA/Cr ratio was lower in brain tumours, higher in demyelinating group (Table III). With variance parametrical analysis test, there was a significant statistical difference between brain masses and the other non-neoplastic brain disorders for all ratios (p < 0.05). When we briefly looked at the other metabolites for all neoplastic and non-neoplastic groups; Dominant metabolite for ischemic lesions, was the Lac and also highly seen in high-grade glial tumours and GBM group. Lip was seen in all brain masses except for the low-grade gliomas and might be the dominant metabolite in the high-grade glial tumour group, was also consistent in the ischemic lesions. According to Lac and Lip peaks by using chisquare test; There was a significant

statistical differences between ischemic lesions, high grade glial tumours and the GBM group (p < 0.05). The others presented non significant statistical differences under p > 0.05. In demyelinating and the low-grade group; We had reliably increased MI peaks, low-grade tumours and the metabolic disease group showed a high Glx peak. According to MI and Glx peaks by using chi-square test, there was a significant statistical differences between demyelinating lesions, lowgrade tumours and the metabolic disease group (p < 0.05). The other groups in this research presented non-significant statistical differences for MI and Glx peaks (p > 0.05). Discussion H-MRS has a potential for definite diagnosis without surgical tissue sampling, also serves a significant role before neurosurgical planning, it has the non-invasive potential to


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PROTON MR-SPECTROSCOPY FOR DIFFERENTIATING BRAIN TUMORS — AYDIN et al

9

Table III. — Metabolite ratios in brain lesions.

Cho/NAA Ratio

Cho/Cre Ratio

Cho/MI Ratio

NAA/Cr Ratio

Brain Masses Demyelinating Ischemia Methabolic Benign cystic masses Brain Masses Demyelinating Ischemia Methabolic Benign cystic masses Brain Masses Demyelinating Ischemia Methabolic Benign cystic masses Brain Masses Demyelinating Ischemia Methabolic Benign cystic masses

N 33 5 18 1 5 33 5 18 1 5 33 5 18 1 5 33 5 18 1 5

Mean 2,84 0,59 0,40 0,89 0,34 2,42 1,75 0,58 0,68 1,04 3,51 0,78 1,37 1,05 1,10 0,97 2,72 1,96 2,23 2,69

Std. deviation 2,35 0,17 0,23 . 0,15 1,28 1,05 0,37 . 0,51 3,21 0,30 1,23 . 0,76 0,42 0,91 0,80 . 1,51

F

p

7,132

0,000*

10,227

0,000*

3,312

0,017*

13,518

0,000*

*p < 0.05.

Fig. 4. — Metabolite peaks in ischemic brain lesion.

provide additional metabolic information to improve pre-surgical diagnosis, disease extension and direct anatomical replacement of surgical biopsy if needed (2, 4, 10). MRSI can also help to identify the tumour type and grade, grading of brain tumour and histopathologic classification of brain lesions which has important

implications for clinical management (9, 11, 12). In our study, we find out that MRSI can be used for differentiation of malignant brain masses from the non-neoplastic ones. It can be acquired as an additional pulse sequence and contribute to a multimodality research of morphological and metabolic information, MVS

also provides wider anatomical coverage and better spatial resolution, for the assessment of lesion heterogeneity (1, 2, 7, 10). In MRSI; Resonances of brain metabolites as mentioned above, are briefly analysed for correct diagnosis of brain disorders especially for tumours and non-neoplastic lesions. NAA is a marker of functional neurons and decreases in all kinds of brain damages whether infiltrative, degenerative or destructive processes (3, 5, 8, 11-13). Cho resonances increase in all brain tumours, acute MS plaques and tuberculous abcesses due to increased cell membrane synthesis and turnover, reflect the abnormal processes like proliferating tumoral cells and active demyelinating plaques (1-3, 7, 11, 13). CrPhosphoCr are ubiquitous metabolites of ATP/ADP cycle, therefore Cr is considered to be an indicator of energy metabolism, generally increased in chronic MS plaques, decreased in acute MS and in glial tumours, brain tumours generally show decreased Cr levels (1-3, 6-8, 11-14). Lac is produced in anaerobic glycolysis; is more prominent in high grade tumours, ischemic diseases and acute infarction, Lip usually correlates to the extent of tissue necrosis, only observed in short TE acquisition because of its short relaxation time, generally seen in high-grade neoplasms, metastasis,


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JBR–BTR, 2011, 94 (1)

Table IV. — Metabolite ratios in non malignant brain lesions. Measurement

NAA/Crea

Cho/NAA

Cho/Crea

CHO/MI

Groups Ischemia Demyelinating Metabolic Benign cystic masses Sum Ischemia Demyelinating Metabolic Benign cystic masses Sum Ischemia Demyelinating Metabolic Benign cystic masses Sum Ischemia Demyelinating Metabolic Benign cystic masses Sum

N 16 5 1 5 27 16 5 1 5 27 16 5 1 5 27 18 5 1 5 29

Mean 1,89 2,72 2,23 2,69 2,20 0,41 0,59 0,89 0,34 0,45 0,54 1,75 0,68 1,04 0,86 1,37 0,78 1,05 1,10 1,21

Std.deviation 0,81 0,92 . 1,51 1,01 0,22 0,17 . 0,15 0,22 0,37 1,05 . 0,51 0,71 1,23 0,30 . 0,76 1,03

F

Sig.

1,423

0,262

3,239

0,041*

5,924

0,004*

0,419

0,741

*p < 0.05.

Fig. 5. — Metabolite peaks in demyelinating disorders (MS).

acute MS plaques and brain abscesses, Lip-Lac metabolites routinely were undetectable in healthy brain (1-3, 7 , 10, 12-14). Glx is a neu-

rotransmitter, generally increased in Wilson disease, Ala peak is generally shown in meningiomas and involves in partial oxidation (8, 13), but in our

cases; we had no precise Ala peaks. With the further advancement of MRI technology; new commercial or free software packages may improve the quality of MRS data by increasing the absolute quantification of metabolites or quantification by fitting against the metabolite data bases. In the literature, there were several former studies trying to analyze the efficacy of MRSI in brain tumours and most of the authors used Cho/NAA, Cho/Cr ratios to differentiate brain tumours from nonneoplastic disorders (2-4, 9, 12). There was a general concordance in previous studies that Cho was the best index for grading cerebral gliomas and its peak reliably increased from low-grade to GBM group (2-4, 9, 12, 15). In this research, we had also similar results. For GBM group, the Cho peak was extremely high. The second best discriminator between low-grade glial tumours and malignant gliomas was the amount of lipids while changes of Cr and NAA peaks were less helpful for the analysis of potential malignancy of cerebral tumours (24, 12). In addition to high-grade gliomas, metastases were also assumed to have extremely increased Cho, significantly reduced NAA and Cr (2-4, 9, 15). Besides the similar results to the literature, our metastases group had high lip peak.


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PROTON MR-SPECTROSCOPY FOR DIFFERENTIATING BRAIN TUMORS — AYDIN et al

In the benign cystic cranial masses of non-neoplastic group, we had also strong lipid resonances. MR-Spectroscopy has also an important diagnostic role, especially when it reveals reduced NAA without increased Cho, in these instances MRS findings may spare the patient from biopsy (4, 16, 17). Cho/NAA, Cho/Cr ratios were the valuable tools for determining the malignancy of cranial neoplasms, used by most of the authors (3-5, 912, 15). In most of the former studies, both ratios increased from lowgrade to high-grade gliomas, also extremely higher in metastases and Primitive neuroectodermal tumours (PNET) (3, 5, 8, 9, 11, 15). In this research, Cho/Cr ratio more than 2.5 was presented as malignant (p < 0.05) . We had no PNET type tumour but both ratios were similarly increased in low-high grade glial tumours and in metastases. In our study, the lowest Cho/Cr ratio among the other brain masses was presented in meningiomas. In this group, we had strong elevation of Cho resonance but contrary to literature, there was no precise Cr decrease and Cho/Cr ratio was the lowest in meningiomas, we didn’t see any Ala resonance for both meningiomas. We tried to make another variation by using Cho/MI ratio in order to categorize tumours according to their potential malignancy and tried to differentiate malignant cranial masses from non-neoplastic disorders which were not frequently seen in the past former studies. Among these masses; Cho/MI ratio was the highest in GBM and lowest in Lowgrade glial tumours. It was also extremely high in higher-grade gliomas and metastasis groups. In cranial mass groups; We had an average Cho/MI ratio about 3.51, therefore we could easily conclude that increased ratio was strongly related with malignancy of the tumours. In non-neoplastic group; The highest Cho/MI ratio was in ischemic group, about 1.37. With these datas, one could easily conclude that Cho/MI ratio more than 3 was most likely to be malignant rather than benign (p < 0.05). In our research, Cho/MI ratio was really a diagnostic tool for grading gliomas, categorizing the tumours according to their malignancy rates and differentiation from non-neoplastic lesions. The primary results of our study presented that MR-Spectroscopy had a good sensitivity and specificity among the non-neoplastic brain

disorders except for the cystic cranial masses, Perfusion MR and Diffusion Weighted MR imaging could also be added to MRS findings in order to get more beneficial results (2, 4, 7, 10, 16). When we look at the literature; Elevated Cho levels and reduced NAA levels had been reported in acute MS plaques and had been explained by reactive astrogliosis, inflammation and early axonal degeneration, MI at short TE was also a discriminating metabolite for the acute MS and also increased in cases of glial activation or gliosis, represented dominancy in low-grade astrocytomas related to abnormal astrocyte proliferation, nevertheless might also be seen in Encephalitis, Dementia, Epilepsy and SSPE-PML like brain disorders (1, 7, 10, 13, 14), but in high grade gliomas, metastases and more malignant tumours, its peak sharply declined (1, 2, 4, 15, 17). In our study, we had also elevated MI peaks in non-neoplastic demyelinating group. According to previous studies, there was no significant difference in the level of Cr among the non-neoplastic brain lesions (1, 4, 17). In our series, Cr peak was strongly decreased in demyelinating group. Infarctions as mentioned in the literature show increased Lac, progressive Cho reduction in the chronic phase and also increased NAA peak especially in acute and subacute phases (1, 7, 10). In our ischemic group, Lac was also the dominant metabolite. Glx peak was the highest in the metabolic group. In the non-neoplastic group, Cho peak was highest in metabolic group and Cr was strongly decreased at the demyelinating group. NAA/Cr ratio was also higher in demyelinating group. In the differentiation of low-grade group from the non-neoplastic disorders; Most of the authors considered the Cho peak and Cho/NAA, Cho/Cr ratios as the reference standards, they mostly made the discrimination by the elevated Cho/NAA, Cho/Cr ratios and high Cho peak in the selected voxels (2-4, 9, 12, 13). In our paper with correspondence to these ratios, lowgrade group had significantly higher results than the non-neoplastic brain lesions as seen in the former studies, at the same time we also used the Cho/MI ratio for the differentiation of both groups and although this ratio was lowest in low-grade group through the other neoplastic mass groups, was certainly higher than the non-neoplastic group. In the previous researches, evaluation of non-neoplastic brain disor-

11

ders and differentiation of them from the brain tumours based on the NAA/Cr, Cho/ NAA ratios (1, 2, 4, 7, 10). NAA/Cr ratio more than 2 was presented as non-malignant (p < 0.05) and elevated NAA/Cr ratio was highly specific for the demyelinating diseases (2, 4, 7, 17). In our experience; NAA/Cr ratio was the highest in demyelinating group and the lowest in brain masses. Cho/NAA ratio above 1.5 was assumed to be malignant in the previous former studies (2-6, 13, 15). In our paper, the non-neoplastic group had precisely lower Cho/NAA ratio than the cranial mass groups. To our belief, Cho/NAA ratio above 2 mostly focused to malignancy (p < 0.05). Some cases of demyelinating lesions were presented as mimicking high-grade gliomas because of histopathologic similarities which include hypercellularity-reactive astrocytes-mitotic and necrotic areas (1, 4, 17), we didn’t have such misclassified cases in our report. As a whole; This study provided that increased Cho/NAA, Cho/MI and Cho/Cr ratios with decreased NAA/Cr ratio should easily be used in the differentiation of malignant ones from the nonneoplastic brain lesions and could aid to the relevant literature with this aspect. Conclusion As a summary, a brain lesion with higher Cho/NAA, Cho/Cr and Cho/MI ratio plus lower NAA/Cr ratio was most likely to be malignant. Additionally, lip and lac peaks were also frequently seen in more malignant lesions. A higher MI peak generally presented low-grade malignancy or a non-neoplastic disorder especially demyelinating lesions. Acknowledgements We thank Mr.Egemen Alper and Erdeniz Yurdakul for their brief assistance in handling of those all MRSI procedures and also we are so grateful to the Schering-Germany for their financial supports. References 1. Mader I., Rauer S., Gall P., Klose U.: H-MR spectroscopy of inflammation, infection and ischemia of te brain. Eur J Radiol 2008, 67: 250-257. 2. Majos C., Aguilera C., Alonso J., Sape J.M., Castaner S., Sanchez J.J., et al.: Proton-MR spectroscopy improves Discrimination between tumour and pseudo-tumoral lesion in


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JBR–BTR, 2011, 94 (1) solid brain masses. AJNR, 2009, 30: 544-551. Hartmann W., Herminghaus S., Krings T., Marquardt G., Lanfermann H., Pilatus U., et al.: Clinical application of proton magnetic resonance spectroscopy in the diagnosis of intracranial mass lesions. Neuroradiology 2002, 44: 371-381. Hourani R., Brant L.J., Rizk T., Weingart J.D., Barker P.B., Horska A., et al.: Can Proton MR Spectroscopic and Perfusion imaging differentiate between neoplastic and non-neoplastic brain lesions in adults? AJNR 2008, 29: 366-372. Poptani H., Gupta R., Roy R., Pandey R., Jain V.K., Chhabra D.K.: Characterization of Intracranial mass lesions with in vivo Proton MR Spectroscopy. AJNR 1995, 16: 15931603. Sibtain N.A., Howe F.A., Saunders D.E.: The clinical value of proton magnetic resonance spectroscopy in adult brain tumours. Clin Radiol, 2007, 62: 109-119. Papanagiotou P., Grunwald I.Q., Farmakis G., Hartmann K.M., Politi M., Roth C., et al.: MR-

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Spektroskopie bei entzündlichen Hirnerkrankungen. Radiologe, 2008, 48: 582-587. Callot V., Galanaud D., Le Fur Y., Gouny S.C., Ranjeva J.P., Cozzone P.J.: H-MR spectroscopy of human brain tumours , A practical approach. Eur J Radiol, 2008, 67: 268-274. Majos C., Alonso J., Aguilera C., Serrallonga M., Martin J.P., Acebes J.J., et al.: Proton magnetic resonance of human brain tumours, Assessment of differences between tumour types and its applicability in brain tumour categorization. Eur Radiol, 2003, 13: 582-591. Nagar V.A., Ye J., Xu M., Ng W.H., Yeo T.T., Ong P.L., et al.: Multi-voxel MR Spectroscopic ImagingDistinguishing Intracranial Tumours from Non-neoplastic Diseases. Ann Acad Med Singapore, 2007, 36: 309313. Magalhaes A., Godfrey W., Shen Y., Hu J., Smith W.: Proton magnetic resonance Spectroscopy of brain Tumours correlated with pathology. Acad Radiol, 2005, 12: 51-57. Parmar H., Lim T.C., Yin H., Chua V., Khin LW., Raidy T., et al.: Multi-voxel MR Spectroscopic imaging of the

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brain: Utility in clinical setting-initial results. Eur J Radiol, 2005, 55: 401408. Papanagiotou P., Backens M., Grunwald I.Q., Farmakis G., Politi M., Roth C., et al.: MR-Spektroskopie bei Hirntumoren. Radiologe, 2007, 47: 520-529. Faria A.V., Reis F., Zanardi V., Menezes J.R., Cendes F.: The pattern of Proton-Magnetic resonance Spectroscopy in non-neoplastic Encephalic lesions. Arq Neuro psiquiatr, 2004, 62(2b): 429-436. Venkatesh S.K., Gupta R.K., Pal L., Husain N., Husain M.: Spectroscopic increase in choline signal, is a nonspecific marker for differentiation of infective-inflammatory from neoplastic lesions of the brain. J Magn Reson Imaging, 2001, 14: 8-15. Lai P.H., Hsu S.S., Ding S.W., Ko C.W., Fu J.H., Weng M.J., et al.: Proton magnetic resonance spectroscopy and diffusion-weighted imaging in intracranial cystic mass lesions. Surg Neurol, 2007, 68: 25-36. Narayana P.A.: Magnetic resonance Spectroscopy in the monitoring of Multipl Sclerosis. J Neuroimaging, 2005, 15: 46-57.

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JBR–BTR, 2011, 94: 13-14.

PLEUROPULMONARY BLASTOMA PRESENTING AS A COMPLICATED PLEURAL EFFUSION J. O’Brien, D. Rea, R. Hayes1 Pleuropulmonary blastoma (PPB) is a rare tumour of mesenchymal cells. We present a case of PPB in a child, which presented to the emergency department with an extensive pleural effusion. We discuss the radiological features, pathology, classification and treatment of this condition. This case reiterates the importance of considering this diagnosis prior to surgical intervention, to improve the long term prognosis of this aggressive disease. Key-word: Blastoma.

Case report A 3year-old previously healthy girl was referred to the emergency department with a 2-day history of increasing dyspnoea and drowsiness. Laboratory investigations revealed an elevated white cell count of 20cells/mm3, with normal haemoglobin and platelet levels. ESR was elevated at 110 mm/hour. A chest xray revealed extensive opacification of the right hemithorax with minimal aerated lung in the right apex. There was marked mediastinal shift to the left and patchy left lower lobe consolidation. There was also a small air pocket projected to the right of the midline which was suggested to be a pnuematocoele (Fig. 1). An ultrasound of her chest revealed that the right lung was abnormally echogenic with multiple fluid-filled cystic spaces and was surrounded by a large collection of septated fluid which suggesting empyema (Fig. 2). The diagnosis of underlying lung abscess was proposed. A subsequent Computed Tomography (CT) thorax was performed (Fig. 3) which confirmed mediastinal shift to the left, with a thick rind of peripulmonary fluid. Furthermore, the right lower lobe parenchyma appeared abnormal with a mixed solid and cystic appearance. The patient was referred for thoracotomy, with a histopathological diagnosis of pleuropulmonary blastoma. Discussion Pulmonary blastoma is a rare aggressive neoplasm which usually presents as a well-defined lung lesion or pleural effusion and

Fig. 1. — Chest X-ray demonstrating extensive opacification of the right hemithorax with marked mediastinal shift to the left and patchy left lower lobe consolidation. There was an area of aerated lung at the right apex and also a small air pocket projected to the right of the midline.

accounts for 0.25-0.5% of lung malignancies. These tumours are composed of malignant immature epithelial or mesenchymal cells which may bear a resemblance to early embryological lung tissue. They can be classified into three subgroups: Classic pulmonary blastoma (PB), well-differentiated fetal adenocarcinoma (WDFA) and pleuropulmonary blastoma (PPB) (1). Classic pulmonary blastoma is the most common subtype and often presents with non-specific respiratory symptoms with two peak age incidences in the first and fourth decades of life. Chest x-rays reveal a solitary lung mass or nodule, however 40% may be diagnosed coincidentally.

