Jams Journal April-June 2013

Journal of Applied MEDICINE & SURGERY Volume 2, Issue 4, April-June 2013

Journal of Applied MEDICINE & SURGERY

Volume 2, Issue 4 April-June 2013

JAMS Editorial Panel Editor-in-Chief SM Rajendran Executive Editor Jayakar Thomas

Associate Editor S Bhuminathan

JAMS Advisory Board Radiology Chandrasekar V

Anatomy Johnson WMS

Biochemistry Shanthi B

Microbiology Chitralekha S

Dentistry Masthan KMK

Orthopaedics Sivakumar A

Pharmacology Inbaraj SD

Pathology Monika G

Community Medicine Steven T

Nephrology Jayakumar M

Surgery Ravishankar S

Forensic Medicine Tahera Begum

Anatomy Jayanthi V

Physiology Saikumar P

IJCP’s Editorial Panel Padma Shri and Dr BC Roy National Awardee Dr KK Aggarwal Group Editor-in-Chief Dr Sanjiv Chopra Prof. of Medicine & Faculty Dean Harvard Medical School Group Consultant Editor

Dr Veena Aggarwal MD, Group Executive Editor Dr Deepak Chopra Chief Editorial Advisor

Advisory Bodies Heart Care Foundation of India, Non-Resident Indians Chamber of Commerce and Industry, World Fellowship of Religions

Contents FROM THE DESK OF EDITOR-IN-CHIEF

4

FROM THE DESK OF EXECUTIVE EDITOR

5

FROM THE DESK OF IJCP GROUP EDITOR-IN-CHIEF

6

CLINICAL STUDY Premenstrual Syndrome

7

Prevalence and Antibiotic Sensitivity Pattern of Bacteria Isolated from Nosocomial Infections in a Surgical Ward

9

CASE REPORT Gastric Rupture Following Blunt Trauma Abdomen

14

Post-coital Generalized Pruritus as a First Symptom of Polycythemia Vera: A Very Rare Case

16

Scalpel Cautery Adenotonsillectomy Done in a Case of Crouzon’s Syndrome

21

Thirty-six Cases of Vocal Polyps Treated by Microflap Phonomicrolaryngeal Surgery

27

Wireless Capsule Endoscopy Requiring Surgical Intervention

32

Acute Fatty Liver of Pregnancy with Overlapping HELLP Syndrome Associated with Transient Proximal Myopathy

35

Ruptured Pulmonary Hydatid Cyst: The Camalote Sign

39

Glomus Tumor: A Case Report with Review of Literature

42

Unusually Large Functional Adrenal Adenoma: A Rare Case Report with Review of Literature

45

Hirschsprung’s Enterocolitis Presenting as Fulminant Sepsis in A Neonate

48

Laurence-Moon-Biedl Syndrome

50

Nevus of Ota is a Rare Nevus, Q-switched Laser is the Best Available Option for Successful Treatment

52

Scorpion Bite causing Acute Severe Myocarditis: A Rare Complication

55

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FROM THE DESK OF EDITOR-IN-CHIEF

Prof. SM Rajendran

MD, FRCP, (Glasgow), FIMSA Registrar, Bharath University, Chennai Former Prof. & Head, Dept. of Medicine and Diabetology Stanley Medical College and Hospital, Chennai

Dear Readers Journal of Applied Medicine & Surgery is marching forwards in the practice of Medicine. All over the South as well as in the North, Post Graduates and Under Graduates viewers are benefiting by viewing this Journal.

The information is rich and soon it will become a powerful tool for Clinical Medicine.

Our Journal will always be open to observe and we welcome your suggestions and comments.

Sree Balaji Medical College and Hospital of Bharath University has dedicated itself for academic and research work and our main vision is that Clinical Medicine reaches you all.

Sincere thanks to our Chair Person Dr Mrs. J. Srinisha for her continuing encouragement and taking keen interest and personal care to bring out each issue.

My wishes to all Readers Thanking you, Registrar Bharath University

4

Journal of Applied Medicine & Surgery, Vol. 2, Issue 4 April-June 2013

FROM THE DESK OF EXECUTIVE EDITOR

Prof. Jayakar Thomas

Executive Editor MD, DD, MNAMS, PhD, FAAD, FRCP (Edin) FRCP (Glasgow), FRCP (London), FRCP (Ireland), FRCPCH (London)

Dear Readers The journey through our academic pursuit through JAMS continues.

I am thankful to the untiring efforts of our reviewers and the encouragement from the Editor-in-Chief Dr SM Rajendran and the Associate Editor Dr. Bhuminathan.

Our Chairperson Dr. Srinisha needs special mention for her unlimited support and back-up.

I render my heartfelt thanks to all of you.

Things are happening and all of you should join the celebrations.

I wish you happy reading.

Journal of Applied Medicine & Surgery, Vol. 2, Issue 4 April-June 2013

5

FROM THE DESK OF IJCP GROUP EDITOR-IN-CHIEF

Dr KK Aggarwal

Padma Shri and Dr BC Roy National Awardee Sr. Physician and Cardiologist, Moolchand Medcity President, Heart Care Foundation of India Group Editor-in-Chief, IJCP Group and eMedinewS National Vice President, Elect. IMA Director, IMA AKN Sinha Institute (08-09) Hony. Finance Secretary, IMA (07-08) Chairman, IMA AMS (06-07) President, Delhi Medical Association (05-06) emedinews@gmail.com http://twitter.com/DrKKAggarwal Krishan Kumar Aggarwal (Facebook)

Surgery Round Up zz Prophylactic platelet transfusion to continue: Two randomized trials in patients with severe thrombocytopenia due to acute myeloid leukemia, chemotherapy or autologous hematopoietic cell transplantation has shown that compared with patients who were transfused for a threshold platelet count of ≤10,000/µl, patients who were only transfused for active bleeding had more frequent episodes of bleeding and more severe bleeding.

The current practice of prophylactic platelet transfusion at a threshold platelet count of 10,000/µl in this population should therefore continue.1,2 zz Blood transfusion thresholds for acute upper gastrointestinal bleeding: A randomized trial of patients with and without cardiovascular disease suggests that in patients who are not at increased risk for complications from anemia, outcomes are improved if a lower hemoglobin threshold (<7 g/dl rather than <9 g/dl) is used for initiating blood transfusion. Patients transfused when the hemoglobin was <7 g/dl had lower mortality rates, were less likely to have further bleeding and were less likely to suffer complications.3 zz Breast tissue density and breast conserving surgery: In a prospective study of 1,052 women undergoing surgery for invasive breast cancer, patients with the highest breast density were significantly less likely to be treated with an initial breast conserving surgery (BCS) compared with those with less dense breast tissue (52 vs 74%).4 However, breast density was not associated with positive margins on BCS or with conversion to a mastectomy.

Thus, breast density should not be a criterion for BCS. In addition, patients with high breast density were more likely to receive a preoperative magnetic resonance imaging (MRI), although this did not reduce the risk of positive margins.

References 1. Stanworth SJ, Estcourt LJ, Powter G, et al. A no-prophylaxis platelet-transfusion strategy for hematologic cancers. N Engl J Med 2013;368:1771.

2. Wandt H, Schaefer-Eckart K, Wendelin K, et al. Therapeutic platelet transfusion versus routine prophylactic transfusion in patients with haematological malignancies: an open-label, multicentre, randomised study. Lancet 2012;380:1309.

3. Villanueva C, Colomo A, Bosch A, et al. Transfusion strategies for acute upper gastrointestinal bleeding. N Engl J Med 2013;368:11.

4. Kapoor NS, Eaton A, King TA, et al. Should breast density influence patient selection for breast-conserving surgery? Ann Surg Oncol 2013;20:600.

6

Journal of Applied Medicine & Surgery, Vol. 2, Issue 4 April-June 2013

CLINICAL STUDY

Premenstrual Syndrome K Saraswathi*, S Nambi**, L Nandiniâ&#x20AC;

ABSTRACT Premenstrual syndrome (PMS) or the more severe premenstrual dysphoric disorder is caused by hormonal disturbances and alterations in the psychosocial factors before the onset of each menstrual cycle. It affects millions of women in terms of change in mood, somatic and behavioral symptoms. A study was conducted to assess the prevalence rate of PMS among a randomly selected sample population of 50 girls undergoing MBBS in Sree Balaji Medical College and Hospital, Chennai and 50 working women (teachers, technicians, administrators, etc.) who were also selected randomly. It was seen that PMS is not uncommon among women in reproductive age group, both young and middle-aged (13.66%) and that regular fitness regimens like exercise, yoga and meditation help reduce the severity of symptom profile, which improves the quality-of-life.

Key words: Premenstrual syndrome, menstrual cycle, hormonal disturbances, psychosocial factors, quality-of-life

P

remenstrual syndrome (PMS) or the more severe premenstrual dysphoric disorder affects millions of women in terms of change in mood, somatic and behavioral symptoms that occur during the luteal phase of the menstrual cycle. The symptoms of PMS include both physical and psychological. These symptoms often affect the activities of daily living and quality-of-life. Hormonal disturbances and alterations in the psychosocial factors before the onset of each menstrual cycle seem to play an important role in causation of PMS. Aims and Objectives zz To assess the prevalence rate of PMS among college girls and working women. zz To find out the most common symptoms in each group. zz To compare the psychological and somatic symptoms of PMS and to find out methods to reduce the severity of symptom pattern.

Materials and Methods A randomly selected sample population of 50 girls studying for MBBS in Sree Balaji Medical College and

Hospital, Chennai was compared with 50 randomly selected working women (teachers, technicians, administrators, etc.). The study was conducted between January to February 2013. A structured proforma to assess general information and a PMS questionnaire by Kelly Wallace were used for the study.

Results and Discussion Table 1 describes the demographic profile of the study population. The average prevalence rate of PMS among the sample population was found to be 13.66%.

Prevalence among college girls was 14.76% and among working women, it was 12.57% (Fig. 1).

Common symptoms reported have been grouped into somatic and psychological (Tables 2 and 3). Common symptoms observed in the study in college girls were acne, anxiety, depression and irritation.