From: 1. Dept. of Radiology, Our Lady’s Hospital for Sick Children, Crumlin, Dublin, Ireland. Address for correspondence: Dr J. O’Brien, M.D., Dept. of Radiology, Our Lady’s Hospital for Sick Children, Dublin, Ireland. E-mail: juliemobrien@gmail.com

WDFA usually affects adults with a history of smoking and may be mistaken for bronchogenic carcinoma. Pleuropulmonary blastoma is a distinct tumour of childhood and can be classified into three subtypes depending on the histological appearance. Type I is a purely cystic tumour, type II has mixed solid and cystic components and type III is a solid tumour (2). There have been reports of a better prognosis associated with type I disease in comparison with Types II and III, however this has not proven to be statistically significant and may be due to the lack of data and the rarity of this tumour. On CT, PPB appears as a mixed solid and cystic lesion with a necrotic centre and variable contrast enhancement, or a persistent pleural effusion and can cause complete opacification of a hemithorax, with co-existing mediastinal shift. Differential diagnosis depends on the presentation, however, conditions such as congenital cystic adenomatoid malformation (CCAM)


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Fig. 2. — Longitudinal ultrasound scan over right lung demonstrating echogenic parenchyma with septated fluid.

should also be considered and it can be difficult to distinguish this from PPB type I (3). It is also important to note that, these lesions can often arise in association with congenital lung lesions such as the previously mentioned CCAM (4). In the case of an opaque hemi-thorax with an associated rib lesion, consideration should also be given to lymphoma or Ewing’s sarcoma. There are no specific genetic markers yet identified despite extensive investigation (5), however 25% cases are associated with familial malignancies including sarcomas, medulloblastomas, lymphoma, leukaemia and PPB. Patients are often commenced on treatment for an empyema with a poor response. Surgery is the mainstay of treatment, with a combination of

Fig. 3. — Axial post-contrast CT scan of the thorax confirms a mixed solid/cystic mass in the right hemithorax with surrounding peripulmonary fluid. There is also significant shift of the mediastinum to the left.

chemotherapy and radiotherapy, however it is an aggressive tumour with a relatively poor prognosis, which increases with tumour masses larger than 5 cm at diagnosis. Unfortunately, a pre-operative diagnosis is rarely made, and the diagnosis is usually seen retrospectively on histopathological analysis of the surgical specimen which has often not achieved definitive surgical margins. Since an adequate resection is required to prevent tumour seeding, a pre-operative consideration should be given to this diagnosis the presence of these features to ensure a better prognosis. References 1.

Walker R., Suvarna K., Matthews S.: Pulmonary blastoma: presentation of

2.

3.

4.

5.

two atypical cases and review of the literature. BJR, 2005, 78: 437-440. Priest J.R., McDermott M.B., Bhatia S., Watterson J., Mannivel J.C., Dehner L.P.: Pleuropulmonary blastoma: a clinicopathologic study of 50 cases. Cancer, 1997, 80 (1): 147161. Orazi C., Inserra A., Schingo PM., De Sio L., Cutrera R., Boldrini R., Malena S.: Pleuropulmonary blastoma, a distinctive neoplasm of childhood: report of three cases. Pediatr Radiol, 2007, 37: 337-344. Naffaa L., Donnelly L.: Imaging findings in pleuropulmonary blastoma. Pediatr Radiol, 2005, 35: 387-391. Taube J.M., Griffin C.A., Yonescu R., Morsberger L., Argani P., Askin F.B., Batista D.A.: Pleuropulmonary blastoma: cytogenetic and spectral karyotype analysis. Pediatr Dev Pathol, 2006, 9: 453-461.


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JBR–BTR, 2010, 93: 15-17.

PRIMARY PELVIC HYDATID CYST WITH SCIATIC COMPRESSION F. Nouira, T. Chouikh, A. Charieg, S. Ghorbel, S. Jlidi, B. Chaouachi1 Hydatid cysts are endemic in certain regions of the world and particulary in North Africa. They are usually located in the liver, lung, and spleen, though many uncommon locations have been reported. This is the first report of a child with primary pelvic hydatid disease causing a sciatic compression. Key-word: Echinococcosis.

Hydatid disease caused by echinococcus granulosus is endemic in Tunisia where the incidence is 241cases/year (1). It is therefore among common surgical and diagnosis problems in Tunisia. Hydatid disease has a predilection to locate in the liver and lung (90%). It can also be encountered in almost every part of the body from the crown of the head (1) to the big toe (2). Pelvic localizations represent 12% of all locations in the Tunisian publications and 1% in the European ones (3). We report on one case of pelvic hydatic cyst and expose the clinical and radiological characteristics of the disease and the therapeutic management particular of this rare location. Case report An 8- year-old boy with no specific illness was admitted for exploration of sciatica pain and limping lasting for 2 months in a context of weight loss and asthenia. Abdominal examination showed a painless pelvic non mobile mass without hepatomegaly or splenomegaly. At digital rectal examination regular para-rectal mass without sphincter troubles was detected. The neurological examination showed left sciatica pain, limping and negative Achilles’s reflex. The electromyography pointed to a lesion at the L5-S1 level. Biological explorations demonstrated normal blood concentration of αfoetoprotein and βHCG were normal. Chest X ray was normal. Abdominal Ultrasound revealed a 10 x 9 cm cystic pelvic mass without

Fig. 1. — Pelvic mass (9 cm) circumscribed by a thin wall pushing back the bladder and rectosigmoid (white arrow).

septas and with vegetations circumscribed by a proper wall. Post gadolinium abdominal MRI (Fig. 1) shows an oval pelvic mass (9 cm) circumscribed by a thin enhancing wall with a mass effect on the bladder and the rectosigmoid

From: 1. Department of Pediatric Surgery “B”, Children’s Hospital, Tunis, Tunisia. Address for correspondence: Dr F. Nouira, M.D., Department of Pediatric Surgery “B”, Hôpital d’enfants de Tunis, Bab saadoun jebbari 1007, Tunis Tunisie. E-mail: nouirafaouzi@yahoo.fr

colon and compression of the homolateral sciatica. At surgical exploration (Fig. 2) a retroperitoneal mass pushing the rectum to the right and the iliac vessels to the left was found. The aspect of prominent dome evocated hydatic cyst. The surrounding structures were protected with gauzes soaked in normal saline solution as a filter against macroscopic spillage. The cyst was then punctured and aspirated, taking extreme care to prevent inadvertent spillage of intracystic fluid. The hypertonic saline (20% NaCl)


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Fig. 2. — The cyst was opened and a discus proligerus was extracted.

solution was injected into the cyst as a scolicidal agent. After 5 minutes, a suction device with side holes was inserted into the cyst and the fluid was evacuated. The germinal membrane was then pulled out. The remaining protuberant pericystic wall was widely excised with insertion of an omental flap into the remaining cystic cavity. Postoperative recovery was prompt and uneventful with decrease of pain and limping. The patient received albendazole treatment for one year. Follow up after 2 years showed a regression of the neurological symptomatology. Discussion Retroperitoneal and retrovesical locations of a hydatid cyst are rare even in endemic areas. According to the theory of Deve, fissuring or rupture of a primary hepatic, splenic or mesenteric cyst would seed its contents in the abdominal cavity (4). This primary cyst might then heal and even disappear, leaving a scar that could be overlooked. The pouch of Douglas would then be the preferred site for the development of a secondary cyst in the pelvis, initially intraperitoneal and later subperitoneal (5). In the absence of a primary visceral lesion and of peritoneal seeding, hematogenous dissemina-

tion could explain the pathogenesis of a solitary retroperitoneal lesion. Oncospheres hatch and penetrate the intestinal wall disseminating primarily to the liver, secondarily to the lung and finally anywhere to form unilocular cysts. They also can pass through the liver and lung barriers, without seeding these structures, and develop an implant anywhere (6, 7). Other pathogenic hypotheses for an isolated retroperitoneal or retrovesical cyst have also been proposed (8, 6): migration of the larvae from the intestinal lymph vessels to the thoracic channel and then anywhere in the body through the hemorrhoidal vessels to achieve a prerectal or retrovesical location or from the rectal ampulla. Retroperitoneal cysts generally presents as a palpable mass or with flank pain as in our case. A mass was palpable on digital rectal examination. Digestive symptoms, such as constipation, abdominal pain due to the compression effect of the mass are reported. Neurological compression is in theory possible but exceptionally reported. Our patient presented limping and sciatica pain and neurological defect negative Achilles’s reflex of the left foot. The imaging findings frequently suggest hydatid disease but are usually inconclusive and a differential diagnosis may not be made before surgery. A retrovesical cyst mimics

many processes, including embryonal cyst, lymphangioma, and digestive duplicity. Serological tests are undeniable tools for diagnosis and followup of hydatitose. Enzyme-linked immunosorbent assay (ELISA) and immunoelectrophoresis are available. Indirect hemagglutination and ELISA are the most sensitive immunological methods to diagnose human hydatidosis but a false-positive reaction secondary to crossreactivity with other parasitic infections is possible. In the United States the Centers for Disease Control currently recommends a combination of specific ELISA and Western blot serology (9). According to the World Health Organization study group on echinococcosis, surgery is still the treatment of choice to provide complete cure. The optimal treatment of primary retroperitoneal hydatic cyst is complete removal of the cyst without contamination of the field and without sacrificing the organs involved through the appropriate abdominal incision (10). Removal of germinal epithelium and fluid with scoleces may cause hydatid dissemination and allergic manifestations, even anaphylactic shock. Ideally then, total cyst excision or pericystectomy should be performed (3, 11). If the localization of the cyst and invasion to vital structures prevent the total excision, partial pericystectomy is the treatment of choice after injection of scolocidal agent followed by removal of germinative membrane (12-14). Prophylactic measures, such as irrigation with a scolicidal solution are strongly recommended. Hypertonic 30% saline solution for local irrigation, evacuation of the cyst content may be necessary when the hydatid fluid is under high tension. Aspiration of the cyst has been considered an option to standard surgical therapy for elderly patients and an alternative to partial cyst excision or pericystectomy in patients with unresectable disease in the liver. Aspiration of a third of the cyst volume is followed by instillation of the same volume of 95% ethanol within the cyst. Chowbey et al reported a patient whose RHC was removed endoscopically, but this procedure was applied to only few cases and requires further study (12). Preoperative treatment with benzimidazoles (albendazole,


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mebendazole) has been reported to soften the cyst and to reduce intracystic pressure, enabling the surgeons to remove the endocyst more easily (10). Postoperative treatment with benzimidazoles of patients can reduce the rate of recurrence (10, 13). It is particularly recommended if there is cyst spillage during surgery or partial cyst removal (12). The prevalence of long-term recurrence ranges between 2 and 25%. Recurrence can be due to incomplete cyst removal or previously undetected cysts (10). Conclusion In endemic regions, HC should be considered in the differential diagnosis of retroperitoneal cystic lesions. Clinical symptoms are no specific, and appear long time at a late stage of the development of the parasit. Abdomino-pelvic US is the most useful diagnostic tool, and MRI is efficient in the differential diagnosis. Total cyst excision should be tried in all cases. When this is not possible, removal of all germinative membranes and partial pericystectomy

with the use of scolocidal agents are the treatments of choice. Additional adjuvant medical therapy is essential to avoid recurrence.

17

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References 1. Beggs I.: The radiological appearances of hydatic disease of the liver. Clin Radiol, 1983, 34: 555-563. 2. Gharbi H., Cheikh M., Hamza R.: Les locations rares de l'hydatidose chez l'enfant. Ann Radiol, 1977, 20: 151-157. 3. Bennani S., El Mrini M., Raji A., et al.: Les kystes hydatiques rétro-vésicaux et rétro-péritonéaux isolés: à propos de cinq cas. Ann Urol, 1992, 26: 244349. 4. Deve F.: L'échinococcose secondaire. Société d'Editions Scientifiques. Paris, 190. 5. Kotoulas, G., Gouliamos, A., Kalovidouris, et al. Computed tomographic localization of pelvic hydatid disease. Eur J Rad, 1990, 11: 38. 6. Ouadfel, J., Assem, A., Errougani, A., et al: Le kyste hydatique rétropéritonéal isolé. Ann Chir, 1990, 44: 243. 7. Haddad, S. I. and Khairallah, A.: Surgical consideration of hydatid disease: report of some unusual cases. Ann Surg, 1940, 111: 597. 8. Fernandez A., Silmi-Moyano A., Rodriguez-Vallejo J.M., et al:

10.

11.

12.

13.

14.

Hidatidosis retrovesical. Actas Urol Esp, 1983, 7: 165. King C.H.: Cestodes. In: Principles and Practice of Infectious Diseases, 4th ed. Edited by Mandell G.L., Bennett J. E. and Dolin R. New York: Churchill Livingstone, 1995, pp 25442553. Pawlowski Z.S., Eckert J., Vuitton D., et al.: Echinococcosis inhumans: clinical aspects, diagnosis and treatment. A Public HealthProblem of Global Concern. Paris, France: World Organisation for Animal Health and World Health Organisation, 2001, 20: 71. Njeh M., Hajri M., Chebil M., et al.: Le kyste hydatique rétro-vésical. A propos de deux cas. Ann Urol, 1993b, 27: 97. Chowbey P.K., Wadhwa A., Shah S., et al.: Endoscopic management of a retroperitoneal hydatid cyst. J Laparoendosc, 2004, 14: 236-240. Buttenschoen K., Buttenschoen D.: Echinococcus granulosus infection: the challenge of surgical treatment. Arch Surg, 2003, 388: 218-230. Durakbasa C.U., Tireli G.A., Sehiralti V., et al.: An audit on pediatric hydatid disease of uncommon localization: incidence, diagnosis, surgical approach, and outcome. J Pediatr Surg, 2006, 41: 1457-1463.


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UTERUS DIDELPHYS WITH OBSTRUCTED HEMIVAGINA AND RENAL AGENESIS: MRI FINDINGS A. Talebian Yazdi, K. De Smet, C. Ernst, B. Desprechins, J. de Mey1 Müllerian duct abnormalities (MDA) are developmental disorders leading to dysmorphism of the female genital tract. Currently the Buttram and Gibbons classification of these entities is widely used. We present a case of a young girl with uterus didelphys and ipsilateral renal agenesis. Key-word: Uterus, abnormalities.

Müllerian duct abnormalities (MDA) are developmental disorders leading to dysmorphism of the female genital tract. The disruption of the normal embryologic fusion of the paramesonephric (Müllerian) ducts or the non-resorption of the uterine septum is believed to be the crucial element in this entity (1, 2). Buttram and Gibbons have created a widely used classification of MDA (3) MDA are rare syndromes and scarcely reported on in English literature. But the introduction of the modern MRI equipment has led to improved recognition and familiarity with these complex malformations. Clinically the patients present with nonspecific symptoms around the time of menarche. Imaging can be helpful in the correct diagnosis, preventing delayed surgical intervention and possible infertility. In this report we present a case of uterus didelphys with an obstructed hemivagina and ipsilateral renal agenesis (Buttram and Gibbons class III) and a brief review of literature. Case report A 12-year-old female patient presented at our emergency department with intermittent abdominal pain and heavy vaginal blood loss. The menstrual cycle had started 6 months before and was inconspicuous until the last 2 months. At that time menstruation was accompanied by intense pains and heavy blood loss. Initial clinical workup did not lead to a specific diagnosis. Pelvic ultrasound was performed and showed hematometra with suspicion of uter-

Fig. 1. — Axial T2WI demonstrating the presence of a didelphic uterus with a small right (short arrow) horn and an abnormally distended left horn (large arrow) due to a fluid collection consistent with old menstrual blood.

ine dysmorphism. The patient was scheduled for a MRI of the pelvis. The MRI protocol consisted of standard T1 and T2 sequences with and without fat suppression and reconstructions in the three anatomical planes. A total duplication of the uterus was demonstrated. The vagina was divided into separate chambers by a septum. The left hemivagina and hemi-uterus were distended and contained a fluid collection. The signal characteristics of this collection were compatible with old (menstrual) blood (Fig. 1-4). The right hemi-uterus had a normal appearance; the right hemivagina was displaced by the fluid containing left hemivagina. The ovaries had a normal appearance.

From: 1. Department of Radiology, University Hospital Brussels, UZ Brussel, Brussels, Belgium Address for correspondence: Dr. A. Talebian Yazdi, MD, Department of Radiology, University Hospital Brussels, UZ Brussel, Laarbeeklaan 101, B-1090 Brussel, Belgium E-mail: atalebiany@hotmail.com

Ipsilaterally to the affected side of the internal genitalia, absence of the left kidney was noted (Fig. 5). The MRI results were consistent with uterus didelphys with an obstructed hemivagina and ipsilateral renal agenesis. The patient underwent gynaecologic intervention, the obstructed hemivagina was opened and the septum between the two vaginas was excised. There were no major complications and the next day the patient left our hospital in good condition. Discussion MDA are developmental disorders that can lead to a variety of abnormalities in the female urogenital tract. The reported incidence in English literature is 0.5-5% (1, 2). Buttram and Gibbons have classified the various MDA into 6 categories (3). The presented case illustrates the imaging findings of uterus didelphys with an imperforate hemivagina and


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Fig. 4. — Coronal T2WI and drawing, demonstrating the morphology of the didelphic uterus with the small right (short arrow) horn and the abnormally distended left horn (large arrow). The vagina (arrowhead) is also abnormally distended due to the persistent vaginal septum.

Fig. 2. — Right parasagittal T2WI demonstrating the small right horn of the didelphic uterus (arrow).

Fig. 5. — Coronal T2WI demonstrating the absence of the left kidney in the left renal fossa (asterisk).

Fig. 3. — Left parasagittal T2WI demonstrating the abnormally distended left horn of the didelphic uterus (arrow) due to a fluid collection consistent with old menstrual blood.

an absent left kidney (Buttram and Gibbons class III anomaly). The presence of two separate and divergent uterine horns, two cervices and a duplicated vagina with partial obstruction are characteristic for this entity. Knowledge of the normal embryologic events aids in the understanding of this complex dimorphic syndrome. The cascade of events in the development of the female genital tract occurs during the 6th and 22nd week of gesta-

tional age. The paramesonephric (Müllerian) ducts move toward the midline, fuse and insert in the urogenital sinus. Any disruption in this cascade of events leads to abnormalities of the genital tract. In the specific case of uterus didelphys there is a complete nonfusion of the Müllerian ducts leading to the duplication, of the uterus and the vagina. Disorders of the urologic tract, in our case the absence of the left kidney, could be explained by the close


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embryologic relationship between the mesonephric (Wolffian) ducts and the paramesonephric ducts. Various teratologic influences are believed to cause concomitant urologic and gynaecologic malformations (4-6) MRI, with its superior soft tissue resolution, often leads to a straightforward diagnosis of complex Müllerian duct abnormalities. Therefore it is considered the best noninvasive tool in the work-up of malformations of the urogenital tract. Knowledge of the main imaging findings of uterus didelphys and other MDA will aid in the timely and correct diagnosis. Complications of delayed diagnosis are infection with subsequent abscess formation and hysterectomy with or without oophorectomy leading to infertility.

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Conclusion Müllerian duct abnormalities encompass a variety of dimorphic changes in the female genital tract, often with associated urologic anomalies. In this paper the MR characteristics of uterus didelphys with an imperforate hemivagina and an absent left kidney were discussed. Familiarity of the radiologist with the imaging findings of this rare syndrome is important, because delayed diagnosis increases the chance of complication and possible infertility. References 1.

Nahum G.G. Uterine anomalies: how common are they and what is

2.

3.

4.

5.

6.

their distribution among subtypes? J Reprod Med, 1998, 43: 877–887. Stampe Sorensen S. Estimated prevalence of müllerian anomalies. Acta Obstet Gynecol Scand, 1988, 67: 441-445. Buttram V.C. Jr, Gibbons W.E. Müllerian anomalies: a proposed classification (an analysis of 144 cases). Fertil Steril, 1979, 32: 40-46. Anderson K.A., McAninch J.W. Uterus didelphia with left hematocolpos and ipsilateral renal agenesis. J Urol, 1982, 127: 550-553. Yoder I.C., Pfister R.C. Unilateral hematocolpos and ipsilateral renal agenesis: report of two cases and review of the literature. AJR, 1976, 127: 303-308. Eisenberg E., Farber M., Mitchell Jr, G.W. Complete duplication of the uterus and cervix with a unilaterally imperforate vagina. Obstet Gynecol 1982, 60: 259-262.


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ACUTE ADRENAL INSUFFICIENCY DUE TO BILATERAL ADRENAL HEMORRHAGE X. Zhu1, I.C. van der Schaaf1, J.A. van der Valk3, A.K. Bartelink3, M. Nix4 We report a rare case of bilateral hemorrhage of adrenal glands diagnosed in a 61-year-old female. The case presented with classic clinical findings and typical imaging features. Key-words: Adrenal gland – hemorrhage.

Adrenal hemorrhage is an uncommon event observed in patients of all ages. It usually occurs as a complication of physiologic stress, trauma, or a coagulopathic state. Although most cases of adrenal hemorrhage have a benign clinical course, bilateral adrenal hemorrhage could result in an Addisonian crisis and is a potentially life threatening disease. Therefore, it is important to promptly recognize adrenal hemorrhage on imaging studies and to realize its clinical implications. The reported case illustrates the typical imaging findings of bilateral adrenal hemorrhage causing adrenal insufficiency. Case report A 61-year-old woman was referred to our hospital with fever and acute low abdominal pain. Laboratory tests revealed elevated inflammatory markers. An abdominal CT scan demonstrated perforated sigmoid diverticulitis with a small abscess collection. The patient underwent a Hartmann procedure. She was readmitted two weeks later with fever, abdominal pain, complaints of confusion, nausea, general weakness and anorexia. Laboratory tests showed increased inflammatory markers and hyponatremia. Potassium was normal. A contrast enhanced abdominal CT revealed a wound infection in the laparatomy area, possibly explaining the abdominal pain. On this abdominal CT both adrenal glands appeared significantly increased in size with hazy borders when compared to the preoperative CT (Fig. 1 and 2). The diagnosis of bilateral adrenal hemorrhage with secondary insufficiency

Fig. 1. — Admission (pre-operative) scan: contrast enhanced CT scan showing a normal aspect of both adrenal glands (arrowheads).

Fig. 2. — Follow up scan two weeks postoperative: contrast enhanced CT scan showing bilateral enlarged adrenal glands with less sharp borders compared to Fig. 1.

of the adrenal glands was proposed and could explain both the hyponatremia and clinical symptoms of confusion, weakness and anorexia.

From: 1. Department of Urology, Erasmus Medical Centre, Rotterdam, The Netherlands; 2. Department of Radiology, University Medical Centre Utrecht, Utrecht, The Netherlands; 3. Department of Internal Medicine, Meander Medical Centre, Amersfoort, The Netherlands. 4. Department of Radiology, Meander Medical Centre, Amersfoort, The Netherlands. Address for correspondence: Dr Y. Zhu, MD, Department of Urology, Erasmus Medical Centre, Room NH 227, P.O. Box 2040, 3000 CA Rotterdam,The Netherlands, Email: xiaoyezhu@hotmail.com

Hyponatremia was first treated which improved symptoms of confusion. The wound infection was treated surgically. The adrenal failure was proved through a low dose short stimulation test with a synthetic ACTH analog (tetracosactide). The patient was substituted with hydrocortisone and fludrocortisone. The patient was discharged from the hospital five days later. Follow up and treatment of the adrenal insufficiency was performed in the outpa-


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Fig. 3. — Follow up scan three months postoperative: unenhanced CT scan showing a normalized size and attenuation values of both adrenal glands (A = right, B = left).

tient clinic. Three months later the CT scan showed normalization of size and shape of both adrenal glands (Fig. 3a and b). Seven months after the adrenal hemorrhage the patient still needed corticosteroid supplementation. Discussion Adrenal hemorrhage can occur as a complication of acute stress, neonatal stress, blunt trauma, sepsis, underlying tumor, coagulopathic state, or idiopathic disease (1). Trauma accounts for 80% of cases of adrenal hemorrhage (2). Recent studies, using advanced imaging modalities, report an incidence of adrenal hemorrhage ranging from 0.8% to 2% in a trauma setting (3). Symptoms of adrenal hemorrhage are unclear and may include acute abdominal pain, nausea, vomiting, hypotension, hypertension, agitation, mental status change, and low-grade fever. Laboratory tests are often non-specific and rarely show a significant drop in haemoglobin level. Also it may induce hyponatremia and hyperkalemia (4). Since both clinical presentation and laboratory tests are indefinite, adrenal hemorrhage could be easily overlooked. Although most cases carry a benign clinical course, bilateral adrenal hemorrhage may lead to acute adrenal crisis (Addisonian crisis), shock, and death unless it is recognized promptly and treated appropriately. Bilateral hemorrhaqe is particularly associated with anticoagulation therapy or a blood dyscrasia, less commonly it is associated with stress of surgery. The incidence of adrenal insufficiency in case of bilat-

eral adrenal hemorrhage ranges from 16.7% to 50% (5). Hence, early recognition of bilateral hemorrhage by means of abdominal imaging is important. CT scan, US, or MRI can be utilized to diagnose adrenal hemorrhage (2, 6). CT scan is preferred in all age groups except for neonates where ultrasound may be more beneficial. Findings of adrenal hemorrhage include peri-adrenal fat stranding and thickening of adjacent diaphragmatic crura. Also it is characterized by increased volume of the adrenal gland by a round or oval mass and hyper attenuating foci in the range of +50 to +90 HU (7). Differential diagnoses to be considered include leaking aortic aneurysm, bleeding from retroperitoneal organs, renal vein thrombosis, and abdominal abscess. Depending on the cause of adrenal hemorrhage, most cases will not require surgical exploration. Nonoperative management is recommended in the absence of ongoing bleeding and includes supportive care and evaluation of adrenal function (1). ln case of adrenal insufficiency, corticosteroids replacement should be applied. Adrenal hematomas decrease in size and attenuation over time, and often resolve spontaneously (6). Patients with adrenal insufficiency after bilateral adrenal hemorrhage should continue oral steroid replacement on discharge. However, one study with a long-term follow-up of four patients has demonstrated that some patients can recover adrenal function (8). ln conclusion, the clinical presentation of adrenal hemorrhage may

be indifferent and laboratory tests are often non-specific. Therefore, the diagnosis could be easily overlooked. On abdominal imaging – mostly CT – findings are quite typical but may be missed because the indication and the radiologist's attention are focused on other organs. Radiologists should be aware of this potentially lethal condition and careful evaluation of the adrenals in each abdominal imaging study is strongly recommended. References 1.

2.

3.

4. 5.

6.

7. 8.

Simon D.R., Palese M.A. Clinical update on the management of adrenal hemorrhage. Curr Urol Rep, 2009, 10: 78-83. Mayo-Smith W.W., Boland G.W., Noto R.B., Lee M.J. State-of-the-art adrenal imaging. Radiographics, 2001, 21: 995-1012. Rana A.I., Kenney P.J., Lockhart M.E. et al. Adrenal gland hematomas in trauma patients. Radiology, 2004, 230: 669-675. Arlt W., Allolio B. Adrenal insufficiency. Lancet 2003,361: 1881-1883. Mehrazin R., Derweesh I.H., Kincade M.C. et al. Adrenal trauma: Elvis Presley Memorial Trauma Center experience. Urology, 2007, 70: 851-855. Kawashima A., Sandler C.M., Ernst R.D. et al. Imaging of nontraumatic hemorrhage of the adrenal gland. Radiographics, 1999, 19: 949-963. Dunnick N.R., Korobkin M. Imaging of adrenal incidentalomas: current status. AJR, 2002, 179: 559-568. Jahangir-Hekmat M., Taylor H.C., Levin H., Wilbur M., Llerena L.A. Adrenal insufficiency attributable to adrenal hemorrhage: long-term follow-up with reference to glucocorticoid and mineralocorticoid function and replacement. Endocr Pract, 2004, 10: 55-61.


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JBR–BTR, 2011, 94: 23-25.

HEPATIC ALVEOLAR ECHINOCOCCOSIS: A DIAGNOSTIC CHALLENGE O. Yapici, S.M. Erturk, M. Ulusay, A. Ozel, A. Halefoglu, Z. Karpat, M. Basak1 We report a case of alveolar echinococcosis involving the liver in a 61-year-old male. Alveolar echinococcosis is a rare chronic and progressive disease, which can involve mostly liver and in rare cases lung and brain. It is caused by Echinococcus multilocularis. In this report we describe the imaging findings of hepatic involvement of alveolar echinococcosis by ultrasonography, computed tomography and magnetic resonance imaging. Key-word: Echinococcosis.

Case report Alveolar echinococcosis is a rare, but potentially fatal, chronic and progressive disease, caused by infestation of the liver by Echinococcus multilocularis. Humans are the intermediate host that become infected by ingestion of the wild berries, plants or water which is contaminated with eggs of the parasites or by direct contact with the definitive host, which is the wild canine (foxes, wolves). The disease is usually seen in central and northern Europe, North America, Canada, China and Japan (1). It is endemic in eastern Turkey (2). An invasive tumor-like multi-vesiculated lesion develops during a long, asymptomatic period which is characteristic for the disease. In this study we present a case of Echinococcus multilocularis infestation, forming a tumor-like lesion in the liver. A 61- year-old man was referred to our clinic with right-upper quadrant pain, weight loss and fatigue of almost 1 year duration. Physical examination revealed no significant finding. Abnormal laboratory findings were as follows: erythrocyte sedimentation rate 52 mm/h (525 mm/h), gamma-glutamyl transferase 122 IU/L (8-61 IU/L), lactate dehydrogenase 500 IU/L (240480 IU/L), serum aspartate transaminase 52 IU/L (0-37 IU/L), white blood cell count 10.75 x10^3/Ul (3.810.0 x 10^3/Ul), neutrophil percentage 32.8 (45-78%), eosinophil percentage 34.8 (0.8- 7.0%), haemoglobin 11.6 g/dL (13.0-17.5 g/dL), CA 125 level 48.2 U/ml (0-30.2 U/ml), CA 199 level 71.4 U/ml (0-37 U/ml). Hepatitis B surface antigen level was 287.8 mIU/ml in the serum (positive: > 10 mIU/ml). Ultrasonography (Aplio XV, Toshiba, Tokyo, Japan) revealed a

large heterogeneous liver mass almost completely filling the right lobe with isoechoic and hyperechoic areas relative to the normal liver parenchyma. There was a small hypoechoic cystic space with irregular margins on the right upper peripheral region (Fig. 1A). The mass had irregular, indistinct borders. It demonstrated no significant finding by colored Doppler ultrasonography. Because the use of sonographic contrast agents were not allowed by the ministry of health in Turkey, contrast enhanced ultrasonography could not be performed. Unenhanced abdominal computed tomography (Siemens Somatom Sensation 16-detector row CT, Erlangen, Germany) revealed an area of heterogeneous density in the right lobe of the liver with some millimetric calcifications located centrally (Fig. 1B). The margins were indistinct and ill-defined. There was no prominent contrast enhancement after the iodinated contrast medium injection (Fig. 1C). Magnetic Resonance imaging (Signa Excite HD 1.5T; GE Healthcare, Milwaukee, WI, USA) of the liver revealed a heterogeneous hypointense mass with irregular margins on both unenhanced T1 and T2- weighted images (Fig. 2A-B). The lesion measured 16 cm x 12 cm; it was almost totally filling the right upper lobe and extending into the left lobe. It had a right peripheral cystic necrotic area. After the administration of hepatocyte-specific contrast agent (Primovist 0.25 mmol/ml - Bayer Schering, Berlin,Germany), the lesion revealed no contrast enhancement in arterial (Fig. 2C) and portal venous phases. There was no contrast enhancement in the late hepatobiliary phase (Fig. 2D), indicating that the mass does not contain any hepatocytes.

From: Department of Radiology, Sisli Etfal Training and Research Hospital, Istanbul, Turkey. Address for correspondence: Dr O. Yapici, Department of Radiology, Sisli Etfal Training and Research Hospital, Istanbul, Turkey. E-mail: ozgevapici@hotmail.com

Following the radiological workup, the patient was referred to the liver biopsy. The biopsy was completed without any complication. The histopathologic work-up, as the most reliable method to have the definitive diagnosis, showed hepatic alveolar echinococcosis. Discussion Hepatic infestation of echinococcus multilocularis appears as an ill-defined infiltrative disease usually consisting of cystic and solid components. Microcalcifications are commonly seen (3). Necrotic areas frequently develop due to ischemia as the lesion size increase. The surrounding vascular structures and biliary system may be infiltrated by the disease or they may even be displaced due to the mass effect of the lesion, which imitates a slowgrowing tumor (3). In most cases, lesions are hyperechoic with heterogeneous echopattern and indistinct margins on ultrasonography (USG) (1). Cavitary or cystic to vesicular appearance can be seen within the masses. Color Doppler USG shows the absence of vascular flow in the solid components of the lesions (4). CT imaging of the liver infestation shows heterogeneous, ill-defined, hypodense areas which have poor or no enhancement after intravenous contrast administration (1). In 90% of the infected cases, calcifications are found (1). Pseudocystic necrotic areas and dilatation of the intrahepatic bile ducts can also be identified (3). The fibrotic changes of the disease process and extension of the lesion within the liver parenchyma can be demonstrated by MRI (3). Fibrosis and parasitic tissue have low signal intensity on T1-weighted images (3). On T2-weighted images, lesions may be hypointense, hyperintense or isointense (1). Central necrotic zones and cystic peripheral extensions are better identified on T2-weighted images (3).


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A

JBR–BTR, 2011, 94 (1)

B Fig. 1. — A: Axial grey-scale ultrasound image reveals a large hepatic mass with heterogenous echogenity. B: On unenhanced CT image the mass shows heterogeneous hypodensity and contains calcific areas. C: After intravenous administration of the iodinated contrast material, the mass does not show any enhancement.

C There is poor or no enhancement after intravenous administration of gadolinium (1). CT and MRI findings of alveolar echinococcosis can overlap with those seen in malignant liver neoplasms such as hepatocellular carcinoma (HCC), intrahepatic cholangiocarcinoma (IHCC), solitary metastasis and primary lymphoma of the liver. On unenhanced CT scans, HCC is usually demonstrated as a large, hypodense mass, often with central areas of low attenuation representing areas of necrosis (5). On MRI, HCC lesions typically appear hypointense on T1-weighted sequences; hyperintense on T2weighted sequences. On both CT and MRI, on contrast-enhanced images, increased arterial enhancement and a rapid washout is observed (6). Intrahepatic cholangiocarcinoma appears as a homogenous, hypodense mass on unenhanced CT images. On MRI it is seen as a hypointense mass on T1-weighted images and as a hyperintense mass

on T2-weighted images (7, 8). After the intravenous contrast agent administration, the enhancement patterns depend on the size of the lesion (8). Larger lesions (> 4 cm) show peripheral enhancement which progress centripetally, while smaller lesions (2-4 cm) enhance homogenously (8). On unenhanced CT scans, primary lymphoma of the liver is often a poorly defined mass of low attenuation. Necrosis and calcification may be observed (9). On T1- weighted MR images lesions are hypointense. On T2- weighted images, low to moderately high signal intensity is seen. After intravenous contrast administration, enhancement is predominantly peripheral (10). Most of the solitary liver metastases appear hypodense relative to liver parenchyma on unenhanced CT images (11). On T1-weighted MR images the lesions are usually hypointense. Lesions frequently show heterogeneously moderate to moderately high signal intensity on T2-weighted images. Since the majority of the metastatic liver

lesions are hypovascular, they show a diminished late enhancement on contrast-enhanced dynamic imaging (12, 13). Unlike E. granulosus infection, which has typical radiological patterns such as daughter cysts and detached membranes, alveolar echinococcosis is hard to diagnose in most cases (14). Unfortunately, no pathognomonic feature of hepatic alveolar echinococcosis is identified. The clinical course may resemble that of a slowly developing tumor (15). The definite diagnosis can be reached by correlating radiological findings with the clinical and laboratory data, and in some cases by the histopathologic work-up. Even the cyst aspiration for diagnosis was considered as a possible risk of anaphylaxis or recurrence due to the spillage, USG guided fine needle aspiration biopsy is used to confirm the diagnosis in some cases (16, 17). Especially in endemic regions, if a complex appearing liver lesion is detected, alveolar echinococcosis must be definitely included in the differential diagnosis. References 1. Czermak B.V., Akhan O., Hiemetzberger R., Zelger B., Vogel W., Jaschke W., Rieger M., Kim S.Y., Lim J.H.: Echinococcosis of the liver. Abdom Imaging, 2008, 33: 133-143. 2. Ozkok A., Gul E., Okumus G., Yekeler E., Gulluoglu M.G., Kiyan E., Arseven O.: Disseminated alveolar echinococcosis mimicking a metastatic malignancy. Intern Med, 2008, 47: 1495-1497.


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Fig. 2. — On T1-weighted (A) and T2-weighted (B) MR images the mass shows heterogenous signal intensity. On T1-weighted MR images obtained after intravenous administration of gadolinium-containing hepatocyte specific contrast material (C), the mass does not enhance in the arterial phase. On images obtained during the hepatobiliary phase (D), there is no enhancement. neither.

3. Etlik O., Bay A., Arslan H., Harman M., Kosem M., Temizoz O., Dogan E.: Contrast-enhanced CT and MRI findings of atypical hepatic Echinococcus alveolaris infestation. Pediatr Radiol, 2005, 35: 546-549. 4. Coskun A., Ozturk M., Karahan O.I., Erdogan N., Isin S., Gulec M.: Alveolar echinococcosis of the liver: correlative color Doppler US, CT, and MRI study. Acta Radiol, 2004, 45: 492498. 5. McTavish J.D., Ros P.R.: Hepatic Mass Lesions. In: Haaga J.R., Lanzier C.F., Gilkeson R.C. (eds). CT and MR Imaging of the Whole Body (4th ed). St Louis, MI, USA: Mosby, 2003, 12841286. 6. Van den Bos I.C., Hussain S.M., de Man R.A., Zondervan P.E., et al.: Magnetic Resonance Imaging of Liver Lesions: Exceptions and Atypical Lesions. Curr Probl Diagn Radiol, 2008, 37: 95-103. 7. Fan Z.M., Yamashita Y., Harada M., et al.: Intrahepatic cholangiocarcinoma: spin-echo and contrast enhanced

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9. 10.

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dynamic MR imaging. AJR, 1993, 161: 313-317. Adjei O.N., Tamura S., Sugimura H., et al.: Contrast enhanced MR imaging of intrahepatic cholangiocarcinoma. Clin Radiol, 1995, 50: 6-10. Sanders L.M., Botet J.F., Straus D.J., et al.: CT of Primary Lymphoma of the Liver. AJR, 1989, 152: 973-976. Semelka R.C., Martin D.R., Balci N.C.: Focal lesions in normal liver. J Gastroenterol Hepatol, 2005, 20, 1478-1487. Ros P.R., Taylor H.M.: Malignant Tumors of the Liver. In Gore RM, Levine SM (eds): Textbook of Gastrointestinal Radiology (2nd ed). Philadelphia: W.B. Saunders Company, 2000, 1523-1568. Hamm B., Thoeni R.F., Gould R.G., et al.: Focal liver lesions: characterization with nonenhanced and dynamic contrast material-enhanced MR imaging. Radiology, 1994,, 190: 417423. Danet I.M., Semelka R.C., Nagase L.L., Woosely J.T.,

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Leonardou P., Armao D.: Liver metastases from pancreatic adenocarcinoma: MR imaging characteristics. J Magn Reson Imaging, 2003, 18: 181-188. Czermak B.V., Unsinn K.M., Gotwald T., Niehoff A.N., Freund M.C., Waldenberger P., Vogel W., Jaschke W.R.: Echinococcus granulosus Revisited. AJR, 2001, 177: 10511056. Czermak B.V., Unsinn K.M., Gotwald T., Niehoff A.N., Freund M.C., Waldenberger P., Vogel W., Jaschke W.R.: Echinococcus multilocularis Revisited. AJR, 2001, 176: 1207-1212. Bilgen C., Oner K., Ovul I., Kirazli T.: Vertebral hydatid disease presenting as a parapharyngeal and neck mass: a case report. Otolarygol Head Neck Surg, 2002, 126: 89-90. Celik A., Turanli M., Kutun S., et al.: Unusual location of hydatid cyst: soft tissue mass in the neck. Eur Arch Otorhinolaryngol, 2006, 263: 11471150.