In working women, breast tenderness, mood swings, irritation and weakness were the common symptoms observed. Overall the psychological symptoms were Table 1. Demographic Profile Profile factors

*Professor and Head Dept. of Obstetrics and Gynecology **Professor and Head Dept. of Psychiatry â&#x20AC; Postgraduate Dept. of Obstetrics and Gynecology Sree Balaji Medical College and Hospital, Chromepet, Chennai

Journal of Applied Medicine & Surgery, Vol. 2, Issue 4 April-June 2013

College girls

Working women

Age (years)

18-20

35-45

Marital status

Single

Married

12

12

Number of persons in the sample undergoing fitness regime

7

CLINICAL STUDY

13.66%

14.76%

College girls Working women Average prevalence

12.57%

more disturbing than physical symptoms in both groups during PMS. In both groups, a reduction of symptoms during PMS was found in those who followed regular physical or mental fitness regime.

Conclusion zz

Figure 1. Prevalence of PMS. zz

Table 2. Somatic Symptoms Symptoms

Percentage (%)

Fatigue

53

Backache

48

Acne

34

Breast tenderness

31

Weight gain

22

Table 3. Psychological Symptoms Symptoms

Percentage (%)

Irritation

56

Anxiety

48

Mood swings

43

Depression

29

zz

PMS is not uncommon among women in reproductive age group both young and middle aged (13.66%).

Both psychological and somatic symptoms are common during PMS. Regular fitness regimens like exercise, yoga and meditation reduce the severity of symptom profile, which will improve the quality-of-life.

Suggested Reading 1. Chandra P, Chaturvedi SK, Gururaj G. Premenstrual experiences, explanatory models and help seeking in Indian women. Indian J Soc Psychiatr 1995;11:73-7.

2. Chandra P, Chaturvedi SK, Gururaj G. Identification and assessment of premenstrual symptoms and syndromes in women: an epidemiological survey approach to investigation. NIMHANS J 1994;12(1):1-8.

3. Brockington I. Menstrual psychosis. Arch Womens Ment Health 1998;1:3-13.

n

8

n

n

Journal of Applied Medicine & Surgery, Vol. 2, Issue 4 April-June 2013

CLINICAL STUDY

Prevalence and Antibiotic Sensitivity Pattern of Bacteria Isolated from Nosocomial Infections in a Surgical Ward Rama Sikka*, JK Mann**, Deep†, MG Vashist‡, Uma Chaudhary¶, Antriksh Deep§

ABSTRACT Background and aims: Nosocomial infections among surgical patients are common and many are preventable. It is critical to understand microbiology of these infections in order to create appropriate strategies to reduce this risk. This study was planned to delineate the occurrence, microbiology and sensitivity pattern of such infections among surgical patients.

Subject and methods: Various from 130 patients admitted to the surgery ward were cultured, identified and antibiotic sensitivity was performed by standard methods. Results: From 130 patients, 146 isolates were recovered. Of these 140 (95.9%) were bacterial and six (4.1%) were of Candida spp. Most frequently observed nosocomial infections were SSIs (55.4%) followed by infections of urinary tract (28.4%), respiratory tract infections (10.8%) and bacteremia was observed in only 5.4% patients. The predominant pathogen isolated from polymicrobial episodes were E. coli, S. aureus, K. pneumoniae, A. baumanii and P. aeruginosa. Resistance to β-lactams was high and carbapenems were found to be most effective drugs against GNBs. Conclusions: Gram-negative organisms are the predominant pathogens causing infections in surgical patients. The increasing trend of resistance to β-lactams is posing a great problem. So for proper management of critically ill patients and patients undergoing various operative procedures and other medical interventions, hospital antibiotic policies need frequent revisions.

Key words: Nosocomial infections, surgical site infections, antimicrobial resistance

N

osocomial infections or healthcare-associated infections encompass all clinically evident infections that do not originate from patient’s original admitting diagnosis.1 The incidence of nosocomial infections is about 5-10% in most developed nations while in India, one in four patients admitted into hospital acquire nosocomial infection.2 Common nosocomial infections in surgical patients include surgical site infections (SSIs), urinary tract infections (UTIs), pneumonias and blood stream infections (BSIs). In 1986, NNIS report, the overall incidence of nosocomial infections was 33.5 per 1,000 discharges, the range extended from 13.3 per 1,000 discharges to 46.7 per 1,000 discharges in surgical patients.

The higher incidence of infections among surgical

*Professor **Resident † Senior Resident, Dept. of Microbiology ‡ Senior Professor, Dept. of Surgery ¶ Senior Professor and Head § Associate Professor Dept. of Microbiology Pt. BD Sharma PGIMS, Rohtak, Haryana Address for correspondence Dr Rama Sikka Professor, Dept. of Microbiology Pt. BD Sharma PGIMS, Rohtak, Haryana E-mail: ramasehgal30@yahoo.com

Journal of Applied Medicine & Surgery, Vol. 2, Issue 4 April-June 2013

patients was largely attributable to SSIs.3 SSIs account for approximately a quarter of all nosocomial infections.

These infections can range from superficial wound infections, which have minimal mortality rates but add a considerable cost to patient care, to necrotizing soft tissue infections, which are associated with prolonged hospitalization, significant healthcare expense and a high mortality rate.4 The incidence of infection varies from surgeon-to-surgeon, from hospital-to-hospital, from one surgical procedure to another, and most importantly from one patient to another. In clean surgical procedures, in which the gastrointestinal, gynecologic and respiratory tracts have not been entered, Staphylococcus aureus from the exogenous environment or the patient’s skin flora is the usual cause of infection. In other categories of surgical procedures, including clean contaminated, contaminated and dirty, the polymicrobial aerobic and anaerobic flora closely resembling the normal endogenous microflora of the surgically resected organ are the most frequently isolated pathogens.5 Emmerson et al in 1996 reported that surgical wound infections accounted for 12.3% of all hospital acquired infections.6 Basa et al in their study observed that department of surgery had the highest infection rate among all clinical departments and 50% of the cases were due to gram-negative bacteria.7 UTIs also accounts for a large number of nosocomial infections in surgical patients. The single most 9

CLINICAL STUDY important factor for nosocomial bacteriuria and UTI is the presence of indwelling urinary catheter.8 This issue of nosocomial infections in surgical patients is further complicated by emergence of polyantimicrobial resistant strains of hospital pathogens.9 Multiple antibiotic resistance to all useful classes of antibiotics has gradually increased among a number of gramnegative hospital pathogens especially the Klebsiella spp., Enterobacter spp., Pseudomonas aeruginosa and Acinetobacter spp.10 Having a knowledge of spectrum of organisms causing SSIs and their resistance pattern is important when considering strategies for controlling the development and spread of resistance. Keeping in mind the above facts, this study was planned to delineate the occurrence, microbiology and sensitivity pattern of such infections for a better management thereby, reducing both mortality and costs.

Subject and Methods Study Center

This was a prospective study carried out at a teaching tertiary care hospital in India over a period of one and a half year.

Patient Selection

One hundred thirty patients admitted to surgical ward, who developed signs and symptoms of SSIs, pyrexia, bacteremia, pyuria or respiratory infection after 48 hours of admission were included in the study. Patients with any signs of infection at the time of admission were excluded from study.

Processing of Specimen

Pus sample or two wound swabs, urine, sputum and blood were collected aseptically from the operated patients and were transported immediately to the Microbiology lab. The pathogens were identified by standard laboratory procedures including Gram’s staining, motility, colony characters and biochemical reactions.11 Antibiotic susceptibility testing was done

by Kirby-Bauer disc diffusion method.12 Following antimicrobials were used: For Gram-positive Aerobic Isolates

Penicillin (2 units), oxacillin (1 µg), cephalexin (30 µg) doxycycline (30 µg), erythromycin (15 µg), clindamycin (2 µg), linezolid (30 µg), cotrimoxazole (25 µg), vancomycin (30 µg), ofloxacin (5 µg), 10

gatifloxacin (5 µg), amoxycillin/clavulanic acid (20 µg/10 µg), pristinamycin (15 µg), nitrofurantoin (50 µg) and norfloxacin (10 µg).

For Gram-negative Aerobic Isolates

Amoxycillin (10 µg), cephalexin (30 µg), gentamicin (10 µg), amikacin (30 µg), amoxycillin/clavulanic acid (20 µg/10 µg), piperacillin/tazobactam (100 µg/ 10 µg), cefepime (30 µg), ceftazidime/clavulanic acid (30 µg/10 µg), cefoperazone/sulbactam (75 µg/30 µg), cefotaxime (30 µg), ciprofloxacin (5 µg), meropenem (10 µg), aztreonam (30 µg), ceftazidime (30 µg), netilmicin (30 µg), gatifloxacin (5 µg), nitrofurantoin (50 µg) and norfloxacin (10 µg).

For Pseudomonas Species

Ceftazidime (30 µg), gentamicin (10 µg), amikacin (30 µg), piperacillin/tazobactam (100 µg/10 µg), cefepime (30 µg), aztreonam (30 µg), ofloxacin (5 µg), imipenem (10 µg), ceftriaxone (30 µg), netilmicin (30 µg), ceftizoxime (30 µg) and norfloxacin (10 µg).

All the antibiotic discs used were obtained from Hi-Media Laboratories Pvt. Ltd. Results A total of 130 patients were investigated in the present study, out of which 103 (79.2%) were males and 27 (20.8%) were females and the age ranged between 7-80 years (20.8% of patients in age group of 31-40 years). Maximum number of operated patients suffered wound infections (55.4%) followed by infections of urinary tract (28.4%). Infection of respiratory tract and bacteremia was observed in only 10.8% and 5.4% patients, respectively.