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WERNICKE’S ENCEPHALOPATHY: A CASE REPORT AND MRI FINDINGS S. Yucebilgin1, T. Cirpan1, C.Y. Sanhal1, E. Ozan2, T. Acar2, S. Ozsener1 Wernicke’s encephalopathy (WE) is a serious, potentially fatal acute or subacute neurological disorder caused by thiamine (Vitamin B1) deficiency. Although it is most frequently observed in patients who are chronic alcoholics, WE may also be associated with hyperemesis gravidarum. We report magnetic resonance imaging (MRI) and diffusion weighted imaging (DWI) findings in this case of WE in a pregnant patient with hyperemesis gravidarum. We conclude that DWI should be included in the imaging protocols of patients suspected to suffer from Wernicke’s encephalopathy. Key-words: Pregnancy, complications – Brain, diseases.

Nausea and vomiting generally peak between the 8th and 12th weeks of pregnancy, and they are usually mild and self-limited (1). Hyperemesis gravidarum, however, is a severe condition with vomiting pernicious enough to cause weight loss, dehydration and alkalosis. It affects among 0.3-2% of all pregnant patients (2). Wernicke’s encephalopathy (WE) is a disorder due to thiamine deficiency associated with alcoholism and malnutrition but can also arise during the first trimester of pregnancy, due to hyperemesis gravidarum. We present conventional magnetic resonanace imaging (MRI) and diffusion weighted imaging (DWI) findings in this unusual case of WE.

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Fig. 1. — A. T2-weighted transaxial image demonstrating symmetrical bilateral hyperintense lesions in both dorsomedial thalamic regions. B. FLAIR image demonstrating symmetrical hyperintense lesions within dorsomedial thalamic regions.

Case report A 37-year-old gravida 1 para 0 woman in her 5th week of gestation presented with the diagnosis of hyperemesis gravidarum after uncontrolled anorexia, constant nausea, vomiting and intermittent diarrhea had persisted longer than 5 days. Her physical examination was normal. Laboratory studies were remarkable only for an elevated alanine aminotransferase (ALT) level of 60 U/L [normal range 1031 U/L] and ketonuria. Other parameters including thyroid function tests, urine and stool culture for bacterias, abdominopelvic ultrasound, serological tests for hepatitis and immunological markers for liver diseases were normal. On hospital day 6, ultrasound scan revealed a 6 week-old heart beating fetus. Routine hyperemesis gravidarum

therapy continued to the 10th week of gestation. At that time the patient became more depressive (had attacks of crying) and less cooperative. She had physchiatric consultation but no drugs or other medications were given. Her complete neurological examination was unremarkable, however a cranial MRI was offered to exclude any intracranial pathologies. The patient refused MRI with pretending her claustrophobia. Her general state of nause, vomiting and insomnia worsened over the next 2 weeks with 4 kilograms loss. During the 12th gestational week, she developed signs of confusion, depression, muscle weakness, ataxia and a tendancy to fall backwards, and a sudden decrease in vision. Fundoscopic examination

From: 1. Department of Obstetrics and Gynecology, 2. Department of Radiology, Ege University, Izmir, Turkey. Address for correspondence: Dr C.Y. Sanhal, M.D., Department of Obstetrics and Gynecology, Ege University, Bornova Asfaltı, Izmir, Turkey. E-mail: cemsanhal@yahoo.com

revealed loss of border-lines of the discs and papiledema. Vision acuity decreased to 15 cm. The patient was not able to stand and walk because of the truncal ataxia. Total parenteral nutrition and electrolyte replacement therapy were started. With the dramatic loss in her vision, she accepted MRI. Because of the increased rates of thrombotic events in pregnancy, venous sinus thrombosis was suggested initially. Magnetic resonance venography and magnetic resonance angiography showed normal vessels. T2-weighted and fluid attenuated inversion recovery (FLAIR) images demonstrated symmetrical hyperintense lesions within dorsomedial thalamic region (Fig. 1). DWI showed symmetrical pathologic thalamic hyperintensities and the apparent diffusion coefficient (ADC) map images showed signal reductions suggesting restricted diffusion within these regions (Fig. 2). As the neurologic signs and MRI findings pointed to a diagnosis of WE, the patient was transferred to neurology department. Laboratory


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WERNICKE’S ENCEPHALOPATHY — YUCEBILGIN et al

Fig. 2. — DWI image showing hyperintensities in both thalami.

estimation of serum thiamine was not available before administration of it. Her vision was back three days after the initial rapid intravenous infusion of 250 mg thiamine and the oral treatment of 250 mg/day with other vitamin B complexes. By the second week, her symptoms lost. She could stand and walk without help. Discussion WE is a rare but known complication of hyperemesis gravidarum resulting from the combination of poor nutritional intake, frequent vomiting, and increased metabolic demands of pregnancy (3). WE can also be precipitated by parenteral nutrition or infusion of glucosecontaining solutions without prior administration of thiamine (4, 5). The clinical features are nonspecific but include the classical triad of ocular abnormalities, confusion and ataxia. It should be remembered that the clinical triad is not obligatory. Symptoms may include lethargy, fatigue, apathy, impaired awareness, equilibrium loss, disorientation, difficulty to concentrate, retrograd amnesia, anorexia, muscu-

lar weakness, peripheral numbness, paresthesia, disorientation, hallucinations, confabulation, memory loss, impaired linguistic processing, anterograde amnesia and global intellectual impairment (6). The diagnosis of WE is based on the clinical manifestations and rapid reversal of symptoms with thiamine. Early diagnosis is essential in WE in order to avoid persistent brain damage (7). Determination of blood transketolase activity and thiamine pyrophosphate reflects the thiamine status in the body. But only few centers have this diagnostic tool due to technological complexity and cost (8). Hence, MRI plays an important role in the diagnosis of WE. Common MRI findings of WE are symmetrically increased signal intensities in the mesencephalic tegmentum, mamillary body and medial thalamus on proton-density and T2weighted images. In addition, due to the possibility that cerebrospinal fluid may mask high signal lesions on T2-weighted and proton-weighted images, fluid-attenuated inversionrecovery (FLAIR) sequences were found to be better in detecting the lesion conspicuously (9). Diffusionweighted imaging (DWI) is based on an echo-planar MRI technique, and it is highly sensitive to intracellular edema. DWI changes with decreased signal intensity on ADC (apparent diffusion coefficient) maps are associated with restricted diffusion and cytotoxic edema, while increased ADC values represent vasogenic edema. The importance of ADC values in the diagnosis of WE is unclear, since decreased or increased ADC values have been reported and both cytotoxic and vasogenic edema patterns are present in lesions seen in WE (7, 10). Our patient showed high signal intensities within the thalami on DWI images and demonstrated decreased ADC values. We assume that the high signals seen on DWI images were, at least in part, caused by true restricted diffusion representing cytotoxic edema.

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In conclusion we presented an unusual case of WE in a pregnant patient with hyperemesis gravidarum. This case emphasizes that, while conventional MRI is helpful, DWI should be considered as a valuable additional imaging sequence in patients suspected to suffer from WE. References 1. Hill J.B., Yost N.P., Wendel G.D. Jr.: Acute renal failure in association with severe hyperemesis gravidarum. Obstet Gynecol, 2002, 100: 1119-1121. 2. Eliakim R., Abulafia O., Sherer D.M.: Hyperemesis gravidarum: a current review. Am J Perinatol, 2000, 17: 207218. 3. Chiossi G., Neri I., Cavazzuti M., Basso G., Facchinetti F.: Hyperemesis gravidarum complicated by Wernicke encephalopathy: background, case report, and review of the literature. Obstet Gynecol Surv, 2006, 61: 255268. 4. Reuler J.B., Girard D.E., Cooney T.G: Wernicke’s encephalopathy. N Engl J Med, 1985, 312: 1035-1039. 5. Togay-Isikay C., Yigit A., Mutluer N.: Wernicke's encephalopathy due to hyperemesis gravidarum: an underrecognised condition. Aust N Z J Obstet Gynaecol, 2001, 41: 453-456. 6. Accetta S.G., Abeche A.M., Buchabqui J.A., et al.: Memory loss and ataxia after hyperemesis gravidarum: a case of Wernicke-Korsakoff syndrome. Eur J Obstet Gynecol Reprod Biol, 2002, 102: 100-101. 7. Halavaara J., Brander A., Lyytinen J., Setälä K., Kallela M.: Wernicke's encephalopathy: is diffusion-weighted MRI useful?. Neuroradiology, 2003, 45: 519-523. 8. Fei G.-q., Zhong C., Jin L., et al.: Clinical characteristics and MR imaging features of nonalcoholic Wernicke's encephalopathy. Am J Neuroradiol, 2008, 29: 164-169. 9. Ashikaga R., Araki Y., Ono Y., et al.: FLAIR appearance of Wernicke encephalopathy. Radiat Med, 1997, 15: 251253. 10. Lapergue B., Klein I., Olivot J.M., Amarenco P.: Diffusion weighted imaging of cerebellar lesions in Wernicke's encephalopathy. J Neuroradiol, 2006, 33: 126-128.


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DIFFUSE “VERTEBRA-WITHIN-VERTEBRA” APPEARANCE AT THE ADULT AGE DUE TO BIPHOSPHONATE (PAMIDRONATE) ADMINISTRATION DURING EARLY ADOLESCENCE B. Coulier1 We report a rare case of diffuse alteration of the vertebral bony structure fortuitously found in a 20-year-old patient and essentially characterized by an impressive “vertebra-within-vertebra” appearance. This aspect was found being the result of an unusual use of intravenous perfusions of biphosphonate (Pamidronate) during early adolescence for reflex sympathetic dystrophy after tibial fracture. The clinical applications of biphosphonates are briefly reminded and the physiopathology of the induced bone changes is explained. Key-words: Biphosphonates – Bones, growth and development – Children, skeletal system.

Biphosphonates are not commonly used in childhood and adolescence youth except for very specific entities like osteogenesis imperfecta, recurrent multifocal osteomyelitis and juvenile osteoporosis. We report a very rare case of diffuse alteration of the vertebral bony structure fortuitously found in a 20-year-old patient and essentially characterized by an impressive “vertebra-within-vertebra” appearance. This aspect was found being the result of an unusual use of intravenous perfusions of biphosphonate (Pamidronate) during early adolescence (at the age of eleven) for – probably overestimated – reflex sympathetic dystrophy after tibial fracture. The clinical applications of biphosphonates are briefly remembered and the physiopathology of the induced bone changes is explained. Case report A 20-year-old patient was referred for X-ray evaluation of spine after a car accident. He presented with complaints of moderate low back pain. Plain films failed to reveal any vertebral fracture but demonstrated atypical uniform diffuse alterations of the vertebral structure. All vertebral bodies were delineated by a double bony cortex creating an impressing "bone-within-bone" or "vertebra-within-vertebra" appearance. Duplication of the bony cortex was also found at the level of the iliac crests, acetabular roofs, sacroiliac joints and vertebral spinous processes (Fig. 1, 2). The patient was unsuccessfully questioned about the occurrence of serious medical

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Fig. 1. — Lateral plains films of the thoracic (A) and lumbar (B) spine. Vertebral bodies are all delineated by a double bony cortex creating an impressing "bone-withinbone" or "vertebra-within-vertebra" configuration (black arrows). Duplication of the bony cortex is also visible at the level the vertebral spinous processes (white arrows).

events during childhood or early adolescence. Fortunately a revue of his medical records revealed a history of tibiofibular fracture (not

From: 1. Department of Diagnostic Radiology , Clinique St Luc, Bouge (Namur), Belgium. Address for correspondence: Dr B. Coulier, M.D., Department of Diagnostic Radiology, Clinique St Luc, Rue St Luc 8, 5004 Bouge (Namur). Belgium. E-mail: bcoulier@skynet.be

illustrated) at the age of eleven. Two months after trauma a diagnosis of – probably overestimated – reflex sympathetic dystrophy (RSD) was evocated on the basis of clinical signs, plain films and bone scintigraphy (not illustrated). The patient received intravenous perfusions of biphosphonate (pamidronate).


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DIFFUSE “VERTEBRA-WITHIN-VERBEBRA” APPEARANCE — COULIER

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Fig. 2. — A to C: Postero anterior plains films of the lumbar spine (B). Vertebral bodies express the typical “vertebra-within-vertebra" configuration (small black arrows). Duplication of the bony cortex is also found at the level of the iliac crests (black arrow), acetabular roofs (white arrow) and sacroiliac joints (small white arrows).

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Fig. 3. — Digital plain films of the ankle obtained two (A) and twenty months (B) after the administration of biphosphonate. A typical metaphyseal bandlike sclerosis (black arrow) or “zebra line” has developed near the cartilaginous grow plate after two months and this band typically migrated and significantly decreased after 20 months. White arrow = healed fracture.

Digital plain films of the ankle obtained two (Fig. 3A) and twenty months (Fig. 3B) after the administration of biphosphonate were retrieved from our picture archiving system. A typical metaphyseal bandlike sclerosis or “zebra line” had developed near the cartilaginous

grow plate after two months and this band had typically migrated and significantly decreased after 20 months. Discussion Bisphosphonates are structural analogues of inorganic pyrophos-

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phate. They are resistant to enzymatic and chemical breakdown and inhibit bone resorption by selective adsorption to mineral surfaces and subsequent incorporation within bone-resorbing osteoclasts where they interfere with various biochemical processes. After it was shown that they inhibited experimentally induced calcification and bone resorption, their potential application to clinical disorders was obvious, but it took about 30 years for them to become well established (1). Biphosphonates were first clinically used to inhibit calcification in myositis ossificans, to prevent subsequent heterotopic ossification and improve mobility in patients who had undergone total hip replacement surgery and finally as agents for bone imaging for which they still remain outstandingly useful for detecting bone metastases and other bone lesions (1). Their most impressive clinical application has been as inhibitors of bone resorption. They so became the treatment of choice for a variety of bone diseases in which excessive osteoclast activity is an important pathologic feature (1, 2). For example, they are the most important drugs used in the treatment of Paget disease (2). Bisphosphonates are also remarkably effective in malignancies where they significantly reduce the incidence of pathologic fracture, spinal cord compression and hypercalcemia in myeloma or in patients presenting with metastasis of breast, prostate and lung cancer, renal cell carcinoma and other solid tumors (1, 2). Bisphosphonates have also emerged in the past few years as the leading effective treatments for postmenopausal and other forms of osteoporosis (1, 2). They can increase bone mass and reduce fracture rates at the spine by 30% to 50% and at other sites in postmenopausal women. They also prevent bone loss associated with glucocorticosteroid administration. In pediatrics, pamidronate has proved remarkably effective in increasing bone in children with the inherited osteogenesis imperfecta (1). Good results have also been reported in patients presenting with juvenile osteoporis (2) and chronic recurrent multifocal osteomyelitis (3). Reflex sympathetic dystrophy (RSD) is characterized by spontaneous pain, swelling, dysaethesia, and allodynia (4). Other features relate to the autonomic nervous system and include cyanosis, mottling,


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sweating and reduction in temperature. In some cases more permanent and serious features may develop including muscle atrophy, demineralization of bone, and contractures of soft tissue around the affected joint. There is currently great controversy over the pathogenesis of RSD (5). Some authors believe that the disease is the result of a posttraumatic reflexive neuronal mechanism which leads to abnormal pain perception and exacerbated efferent sympathetic activity (5). Innumerable conditions are associated with the development of RSD but in more than 60% of cases described in adults there is a history of trauma. Among pediatrics RSD is considered as being a rare and under-diagnosed event. The history of trauma is less common and, when present, usually of lesser intensity. RSD usually involves children in late childhood or early adolescence, with a mean age of onset of 12-13 years and a higher frequency in girls than boys (5). Bisphosphonates were proposed in the treatment of RSD due to their action as potent osteoclast-blocking agents (6). Pamidronate appeared to be effective and well tolerated in the treatment of refractory RSD (6-8). Use of pamidronate as the firstintention treatment for RSD can also be proposed (2). However, there are two limiting factors: the cost of amidronate and the need for intravenous administration. Osteonecrosis of the jaw (ONJ) has been described as a complication of bisphosphonate therapy in adults but not among paediatric patients up to now (9). The occurrence of metaphyseal bandlike sclerosis (and in minder proportion of metaphyseal undertubulation) induced by biphosphonates is probably the result of the establishment of a new equilibrium between osteoblastic and osteoclastic activity within the bone after an initial phase of inhibition of osteoclastic activity without equal decrease in the osteoblastic activity (10). The relative resultant increase in bone formation in addition to the already high level of osteoblastic activity near the growth plates result in the development of sclerosis in the growing child. Multiple linear bands of increased bone density have also been described at the metaphysic of long bones in children receiving cyclical pamidronate infusions. The same

JBR–BTR, 2011, 94 (1)

phenomenon in areas of concentric bone growth (epi- and apophyses and vertebral bodies) causes ringlike sclerosis resulting in bone-withinbone or vertebra-within-vertebra appearance (11-14). A gradual decrease in the degree of sclerosis has been observed after discontinuation of medication before the closure of the growth plates and in patients receiving medication after closure. This observation is probably due to a return to a normal level of bone turnover with a gradual replacement of the sclerotic bone by bone with normal density. The disappearance of metaphyseal sclerosis in follow-up studies in individual subjects has suggested that sclerosis was a completely reversible phenomenon. A bone-within-bone appearance is a rare finding. In children, this appearance can occur after bone infarction in sickle cell anemia and Gaucher disease. In these disorders, however, this abnormality is most commonly seen in the diaphyses of the long tubular bones and is rarely generalized. It can also accompany heavy metal intoxication and can be a normal finding in the spine of neonates (10). Sclerosing bone dysplasias must also be mentioned. These diseases are classified into three groups: dysplasias of endochondral bone formation (essentially affecting the spongiosa), dysplasias of intramembranous bone formation (essentially affecting the diaphysis) and mixed sclerosing dysplasia. Among dysplasias of endochondral bone formation type II autosomal-dominant (adult type) osteopetrosis and osteopathia striata can commonly show the bone-within-bone appearance and/or dense striations (15). The persistence of bone-withinbone in the axial skeleton of the reported case appears rather remarkable and has, to our knowledge only exceptionally been reported (10, 14). The reason probably resides in the fact that biphosphonates are not currently used as first line treatment of reflex sympathetic dystrophy during early adolescence. Moreover the opportunity to fortuitously obtain plain films of the spine in a young adult who has received this atypical treatment during grow is extremely uncommon. Bibliography 1. Russell R.G.: Bisphosphonates: mode of action and pharmacology. Pediatrics, 2007,119: 150-162.