Pathogens

A total of 146 isolates were recovered, of which 140 (95.9%) were bacterial and six (4.1%) were of Candida spp. and among the bacterial isolates, Escherichia coli (54.9%) was the commonest one. It was observed that majority of episodes were monomicrobial (85.7%) rather than polymicrobial (14.3%). Most frequently observed nosocomial infections were SSIs (55.4%) followed by infections of urinary tract (28.4%), respiratory tract infections (10.8%) and bacteremia was observed in only 5.4% patients. Most frequently isolated organisms from surgical site were E. coli (58%), Klebsiella pneumoniae (9.9%), S. aureus (8.6%), Enterobacter spp. and P. aeruginosa (6.2% each), Journal of Applied Medicine & Surgery, Vol. 2, Issue 4 April-June 2013

CLINICAL STUDY Acinetobacter baumanii (4.9%), Citrobacter spp. (3.7%) and Proteus vulgaris (2.5%). Among the urine isolates the most common isolate was E. coli (50%) and Candida was the second most common isolate. K. pneumoniae (9.9%) was the most commonly isolated organism from sputum sample (Table 1). The predominant pathogen isolated from polymicrobial episodes were E. coli, S. aureus, K. pneumoniae, A. baumanii and P. aeruginosa, Enterobacter spp., Citrobacter spp. and P. vulgaris were observed only in single episode each.

About 85-100% urinary isolates were also resistant to norfloxacin but resistance against nitrofurantoin was relatively low (35-75%). Isolates of P. aeruginosa showed high level of resistance to cephalosporins Table 1. Site-wise Distribution of Various Isolates in Nosocomial Infections among Surgical Patients Type of isolate

Antimicrobial Susceptibility Pattern

On performing antimicrobial susceptibility testing of GNBs other than P. aeruginosa showed high frequency of resistance to b-lactams, quinolones as well as b-lactam- b-lactamase inhibitor was observed.

Resistance to aztreonam was also found to be very high (90-100%). However, least resistance was observed to carbapenems with maximum resistance being observed in 25% isolates of Enterobacter spp. (Table 2).

Site Surgical

Urine

Respiratory tract

Blood

E. coli

47

22

2

1

K. pneumoniae

8

4

4

1

Enterobacter spp.

5

4

3

-

P. aeruginosa

5

2

-

1

Citrobacter spp.

3

5

2

1

A. baumanii

4

1

3

1

P. vulgaris

2

-

-

-

S. aureus

7

-

-

2

Candida spp.

-

6

-

-

81

44

14

7

Total

Table 2. Resistance Pattern of Gram-negative Bacteria other than P. aeruginosa from Surgical Patients (%age) Bacterial isolates

Am

G

Ak

Cf

Gf

Ac

Cp

Ca

Cps

Ce

Cpm

Pt

Ao

Nt

Mr

E. coli (72)

98.6

95.8

58.3

93.1

88.8

84.7

98.6

91.6

80.5

94.4

91.6

66.6

98.6

93.1

4.2

K. pneumoniae (17)

100

94.1

76.4

88.2

94.1

88.2

100

100

94.1

94.1

82.3

76.4

100

94.1

0

Enterobacter spp. (12)

100

100

91.6

100

91.6

83.3

100

100

83.3

100

83.3

75

100

91.6

25

Citrobacter spp. (11)

100

81.8

72.7

81.8

72.7

81.8

100

72.7

72.7

100

90.9

90.9

90.9

81.8

18.2

Acinetobacter spp. (9)

100

100

100

100

100

100

100

88.8

77.7

100

100

77.7

100

88.8

22.2

P. vulgaris (2)

100

100

50

50

100

0

100

100

100

100

100

100

100

100

0

Am: Amoxycillin; G: Gentamicin; Ak: Amikacin; Cf: Ciprofloxacin; Gf: Gatifloxacin; Ac: Amoxycillin/clavulanic acid; Cp: Cephalexin; Ca: Ceftazidime; Cps: Cefoperazone/Sulbactam; Ce: Cefotaxime; Cpm: Cefepime Pt: Piperacillin/Tazobactam; Ao: Aztreonam; Nt: Netilmicin; Mr: Meropenem.

Table 3. Resistance Pattern of P. aeruginosa Isolates Obtained from Various Clinical Specimens Antibiotics

Pus (n = 5) (n)

Urine (n = 2)

(%)

(n)

(%)

Blood (n = 1) (n)

Total (n = 8)

(%)

(n)

(%)

Ceftazidime

5

100

2

100

0

0

7

87.5

Cefepime

5

100

1

50

1

100

7

87.5

Ceftriaxone

5

100

1

50

1

100

7

87.5

Ceftizoxime

5

100

0

0

1

100

6

75

Gentamicin

5

100

2

100

1

100

8

100

Amikacin

5

100

0

0

1

100

6

75

Netilmicin

5

100

1

50

1

100

7

87.5

Aztreonam

5

100

2

100

1

100

8

100

Imipenem

0

0

0

0

0

0

0

0

Piperacillin/Tazobactam

3

60

1

50

0

0

4

50

Ofloxacin

5

100

1

50

1

100

7

87.5

Journal of Applied Medicine & Surgery, Vol. 2, Issue 4 April-June 2013

11

CLINICAL STUDY and aztreonam. Although, no isolate from urine sample was resistant to amikacin but they were resistant to gentamicin. Imipenem was the most effective drug as no isolate was resistant to it (Table 3).

Among the 18 isolates of S. aureus, 44.4% were resistant to oxacillin and ofloxacin, whereas no isolate was resistant to vancomycin and linezolid.

Discussion Nosocomial infections become prominent in surgical wards because of surgical intervention and operative procedures and furthermore hospitals particularly acute care units, surgical and medical units are important breeding ground for the development and spread of antibiotic resistant bacteria. A total of 146 isolates were recovered from 130 patients enrolled in the present study over a period of one and a half year; out of which 95.9% were bacterial and 4.1% were fungal.

The incidence of fungal infection was similar to Kamat et al9 but lower than other studies, which may be due to shorter period of stay of our study group in hospital.

Most frequently observed nosocomial infections in the current study were SSIs (55.4%) followed by infections of urinary tract (28.4%), respiratory tract infections (10.8%) and bacteremia, which was observed in only 5.4% patients. The prevalence of SSIs was comparable to Kamat et al,9 who reported prevalence of 46.7% in surgical patients. However, Littaua et al13 reported much lower incidence (2.9%) but they included both surgical and medical admissions in their study group. The protocols for preoperative, operative and postoperative prophylaxis, the pre- and postoperative stay of patients vary in different institution and all these factors have great impact on development of SSIs. Prevalence of UTIs was also comparable to other authors.9,14 However, difference in different types of NIs in different countries may be due to different system employed for epidemiological control of hospital infections. The cultures of wound swab and urine sample yielded polymicrobial growth in 14.3% cases in our study. Singh et al15 and Moraes et al16 have reported polymicrobial growth in 11.3% and 23.3% cases, respectively.

Gram-negative pathogens dominated the whole spectrum, being isolated in 91.35% of cases.

We believe that this distribution is most likely a consequence of gram-negative bacteria gaining 12

foothold in nosocomial infections in our hospital.

E. coli remained the most common pathogen followed

by K. pneumoniae and S. aureus in SSIs. Littaua et al13 and NNIS report (1984)3 have also reported the similar results. However, Daschner et al17 and Stratchounski et al18 reported S. aureus and P. aeruginosa as the most common pathogen in SSIs, respectively. The difference in findings can be explained by the fact that the study group of these authors comprised of intensive care unit (ICU) patients while patients enrolled in our study were admitted in surgical ward. Breaking the skin barriers during surgery or biopsy allows the normal flora of skin like S. aureus to proliferate and moreover surgery involving gastrointestinal tract often gives chance to microflora of gut to cause SSIs. The most frequent isolate among patients of UTI was E. coli (50%) and Candida was the second most common isolate. Catheterization of patients following major surgeries is a common practice and is responsible for causing UTI in most cases. Laupland et al19 and Hsueh et al20 have also reported similar results. In our study, among the cases of pneumonia, K. pneumoniae (9.9%) was the most commonly isolated organism but some workers have found P. aeruginosa as most common isolate among pneumonia patients.

In our setup, P. aeruginosa is more commonly isolated from ICU patients than from wards. Antimicrobial resistance pattern of GNBs showed high level of resistance to quinolones, gentamicin and cephalosporins.

High level of resistance to cephalosporins suggests that resistance observed was mainly due to production of b-lactamases. However, resistance to amikacin was relatively less viz. 57.97% and carbapenems remained the most effective drug against gramnegative organisms. All the strains of P. aeruginosa were sensitive to imipenem but 60% strains showed resistance to piperacillin-tazobactam. Shenoy et al also reported that all strains of P. aeruginosa in their study were sensitive to imipenem and meropenem.21 Domingo et al also found imipenem as most effective drug against P. aeruginosa.22 Looking at the oxacillin resistance among S. aureus, it must be assumed in our setup that almost 40-50% strains are likely to be methicillin-resistant S. aureus (MRSA).

These data reveal high level resistance to b-lactams among gram-negative and emerging MRSA. These observations are alarming since virtually all the patients are prescribed first- or second-generation cephalosporins as prophylaxis before surgery. Linezolid and vancomycin were the most effective drugs against S. aureus and resistance to these drugs is also soon Journal of Applied Medicine & Surgery, Vol. 2, Issue 4 April-June 2013

CLINICAL STUDY expected. On observing the multidrug resistance (MDR), it was found that all the bacterial isolates were MDR. This finding is of great concern because if the situation remains the same we will be left with no therapeutic options in the future.

Conclusion Despite aggressive efforts to limit the rapid rise of antimicrobial resistance, the problem of developing resistance to multiple antibiotics continues to worsen as demonstrated by various studies including the present study. However, the current situation is the result of ineffective infection control measures and antibiotic policies. So for proper management of critically ill patients and patients undergoing various operative procedures and other medical interventions, hospital antibiotic policies need frequent revisions.

Hospital wide antibiograms may mask unit-specific susceptibility pattern. These unit-specific antibiograms may help surgeon in selection of empirical therapy in surgical patients.

References 1. Emori TG, Gaynes RP. An overview of nosocomial infections, including the role of the microbiology laboratory. Clin Microbiol Rev 1993;6(4):428-42.