2. Devogelaer J.P.: Treatment of bone diseases with bisphosphonates, excluding osteoporosis. Curr Opin Rheumatol, 2000, 12: 331-335. 3. Gleeson H., Wiltshire E., Briody J., et al.: Childhood chronic recurrent multifocal osteomyelitis: pamidronate therapy decreases pain and improves vertebral shape. J Rheumatol, 2008, 35: 707-712. 4. Maillefert J.F., Chatard C., Owen S., et al.: Treatment of refractory reflex sympathetic dystrophy with pamidronate. Ann Rheum Dis, 1995, 54: 687. 5. Lotito A.P., Campos L.M., Dias M.H., Silva C.A.: Reflex sympathetic dystrophy. J Pediatr (Rio J), 2004, 80: 159162. 6. Kubalek I., Fain O., Paries J., Kettaneh A., Thomas M.: Treatment of reflex sympathetic dystrophy with pamidronate: 29 cases. Rheumatology (Oxford), 2001, 40: 1394-1397. 7. Simm P.J., Briody J., McQuade M., Munns C.F.: The successful use of pamidronate in an 11-year-old girl with complex regional pain syndrome: response to treatment demonstrated by serial peripheral quantitative computerised tomographic scans. Bone, 2010,46: 885888. 8. Adami S., Fossaluzza V., Gatti D., Fracassi E., Braga V.: Bisphosphonate therapy of reflex sympathetic dystrophy syndrome. Ann Rheum Dis, 1997, 56: 201-204. 9. Chahine C., Cheung M.S., Head T.W., et al.: Tooth extraction socket healing in pediatric patients treated with intravenous pamidronate. J Pediatr, 2008, 153: 719-720. 10. van Persijn van Meerten E.L., Kroon H.M., Papapoulos S.E.: Epiand metaphyseal changes in children caused by administration of bisphosphonates. Radiology, 1992 ,184: 249254. 11. Davies J.H., Gregory J.W.: Radiographic long bone appearance in a child administered cyclical pamidronate. Arch Dis Child, 2003, 88: 854. 12. Srinivasan R.: Pamidronate lines. Indian Pediatr, 2005, 42: 959-960. 13. Grissom L.E., Harcke H.T.: Radiographic features of bisphosphonate therapy in pediatric patients. Pediatr Radiol, 2003,33: 226-229. 14. Devogelaer J.P., Malghem J., Maldague B., Nagant de Deuxchaisnes C.: Radiological manifestations of bisphosphonate treatment with APD in a child suffering from osteogenesis imperfecta. Skeletal Radiol, 1987, 16: 360-363. 15. Vanhoenacker F.M., De Beuckeleer L.H., Van Hul W., et al.: Sclerosing bone dysplasias: Genetic and radioclinical features. Eur Radiol, 2000;10: 1423-1433.


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JBR–BTR, 2011, 94: 31-33.

INCIDENTAL RADIOLOGICAL FINDING OF A RENAL TUMOUR LEADING TO THE DIAGNOSIS OF BIRT-HOGG-DUBE SYNDROME M. Schreuer1,2, M. Lemmerling2, W. Pauwels3, D. Dewilde2, C. Heyse1,2, K.L. Verstraete1 Birt-Hogg-Dubé (BHD) syndrome is a rare autosomal dominant condition characterised by benign tumours of the hair follicle, renal cancer, pulmonary cysts and spontaneous pneumothorax. We report the diagnosis of a BHD syndrome achieved after incidental radiological finding of a renal tumour in a 24-year old man. The patient also displayed recurrent pneumothoraces and showed to have cysts in the basis of both lungs. The association of recurrent pneumothoraces and renal neoplastic disease should alert for the possible presence of this syndrome. Key-word: Oncocytoma.

Case report A 24-year-old man was admitted to the hospital for investigation of retrosternal pain suggestive of pericarditis. Initial investigations revealed a CRP level of 3.4 mg/dl and discrete ECG changes confirming the suspicion of pericarditis. A contrast enhanced computed tomography (CT) examination of the chest and the upper abdomen was performed to rule out conditions such as pulmonary embolism and aortic dissection, but the examination showed no abnormalities. However, it was incidentally noted that a solid contrast captating nodule of 2 cm was present posteriorly in the interpolar region of the left kidney (Fig. 1A). The kidney otherwise appeared normal and no other abdominal abnormalities were seen. The patient subsequently underwent a gadolinium enhanced magnetic resonance imaging (MRI) study of the kidneys on which the presence of the nodule was confirmed. The mass was hypointense on the T1- and T2-weighted images (Fig. 1B), and showed slight enhancement after intravenous injection of gadolinium (Fig. 1C). A partial nephrectomy was performed. The anatomopathological analysis of the nodule showed an oncocytoma. Postoperatively our young patient surprisingly showed a left-sided pneumothorax. The pneumothorax resolved within 5 days after chest drain insertion. The history of our patient revealed that one month prior to this admission, our patient was treated

for a primary left-sided pneumothorax with thoracoscopic pleurectomy and basal bullectomy after chest drainage had failed (Fig. 1D). The patient also had a known history of ulcerative colitis, for which he had been under treatment for several years. Furthermore, family history revealed that our patients father died at a young age due to renal cancer. Because of the wide variety of clinical problems present in a young patient and because of his family history of renal cancer, our patient was referred for genetic counselling. Molecular genetic research confirmed the diagnosis of Birt-HoggDubé (BHD) syndrome, a rare autosomal dominant disorder. Discussion Birt-Hogg-Dubé syndrome is a rare autosomal dominant condition characterised by benign tumours of the hair follicle, renal tumours of different histological types, pulmonary cysts, and spontaneous pneumothoraces. The condition is caused by germline mutations in the FLCN gene, which encodes folliculin. At present about 200 families with BHD syndrome with pathogenic FLCN mutations have been reported worldwide. BHD syndrome is probably under-diagnosed because of the wide variability in its clinical expression. Patients might present with renal cancer or pneumothorax, conditions that generally occur sporadically. The skin lesions usually appear after the age of 20 years, as multiple dome-shaped, whitish papules on

From: 1. Department of Radiology UZ Gent, 2. Department of Radiology AZ Sint-Lucas Hospital Gent, 3. Department of Gastroenterology AZ Sint-Lucas Hospital Gent, Belgium. Address for correspondence: Dr M. Lemmerling, Department of Radiology, AZ SintLucas Hospital Gent, Groenebriel 1, B-9000 Gent, Belgium. E-mail: marc.lemmerling@azstlucas.be

nose and cheeks. About 25% of the FLCN-mutation carriers do not manifest skin lesions (1). The most threatening complication of BHD syndrome is renal cancer. In a series of 124 individuals with BHD, 27% of the patients had renal tumours at a mean age of 50.4 years (range 31-74 years) (2). The earliest reported age at diagnosis of renal cancer in a patient with BHD syndrome is 20 years (3). A unique characteristic of this condition is the mixture of histological types of renal tumours seen in a single kidney or patient, with chromophobe renal cell carcinoma (RCC), clear cell RCC and oncocytoma respectively accounting for up to 34%, 9% and 8% of the cases (4). In addition to this, the presence of different cell populations within an individual tumour, the so-called hybrid oncocytic tumour (50%), is frequently observed in BHD patients. Furthermore, kidney tumours in BHD syndrome usually occur earlier than sporadic tumours and are generally multiple and bilateral (5). This is not the case in our patient. Therefore a familial history of renal cancer, the diagnosis of early-onset renal cancer (< 50 years), or the presence of multiple bilateral kidney tumours should raise suspicion of BHD syndrome, particularly if the predominant histological type is chromophobe renal cell carcinoma or hybrid oncocytic tumour. CT and MRI usually show multiple, bilateral, heterogeneous, solid renal masses that demonstrate heterogeneous enhancement after contrast administration. In our patient the family history of a malignant renal tumour and the discovery of a benign oncocytoma at the age of 24, were clues to the diagnosis of BHD syndrome. On CT examination of the thorax, more than 80% of adult patients with BHD have multiple lung cysts. It is known that pulmonary cysts may rupture under the pressure of


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A

JBR–BTR, 2011, 94 (1)

D

B

E

C

ventilation and subsequently cause pneumothorax. On CT-scan the lung cysts are seen as well-circumscribed, round, air-filled structures. In contrast with sporadic primary pneumothorax, where pulmonary cysts are usually located in the apical

Fig. 1. — Axial contrast-enhanced CT scan of the abdomen (A) shows a well-defined, exophytic, solid mass posteriorly in the interpolar region of the left kidney, less attenuating than the surrounding renal parenchyma (arrow). On the axial T2-weighted MR image (B) a well-defined hypo-intense lesion is seen in the left kidney (arrow). The axial contrast-enhanced T1-weighted MR image (C) shows slight homogeneous enhancement of the mass in the left kidney (arrow). The AP conventional chest x-ray (D) demonstrates a left-sided pneumothorax (arrows). The axial CT image of the chest displayed in lung window setting (D) shows well-circumscribed, round, air-filled structures in the left lung basis: lung cysts (arrow).

zones, the lung cysts in BHD are typically found in the basal lung regions (1). In our patient such cysts were seen in the basis bilaterally, with the largest one showing a diameter of 1.5 cm (Fig. 1E). Another clue to the diagnosis in our case was

therefore given by the finding of recurrent spontaneous pneumothoraces on radiographic imaging. The typical skin lesions that are associated with BHD syndrome were in this case never used as a clue to the diagnosis of this rare clinico-


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BIRTH-HOGG-DUBE SYNDROME — SCHREUER et al

pathologic condition. Concerning the initial complaints, the retrosternal pain was most likely the result of a viral pericarditis of which our patient steadily recovered. This pericarditis episode was probably unrelated to the syndrome. Finally, since our patient is known with ulcerative colitis, we also searched for a possible link between ulcerative colitis and BHD syndrome, but as far as we know no other cases of BHD syndrome have been reported in combination with inflammatory bowel disease. Throughout the years, a number of pathological conditions have been linked to BHD syndrome. Intestinal polyps have been described more than once in combination with BHD syndrome, but no association was demonstrated so far. The general consensus now is that patients with BHD syndrome are not at risk for colon polyps or colonic adenocarcinoma (6, 7). Due to the risk of renal cancers for individuals who have a family history of BHD syndrome, different surveillance programs have been suggested aimed at early recognition and treatment of cancer. A yearly MRI scan of the kidneys starting at the age of 20 years is probably best, but the exact role of

CT and ultrasonography has not been fully investigated (1). In conclusion, we describe the case of a patient with the rare BirtHogg-Dubé syndrome, a probably underdiagnosed syndrome that is passed on to following generations in an autosomal dominant fashion. Recurrent pneumothoraces with cysts in the basis of the lungs rather than in the apical regions in association with renal neoplastic disease should alert for the possible presence of this syndrome. References 1.

Menko F.H., van Steensel M.A., Giraud S., Friis-Hansen L., Richard S., Ungari S., Nordenskjöld M., Hansen T.V., Solly J., Maher ER.: BirtHogg-Dubé syndrome: diagnosis and management. Lancet Oncol, 2009, 10: 1199-1206. 2. Pavlovich C.P., Grubb R.L., Hurley K., Glenn G.M., Toro J., Schmidt L.S., Torres-Cabala C., Merino M.J., Zbar B., Choyke P., Walther M.M., Linehan W.M.: Evaluation and management of renal tumors in the BirtHogg-Dubé syndrome. J Urol, 2005, 173: 1482-1486. 3. Khoo S.K., Giraud S., Kahnoski K., Chen J., Motorna O., Nickolov R., Binet O., Lambert D., Friedel J., Lévy R., Ferlicot S., Wolkenstein P.,

33

Hammel P., Bergerheim U., Hedblad M.A., Bradley M., Teh B.T., Nordenskjöld M., Richard S.: Clinical and genetic studies of Birt-HoggDubé syndrome. J Med Genet, 2002, 39: 906-912. 4. Pavlovich C.P., Walther M.M., Eyler R.A., Hewitt S.M., Zbar B., Linehan W.M., Merino M.J.: Renal tumors in the Birt-Hogg-dubé syndrome. Am J Surg Pathol, 2002, 26: 1542-1552. 5. Weirich G., Junker K., Salles P.G.O., Lim S.D., de Peralta-Venturina M.N., Alvarado-Cabrero I., Jimenez R.E., Cabras A.D., Höfler H., Schubert J., Amin M.B.: Comparative genomic hybridization analysis of renal oncocytomas (RO), chromophobe renal cell carcinomas (CHRCC) and tumors with hybrid histology – Hybrid Oncocytic Tumors (HOT). Modern Pathology, 2002, 15: 186. 6. Adley B.P., Smith N.D., Nayar R., Yang X.J.: Birt-Hogg-Dubé syndrome: clinicopathologic findings and genetic alterations. Arch Pathol Lab Med, 2006, 130: 1865-1870. 7. Zbar B., Alvord W.G., Glenn G., Turner M., Pavlovich C.P., Schmidt L., Walther M., Choyke P., Weirich G., Hewitt S.M., Duray P., Gabril F., Greenberg C., Merino M.J., Toro J., Linehan W.M.: Risk of renal and colonic neoplasm and spontaneous pneumothorax in the Birt-Hogg-Dubé syndrome. Cancer Epidemiol Biomarkers Prev, 2002, 11: 393-400.


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JBR–BTR, 2011, 94: 34-36.

LETTER TO THE EDITOR R. D’Hauwe1, E. Lerut2, L. De Wever1, R. Oyen1, F. Claus1

Dear Editor, We would like to comment on a case of Birt-Hogg-Dubé (BHD) syndrome reported in this issue by M. Schreuer et al, JBR-BTR, 2011, 94: 31-33. Individuals with this rare autosomal dominant inherited disorder – named after three dermatologists who described the syndrome in 1977 – are susceptible to develop 1) noncancerous tumors of the hair follicles, 2) renal tumors (predominantly chromophobe renal cell carcinoma) and 3) thin-walled cystic lung lesions. The diagnosis of BHD is based on these clinical findings and confirmed by molecular genetic testing. We would like to take the opportunity to emphasize the unique position of the radiologist to suggest this syndrome in patients imaged and diagnosed with both multiple solid renal tumors and cystic pulmonary lesions. Hereditary renal cancer is a true challenge for radiologists and surgeons (1-3). A priori knowledge of the genetic predisposition allows screening the patient and his relatives to detect cancer at an early stage in order to treat synchronous and metachronous renal cancerous lesions with nephron-sparing surgical techniques, such as partial nephrectomy and radiofrequency ablation. The main hereditary syndromes known to have an increased risk to develop renal cell carcinoma (RCC) are listed in Table I. Patients with Von Hippel Lindau (VHL) disease and tuberous sclerosis (TS) have an increased risk of developing clear cell RCC’s, lesions characterized by strong, peripheral enhancement of the viable tumor parts in the arterial imaging phase (on CT and MRI) and often central foci of degeneration and necrosis. In the majority of cases, the diagnosis of VHL or TS is based on symptomatic extrarenal manifestations. Two subtypes of hereditary papillary RCC syndromes are known, type I presenting with multiple bilateral tumors, and type II

Fig. 1. — BHD syndrome in 3 patients diagnosed with multifocal chromophobe RCC’s and cystic lung lesions. Top row: 39-year-old female showing bilateral, homogeneously, arterial middle-level enhancing (left image) and venous hypo-enhancing (right image) chromophobe renal cell cancers. Middle row: 77-year-old male, coronal CT showing bilateral hyper-enhancing RCC’s (left image) and multiple lung cysts (right image). Bottom Row: 43-year-old male, coronal contrast enhanced MRI showing two left-sided RCC’s (left image) and bilateral pulmonary cysts complicated by left-sided pneumothorax (right image).

solitary tumors with a more aggressive behavior and an association of uterine leiomyomas and sarcomas.

From: Department of 1. Radiology, 2. Pathology, University Hospitals Leuven, Leuven, Belgium. Address for correspondence: Dr F. Claus, M.D., Dienst Radiologie UZ-Leuven, Herestraat 49, B-3000 Leuven, Belgium. E-mail: filip.claus@uzleuven.be

The RCC’s observed in both papillary subtypes are typically hypovascular malignancies on contrast enhanced cross-sectional imaging. Both the clear and papillary cell type RRC’s originate from the cells of the proximal renal tubule. On the contrary, chromophobe renal carcinomas, as observed in BHD syndrome, originate from the intercalated cells of


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LETTER TO THE EDITOR — D’HAUWE et al

35

Table I. — Hereditary RCC syndromes. predominant RCC

cell of origin

other renal lesions

typical imaging clues

Von Hippel Lindau

clear cell

proximal renal tubule

cysts

hyper-enhancing renal mass(es) + renal cysts + extrarenal manifestations (haemangioblastoma, pancreatic cysts and neuroendocrine tumors)

Tuberous sclerosis

clear cell

proximal renal tubule

angiomyolipoma, cysts, papillary - chromphobe RCC, oncocytoma

hyper-enhancing renal mass(es) + renal cysts, angiomyolipoma + extrarenal manifestations (hamartoma, lymphangioleiomyomatosis)

Hereditary papillary RCC’s

papillary

proximal renal tubule

none

hypo-enhancing renal mass(es)

Birt-Hogg-Dubé

chromophobe ➝

intercalated cell of oncocytoma, clear renal collecting duct cell and papillary RCC

middle-level enhancing renal mass(es) + thin-walled lung cysts (pneumothorax)

Fig. 2. — 65-year-old male presenting with renal dysfunction and bilateral renal tumors; top row: arterial and venous CT, bottom row: arterial and venous MRI. No extrarenal abnormal findings were present. Biopsy confirmed the diagnosis of renal oncocytosis.


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36

JBR–BTR, 2011, 94 (1)

Table II. — Incidence of pulmonary lesions in patients with Birt-Hogg-DubÊ syndrome diagnosed with RCC. number of patients

pulmonary lesions (cysts, pneumothorax)

age at diagnosis RCC (youngest in case > 1)

Kluijt I. et al. Clinical Genetics, 2009

3

3/3

27

Westermann D.H. et al. Urologe, 2010

1

1/1

47

Bielefeld G. et al. Rev Med Interne, 2010

1

1/1

20

Warwick G. et al. J Med Case Reports, 2010

1

1/1

50

Imada K. et al. Br J Dermatol, 2009

1

0/1

68

Janitzky A. et al. Urol J, 2008

1

1/1

67

Souza C. et al. AJR, 2005

1

1/1

27

Stavrakoglou A. et al. QJM, 2010

1

1/1

56

Welsch M.J. et al. Int J Dermatol, 2005

1

1/1

52

Patients identified at our hospital

6

6/6

39

the renal collecting duct and often show a middle-level enhancement in the arterial phase and have lower attenuation than renal parenchyma in the venous phase, with a more homogeneous pattern in the latter phase compared to the clear cell type. A history of multiple chromophobe renal carcinomas is characteristic for BHD and should prompt radiologists to review available lung imaging. The incidence of thinwalled pulmonary cysts (resembling bullous emphysema) is very high in BHD patients presenting with renal cancer and a highly specific imaging key feature to suggest the diagnosis. Figure 1 shows the renal and pulmonary imaging findings in 3 patients with proven BHD. Table II lists a literature review on the coexistence of pulmonary lesions in BHD patients diagnosed with renal cancer and for whom pulmonary imaging findings were reported, together with the imaging findings of 6 patients with BHD identified at our hospital. Pulmonary lesions were present in 16 of the 17 cases (94%). It is important to add that there is another disease entity which

predisposes to develop tumors originating from the intercalated cells of the renal collecting duct, referred to as renal oncocy(toma)tosis (4-6). In this extremely rare hereditary syndrome, of which the causative gene is less known, patients typically present with renal dysfunction and multiple renal oncocytic tumors / oncocytomas on imaging. Since chromophobic and oncocytic tumors are believed to originate from the same precursor cell, differentiating BDH from renal oncocytosis syndrome can be difficult. The absence of extrarenal manifestations ( VHL and TS) and cystic lung lesions ( BHD) is an important imaging discriminator to suggest renal oncocytosis. Figure 2 shows cross-sectional images for a biopsy proven case of renal oncocytosis. In summary, the combination of meta- or synchronous solid renal tumors and cystic pulmonary lesions should prompt the radiologist to suggest the diagnosis of an underlying BHD syndrome. Recognizing this disease entity is important for patient follow-up and family screening.

References 1. Choyke P.L., Glenn G.M., Walther M.M., Zbar B., Linehan W.M.: Hereditary renal cancers. Radiology, 2003, 226: 33-46. 2. Pavlovich C.P., Schmidt L.S. Searching for the hereditary causes of renal-cell carcinoma. N at Rev Cancer, 2004, 4: 381-393. 3. Bottaro D.P., Linehan W.M. Multifocal renal cancer: genetic basis and its medical relevance. Clin Cancer Res, 2005, 15, 11: 7206-7208. 4. Tickoo S.K., Reuter V.E., Amin M.B., Srigley J.R., Epstein J.I., Min K.W., Rubin M.A., Ro J.Y. Renal oncocytosis: a morphologic study of fourteen cases. A m J Surg Pathol, 1999, 23: 1094-1101. 5. Al-Saleem T., Cairns P., Dulaimi E.A., Feder M., Testa J.R., Uzzo R.G.: The genetics of renal oncocytosis: a possible model for neoplastic progression. Cancer G enet Cytogenet, 2004, 1, 152: 23-28. 6. Gobbo S., Eble J.N., Delahunt B., Grignon D.J., Samaratunga H., Martignoni G., Zhang S., Wang M., Brunelli M., Cossu-Rocca P., Cheng L.: Renal cell neoplasms of oncocytosis have distinct morphologic, immunohistochemical, and cytogenetic profiles. A m J Surg Pathol, 2010, 34: 620626.