2. Saranya NK. Nosocomial infections. Available at: medscape.com/viewarticle/535488. Accessed on 2009.

3. Dellinger EP. Nosocomial infection: Discussion. Available at: http://www.medscape.com/viewarticle/535488.

Accessed on 2009.

4. Malangoni MA. Surgical site infections: the cutting edge.

Infect Dis Clin Pract 2001;10(6):319-23.

5. Nichols RL. Preventing surgical site infections: a surgeonâ&#x20AC;&#x2122;s perspective. Emerg Infect Dis 2001;7(2):220-4.

6. Emmerson AM, Enstone JE, Griffin M, Kelsey MC, Smyth ET. The Second National Prevalence Survey of infection in hospitals - overview of the results. J Hosp Infect 1996;32(3):175-90.

10. Struelens MJ. The epidemiology of antimicrobial resistance in hospital acquired infections: problems and possible solutions. BMJ 1998;317(7159):652-4.

11. Collee JG, Miles RS, Watt B. Tests for identification of bacteria. In: Mackie and McCartney Practical Medical Microbiology. 14th edition, Collee JG, Fraser AG, Marmion BP, Simmons A (Eds.), Churchill Livingstone: New York 1996:p.131-49, 166-7.

12. Bauer AW, Kirby WM, Sherris JC, Turck M. Antibiotic susceptibility testing by a standardized single disk method. Am J Clin Pathol 1966;45(4):493-6.

13. Littaua R, Tupasi T. Nosocomial infections at the Makati Medical Center: a prospective study and analysis of risk factors. Phil J Microbiol Infect Dis 1986;15(l):l-6.

14. National Nosocomial Infections Surveillance (NNIS) report, data summary from October 1986-April 1997, issued May 1997. A report from the NNIS System. Am J Infect Control 1997;25(6):477-87.

15. Singh AK, Sen MR, Anupurba S, Bhattacharya P. Antibiotic sensitivity pattern of the bacteria isolated from nosocomial infections in ICU. J Commun Dis 2002;34(4):257-63.

16. Moraes BA, Cravo CA, Loureiro MM, Solari CA, Asensi MD. Epidemiological analysis of bacterial strains involved in hospital infection in a university hospital from Brazil.

Rev Inst Med Trop Sao Paulo 2000;42(4):201-7.

17. Daschner FD, Frey P, Wolff G, Baumann PC, Suter P. Nosocomial infections in intensive care wards: a multicenter prospective study. Intensive Care Med 1982;8(1):5-9.

18. Stratchounski LS, Kozlov RS, Rechedko GK, Stetsiouk OU, Chavrikova EP; Russian NPRS Study Group.

Antimicrobial resistance patterns among aerobic gramnegative bacilli isolated from patients in intensive care units: results of a multicenter study in Russia. Clin Microbiol Infect 1998;4(9):497-507.

19. Laupland KB, Bagshaw SM, Gregson DB, Kirkpatrick AW, Ross T, Church DL. Intensive care unit-acquired urinary tract infections in a regional critical care system.

Crit Care 2005;9(2):R60-5.

7. Basa GM. An epidemiologic and clinical investigation of nosocomial infection at the Cebu (Velez) General Hospital. Phil J lnt Med 1984;22:254-61.

20. Hsueh PR, Chen ML, Sun CC, Chen WH, Pan HJ, Yang LS, et al. Antimicrobial drug resistance in pathogens causing nosocomial infections at a university hospital in Taiwan, 1981-1999. Emerg Infect Dis 2002;8(1):63-8.

8. Richards MJ, Edwards JR, Culver DH, Gaynes RP. Nosocomial infections in medical intensive care units in the United States. National Nosocomial Infections Surveillance System. Crit Care Med 1999;27(5):887-92.

21. Shenoy S, Baliga S, Saldanha DR, Prashanth HV. Antibiotic sensitivity patterns of Pseudomonas aeruginosa strains isolated from various clinical specimens. Indian J Med Sci 2002;56(9):427-30.

9. Kamat U, Ferreira A, Savio R, Motghare D. Antimicrobial resistance among nosocomial isolates in a teaching hospital in Goa. Indian J Community Med 2008;33(2):89-92.

22. Domingo KB, Mendoza MT, Torres TT. Catheterrelated urinary tract infections: incidence, risk factors and microbiological profile. Phil J Microbial Infect Dis 1999;8(4):133-8.

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13

CASE REPORT

Gastric Rupture Following Blunt Trauma Abdomen Ritesh Maheshwari*, Ashutosh Sayana**, Pankaj Mahesh†

ABSTRACT Blunt trauma abdomen is a common sequel to road traffic accident. The variety of intra-abdominal injuries following blunt trauma abdomen is diverse. We report a stomach laceration following a trivial trauma to abdomen in a otherwise healthy young male.

Key words: Blunt trauma abdomen, road traffic accident, intra-abdominal injuries

G

lobally, injury accounts for 10% of all deaths.

It is estimated that by 2020, 8.4 million people will die per year from injury. Injuries from traffic collisions will be the third most common cause of disability worldwide and second most common cause in the developing world.1 Blunt trauma abdomen is a common sequel to road traffic accidents and may cause various types of injuries ranging from solid organs to hollow viscera. We report a case of gastric rupture, which occurred following trivial trauma to abdomen.

Grading of Gastric Injuries Grade I

II

Hematoma Contusion/Hematoma without devascularization Laceration

Partial thickness

Laceration

<2 cm in GE junction/pylorus <5 cm in proximal one-third <10 cm in distal two-third

III

Laceration

≥5 cm in proximal one-third

Case Report A 20-year-old male, laborer by occupation, presented with pain and progressive distension of abdomen, hematemesis (1 episode) following cycle handle bar injury on collision with scooter about six hours prior to presentation. Patient was conscious, well-oriented to time, place and person. He had tachycardia with systolic blood pressure (BP) of 90 mmHg and Glasgow coma scale of 15. There were no apparent external injuries except a small bruise in epigastrium. Abdomen was tense and tender with obliteration of liver dullness and absent bowel sounds.

*Senior Resident **Associate Professor Dept. of Surgery, Government Medical College, Haldwani, Uttranchal † Consultant Radiologist Parakh Imaging Centre, Haldwani, Uttranchal Address for correspondence Dr Ritesh Maheshwari Senior Resident, Dept. of Surgery, Government Medical College Rampur Road Haldwani, Uttranchal E-mail: rsm1819@rediffmail.com

14

>2 cm in GE junction/pylorus ≥10 cm in distal two-third

IV Vascular

Tissue loss/Devascularization ≤two-third stomach

V

Tissue loss/Devascularization ≤two-third stomach

Vascular

Patient was initially stabilized and chest X-ray revealed gas under diaphragm; sonography showed free fluid with internal echoes. Patient was taken up for laparotomy and was found to have serosanguineous and bilious fluid with undigested food particles, full thickness stomach laceration approximately 4 cm proximal to pylorus involving nearly two-thirds of circumference and small hematoma of transverse mesocolon. Distal gastrectomy with double layer closure of duodenal stump with anastomosis of proximal stump to jejunum (end to side) was done. Hematoma of transverse mesocolon required no intervention. Postoperatively patient progressed well and was discharged on 10th postoperative day. Histopathologic study of the resected specimen revealed few neutrophilic infiltrates suggestive of acute inflammation.

Journal of Applied Medicine & Surgery, Vol. 2, Issue 4 April-June 2013

CASE REPORT usual state of relative emptiness.2 When the stomach is distended, as by a recent meal, blunt trauma to the upper abdomen can lead to an increase in intragastric pressure sufficient to cause rupture.

A history of a recent meal prior to gastric rupture is a common theme related to this injury; this signifies the importance of gastric distension as a predisposing factor to rupture.

Blunt gastric rupture can occur in any portion of the stomach. It usually occurs as a single lesion, which is commonly debrided and repaired by primary closure.

The site most commonly affected is the anterior wall (40%), followed by the greater curvature (23%), the lesser curvature (15%) and the posterior wall (15%).

Figure 1. Full thickness stomach laceration seen on laparotomy.

Discussion Blunt abdominal trauma usually results from motor vehicle collisions, assaults, recreational accidents or falls. The most commonly injured organs are the spleen, liver, retroperitoneum, small bowel, kidneys, bladder, colorectum, diaphragm and pancreas. Physical examination findings are notoriously unreliable for several reasons; few examples are the presence of distracting injuries, an altered mental state and drug and alcohol intoxication in the patient. Men tend to be affected slightly more often than women.

Blunt force injuries to the abdomen can generally be explained by three mechanisms. The first is when rapid deceleration causes differential movement among adjacent structures. As a result, shear forces are created and cause hollow, solid, visceral organs and vascular pedicles to tear, especially at relatively fixed points of attachment. The second is when intra-abdominal contents are crushed between the anterior abdominal wall and the vertebral column or posterior thoracic cage.

This produces a crushing effect, to which solid viscera (e.g., spleen, liver, kidneys) are especially vulnerable.

The third is external compression forces that result in a sudden and dramatic rise in intra-abdominal pressure and culminate in rupture of a hollow viscous organ.

Isolated gastric injury in blunt trauma abdomen is rarest of rare injury. The rarity of gastric rupture from blunt trauma is thought to be related to the stomachâ&#x20AC;&#x2122;s relatively protected anatomical position, high-degree of mobility, thick muscular wall and Journal of Applied Medicine & Surgery, Vol. 2, Issue 4 April-June 2013

In our review of the literature, only two other cases have been reported where a patient was found to have sustained two separate gastric perforations from blunt trauma.3Â Furthermore, to our knowledge, there has been no reported case of a patient requiring a partial gastrectomy because of the extent of the gastric damage. Although patient had a full stomach prior to the injury, the patient was not found to have any predisposing weakness of the gastric wall that may be seen with ulcer disease, gastric cancer or previous gastric surgery.

Associated injuries are frequently seen in cases of gastric rupture. Splenic injury is generally the most common associated injury; thoracic injury is the second most common. The majority of complications related to gastric rupture are septic in nature.4Â The mortality associated with gastric rupture has been reported to range from 0 to 66%.2-6 References 1. Murray CJ, Lopez AD. Regional patterns of disabilityfree life expectancy and disability-adjusted life expectancy: global Burden of Disease Study. Lancet 1997;349(9062):1347-52.

2. Yajko RD, Seydel F, Trimble C. Rupture of the stomach from blunt abdominal trauma. J Trauma 1975;15(3): 177-83.

3. Semel L, Frittelli G. Gastric rupture from blunt abdominal trauma. N Y State J Med 1981;81(6):938-9.

4. Brunsting LA, Morton JH. Gastric rupture from blunt abdominal trauma. J Trauma 1987;27(8):887-91.

5. Courcy PA, Soderstrom C, Brotman S. Gastric rupture from blunt trauma. A plea for minimal diagnostics and early surgery. Am Surg 1984;50(8):424-7.

6. Richardson G, Schiller WR, Shuck J. Gastric rupture from blunt trauma. Rocky Mt Med J 1979;76(6):309-10.

15

CASE REPORT

Post-coital Generalized Pruritus as a First Symptom of Polycythemia Vera: A Very Rare Case OP Patidar*, Rekha Patidar*, RP Patidarâ&#x20AC;

ABSTRACT Polycythemia vera (PV) is most common of chronic myeloproliferative disorder that involves the multipotent hemaotopoietic progenitor cells. PV has indolent course and recognized either by incidental discovery of high hemoglobin or hematocrit.

PV may present with aquagenic pruritus (AP) for years together without any other sign and symptoms. So, advice of simple complete blood count (CBC) as a routine in every case of pruritus can be helpful to diagnose it timely there by dreaded complications of PV, related to hyperviscosity of blood like thrombosis both arterial and venous can be managed antecedently.

A 50-year-old male doctor diagnosed as a case of PV presented to us with post-coital generalized pruritus (PCP) as a first, rarest symptoms and he remained undiagnosed for 10 years till he developed other features of PV like aquagenic pruritus, headache red congestion in eyes and erythromelalgia symptoms complex erythema, burning pain and warmness of lower extremities.

Then he was investigated and found high hemoglobin or hematocrit, JAK2 genetic mutation changes were present, bone marrow biopsy and other biochemical investigations confirmed the diagnosis of PV. Initially, he was managed with repeated phlebotomy to bring down high hematocrit value in acceptable range (approximately 45%). Simultaneously, he was put on hydroxyurea 500 mg b.i.d. Since then, his symptoms improved and monthly CBC is done to monitor the hematocrit. So, advice of simple CBC is highly informative in every case of generalized pruritus.

Key words: Polycythemia vera, post-coital generalized pruritus, aquagenic pruritus, JAK2V617F genetic mutation, serum erythropoietin level

P

olycythemia vera (PV) also know as erythremia, or primary polycythemia is an acquired myeloproliferative disorder that causes overproduction of all three hemaotopoietic cell line, most prominently red blood cells. The hematocrit is elevated when values exceeds 54% males and 51% in females. The true erythrocytosis with elevated red blood cell (RBC) mass is distinguished from spurious erythrocytosis caused by a constricted plasma volume. PV is bone marrow disorder characterized by autonomous overproduction of erythroid cells.

Erythroid production is independent of erythropoietin and erythropoietin level is always low or normal in primary polycythemia and high in secondary

*Assistant Professor Dept. of General Medicine **Assistant Professor Dept. of General Surgery â&#x20AC; Senior Resident Dept. of Pediatric Jhalawar Hospital and Medical College Society, Jhalawar, Rajasthan Address for correspondence Dr OP Patidar Gayatri Colony, NH-12 Jhalawar - 326 001, Rajasthan E-mail: dr.oppatidar.patidar@gmail.com

16

polycythemia. PV occurs in all age group (including children), although the incidence increases with age.

One study found the median age at diagnosis to be 60 years, while another study found that the highest incidence was in people aged 70-79 years, and the disease was more common in men than women.

Familial transmission occurs but is infrequent. Patient with PV can be a symptomatic. A classic symptom of PV is itching, particularly after exposure to warm water aquagenic pruritus (AP), especially after taking bath, which may be due to abnormal histamine release1,2 or prostaglandin production.3 Such itching is present approximately 40% of patient with PV. Gouty-arthritis may be present in upto 20% of patient. Peptic ulcer disease is also common in patients with PV, the reason for this are unclear, but may be related to on increased susceptibility to infection with ulcer causing bacterium Helicobacter pylori. Another possible mechanism for the development, for peptic ulcer is increased histamine release and gastric hyperacidity related with PV. A rare but classic symptoms of PV (and the related myeloproliferative disease essential thrombocythemia) is erythromelalgia.4 This is a sudden sever burning pain Journal of Applied Medicine & Surgery, Vol. 2, Issue 4 April-June 2013

CASE REPORT with feelings of warmness in the hands and feet, usually accompanied by a reddish or bluish coloration of skin.

Erythromelalgia is caused by an increased platelet count or increased platelet â&#x20AC;&#x2DC;Stickinessâ&#x20AC;&#x2122; (aggregation), resulting in the formation of tiny blood clots in the vessels of the extremity, it respond rapidly to treatment with aspirin.

Most patient present with symptoms related to expanded blood volume and increased blood viscosity are headache, dizziness, tinnitus, blurred, vision, transient ischemic attacks (TIAs) lack of concentration and fatigue patient may be present with epistaxis. This is probably related to engorgement of mucosal blood vessels in combination with abnormal hemostasis due to qualitative abnormalities in platelet function.

A mutation in JAK2 kinase (V617F), a signaling molecule that makes the erythroid precursors hypersensitive to erythropoietin, has been demonstrated in 95% cases, is almost certainly involved in pathogenesis.5,6 This mutation may be helpful in making a diagnosis or as a target for future therapy. Patient with PV are prone to the development of blood clot (thrombosis). A major thrombotic complication (e.g., heart attack, stroke, deep venous thrombosis or Budd-Chiari syndrome) may some time be the first symptoms or indication that a person has PV. With the exception of AP, no symptom distinguish PV from other causes of erythrocytosis. In every case of generalized pruritus, especially of aquagenic origin, the complete blood count (CBC) must be advised, so, PV can be diagnosed timely and development of dreaded complication may be prevented.

Case Report A 50-year-old male doctor resident of Malwa Plateau in India, after five years of uncomplicated marital life, he developed post-coital generalized pruritus (PCP), it was without wheal and erythema formation and predominantly over lower extremities, use to persist for approximately one hour after the course. This symptom alone persisted for five years for which he took consultation from dermatologist and general physician, clinically they diagnose him as a case of cholinergic urticaria without suggesting him any investigation like complete blood count (CBC), etc. and although he never had wheal and erythema formation and he didnâ&#x20AC;&#x2122;t respond to any treatment, which was being prescribed by them e.g., antihistamines, some body lotion, etc.

Journal of Applied Medicine & Surgery, Vol. 2, Issue 4 April-June 2013

After five years of first complaint of generalized postcoital pruritus he developed AP it was generalized but sparing only hands and face but predominantly over lower extremities and trunk. Initially, AP was started in winter season only but later on persisted for whole years regardless of temperature of water but definitely it was some what more with lukewarm water in winter seasons. It has progressed in 10 years duration upto very severe extent that even exposure to a single drop of water can evoke intense pruritus. This AP use to persist for one hour after taking bath, and it was so intense that after bath he was not able to do his poojapath (pray to God) and ultimately he stopped it.

After 15 years of this initial rarest presenting symptoms PCP he noticed chronic redness in eyes, and painful burning sensation in lower extremities with chronic headache. He had one episode of the true vertigo in his consultation room. He use to have complaints of difficulty in concentrating upon himself on his routine work of consultation of patient and he felt that his mental acuity is affected. During this period he developed mild-to-moderate hypertension and problems related to acid peptic disease and dyspnea on climbing the stairs of his own house, which was not earlier, for dyspnea he took consultation from cardiologist and investigations like ECG, 2D echo, treadmill test (TMT) was done and found to be normal and no any doctor has suggested him a simple test CBC. Two years back he noticed that he is not able to sleep on right lateral aspect and felt an abdominal discomfort in the form that some mass moves down from left to right when he changes his sleep posture from left lateral aspect to right lateral aspect. This problem was increasing progressively, so, he started to sleep always in left lateral position in which he felt comfortable, but this problem along with severe erythromelalgia (a symptom complex erythema, burning pain and warmness of lower extremities) and AP was progressively increased upto very severe extent that they were creating trouble to his daily activity of life and posture of sleep was affected.

Because of abdominal discomfort he underwent USG abdomen and found moderate splenomegaly for which was suggested CBC, peripheral blood film (PBF), MP and other routine investigations in which high hemoglobin (Hb) or hematocrit with increased total leukocyte and platelet count was detected. Then 17

CASE REPORT he took consultation from hematologist, where all relevant investigation of PV, to exclude other cause of splenomegaly and to establish complication of PV was done. Then diagnosis of primary polycythemia (PV) was confirmed by CBC, bone marrow biopsy, plasma erythropoietin level, genetic mutation changes.

He never had any epistaxis and he never developed pruritus during his daily treadmill exercise and during this decade, twice he went to hill stations to enjoy snow fall along with his family, where cold wave was running, there he didn’t felt any aggravation.

zz

Investigation zz

zz

zz

zz

zz

zz

zz

18

CBC: Hb 19 g/dl, hematocrit 60%, white blood cell (WBC) count 14.5 × 109/l, RBC 8.34 × 1012/l, platelet 590 × 109/l, MPV 8.4 bt, erythrocyte sedimentation rate (ESR) 2 mm at end of one hour.

Differential WBC count: Blast 0%, promyelocyte 0%, myelocyte 0%, metamyelocyte 01%, band form 06%, neutrophils 80%, eosinophils 3%, basophils 00%, lymphocytes 08%, monocytes 02%, normoblast 00/100 WBC, platelet count on smear increased. Reticulocyte count 2.0%, MCV 72 b1, MCH 22.7 pg, MCHC 31.6%, RDW 17.8%.