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JBR–BTR, 2011, 94: 37.

IMAGES IN CLINICAL RADIOLOGY Pedunculated pleural lipoma L. Cardinale, F. Avogliero, F. Solitro, C. Fava1

A

A 70-year-old woman was hospitalized for dyspnea and chest pain. After radiological examinations and bronchoscopy, she was diagnosed with a stage IVsmall-cell carcinoma of the lung. Collaterally, chest CT showed an hypodense pleural sessile mass, with smooth, well defined borders and homogeneous fat attenuation of approximately –100 HU; it showed no enhancement after intravenous contrast material administration (Fig. A). The lesion was suggestive for pleural lipoma. At the next CT examination pleural effusion, due to a primary disease progression, was detected. The mass, floating into pleural effusion, had now a polypoid appearance and was inserted into the parietal pleura with a small pedicle (Fig. B,C). Comment

B

C

Most patients with pleural lipoma are asymptomatic until incidental detection at radiological examinations; the rare cases with symptoms in bulky masses may include nonproductive cough, back pain, dyspnea. Definitive diagnosis is made at CT when the lesion in question shows an homogeneous fat attenuation of approximately −100 HU, smooth borders abutting on the lungs, tapering or obtuse margins with the pleural surface and contact with the pleura with at least one quarter of the mass’s circumference. A pedicle is rarely visible. Biopsy is recommended for those lesions with an non-homogeneous density (for the risk of atypical lipomatous tumors and liposarcomas). Another pedunculated neoplasm which enters into the differential diagnosis with lipoma is the localized fibrous tumor of the pleura. However, the fibrous tumor has not the peculiar fat attenuation.

1. University of Turin, Department of Clinical & Biological Sciences, Radiology Unit, San Luigi Hospital, Orbassano (Torino), Italy.


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JBR–BTR, 2011, 94: 38.

IMAGES IN CLINICAL RADIOLOGY Denim sandblasters’ pneumoconiosis M. Apaydin1, M. Varer1, S. Ayik

A

A 28-year-old man with long standing dyspnea for 4 years and a history of dry cough, sweating and loss of weight was admitted to the hospital. Physical examination showed fine crackles at the end of inspiration. The laboratory tests revealed increased low density lipoprotein level with slight increase in erytrocyte sedimentation rate. Sputum smears for blood culture and tuberculosis were negative. He was referred to the radiology department for imaging studies. Chest radiography revealed bilateral reticulonodular infiltrates in upper and middle zones. High resolution computed tomography showed bilateral diffuse intralobular micronodules in upper and mid lung zones with interlobular septal lines also bilateral pleural thickening was seen (A). Right middle lung zone showed hyperaeration (B). Also he had bilateral hilar, right paratracheal, prevascular and subcarinal lymphadenopathies (C). He had been working in producing sandblasted denims for 10 years. The diagnosis was based on clinical history, occupational exposure to silica dust, and chest x-ray findings after other possible diagnoses were ruled out. Comment

B

C

Denim sandblasting changes the color of the denim and gives worn appearance. Quartz is used for denim sandblasting and cause the most common form of pneumoconiosis called silicosis by inhaled dust containing crystalline silica. It easily develops in small and unventilated unhealthy working place conditions with extended exposure to tiny sand particles. Silicosis is untreatable and causes death by impairing general health status. It can be seen in underdeveloped as well as developed countries. Mining, drilling, construction, pottery making are the most known etiologic factors. Also, it can be accombined with autoimmune disease. Tuberculosis is a frequent complication of this disease and there is a strong link between lung cancer and silicosis. Three main clinical presentatition was described; acute/silicoproteinosis, classic, accelerated types. Radiologic findings depends the stage and type of the disease.The most common presentation is classic chronic silicosis like our case. These patients are usually become clinically and radiologically symptommatic after 10-20 years of chronic silica exposure. Smooth nodular infiltrations, interlobular and intralobular septal thickening, axial interstitium thickening, ground glass consolidation, honeycombing, pleural thickening, low-attenuated areas, mediastinal lymphadenopathies are the most detected abnormalities on CT. Denim sandblasters’ silicosis is a rare cause of pneumoconiosis. There is no cure for this preventable enviromental disease. So it is very important to be aware of clinical and radiological findings of denim sandblasters’ silicosis.

1. Department of Radiology, 2. Sleep and Chest Disorders Unit, Izmir Ataturk Education and Reseach Hospital, Izmir, Turkey.


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JBR–BTR, 2011, 94: 39.

IMAGES IN CLINICAL RADIOLOGY Dilated cisterna chyli F.M. Vanhoenacker1,2, M. Eyselbergs1,2, C. Petre1

A

A 46-year-old man was referred to our department because of chronic low back pain. A magnetic resonance (MR) examination of the spine revealed bilateral spondylolysis at the level of the lumbar vertebra L4, with associated anterolisthesis as well as degenerative disk disease at L4-L5. Additionally an oval-shaped hypointense structure was seen in the prevertebral space extending from the L1-L2 to L2-L3 level (arrows) on sagittal T1-weighted MR imaging (Fig. A). Axial T2-weighted MR imaging with a HASTE sequence showed a well delineated structure (arrow) adjacent and to the right of the abdominal aorta (Fig. B). The location, shape and fluid content were consistent with a focal dilatation of the cisterna chyli. Conservative therapy for low back pain was initiated. Because of the benign nature of the prevertebral structure, no further therapy was required. Comment

B

The cisterna chyli (CC) consists of a focal dilatation of the lymphatic channels that lies in the prevertebral space in a retrocrural position, usually at the L1-L2 vertebral level. It may – however – extend in a craniocaudal direction as far superior as T10 and as far inferior as L4. The CC receives lymphatic fluid from two lumbar lymphatic trunks draining the lower extremities and one intestinal trunk. The latter acts as a conduit for the lipid products of digestion. In the cephalad direction, it continues as the thoracic duct. There is large variation in the morphological appearance of the lymphatic channels at the thoracolumbar level. Therefore, the more descriptive term “abdominal confluence of the lymphatic trunks” or “receptaculum chyli” may be etymologically more correct than the term “cisterna chyli”. The CC is identified in 20% at autopsy, whereas lymphangiographic studies demonstrate the structure in 52% of patients. The presence of a visible CC (having a diameter of 5 mm or more) at cross-sectional imaging is less frequent. Previous studies showed a CC in up to 16,1 % of abdominal computed tomography (CT) examinations and in 15 % of abdominal MR examinations. The majority of these lesions show CT attenuation values of less than 15 Hounsfield units (HU). Twenty percent of the CC show CT densities of 15 HU and higher, simulating retrocrural adenopathy. The depiction of the fluid content on fluid-sensitive MR pulse sequences allows correct characterization of the structure. The morphology of the lesion may vary from a single straight or sausage-shaped tube to a focal round collection. Some lesions –however- do present as multiple parallel or converging tubes, tortuous tubes, or focal plexuses. There is no substantial enhancement on dynamic imaging obtained within 5 minutes after intravenous injection of contrast medium. Delayed images may show enhancement of the cistern lumen. PET-CT shows a low metabolic activity. In conclusion, the radiologist should be aware of the imaging appearance of a CC. The typical prevertebral retrocrural location at the thoracolumbar junction and the fluid content are the clues to the correct identification, avoiding misinterpretation of this normal structure as an enlarged retrocrural lymph node or other retroperitoneal soft tissue mass. 1. Department of Radiology, AZ Sint-Maarten, Duffel-Mechelen, Duffel, 2. Department of Radiology, Antwerp University Hospital, University of Antwerp, Edegem, Belgium


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JBR–BTR, 2011, 94: 40.

IMAGES IN CLINICAL RADIOLOGY Iatrogenic facial subcutaneous emphysema after endodontic treatment J. Coulier1, F.C. Deprez2

A

A 74-year-old woman was referred to the ophthalmologic department by her dentist, for an acute left facial swelling after extraction of the left higher canine (tooth n° 23). Patient presented left superior and inferior palpebral oedema extending to left periorbital subcutaneous tissues (Fig. A – CT surface rendering). The skin appeared normal without any erythema and palpation revealed typical subcutaneous gaseous crepitations. Dysphagia and dyspnea were absent. Complete extensive ophthalmologic examination was also normal. Unenhanced facial CT was performed and revealed diffuse left facial subcutaneous emphysema involving both left eyelids, the left malar Bichat fat and left peribuccal subcutaneous fat but also the left infratemporal, pterygomandibular and parapharyngeal spaces (Fig. B – coronal views and Fig. C – axial views). No facial sinuses anomaly or bone interruption was observed on CT series, even in the left superior alveolar bones. These findings associated with recent dental extraction history allowed to diagnose iatrogenic left facial subcutaneous emphysema, caused by a highspeed dental hand-piece use. Treatment consisted on short oral corticotherapy and antibiotherapy (amoxycillin/clavulanic acid). Comment

B

C

Iatrogenic subcutaneous emphysema in the head and neck region may arise after various interventions, including anesthesia, maxillofacial procedures, head–neck surgery and, less commonly, dental surgery involving extraction, implants or other procedures. During dental treatment, the use of compressed air to dry the root canal (with air-cooled high-speed dental hand-pieces) occasionally let the air penetrate soft tissues, dissecting periodontal ligament and gingival tissue, leading to subcutaneous emphysema. Pneumothorax, pneumomediastinum or even pneumoperitoneum can be observed because of the relationship between the cervical prevertebral space and the mediastinum. Life-threatening or even fatal complications can exceptionally occur due to venous (pulmonary) air embolism or paradoxal arterial systemic air embolism. After complicated dental surgery, air spreading occurs rapidly, within seconds to minutes. Patient senses immediate discomfort or pain in the affected area. Subcutaneous emphysema usually resorbs spontaneously within few days. Main complication is subcutaneous tissues infection due to oral flora. Main differential diagnosis of facial swelling after medical procedures is hematoma, allergic reaction or angioedema, but subcutaneous crepitations palpation leads to correct diagnose. CTscan is recommended to confirm and determine the extent of subcutaneous emphysema.

1. Department of Ophtalmology, Cliniques Universitaires St-Luc, Brussels, 2. Department of Radiology, Clinique St-Pierre, Ottignies-LLN, Belgium.


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JBR–BTR, 2011, 94: 41.

IMAGES IN CLINICAL RADIOLOGY The ‘torus palatinus’: a common but relatively unknown entity C. Boulet, M. De Maeseneer, T. Buisseret, M. Shahabpour, J. de Mey1

A

A 60-year-old woman was referred for imaging evaluation of a lump at the midline of the hard palate. The lump was painless but felt hard and lobulated on palpation.Ulcerations were not apparent. It had appeared gradually over time. An open mouth spiral head CT study before and after intravenous contrast injection was performed, with bone reconstructions of the facial skeleton. It revealed a flat based bony thickening at the cleft of the hard palate with a width of 11 mm and a length of 15 mm (Fig. A,B,C). The bony protrusion was covered by a thin layer of mucosa (Fig. D). Contrast enhancement was absent. No other lesions were evident in the oral cavity. The nasal cavity and nasal septum were normal. Comment

B

C

D

A torus (Latin for “lump”) is an exostosis consisting of a dense cortex and a limited amount of bone marrow, usually covered by a thin layer of mucosa. The ’torus palatinus’ presents clinically in three shapes: spindle-shaped, nodular or lobular. They are located at the juncture of the palatine apophysis of the maxillae. A mandibular counterpart can be found (torus mandibularis) on the internal side of the horizontal branch of the mandibula, above the mylohyoid line, in the premolar region. Some authors define it as a congenital anomaly, due to “overactivity” of the osteoblasts with bone being deposited along the line of fusion of the palate. A hereditary autosomal dominant form has been described in the literature, but in up to 70% of the cases, the torus was attributed to environmental factors, mainly related to occlusal stress. The growth of the tori is gradual, being greater in the second or third decade of life. The prevalence is higher in women, which could indicate a linkage to the X-chromosome. In addition the tori seem more common in some ethnic groups (eg. Eskimos, Japanese and African Americans). The diagnosis is usually made during clinical examination by a dentist. It is mostly asymptomatic, but could lead to phonatory disturbances, limitation of mastication, ulceration of the mucosa (mimicking cancer), food deposition and instability of dental prosthetics. Radiographs (perapical, occlusal or panoramic) are not very useful. Clinical examination is sufficient in most of the cases, but in case of doubt CT of the facial skeleton is the best option. MRI does not contribute to the diagnosis. Differential diagnosis with sarcoma or squamous cell carcinoma is not difficult because there is no bone remoddeling, osteolysis, or mucosal ulceration. Removal of the torus is usually not necessary. Surgical treatment is ony indicated with disturbances of phonation, limitation of mastication, sensitivity or ulceration of the overlying mucosa, and instabilty or conflict with dental prosthetics. Removal of the accessory cortical bone of the tori can also be performed in case of need of autogenous bone grafts in periodontal implant surgery.

1. Department of Radiology, UZ Brussel, Jette, Belgium.


images-alsinnawi-_Opmaak 1 16/02/11 10:00 Pagina 1

JBR–BTR, 2011, 94: 42.

IMAGES IN CLINICAL RADIOLOGY An unusual pelvic mass: bladder lymphoma M. Alsinnawi1, M. Quinlan1, A. Brady2, N. Khan1

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B

A 58-year-old woman presented to the emergency department after a minor fall, complaining of lower abdominal pain. A large, tender mass arising from the pelvis was palpable, in association with abdominal wall bruising. The patient believed that she may have had a “lump” there for several years. She had had a total abdominal hysterectomy and bilateral salpingo-oopherectomy for benign fibroids three years previously. She denied any gastrointestinal (GI) or genitourinary symptoms. She had no night sweats or weight loss. Routine blood investigations were normal. A CT scan of her abdomen and pelvis with oral and IV contrast contrast was performed to investigate what was thought to be a rectus sheath haematoma. This has revealed abnormal retroperitoneal lymphadenopathy in the mid and lower Abdomen (Fig. A). Most of her bladder was replaced by a 16 x 12 x 10 cm soft-tissue density mass (Fig. B). There was mild dilatation of both ureters with mild left hydronephrosis. Appearances suggested a malignant process, given the retroperitoneal adenopathy. Ultrasound-guided biopsy of a right-sided 13 x 8 mm solid inguinal lymph node was performed; histology of this was inconclusive. Following this, a rigid cystoscopy confirmed a grossly abnormal bladder with a diffuse, thick mass and a non-friable, pale-looking mucosa which was intact. Multiple resection biopsies were taken. MRI of pelvis the next day showed almost complete absence of a bladder lumen. The bladder wall was diffusely thickened, measuring 10 cms in places (Fig. C). Pathologically-enlarged iliac and retroperitoneal nodes were seen. Abnormal stranding of perivesical fat suggested transmural malignant infiltration. The histology of her bladder biopsies showed dense, diffuse infiltration by small lymphocytes, covered in part by intact urothelium. Microscopy and immunohistochemistry supported the diagnosis of low grade, diffuse, B-cell Non Hodgkin’s Lymphoma (NHL). A diagnosis of primary malignant lymphoma arising in the urinary bladder was made. She was referred on to the Haematology service for further investigation and treatment. Given that her International Prognostic Index is 2-3/5, six cycles of RCHOP (Rituximab, Cyclophosphamide, Doxorubicin, Vincristine and Prednisolone) chemotherapy were planned. After completing her treatment, she will be re-staged with appropriate scans. Comment

About 40% of NHL present in extra-nodal sites, with the skin and the GI tract being the most frequent sites. Primary bladder lymphoma is a rare disease, responsible for only 0.2% of extra-nodal lymphomas. The disease C is typically seen in middle-aged and elderly females. The frequency of NHL in the urinary bladder is higher than Hodgkin’s lymphoma. Presentation can be variable with symptoms including haematuria, pain, dysuria, nocturia, frequency or recurrent UTIs. Bladder lymphomas tend to occur at the base and the trigone of the bladder and usually form a sessile mass with normal urothelium. Cystoscopy commonly shows a solid tumour without surface changes. The surrounding mucosa is typically intact but in a very large tumour the mucosa can be ulcerated. Upper tract dilatation can occur secondary to mass effect and ureteric obstruction. Histopathological studies are essential for the diagnosis. It is often difficult to differentiate lymphoma from sarcoma or carcinoma on frozen section biopsy. Histologically, primary bladder lymphoma is most commonly low-grade NHL of the B-cell type. About 20% of B-cell bladder lymphoma is highgrade. Reported management of primary bladder low-grade NHL is varied. Partial or total cystectomy, radiotherapy, chemotherapy, or combined therapies have all been described. Surgery may be inappropriate in these patients, given the high success rate with either radiotherapy or chemotherapy. Repeat cystoscopy is recommended for follow-up of all patients. In summary, this case highlights the difficulties in diagnostic differentiation relating to large pelvic masses in middle age females. In the differential diagnosis of bladder tumours, malignant lymphoma of the bladder should be considered. Despite the fact that primary bladder lymphoma is a rare condition, we should be aware of the excellent complete remission rates and good prognosis. Department of 1. Urology and 2. Radiology, Mercy University Hospital, Cork, Ireland.


EDOR (Celebrate)_Opmaak 1 16/02/11 10:02 Pagina 1

JBR–BTR, 2011, 94: 43.