Red cell morphology: Hypochromic, microcytosis, anisocytosis otherwise normal.

Bone marrow examination: Increased cellularity with M:E ratio 8:1, myeloid series: hyperplasia, orderly maturation, no blast, no dysplasia. erythroid series: Normoblastic. Megakaryocyts increased. Plasma cells: 1%. Lymphocyte: 10%.

On iron stain: No iron seen. Impression: Marrow is consistent with PV. Bone marrow biopsy: Section reveals packed markedly hypercellular marrow exhibiting panmyelosis. There is erythroid and myeloid hyperplasia, with a slight left shift in the latter.

Megakaryocyts are significantly increased and clustered many contain hyperlobated nuclei.

Diagnosis: Consistent with chronic myeloproliferative disorder.

V617F mutation in JAK2 gene was detected in the leukocytes. Serum erythropoietin level is 4.4 mIU/ ml normal range 3.7-29.5 mIU/ml. Serum iron studies: Serum iron 35 µg/dl normal range 40-140 µg/dl. Total iron binding capacity 384 µg/dl. normal

zz zz

zz

range 245-400 mg/dl. Transferrin saturation 9.1%, normal range 16.5%. Serum ferritin assay 04 ng/ ml. Normal range in male 328-397 nh/ml. Soluble transferrin receptor (sTFR) 9.3 mg/l. Normal range upto 4 mg/l. Lap score point 280 normal range 40-140. Lap score control 80 normal range 40-140. Saturation of arterial blood SaO2 = 98%.

Vitamin B12 1,680 pg/ml normal range 200950 pr/ml. Red cell folate = 320 mg/ml, normal range 175-75 mg/ml, human immunodeficiency virus (HIV) (1+2) Ab-negative. Hepatitis B surface antigen (HBsAg) negative. Hepatocellular carcinoma (HCC) Ab-negative. Urine examination NAD. Blood group B-positive. Blood sugar - 98 mg/dl, serum urea 18 mg/dl, serum creatinine 0.7 mg/dl, serum cholesterol 197 mg/dl, bilirubin 1.8 mg/dl, serum glutamic oxaloacetic transaminase (SGOT) 200 IU, serum glutamic pyruvic transaminase (SGPT) 31 IU, uric acid 8.6 mg/dl.

X-ray chest - Pa view NAD. USG abdomen with portal Doppler; mild hepatomegaly with moderate splenomegaly 160 mm, portal vein 12 mm. No segment of thrombosis seen. Hepatic veins and upper inferior vena cava (IVC) also shows normal caliber and flow pattern.

Upper gastrointestinal (GI) endoscopy and colonoscopy was normal. 2D echo color Doppler study was normal. TMT: Negative for ischemia.

Treatment

When diagnosis of PV was confirmed immediate repeated phlebotomy was done, approximately 5-7 phlebotomy in a period of one week to bring down hematocrit value upto 45%. Simultaneously, hydroxyurea 500 mg b.i.d., clopidogrel 75 mg o.d., esomeprazole 40 mg o.d., telmisartan 40 mg o.d., was started.

Follow-up

In last two and half years no further phlebotomy is required and his hematocrit is maintained in between 40-45%, monthly CBC is done to monitor Hb or hematocrit level in normal range. According to literature best cerebral flow is maintained on hematocrit 40% level. He is carrying out his doctor professional job with normal mental acuity, which was affected prior to Journal of Applied Medicine & Surgery, Vol. 2, Issue 4 April-June 2013

CASE REPORT treatment. Post-coital generalized pruritus has resolved upto majority of extent, still use to occur infrequently with very less severity. AP is still persisting upto 25% of earlier and he is able to do his routine pooja-path daily. Splenomegaly has regressed and his compliant of abdominal discomfort on lying down position is relived. Uric acid level has become normal without taking uricosuric drug.

also observed, which again favors PV. While decreased serum iron and increased serum iron binding capacity, low transferrin saturation, low ferritin assay, high sTFR (9.3 mg/l), increased LAP score (patientâ&#x20AC;&#x2122;s value 280, control value 80), normal SaO2 - 98%, PBF examination shows microcytic, hypochromic, anisocytosis because of iron deficiency state due to high turnover of bone marrow.

Discussion

Patient is nonsmoker, nonalcoholics, non-COPD (chronic obstructive pulmonary disease), nonresident of high altitude with normal serum erythropoietin level and normal renal function rules out other causes of secondary polycythemia. Patient doesnâ&#x20AC;&#x2122;t have any pruritus or urticaria during his daily treadmill exercise, and his TMT was done twice, during or after TMT he never had pruritus so it excludes exercise related (postcoital) urticaria of cholinergic origin.

Although PV is common of chronic myeloproliferative disorder that usually discovered by incidental detection of high Hb or hematocrit value or splenomegaly on routine examination. Aquagenic generalized pruritus especially following shower or bath (more common with warm water) may be a presenting symptom.

Here in this case post-coital generalized pruritus for one hour was earliest and rarest single manifestation, which was persisted for 10 years then he developed other symptoms and signs of PV. Pruritus was always generalized, predominantly over lower limbs and trunk. There was no local pruritus, which is known and reported by many authors but it is of some local infective origin, here it is not so.

After 10 years of this initial rare manifestation when he developed aquagenic pruritus, splenomegaly and conjunctival congestion in eyes. Then he was investigated. CBC revealed high Hb 19 g/dl, hematocrit 60%, total leukocyte count (TLC) 14,500, increased platelet count (5.9 lac/mm3), JAK2V617F genetic mutation was detected. Bone marrow report consistent with PV and bone marrow biopsy diagnosed as a chronic myeloproliferative disorder. USG abdomen and portal Doppler study shows hepatosplenomegaly but no portal hypertension, no thrombosis in portal and splanchnic flow. Hepatic veins and upper IVC also shows normal caliber and normal flow pattern.

Upper GI endoscopy is normal to rule out any lesion of acid-peptic disease, which is common in PV. Other biochemical investigation e.g., serum erythropoietin level is 4.4 mIU/ml, normal is 3.7-29.5 mIU/ml, this normal level is always diagnostic of primary polycythemia. Vitamin B12 level is strikingly elevated because of increased level of transcobalamine III (secreted by WBC), and raised level of uric acid is Journal of Applied Medicine & Surgery, Vol. 2, Issue 4 April-June 2013

No any his brothers/sisters/and his son is having similar sort of illness. Their CBC was done and found to be normal. So, familial transmission, which is rare is also excluded. In the absence of mutation in JAK2 suggest different diagnosis but however JAK2 mutation has been also found in other myeloproliferative disorder e.g., essential thrombocytosis, where only platelet count will be high while other series of cells will be normal.

Myelofibrosis is also to be distinguished by presence of abnormal RBC morphology, nucleated RBC in PBF. Chronic myeloid leukemia is differentiated by presence of marked deviation of WBC count in routine CBC and PBF examination. Other causes of generalized pruritus in hematological disorder origin should be differentiated.

As the condition canâ&#x20AC;&#x2122;t be cured, treatment focused on treating symptoms and reducing thrombotic complication by reducing erythrocyte levels.

Venesection is typically preformed in people with in PV to bring their hematocrit down below 45 for men or 42 for women. It has been observed that phlebotomy also improved cognitive impairment.

Low-dose aspirin (71-81mg) daily is often prescribed.

It also reduces the risk for various thrombotic complications. Chemotherapy for polycythemia may be used, either for maintenance, or when the rate of blood-lettings required to maintain normal hematocrit is not acceptable, or when there is significant 19

CASE REPORT thrombocytosis or intractable pruritus. This is usually with ‘cytoreductive agent’ (hydroxyurea, also known as hydroxycarbamide). In PV cases with chemotherapy, there is increased risk of the transformation to acute myelogenous leukemia (AML). While hydroxyurea is safer in this aspect, there is still some debate about its long-term safety other therapies include interferon injection, and in cases where secondary thrombocytosis (high platelet count) is present, anagrelide may be prescribed. In the past, injection radioactive isotopes (P32) was used another means to suppress the bone marrow. Such treatment is now avoided due to a high rate of AML transformation.

Bone marrow transplantation are rarely undertaken in PV patient, since this condition is nonfatal if treated and monitored, the benefits rarely outweigh the risk involved in such a procedure. There are certain genetic markers, erlotinib may be an additional treatment option for this condition.7 Selective JAK2 inhibitors are being investigated in vitro and clinical trials.8-10 Till date no any reference is available on different internet website for post-coital generalized pruritus.

Cases may be under reported because of shyness of patient. As a author in this case we wants to suggest that in every case of generalized pruritus especially of aquagenic or post-coital related, simple CBC test must be always done so we can avoid development of dreaded complication of thrombotic or platelet dysfunction origin which are 100% preventable if patient is diagnosed timely and managed properly.

If at all post-coital generalized pruritus is a newer observation in PV cases, it needs further studies to understand exact pathophysiology.

Acknowledgment We are highly thankful to Professor (Dr.) MB Agarwal MD, MNAS, Hematologist and Hemato-Oncologist, Bombay Hospital, Mumbai - 400014 for helping us in establishment of diagnosis.

References 1. Steinman HK, Kobza-Black A, Lotti TM, Brunetti L, Panconesi E, Greaves MW. Polycythaemia rubra vera and water-induced pruritus: blood histamine levels and cutaneous fibrinolytic activity before and after water challenge. Br J Dermatol 1987;116(3):329-33.

2. Jackson N, Burt D, Crocker J, Boughton B. Skin mast cells in polycythaemia vera: relationship to the pathogenesis and treatment of pruritus. Br J Dermatol 1987;116(1):21-9.

3. Fjellner B, Hägermark O. Pruritus in polycythemia vera: treatment with aspirin and possibility of platelet involvement. Acta Derm Venereol 1979;59(6):505-12.

4. van Genderen PJ, Michiels JJ. Erythromelalgia: a pathognomonic microvascular thrombotic complication in essential thrombocythemia and polycythemia vera.

Semin Thromb Hemost 1997;23(4):357-63.

5. Baxter EJ, Scott LM, Campbell PJ, East C, Fourouclas N, Swanton S, et al; Cancer Genome Project. Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders. Lancet 2005;365(9464):1054-61.

6. Levine RL, Wadleigh M, Cools J, Ebert BL, Wernig G, Huntly BJ, et al. Activating mutation in the tyrosine kinase JAK2 in polycythemia vera, essential thrombocythemia, and myeloid metaplasia with myelofibrosis. Cancer Cell 2005;7(4):387-97.

7. Li Z, Xu M, Xing S, Ho WT, Ishii T, Li Q, et al. Erlotinib effectively inhibits JAK2V617F activity and polycythemia vera cell growth. J Biol Chem 2007;282(6):3428-32.

8. Verstovsek S, Manshouri T, Quintás-Cardama A, Harris D, Cortes J, Giles FJ, et al. WP1066, a novel JAK2 inhibitor, suppresses proliferation and induces apoptosis in erythroid human cells carrying the JAK2 V617F mutation. Clin Cancer Res 2008;14(3):788-96.

9. Wernig G, Kharas MG, Okabe R, Moore SA, Leeman DS, Cullen DE, et al. Efficacy of TG101348, a selective JAK2 inhibitor, in treatment of a murine model of JAK2V617F-induced polycythemia vera. Cancer Cell 2008;13(4):311-20.

10. Geron I, Abrahamsson AE, Barroga CF, Kavalerchik E, Gotlib J, Hood JD, et al. Selective inhibition of JAK2driven erythroid differentiation of polycythemia vera progenitors. Cancer Cell 2008;13(4):321-30.

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CASE REPORT

Scalpel Cautery Adenotonsillectomy Done in a Case of Crouzonâ&#x20AC;&#x2122;s Syndrome Sudhir M Naik*, Sarika S Naik**

ABSTRACT Background/Objectives: Crouzon syndrome is characterized by premature closure of the cranial sutures, midface hypoplasia, orbital deformities and other associated abnormalities. Children with Crouzon syndrome frequently have obstructive sleep apnea due to the underdevelopment of the midface. Setting: Dept. of ENT, Head and Neck Surgery and Anesthesia, KVG Medical College, Sullia. Case report: A 12-year-old boy of Crouzon syndrome with chronic adenotonsillitis was managed by adenotonsillectomy under general anesthesia by scalpel cautery method. The boy responded well to surgery and the mild sleep disorder disappeared within a week uneventfully. Conclusion: Sleep disorders in this condition can be treated by improving the airway by selective procedures like midface advancement, mandibular expansion, adenotonsillectomy, uvulopalatopharyngoplasty, anterior tongue reduction and endoscopic tracheal granuloma excision.

Key words: Crouzon syndrome, adenotonsillectomy, craniosynostosis, proptosis

C

rouzon syndrome is a rare autosomal dominant skeletal disorder caused by multiple mutations in the fibroblast growth factor receptor 2 (FGFR-2) gene.1 Paternal age older than 35 years is associated with the 60% of all new mutations leading to this condition.1 It occurs in one of every 25,000 live births and accounts for 5% of cases of craniosynostosis.1 Crouzon syndrome has two variants. The classic variant is caused by a mutation of the FGFR2 gene, located on chromosome 10 and the second variant, notable for the presence of abnormal skin pigmentation called acanthosis nigricans, is the result of a mutation in FGFR-3, located on chromosome 4.2 Nucleotide alterations causing amino acid substitutions at the FGFR-2 gene on chromosome 10 lead to the Crouzon phenotype.1 It is commonly inherited as an autosomal dominant trait, with complete penetrance and variable expressivity,3 but 30-60% of cases are sporadic and represent fresh mutations.4 It accounts for approximately 4.8% of all cases of craniosynostosis, with a prevalence of approximately 1/25,000 live births worldwide.3,5 Clinical findings include brachycephalic

* Dept. of ENT, Head and Neck Surgery **Dept. of Anesthesia KVG Medical College, Sullia, Karnataka

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craniosynostosis, significant hypertelorism, proptosis, maxillary hypoplasia, beaked nose and possibly, cleft palate. Intracranial anomalies include hydrocephalus, Chiari I malformation and hindbrain herniation.1 It is characterized by premature closure of the cranial sutures, midface hypoplasia, orbital deformities and other abnormalities.3 The diagnosis is based on clinical findings and radiological examination.3 Early recognition and specific therapeutic interventions are essential to guide the growth and development of the craniofacial region.3 Pathology of the ear and cervical spine is common.2 Infants with Crouzon syndrome do not have anomalies of the hands and feet as do infants with Apertâ&#x20AC;&#x2122;s syndrome.2 The abnormal skull shape is usually noted in the newborn period although occasionally, it may be detected either prenatally or not until later in infancy.

The appearance of an infant with Crouzon syndrome can vary in severity depending on the order and rate of the fusion of the sutures.3 Despite tremendous advances in the establishment of inheritance mode of this condition, prevention and treatment, it remains a significant cause of morbidity worldwide.3 It is important to be aware of the syndrome so as to impart better care.3 Genetic testing and individual study of each patient with suspected syndrome are essential for early diagnosis and management and to differentiate the condition from other syndromic and nonsyndromic craniosynostosis.3 21

CASE REPORT We report a rare case of Crouzonâ&#x20AC;&#x2122;s syndrome in a 12-year-old boy with characteristic features of maxillary hypoplasia, proptosis with minimal cranial deformity and chronic adenotonsillitis.

Case report A 12-year-old boy of normal intelligence presented to the Dept. of ENT, Head and Neck Surgery, KVG Medical College, Sullia with history of sore throat and difficulty in swallowing since one week with similar episodes since three years. No difficulty in speech but occasional difficulty in swallowing due to enlarged tonsils was reported. He had one elder brother with no features of the disease. None of the parents, or other family members had similar facial features.

At the time of his birth, father was 37 years and his mother 29-year-old and they had a nonconsanguineous marriage. History from his parents revealed that his facial features were different from other children of his age. They had noticed the gradual bulging of his eyes after the age of one year. Parents complained of chronic mouth breathing and snoring while sleeping with chronic de-nasal speech.

There was no family history of similar symptoms or any other congenital abnormality. His developmental milestones were normal, but he appeared smaller than other children of his age. Clinical examination showed the cranial vault was regular with no undue prominence. No brachycephaly, flatness of forehead or occiput was seen. The frontal hairline was not low-set.

No ridging of the skull was seen in regions of coronal, lambdoid sutures and anterior to the vertex. Midface flattening with maxillary hypoplasia and a relatively large mandible was seen. The upper lip was small compared to the everted lower lip.

Ophthalmologic evaluation revealed shallow orbits, hypertelorism, bilateral proptosis, mild exotropia, no papillary edema or optic atrophy. His nasal bridge was normal with right septal deviation. His nose was slightly pointed with dorsal hump with normally set ears with normal hearing. The hands and feet were normal with no other abnormality seen. Oral cavity showed U-shaped dental arches with a high-arched palate with hard and soft palates being normal with normal oral hygiene. His dentition was normal with no caries. There was no evidence of temporomandibular dysfunction. Orthopantomographic view confirmed 22

Figure 1. Proptosis and nasal deformity with no maxillary prominence.

Figure 2. Protrusion of the mandible with maxillary flatness.

Figure 3. No deformity of the palate but a large tongue compared to the mouth cavity.

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CASE REPORT

Figure 4. Normal dentition with maxillary hypoplasia.

Figure 7. Normal X-ray of the hand and cranial vault with maxillary hypoplasia.

the clinical findings. A retruded maxilla with a relatively large mandible was seen on X-ray lateral view of the skull. The widely spaced orbits, hypoplastic maxilla and zygoma are due to early fusion of coronal and lambdoid sutures. Cardiovascular, respiratory and abdominal examinations were normal. Routine hematological and biochemical tests showed hemoglobin (Hb) 12.8 g/dl, erythrocyte sedimentation rate (ESR) 10 mm/hour and total leukocyte count (TLC) 7,400 cells/mm3 with polymorphs 57%, lymphocytes 36% and eosinophils 7%. Blood group was O-positive; urine tests were within normal limits.

Figure 5. Normal chest X-ray with maxillary hypoplasia.

Based on the above clinical and radiological findings and in the absence of hand and feet anomalies, a diagnosis of Crouzon syndrome was made. Cardiovascular and respiratory systems were normal on pre-anesthetic examination. He was accepted for adenotonsillectomy in ASA II under general anesthesia. The surgery was done in 45 minutes under scalpel cautery method.

Discussion

Figure 6. Normal cranial vault and ear with maxillary hypoplasia.