CELEBRATE THE POWER OF IMAGING: THE EUROPEAN DAY OF RADIOLOGY1

Imaging is an indispensable tool in modern medicine, yet very few patients know just how important it is. From cancer detection and therapy to diagnosing stroke or serious trauma in time, radiologists contribute to saving lives by covering every field of medicine. To raise public awareness, the European Society of Radiology has launched the 1st European Day of Radiology on February 10, in memory of x-ray discoverer Wilhelm Conrad Röntgen. Vienna, December 2010 – Radiologists, radiographers, nurses or anyone else who has witnessed the difference that can be made by medical imaging cannot fail to appreciate the value of its role in modern healthcare, but the world of radiology is often something of an unsung hero among the general public. Although many procedures are well known by name, and the impact that a single routine examination can have on a patient’s life can be life-altering, the perception of radiology held by most of us is often far less clear than the high-resolution images interpreted by its practitioners. Most people are familiar with the concept of the diagnostic x-ray as the traditional role of radiology, but numerous advances in research and technology have led radiology to play an increasingly prominent part in healthcare, not just in terms of spotting problems, but also as a major contributor to treatment and recovery. Working in tandem with other disciplines, radiology has had a major impact on achievements in such significant areas as early cancer detection, speedy trauma analysis, and precise stroke localisation, among many others. In order to raise awareness among the general public and draw attention to the broad contribution imaging makes to general healthcare, the European Society of Radiology (ESR) decided to launch the 1st European Day of Radiology on February 10th, the anniversary of the

death of Wilhelm Conrad Röntgen, who first discovered x-rays in 1895. The European Day of Radiology will, for the first time, see coordinated press activities undertaken by national radiological societies across Europe to highlight the role and significance of medical imaging. Societies from seventeen countries have accepted the ESR’s invitation to take part in this endeavour, all of whom will launch press releases on key topics, focusing on the recent challenges and achievements in their respective countries. The topics reflect some of the most important issues in radiology, which are just as relevant to potential patients as they are to those working in the field. Cancer detection and therapy, emergency radiology, patient safety and radiation dose reduction, and stroke imaging are all areas of major importance in radiology, and each participating country will take at least one of these subjects as the focus of its press activities, with some societies also deciding to support them with various events held on and around February 10. The national radiological societies of Austria, Belgium, the Czech Republic, Croatia, France, Georgia, Hungary, Ireland, Italy, Lithuania, Poland, Romania, Spain, Sweden, Switzerland, Turkey and the UK all agreed to take part in the initiative, and a total of 23 representatives of these societies have been interviewed by ESR staff to create press releases that will be distributed to the media in their home countries. In many cases the topics will be the same and the general essence will be similar, but the close involvement of the radiological societies means each press release bears a particular national relevance. It is hoped that this will increase the specificity of the campaign’s wider message and help to enhance radiology’s image as an essential and progressive element of modern medical care. Healthcare systems vary from country to country, but the big issues

in radiology are universal. Although several radiological societies will be participating in the European Day of Radiology, the initiative was conceived and coordinated centrally by the ESR, a non-profit organisation based in Vienna, Austria. Formed in 2005 through the merging of two other organisations – the European Association of Radiology and the European Congress of Radiology –, the ESR has spent the last five years promoting and coordinating all manner of radiological activities, bringing together more than 50,000 members worldwide in the process. The date of February 10 was chosen for the European Day of Radiology to draw a link between the beginnings of radiology and its current status, so as to highlight the progress made, as much as to honour the life of the man who made the very first steps in founding the discipline. Wilhelm Conrad Röntgen received the Nobel Prize in Physics in 1901 “in recognition of the extraordinary services he has rendered by the discovery of the remarkable rays subsequently named after him”. Significantly, he refused to take out patents, intending the practical applications of x-rays to benefit mankind to the greatest possible extent, and did not even want his discovery to bear his name. Long after his passing he is still undoubtedly the most important personality in the history of radiology, having laid the foundations for the many and varied advances made in his wake. Through the European Day of Radiology, the ESR and all participating societies will be hoping to contribute to making those advances better known and to bringing the image of radiology into a sharper focus. ESR Press Office Neutorgasse 9 1010 Vienna, Austria press@myESR.org www.myESR.org

1 Responding to the invitation of the European Society of Radiology President Desprechins with the board of the Royal Belgian Radiological Society have organized a press conference in the Museum of Radiology in Brussels on the 10th of February. Considering their appeal to the general public, two topics were chosen for this conference, namely acute stroke and safety image (dose reduction). The summaries of the presentations by three radiologists will appear in the next issue of JBR-BTR.


news (Wet. Prijs Baert)_Opmaak 1 16/02/11 10:03 Pagina 1

JBR–BTR, 2011, 94: 44.

NEWS FROM THE UNIVERSITIES

Wetenschappelijke Prijs em Prof Dr A.L. Baert Dinsdag 7 december 2010 kreeg Dr. Chantal Van Ongeval de 7de “Wetenschappelijke Prijs Em Professor Doctor A.L. Baert” ter waarde van 2.500 euro. De jury heeft haar de titel toegekend “Laureaat Wetenschappelijke Prijs Em Professor Doctor A.L. Baert”, voor haar onderzoekswerk “Performance and optimization of clinical digital mammography”. Dr Van Ongeval is staflid van de dienst Radiologie. Deze tweejaarlijkse prijs bekroont het werk van een radioloog, opgeleid aan één van de vier Nederlandstalige universiteiten in België, op basis van een met goed gevolg verdedigde doctoraatsthesis. De prijs werd voor de 1ste keer uitgereikt in 1998, ex aequo aan Prof Dr Robert Hermans, Dr Marc Lemmerling en Dr Lieven Van Hoe. De volgende laureaten waren: Prof Dr J. Van Goethem (2000) Prof Dr M. De Maeseneer (2002) Prof Dr S. Dymarkowski (2004) Prof Dr M. Van Goethem (2006) Prof Dr G. Maleux (2008) De 8ste “Wetenschappelijke Prijs Em Professor Doctor A.L. Baert” Periode 2011-2012 zal uitgereikt worden in december 2012. Het Algemeen Reglement zal in het najaar 2011 in het Belgisch tijdschrift voor radiologie gepubliceerd worden.

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From left to right: Prof. E. Ponette, Prof. R.F. Dondelinger, Dr Ch. Van Ongeval, Prof. A.L. Baert, Dr P. Cleeren.


Musée (tekst ned.+fr)_Opmaak 1 16/02/11 11:29 Pagina 46

JBR–BTR, 2011, 94: 46-53.

De eerste Wereldoorlog in België Radiologie in "Trench Coat"

La Grande Guerre de 1914-1918 La radiologie belge monte au front

René Van Tiggelen

René Van Tiggelen

Met de medewerking van Luc De Broe, Walter Esch, Françoise Goetghebuer, Georges Mazy, Lieven Mortelmans, François Olier, Laurent Provost, Robert Smets, Ronny Van Loon, Luc Viaene

Avec la collaboration de Luc De Broe, Jan Dirckx, Walter Esch, F. Goetghebuer, Georges Mazy, Lieven Mortelmans, François Olier, Laurent Provost, Robert Smets, Ronny Van Loon, Luc Viaene

Vertaling Jan Dirckx Vormgeving Jacques Louagie Brussel 2011

Mise en pages par Jacques Louagie Bruxelles 2011

Inhoudstafel

Table des matières

Lijst van medewerkers . . . . . . . . . . . . . . . . . . . . . . . Toelichting .............................. Illustratie .............................. Voorwoord .............................. Hoofdstuk 1 Aanvang van de radiologie in het leger . . . . . . . . . . . . . . . . . . . . . . . Hoofdstuk 2 Belgische legerradiologen bij de pioniers . . . . . . . . . . . . . . . . . . . . . . . Hoofdstuk 3 Opvattingen van de Medische Dienst bij de aanvang van de oorlog . . . . . Hoofdstuk 4 De radiologie trekt naar het IJzerfront . . . . . . . . . . . . . . . . . . . . . . Hoofdstuk 5 Hoe werkt de röntgenapparatuur in deze tijd nu eigenlijk? . . . . . . . . . . . . Hoofdstuk 6 Voertuigen voor radiologie . . . . . . . Hoofdstuk 7 Plaatsbepaling en verwijdering van projectielen . . . . . . . . . . . . . . . . Hoofdstuk 8 De gevaren van de X-Stralen . . . . . . Hoofdstuk 9 Moeilijkheden en oplossingen in de radiologie . . . . . . . . . . . . . . . . . Hoofdstuk 10 Ongelukkige tussenkomst van Röntgen . . . . . . . . . . . . . . . . . . . . . . . Hoofdstuk 11 Dr. Etienne Henrard: de radioloog die generaal wordt . . . . . . . . . . . . . . Hoofdstuk 12 Van laborant tot technoloog . . . . . . Hoofdstuk 13 De radiologie beschreven door hen die haar hebben ondergaan . . . Hoofdstuk 14 Positieve gevolgen van het conflict voor de radiologie . . . . . . . . . . . . . . Nawoord .............................. Klein lexicon ter uitleg . . . . . . . . . . . . . . . . . . . . . . . Bronnenvermelding . . . . . . . . . . . . . . . . . . . . . . . . . . Index van eigennamen . . . . . . . . . . . . . . . . . . . . . . . Dankbetuigingen . . . . . . . . . . . . . . . . . . . . . . . . . . . . Inhoudstafel ..............................

I III V VII 1 5 11 15 25 35 51 67 75 81 87 97

103 107 121 125 129 137 141 143

Liste des collaborateurs . . . . . . . . . . . . . . . . . . . . . . Avant-propos . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Illustration . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Préface ................................ Chap. 1 Les débuts de la radiologie militaire . . . . . . . . . . . . . . . . . . . . . . . . . Chap. 2 Les radiologues militaires belges parmi les pionniers . . . . . . . . . . . . . . . Chap. 3 Doctrine du service médical jusqu'au début des hostilités . . . . . . . Chap. 4 La radiologie monte au front de l'Yser . . . . . . . . . . . . . . . . . . . . . . . . Chap. 5 En fait, comment l'appareillage fonctionne-t-il à l'époque ? . . . . . . . . . Chap. 6 Voitures de radiologie . . . . . . . . . . . . . Chap. 7 La localisation des projectiles et leur extraction . . . . . . . . . . . . . . . . . . . Chap. 8 Les dangers des rayons X . . . . . . . . . . Chap. 9 Difficultés radiologiques et solutions . . . . . . . . . . . . . . . . . . . . . . . . Chap. 10 Intervention malheureuse de Röntgen . . . . . . . . . . . . . . . . . . . . . . . . . Chap. 11 Le Dr Etienne Henrard: le radiologue qui devint général . . . . . . . . . . . . . . . . Chap. 12 Du garçon de laboratoire au manipulateur . . . . . . . . . . . . . . . . . . . . Chap. 13 La radiologie décrite par ceux qui l’ont subie . . . . . . . . . . . . . . . . . . . . Chap. 14 Conséquences radiologiques positives du conflit . . . . . . . . . . . . . . . . Postface ................................ Petit lexique explicatif . . . . . . . . . . . . . . . . . . . . . . . . Sources ................................ Index des noms propres . . . . . . . . . . . . . . . . . . . . . . Remerciements . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Table des matières . . . . . . . . . . . . . . . . . . . . . . . . . . .

Inschrijving:

Souscription:

Militair Hospitaal Koningin Astrid Bruynstraat 2, 1120 Brussel info@radiology-museum.be www.radiology-museum.be Tel.: +32 (0)2 264.40.97 Fax: +32 (0)2 264.40.98

Hôpital Militaire Reine Astrid rue Bruyn 2, 1120 Bruxelles info@radiology-museum.be www.radiology-museum.be Tél.: +32 (0)2 264.40.97 Fax: +32 (0)2 264.40.98

I III V VII 1 5 11 15 25 35 51 67 75 81 87 97 103 107 121 125 129 137 141 143

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Musée (tekst ned.+fr)_Opmaak 1 16/02/11 11:29 Pagina 47

VAN TIGGELEN

47

Hoofdstuk 7

Chapitre 7

Plaatsbepaling en verwijdering van projectielen

La localisation des projectiles et leur extraction

Plan van dit hoofdstuk

Plan du chapitre

Plaatsbepaling 1 door driehoeksmeting

Localisation par onder radioscopie met radiografie

1 triangulation

2 met een speciale passer Verwijdering

radioscopique radiographique

2 compas Extraction par

1 electromagneet 2 electrische vibrator 3 electrische localisator

Naast het diagnosticeren van ziektes en breuken stelt zich in oorlogstijd het delikate probleem van de plaatsbepaling van metalen vreemde voorwerpen (projectielen, shrapnels...) die vaak een bron van ontsteking of gangreen vormen en soms tot de dood leiden. Het volstaat niet enkel de aanwezigheid van een projectiel in het lichaam te onderkennen opdat de chirurg het zou kunnen verwijderen. Daarvoor is het eveneens nodig de plaats ervan te bepalen, d.w.z de diepte te bepalen waarop het voorwerp zich bevindt alsook zijn ligging ten opzichte van de spieren, organen en botten in de onmiddellijke omgeving (fig. 56). Het probleem dat zich stelt is dat een radiografie een tweedimensionale projectie vormt van een driedimensionaal volume. Reeds in maart 1896, slechts drie maanden na de ontdekking van de X-stralen, beschrijven A. Buguet en A. Gascard 15 een eerste doeltreffende techniek; de talrijke sindsdien beschreven methodes zijn slechts varianten ervan. Inderdaad, in een literatuuroverzicht door de Amerikaanse militaire arts J. Case 19 uitgevoerd in 1919, worden er meer dan 216 verschillende methodes opgesomd, zonder die methodes waartoe hij geen toegang had erbij te tellen, nl. de methodes uitgewerkt door de Duits-Oostenrijkse, Russische, Scandinavische en Nederlandse legers! Zonder teveel in detail te treden kunnen we zeggen dat sommige technieken de juiste afstanden bepalen ten opzichte van uitgekozen merkpunten op de huid, terwijl andere een metalen zoekapparaat (een vaak ingewikkelde, maar nauwkeurige passer) gebruiken. Deze laat ook toe de resultaten te tonen in de operatiezaal. Laten we vooraf de eerste categorie bespreken, nl. deze die de vrij algemene triangulatiemethode toepast; deze wordt met zeer verschillende toestellen in praktijk gebracht. Hier volgt de uitleg van deze methode in zijn meest eenvoudige toepassing onder radioscopie (fig. 57): S is de patiënt. E is het erachtergeplaatste scherm. P is het projectiel. T is het brandpunt van de X-stralenbuis die parallel aan het scherm kan verplaatst worden van T naar T’. De lengte van de verplaatsing van de buis is gekend. Het merkpunt A bevindt zich op een vast metalen kruis waarvan de schaduw in h op het scherm wordt geprojecteerd. Wanneer de buis zich in T bevindt, wordt op het lichaam S het kruis A geplaatst op zulkdanige wijze dat de normale straal doorheen projectiel P gaat; zo wordt h de projectie van het vreemde lichaam. Vervolgens wordt de buis verplaatst van T naar T’: de schaduw van het projectiel valt nu in p’, de schaduw van het kruis in a.

1 électro-aimant 2 électro-vibreur 3 révélateur électrique

A côté du diagnostic des maladies et des fractures se pose en temps de guerre le délicat problème de la localisation des corps étrangers métalliques (projectiles, shrapnells..) souvent source d'infection, de gangrène voire de mort. La situation est aggravée par les conditions sanitaires et alimentaires précaires des combattants. Il ne suffit pas d'avoir reconnu la présence d'un projectile dans l'organisme pour que le chirurgien puisse l'extraire; il faut, en outre, pouvoir le localiser, c'est-à-dire déterminer la profondeur à laquelle il se trouve ainsi que sa position par rapport aux muscles, organes et os de la région (fig. 56). Le problème est que la radiographie est une projection en deux dimensions d'un volume qui en possède trois. Dès mars 1896, trois mois après la découverte des rayons X, A. Buguet et A. Gascard 15 décrivent déjà la technique du premier procédé rigoureux dont la plupart des nombreuses méthodes publiées depuis ne sont que des variantes. En effet, dans une revue de la littérature effectuée en 1919, un médecin militaire américain, J. Case, 19 dénombre plus de 216 publications différentes, sans compter les méthodes auxquelles il n'a pas eu accès, élaborées par les troupes austro-allemandes, russes, scandinaves et hollandaises! Sans entrer dans trop de détails, disons que certaines techniques déterminent les distances exactes par rapport à des repères cutanés choisis, alors que d'autres utilisent un appareil métallique de repérage (un compas, souvent complexe mais précis). Celuici permet de reporter ses résultats en salle d'opération. Abordons tout d'abord la première catégorie; celle qui a eu recours à la méthode très générale de triangulation, mise en pratique à l'aide des dispositifs les plus variés. Voici le principe de cette méthode dans son application radioscopique la plus simple (fig. 57): S est le sujet. E est l'écran placé derrière lui. P est le projectile. T est le foyer de l'anode du tube à rayon X que l'on peut déplacer transversalement de T en T' parallèlement à l'écran. La longueur du déplacement du tube est connue. Le repère A est sur un croisillon métallique fixe dont l'ombre se voit en h sur l'écran. Quand le tube est en T, on place sur le sujet S le croisillon A de telle façon que le rayon normal passe par le projectile P; ainsi h est la projection normale de ce corps étranger. On décale alors le tube de T en T': l'ombre du projectile vient en p', l'ombre du croisillon en a.


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JBR–BTR, 2011, 94 (1)

La distance p'a donne la proDe afstand p’a geeft de diepte fondeur PA sur une échelle. Cette PA weer op een schaal. Deze échelle est établie une fois pour schaal wordt voor eens en altijd toutes, en grandeur naturelle, en vastgelegd in ware grootte, door menant à partir du point T' une vanaf punt T’ een reeks rechten te série de droites passant par les trekken die doorheen de natudivisions centimétriques naturelurlijke centimeterverdelingen les de l'axe A h. gaan van de as Ah. Cette échelle peut d'ailleurs De schaal kan trouwens worêtre faite pour des positions den uitgevoerd voor verschillende variables de l'écran suivant les opstellingen van het scherm naarbesoins. On détermine aussi gelang de noodwendigheid. Men bien, si on le préfère, la distance kan, indien men het verkiest, du projectile au repère B de la evengoed de afstand van het face d'émergence. projectiel tot het merkpunt B, Cette méthode de trianguwaar de straal het lichaam verlaat, lation est appliquée très bepalen. diversement suivant les auteurs Deze triangulatiemethode qui prennent comme fixe, les uns wordt op zeer verschillende la distance du tube à l'écran, les manieren toegepast volgens het autres la distance du tube au vaste punt waarvan de auteurs croisillon normal. Les uns font un vertrekken; voor de ene is dat de décalage quelconque, les autres afstand van de buis tot het un décalage fixe. Les uns scherm, voor de andere de mesurent la profondeur par un fil afstand van de buis naar het tendu entre T' et p, les autres par richtkruis. De ene wendt een variune échelle, d'autres par un abele verplaatsing aan, de andere tableau, d'autres la calculent. een vaste. De ene meet de diepte Une multitude de variantes door een draad te spannen tussen existent dans la pratique. T’ en p, de andere met een schaal, Les procédés radioscopiques weer een andere met een tabel, possèdent l'avantage de la rapinog een andere maakt berekenindité. Ils peuvent entraîner des gen. De praktijk is gekenmerkt troubles biologiques (voir chapidoor een hele reeks varianten. tre suivant), principalement chez Het voordeel van de radiol'expérimentateur peu ou pas scopie ligt in haar snelheid. Er Fig. 57 Triangulatieprincipe protégé, si cette technique est kunnen evenwel problemen van trop souvent répétée. biologische aard uit voortvloeien Principe de la triangulation Si les indications fournies au (zie volgend hoofdstuk), voorchirurgien par les procédés namelijk door de weinig of niet radioscopiques sont parfois suffisantes, il faut beschermde operator, indien deze techniek te cependant tenir compte que, dans nombre de cas, les veelvuldig wordt herhaald. procédés radiographiques s'imposent parce que la visiHoewel de aanduidingen aan de chirurgen verschaft bilité des projectiles sur l'écran ne permet pas une door de radioscopische techniek soms kunnen volbonne interprétation. Ceci, en plus de la nécessité de staan, dient er nochtans rekening mee gehouden dat in protéger à la fois l'expérimentateur et le blessé des bepaalde gevallen de radiografische techniek zich irradiations trop prolongées, oblige à employer les opdringt omdat de zichtbaarheid van de projectielen op procédés radiographiques. het scherm geen goede interpretatie toelaat. Dit noopt,

Toepassingsschema van de radioscopie bij de plaatsbepaling van een projectiel. Hier wordt de stralingsbuis onder de tafel aangebracht en het radioscopiescherm boven de patiënt. G. Nieuwenglowski (1924). 101 Schéma d'application de la radioscopie à la localisation d'un projectile. Ici le tube radiogène est placé sous la table et l'écran radioscopique au-dessus du patient. G. Nieuwenglowski (1924). 101


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Radioscopische tafel “Ledoux-Lebard” van het militair hospitaal Beveren-aan-de-IJzer (Privé verzameling). Table radioscopique "Ledoux-Lebard" de l'hôpital militaire de Beveren-sur-Yser (Collection privée).

benevens de noodzakelijkheid om de uitvoerder van het experiment en de gewonde te beschermen tegen te langdurige bestraling, tot toepassing van de radiografietechniek. Daar ook zijn veelvuldige technieken voorgesteld, en nog ingewikkelder dan deze die gebruik maken van de radioscopie! We bespreken hier de methode voorgesteld door G. Massiot (fig. 58). De lamp wordt in M geplaatst, men glijdt in de gleuven onder de sokkel van de koepel het kartonnen - of vezelplaatje dat het schietlood draagt. Terwijl de lampdrager verplaatst wordt, wordt een schietlood aangebracht, dat de richtstraal voorstelt, op het punt dat gekozen wordt als spoor van deze straal. Deze plaatsing dient gemerkt te worden door de staaf T, zodanig te verplaatsen dat zijn uiteinde samenvalt met het schietlood en dat zijn richting waarlijk vertikaal is. Een eerste belichting wordt verricht (fig. 58-A) en in die voorwaarden geeft het uiteinde van de staaf het spoor van de richtstraal weer in N, en een schaduw van het projectiel P projecteert zich in O. Een tweede belichting wordt in dezelfde omstandigheden uitgevoerd, na de verplaatsing van de lamp over een willekeurige afstand. Deze tweede belichting maakt een nieuwe afdruk op dezelfde plaat van het spoor van de richtstraal, uitgaande van M’ naar N’ en van een schaduw O’ van hetzelfde projectiel P. Vervolgens wordt de radiografische plaat (R) die eerst onder de patiënt is geplaatst, weggenomen. Zo zijn de betrekkelijke plaatsen van NN’, OO’ bekomen: het komt er nu op aan deze schaduwen te gebruiken om de betrekkelijk hoogte van het projectiel te vinden ten opzichte van het vlak van de plaat. Op een willekeurige tafel wordt het buisstatief geplaatst, nadat men de lamp en de koepel niet meer nodig heeft, voor de volgende handelingen (fig. 58 B).

Là encore, de multiples moyens ont été proposés et le plus souvent encore plus compliqués que ceux empruntant la radioscopie! Nous retiendrons ici la méthode proposée par G. Massiot (fig. 58). L'ampoule étant placée en M, on introduit dans les rainures pratiquées sous le socle de la cupule, la plaquette de carton ou de fibre qui supporte le fil à plomb. En déplaçant le porte-ampoule on amène un fil à plomb, représentant le rayon normal, au point qu'on a choisi comme trace de ce rayon. Il faut repérer cet emplacement en disposant une tige T coulissant horizontalement le long du support et verticalement dans un écrou fendu, de façon que son extrémité se confonde avec le plomb du fil, et que sa direction soit bien verticale. On fait une première exposition (fig. 58-A) et dans ces conditions l'extrémité de la tige donne la trace du rayon normal en N, et une ombre du projectile P se fait en O. Une seconde pose est réalisée dans les mêmes conditions après avoir déplacé l'ampoule d'une quantité arbitraire. Cette seconde exposition donne lieu à l'impression nouvelle sur la même plaque de la trace du rayon normal, émanant de M' en N' et une ombre O' du même projectile P. La plaque radiographique (R) qu'on avait préalablement posée sous le malade est ensuite retirée. Ayant obtenu les positions relatives des points NN', OO': il s'agit maintenant d'utiliser ces ombres à la recherche de la hauteur relative du projectile par rapport au plan de la plaque. On porte sur une table quelconque ce porte-ampoule, après avoir retiré l'ampoule et la cupule dont on n'a plus besoin, pour les manipulations qui vont suivre (fig. 58-B).


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Fig. 58 A. De patiënt wordt onder twee invalshoeken belicht. B. Opzoeken van de plaats van het projectiel in de ruimte. C. Het statief wordt opnieuw boven de onderzochte streek geplaatst. A. Le sujet est exposé sous deux incidences. B. Recherche dans l'espace de la position du projectile. C. Le support est à nouveau placé sur la région examinée. G. Massiot et Biquard (1915).

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De radiografische plaat wordt aangebracht op de hoogte die ze in de opeenvolgende belichtingen ingenomen heeft. Praktisch bekeken volstaat het ze vlak op de tafel te leggen. En op de uiteinden van de merkstaven T en T’ worden de dwarsstaafjes aangebracht, waarvan de ringen precies in de punten M en M’ geplaatst worden, de punten waar opeenvolgend het uitzendpunt van de X-stralen geweest is. Door middel van een schietlood dat neerhangt uit M en M’, wordt de plaatsing van de plaat geregeld zodanig dat ze dezelfde plaats inneemt als de plaats onder de gewonde. Dit wordt vergemakkelijkt door deze plaat te oriënteren totdat de sporen van de schietloden samenvallen met de schaduwen van de uiteinden van de merkstaven. Wat de vrije dradenuiteinden betreft, die worden

La plaque radiographique se place à la hauteur qu'elle occupait dans ces expositions successives; pratiquement, il suffit de la poser à plat sur la table, et on introduit sur les extrémités des tiges repères T et T', les potences dont les anneaux se placent exactement aux points M et M' où se trouvait successivement le centre d'émission des rayons X. Au moyen de fils à plomb tombant des points M et M', la position de la plaque est réglée de façon qu'elle occupe la place qu'elle avait sous le blessé, ce qui est facile en orientant cette plaque jusqu'à ce que les traces des fils à plomb tombent sur les ombres des extrémités des tiges repères. Quant aux extrémités libres des fils, ils sont arrêtés au moyen de poids, aux points où les ombres du projectile sont apparues.


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tegengehouden door een gewicht op die plaatsen waar de schaduwen van het projectiel verschenen zijn. Het kruisingspunt van deze draden stelt de plaats van het projectiel voor. Om de plaats ervan in de ruimte vast te leggen, ten overstaan van welbepaalde coördinaten, worden de uiteinden van de drie staven a,b en c loodrecht aangebracht aan de drie zijden van het buisstatief in het contact met punt P. Met zorg wordt eveneens de aanwijzer C glijdend aangebracht op elke staaf, tegen hun respectievelijke huls. Deze drie staven vormen samen het vlak waarin het projectiel zich bevindt. De draden die geen dienst meer doen worden teruggetrokken. De drie staven worden van mekaar verwijderd zonder de aanwijzer te verplaatsen, om toe te staan het lampenstatief opnieuw over de bestraalde oppervlakte te plaatsen en dat op de plaats die het eerst ingenomen heeft, zoals getoond in fig. 58 C. De drie staven a,b, en c worden naar mekaar toegebracht en zodoende worden drie merkpunten bepaald die met een dermatografisch potlood worden aangebracht. Deze drie punten bepalen een vlak waarin het projectiel zich bevindt en de drie betrekkelijke afstanden van elke aanwijzer aan de stavenhulzen, komen overeen met de diepte van het projectiel met betrekking tot die drie punten. De aanduidingen, gegeven door de betrekkelijke plaatsen van de drie staven, kunnen volstaan maar immobiliseren het radiografiematerieel tot op het ogenblik waarop de chirurg wordt geroepen voor de ingreep.

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Le point de croisement de ces fils représente la place du projectile. Pour en fixer la position dans l'espace, par rapport à des coordonnées bien définies, les extrémités de trois tiges a,b,c, sont amenées perpendiculairement aux trois faces du porte-ampoule au contact avec le point P. On a soin également d'amener les index C coulissant sur chaque tige, contre leur douille respective. Ces trois tiges constituent un plan dans lequel se trouve le projectile. Les fils qui n'ont plus d'utilité sont retirés, les trois tiges sont écartées sans déplacer les index, pour permettre de poser à nouveau le support d'ampoule audessus de la région radiographiée et à l'endroit qu'il occupait primitivement, comme le montre la figure 58C. Les trois tiges a, b, c, sont rapprochées au contact avec la peau, et l'on détermine ainsi trois points de repère qu'on trace avec un crayon dermographique. Ces trois points définissent un plan dans lequel se trouve le projectile, et les trois distances relatives de chaque index aux douilles des tiges, correspondent aux profondeurs du projectile par rapport à ces trois points. Les indications fournies par les positions relatives des trois tiges peuvent être suffisantes, mais immobilisent le matériel radiographique jusqu'au moment où le chirurgien sera appelé à intervenir.

Fig. 59 Plaatsbepalingspasser voor de opsporing van projectielen, van Geneesheer Majoor E. Hirtz. Compas-localisateur pour recherche de projectiles du médecin-major E. Hirtz. Belgian Museum of Radiology


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Fig. 60 Hirtz-passer Compas de E. Hirtz réglé prêt à l'emploi G. Pallardy et coll (1989).

Vandaar het nut van een speciale plaatsbepalingspasser: deze vervolledigt het gebruiksklare radiologiematerieel op het ogenblik van de operatie. Bovendien weten allen die de opzoeking van projectielen uitvoeren dat, eens de huid is ingesneden, het zeer moeilijk is om door te dringen in de dieperliggende weefsels in een welbepaalde richting. Laten we ook opmerken dat voorwerpen, zelfs van een zekere omvang vaak niet gevoeld worden door de verkennende vinger. En zelfs na een zeer moeizame ingreep kan het gebeuren dat men mislukt terwijl men toch zeer dicht bij het voorwerp geweest is. De chirurg heeft dus als gids een apparaat (passer) nodig, dat hem toelaat niet verloren te lopen maar hem zeer precies geleidt naar het gewenste doel. In deze tweede categorie zijn talrijke passers bedacht. Deze van de Franse arts E. Hirtz, ontwikkeld in 1907, vindt een ruimere toepassing (fig. 59 en 60) dan de meeste andere. 58-78 Deze techniek vereist vier opeenvolgende bewerkingen: 1. Beeldopname (fig.61-A). Op een horizontaal geplaatste röntgenplaat (R) wordt, op het snijpunt van de diagonalen, het middelpunt V’ aangeduid. Boven dit punt, aangeduid door een opaak merkpunt, wordt met behulp van een schietlood de focus van een radiografiebuis aangebracht in V. De afstand V V’ wordt onmiddellijk gemeten. Op de plaat wordt de zone opgezocht, die het vreemd voorwerp P omschrijft, waarvan de plaats dient bepaald te worden. Drie merkpunten A, B en C, worden op de huid aangeduid d.m.v. metalen aanwijzers. Deze punten bepalen een willekeurig driehoeksvlak op de plaat R. De buis wordt vervolgens, parallel met de plaat, naar F en F’ verplaatst, en dan

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De là l'utilité d'un compas localisateur spécial qui rentre dans la seconde catégorie et complète le matériel radiologique prêt à l'emploi, lors de l'opération. De plus, tous ceux qui exécutent des recherches de projectiles savent qu'une fois la peau incisée, il est très difficile de progresser dans les parties profondes selon une direction bien déterminée. Il faut noter que les objets, même assez volumineux, ne donnent souvent aucune sensation au doigt explorateur et qu'il arrive d'échouer après une intervention laborieuse alors qu'on est passé bien près du but. Il faut donc un guide pour l'opérateur, un appareil (compas) qui ne lui permette pas de s'égarer et qui le conduise avec précision au point voulu. Dans cette seconde catégorie, de nombreux compas sont imaginés. Celui du médecin français E. Hirtz, développé en 1907, est le plus largement utilisé (fig. 59 et 60). 58, 78 Son emploi nécessite quatre opérations consécutives: 1. Prise du cliché (fig. 61-A). Sur une plaque radiographique (R) horizontale, on marque, au croisement des diagonales, le centre de figure V’. Au-dessus de ce point, signalé par un index opaque, on amène avec le fil à plomb le foyer d’une ampoule radiographique en V. La distance V V’ est mesurée directement. Sur la plaque est disposée la région qui contient le corps étranger P qu’il s’agit de localiser. Trois points de repère cutanés A, B, C, choisis sont marqués par des index métalliques. Ces points déterminent un triangle de plan quelconque par rapport à celui de la plaque R . Chaque fois que cela est possible, il circonscrit le corps P. L’ampoule est transportée successivement en F et F’, parallèlement à un des côtés de la plaque, et de chacun de ces points une


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wordt een röntgenopname verricht. Na ontwikkeling wordt op de radiografie de aanwezigheid van dubbelbeelden van de drie merkpunten A,B, C en van het voorwerp P waargenomen. 2. Uitvoering van de projectieschets (fig. 61-B). Het röntgenbeeld laat toe een grafiek te tekenen, die tot doel heeft de pas uitgevoerde dubbele kegelprojectie om te zetten in een rechthoekige projectie op een horizontaal en een verticaal vlak. We laten de beschrijving van de bekomen ingewikkelde grafiek hier terzijde; volgens haar ontwerper is de uitvoering ervan echter heel eenvoudig. 3. Instelling van de passer (fig. 61C). Overeenkomstig de gegevens op de projectieschets, wordt een plaatsbepalingspasser aangebracht, die schematisch wordt weergegeven in de figuur. Deze bestaat uit drie armen (1, 2 en 3) die rond een centrum O wentelen, drie punten loodrecht op het vlak van de armen, en uit een stift die van uit het middelpunt O, als glijdende plaatsbepalingspeilstift dienst doet. 4. Opsporing van het vreemd voorwerp (fig. 61-D). De ingestelde passer wordt opgesteld voor de opsporing in de zone die berust op een horizontaal vlak T’. Dit is exact dezelfde stand waarin de röntgenopname is verricht. De punten vallen samen met de merkpunten A, B en C op de huid. Met dezelfde nauwkeurigheid gaat de plaatsbepalingspeilstift samenvallen met P. Nochtans, aangezien P ingebed ligt in het weefsel, gaat de peilstift S tegengehouden worden door het lichaamsoppervlak in i; de afstand iP stemt overeen met de diepte van het vreemd voorwerp. Deze wordt opgemeten door de afstand tussen het middelpunt O van de passer en het merkpunt r ingesteld op S. Een insnijding, uitgevoerd in i, gaat de weg openen voor de peilstift S waarvan het eindpunt het voorwerp P gaat raken wanneer het merkpunt r met O samenvalt. Aan de bouw en het gebruik van de passer ligt een meetkundig principe ten grondslag. Bijgevolg dient toegezien te worden dat het vlak van de drie armen 1,2 en 3 horizontaal ligt, terwijl het instrument rust op de merkpunten in de stand van opsporing, en dat de stift S verticaal staat. Hij bepaalt de richtstraal doorheen het vreemd voorwerp.

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radiographie est exécutée. Au développement, on constate sur le cliché la présence d’images doubles pour les trois repères A, B, C, et pour le corps P. 2. Établissement de l’épure (fig. 61-B). Ce cliché radiographique permet de tracer un graphique dont le but est de transformer la double projection conique qui vient d’être exécutée en projection orthogonale sur un plan horizontal et sur un plan vertical. Si la description compliquée du graphique obtenu ne sera pas abordée ici, son exécution, d’après son auteur est d’une simplicité élémentaire! 3. Réglage du compas (fig. 61-C). D’après les données de l’épure on place un compas localisateur schématisé dans la figure. Celui-ci est composé de trois bras (1, 2, 3) pouvant tourner autour d’un centre O, de trois pointes perpendiculaires au plan des bras et d’une tige médiane, sonde localisatrice coulissant à frottement doux à travers le centre O. 4. Recherche du corps étranger (fig. 61-D). Le compas réglé est présenté pour la recherche sur la région reposant sur un plan horizontal T’. Ceci est exactement dans la position où a été exécutée la radiographie. Les pointes vont tomber sur les repères cutanés A, B, C. Avec la même précision, la pointe de la sonde localisatrice coïncidera avec P. Toutefois, comme P est inclus dans les tissus, la sonde S est arrêtée par le tégument en i; la distance iP correspond à la profondeur du corps étranger. Elle s’évalue par la distance entre le centre O du compas et le repère r fixé sur S. Une incision faite en i ouvrira une voie devant la sonde S, dont l’extrémité touchera le corps P lorsque le repère r viendra buter sur O. Il faut faire observer que suite à la conception géométrique qui a présidé à la construction et à l’utilisation du compas, le plan des trois branches 1,2,3, est horizontal lorsque l’instrument repose sur des repères dans la position de recherche et que la tige S est verticale. Elle matérialise le trajet passant par le corps étranger.

Fig. 61 Schema van de verschillende stappen die toelaten de plaats te bepalen van een projectiel, dankzij de Hirtz-passer (E. Henrard, 1929). 74 Schémas des différentes phases permettant de situer un projectile grâce au compas de Hirtz, (E. Henrard, 1929).

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FORTHCOMING COURSES AND MEETINGS NATIONAL CALENDER 19.11.11 Annual RBRS symposium 2011 Imaging of the spine and the spinal cord: state-of-the-art Organization: Dr B. Desprechins 02.02.11 RBRS – Cardiac imaging Information: rodrigo.salgado@scarlet.be 18.03.11 RBRS – Cardiovascular and interventional radiology Antwerp, UZA Information: Dr O. d’Archambeau, olivier.archambeau@uza.be 24-25.03.11 ATELIERS DE COLONOSCOPIE VIRTUELLE Liège, Clinique Saint-Joseph Information: www.chc.be 25.03.11 RBRS – Bone and joints Information: Pieter.Van.Dyck@uza.be 21.04.11 RBRS – Pediatric radiology Antwerp, UZA Information: brigitte.dep@hotmail.be 21.05.11 RBRS – Bone and joints Information: Pieter.Van.Dyck@uza.be

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FORTHCOMING COURSES AND MEETINGS 28.04.11 ESOR GALEN ADVANCED COURSE: Urogenital Cross-Sectional Imaging Barcelona, Spain Information: www.myESR.org/esor 04-07.05.11 ADVANCED MRI 2011-02-01 Graz, Austria Information: www.diagnosticzentrum.com 15.05.11 INTERNATIONAL CONFERENCE OF NON-INVASIVE CARDIOVASCULAR IMAGING 2011 Amsterdam, The Netherlands Information: http://www.escardio.org/ congresses/ICNC10/Pages/welcome.aspx 21.05.11 ESGAR 2011 European Society of Gastrointestinal and Abdominal Radiology Venice, Italy Information: www.esgar.org 08.06.11 CONGRÈS 2011 MEETING CIRA-SFICV 10e réunion annuelle de CIRA conjointement avec la SFICV Montreal, Canada Information & Registration For Canadian and International participants:

Registration (starting January 2011): www.ciraweb.org E-mail: info@ciraweb.org Tel.: 514-282-2744 For participants fromFrance: Registration (starting January 2011): agence@coralys.fr E-mail: agence@coralys.fr Tel.: 04 67 79 24 95 09-11.06.11 18TH EUROPEAN SOCIETY OF MUSCULOSKELETAL RADIOLOGY MEETING (ESSR) Crete, Greece Main theme refresher course: Bone marrow. Additional refresher courses on different MSK topics. Scientific papers on all aspects of MSK Radiology Information: http://www.essr.org/cms/ website.php?id=/en/essr_home.htm 18.06.11 Paris, France 8EME JOURNEE DE RADIOLOGIE HEPATOBILIAIRE DU GROUPE HOSPITALIER BICETRE-PAUL BROUSSE Theme: Les nouveautés technologiques – Le carcinome hépato-cellulaire revisité en 2011 – L’exploration moderne des voies biliaires Information: Secrétariat du Pr M.F. Bellin, veronique.rey@bct.aphp.fr

55 22.06.11 COMPUTER ASSISTED RADIOLOGY AND SURGERY – 25th International Congress and Exhibition 2011 Berlin, Germany Information: http://www.cars-int.org/ 21-24.09.11 USA, San Diego ISS MUSCULOSKELETAL IMAGING COURSE FUNDAMENTALS TO ADVANCED CONCEPT Information: www.internationalskeletalsociety.com 21-25.10.11 JFR – JOURNÉES FRANÇAISES DE RADIOLOGY Paris, France Information: www.sfrnet.org 27.11-02.12.11 RSNA ANNUAL MEETING Chicago, USA Information: www.rsna.org 08.11.11 11TH WORLD FEDERATION OF INTERVENTIONAL AND THERAPEUTIC NEURORADIOLOGY CONGRESS South Africa, Cape Town Information: www.wfitn2011.org

CLASSIFIED SERVICES Experienced, native English teacher in Brussels offers: 1) Proofreading of oral presentations; 2) Business English lessons. References available upon request from commercial firms, civil servants within the EU institutions, and one from the Belgian Thoracic Society. If interested please contact Iain Campbell on: EnglishTuition4U@gmail.com or on: mobile 0493-212244.

CLASSIFIED SERVICES Région Lilloise, groupe radiologique, 5 sites dont scanner. Cherche plusieurs associés en vue de l’ouverture d’une IRM. Conditions financières très intéressantes. Revenus très importants. Pas de garde. Tél.: 00.33.6.13.23.84.69.


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