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Crouzon syndrome was first described by Octave Crouzon in a mother and son with the characteristic triad of calvarial deformities, facial anomalies and proptosis.6 Mutation of the FGFR gene is also responsible for other craniosynostosis such as Apert’s, Pfeiffer’s, Jackson-Weiss and Saether-Chotzen’s syndromes.7 Rarely, acanthosis nigricans may coexist with Crouzon syndrome and is caused by mutation in the transmembrane region of the FGFR-3 gene.8 The sporadic cases are postulated to be associated with 23

CASE REPORT advanced paternal age; some investigators have found that this mutation is more common in the sperm of older men.9

headache and convulsions are also seen.11 A thorough clinical, radiological and genetic analysis is required for early recognition and diagnose of this syndrome.11

Due to variability of phenotypic expression, some patients exhibit complete phenotypic penetrance, whereas other family members may appear normal and still carry a Crouzon mutation as seen in our patient.10 Crouzon’s syndrome has no racial or sex predilections.10 The craniosynostosis of sagittal or metopic types are more predominance in boys, while coronal craniostenosis is more common in girls. The condition is usually detected in the first year of life and sometimes congenital forms are seen at birth due to synostosis in the uterus itself.11

X-rays show obliterated sutures, hypoplastic maxilla with shallow orbits, shortened cranial fossa, enlarged hypophyseal cavity and small paranasal sinuses.13 Prominent cranial markings of the inner surface of the cranial vault may be seen as multiple radiolucencies appearing as depressions (so called digital impressions) resulting in the hammered silver (beaten metal/copper beaten) appearance.13

The coronal sutures usually close first and eventually all cranial sutures close early. The coronal and sagittal sutures are most commonly involved. As the skull grows in planes perpendicular to the cranial sutures, premature suture closure causes skull growth to cease in the plane perpendicular to the closed suture and to proceed parallel to the suture.11 The skull shape becomes asymmetric, with the shape depending on the sutures involved. The characteristic cranial shapes are brachycephaly (disproportionate shortness of the head), scaphocephaly (boat-shaped head), trigonocephaly (triangle-shaped head) or, in severe disease, cloverleaf skull deformity.12 The facial malformations consist of hypoplasia of the maxilla with mandibular prognathism, high-arched palate, bilateral palatal swelling, rarely cleft palate, exaggerated facial angle; the nose appears prominent and pointed (beak-like nose), recalling a ‘parrot beak’ due to short and narrow maxilla.13 Conductive hearing loss and ear infections are common due to middle ear deformities.11 Upper airway obstruction may occur due to midfacial hypoplasia and a narrow epipharynx.11 Ocular abnormalities such as bilateral ocular proptosis due to extremely shallow orbits and hypertelorism due to decrease in the growth of sphenozygomatic and sphenotemporal sutures are seen.14 Optic atrophy is frequently seen in 30-80% of patients. Other ocular abnormalities include divergent strabismus, conjunctivitis or exposure keratoconjunctivitis, as well as unexplained loss of visual accuracy.15 Mental ability and psychomotor development is generally within the normal limits. However, increased intracranial pressure can lead to mental retardation.11 Sometimes 24

A copper beaten skull indicating internal remodeling of the calvaria due to an increase in intracranial pressure as a result of premature cranial suture fusion may be seen.11 On spine radiography, abnormal craniocervical junction, butterfly-shaped vertebrae and fusion of the cervical vertebrae (C2-C3 and C5-C6) may be visible.11 X-ray examination of the metacarpal bones and fingers may reveal slight achondroplasia.13 Ventriculomegaly is frequently noticed in central nervous system imaging, but is usually asymptomatic and does not require treatment.16 Corpus callosum agenesis and optic atrophy are rarely seen on magnetic resonance imaging (MRI).16 Three-dimensional ultrasonography and MRI aid early detection and diagnosis of fetal malformation.17 Prenatal diagnostic testing for a known FGFR gene mutation is a reasonable option for couples at risk for having a child with Crouzon syndrome due to germline mosaicism.17 The syndrome must be differentiated from simple craniosynostosis and other syndromic craniosynostosis.18 Once craniosynostosis is seen radiographically, it is important to determine whether it occurred because of abnormal biology of the cranial suture, possibly caused by an FGFR mutation (primary craniosynostosis).18 The features of Apert’s syndrome are very similar but are characterized by craniosynostosis, midface hypoplasia and symmetric syndactyly of the hands and feet.19 Differential diagnosis is also made with the other syndromes associated with features of craniosynostosis such as Pfeiffer’s syndrome, SaethreChotzen’s syndrome and Jackson-Weiss syndrome.19 All these syndromes involve craniofacial abnormalities as well as other abnormalities, including the hands and feet.19 The management requires a multidisciplinary approach to prevent early fusion of craniofacial suture, thus Journal of Applied Medicine & Surgery, Vol. 2, Issue 4 April-June 2013

CASE REPORT minimizing intracranial pressure and secondary craniofacial deformities.19 Early release of the synostotic sutures of the skull allows adequate cranial volume for brain growth.19 Skull reshaping may need to be repeated as the child grows.19 Depending on the severity of the deformity, midfacial advancement and jaw surgery can be done to provide adequate orbital volume and correcting the occlusion for appropriate function.19 The prognosis depends on the severity of the malformation and the timing of intervention.

Patients usually have a normal lifespan.19 In a study of 244 children 20% of these children with syndromes with respiratory distress required tracheostomy.20 Craniofacial synostosis patients (Crouzon, Pfeiffer or Apert syndrome) had the highest rate of tracheotomy (48%), mandibulofacial dysostoses patients (Treacher Collins or Nager syndrome) being the next highest rate (41%) and patients with oculo-auriculo-vertebral sequence were less likely to undergo tracheotomy (22%).20 Children with craniosynostosis rarely required a surgical airway, unless there was marked associated facial dysmorphism (1%).20 The presence of a cleft palate correlated with reduced risk for tracheotomy. We performed adenotonsillectomy to improve the snoring and sleep disorder problem. Other procedures done to improve airway are midface advancement, mandibular expansion, uvulopalatopharyngoplasty, anterior tongue reduction and endoscopic tracheal granuloma excision.20 Conclusions Crouzon syndrome can present with a wide range of clinical features. The affected boy in this case presented with varying degrees of craniosynostosis, ocular proptosis, hypertelorism, hypoplastic maxilla and chronic tonsillitis. The diagnosis is usually based on clinical and radiographic findings of the craniofacial region.

Molecular genetic testing for mutations in the FGFR-2 gene may be useful adjuncts, particularly when prenatal detection in subsequent family members is desired. Sleep disorders in this condition can be treated by improving the airway by selective procedures like midface advancement, mandibular expansion, adenotonsillectomy, uvulopalatopharyngoplasty, anterior tongue reduction and endoscopic tracheal granuloma excision.

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References 1. Corde Mason A, Bentz ML, Losken W. Craniofacial syndromes. In: Atlas of Pediatric Physical Diagnosis.

Zitelli BJ, Davis HW (Eds.), 4th edition, Mosby: St. Louis, 2002:p.803-17.

2. Reardon W, Winter RM, Rutland P, Pulleyn LJ, Jones BM, Malcolm S. Mutations in the fibroblast growth factor receptor 2 gene cause Crouzon syndrome. Nat Genet 1994;8(1):98-103.

3. Bowling EL, Burstein FD. Crouzon syndrome.

Optometry 2006;77(5):217-22.

4. al-Qattan MM, Phillips JH. Clinical features of Crouzon’s syndrome patients with and without a positive family history of Crouzon’s syndrome. J Craniofac Surg 1997;8(1):11-3.

5. Kaur H, Waraich HS, Sharma CM. Crouzon syndrome: a case report and review of literature. Indian J Otolaryng Head Neck Surg 2006;58(4):381-2.

6. Crouzon O. Dysostose cranio-faciale hereditaire. Bull Soc Med Hop Paris 1912;33:545-55.

7. Preising MN, Schindler S, Friedrich M, Wagener H, Golan I, Lorenz B. On the effect of mutations of the fibroblast growth factor receptors as exemplified by three cases of craniosynostoses. Klin Monbl Augenheilkd 2003;220(10):669-81.

8. Meyers GA, Orlow SJ, Munro IR, Przylepa KA, Jabs EW. Fibroblast growth factor receptor 3 (FGFR3) transmembrane mutation in Crouzon syndrome with acanthosis nigricans. Nat Genet 1995;11(4):462-4.

9. Glaser RL, Jiang W, Boyadjiev SA, Tran AK, Zachary AA, Van Maldergem L, et al. Paternal origin of FGFR2 mutations in sporadic cases of Crouzon syndrome and Pfeiffer syndrome. Am J Hum Genet 2000;66(3):768-77.

10. Cohen MM Jr. Crouzon syndrome. In: Craniosynostosis: Diagnosis, Evaluation, and Management. 2nd edition, Cohen MM Jr (Ed.), Oxford University Press: New York 2000:p.361.

11. da Silva DL, Palheta Neto FX, Carneiro SG, et al.

Crouzon’s syndrome: literature review. Intl Arch Otorhinolaryngol, São Paulo 2008;12:436-41.

12. Developmental defects of the oral and maxillofacial region. In: Oral and Maxillofacial Pathology. 2nd edition Neville BW, Damm DD, Allen CM, Bouquet JE (Eds.), Elsevier Publishers 2004:p.40.

13. Shafer WG, Hine MK, Levy BM. A Textbook of Oral Pathology. 5th edition, Elsevier Publishers 2006:p.984-6.

14. Ahmed I, Afzal A. Diagnosis and evaluation of Crouzon syndrome. J Coll Physicians Surg Pak 2009;19(5):318-20.

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CASE REPORT 15. Abdullah AM. A severe form of Crouzons syndrome: clinical and radiological correlation. Saudi J Ophthalmol 2003;17:183-5.

16. Gupta AK, Koley S, Choudhary S, Bhake A, Saoji V, Salodkar A. A rare association of acanthosis nigricans with Crouzon syndrome. Indian J Dermatol Venereol Leprol 2010;76(1):65-7.

17. JaczyĹ&#x201E;ska R, Kutkowska-KaĹşmierczak A, Obersztyn E, Niemiec KT, Leibschang J, Ceran A, et al. Prenatal diagnosis of Crouzon syndrome - actual diagnostic possibilities. Ginekol Pol 2006;77(2):138-45.

18. Tsai FJ, Yang CF, Wu JY, Tsai CH, Lee CC. Mutation analysis of Crouzon syndrome and identification of one novel mutation in Taiwanese patients. Pediatr Int 2001;43(3):263-6.

19. Katzen JT, McCarthy JG. Syndromes involving craniosynostosis and midface hypoplasia. Otolaryngol Clin North Am 2000;33(6):1257-84, vi.

20. Sculerati N, Gottlieb MD, Zimbler MS, Chibbaro PD, McCarthy JG. Airway management in children with major craniofacial anomalies: Laryngoscope 1998;108(12): 1806-12.

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CASE REPORT

Thirty-six Cases of Vocal Polyps Treated by Microflap Phonomicrolaryngeal Surgery Sudhir M Naik*, Sarika S Naik**

ABSTRACT Background/Objective: Vocal fold polyps usually occur on the anterior or middle part of the membranous vocal fold and are the commonest laryngeal pathology requiring surgical removal. Phonomicrosurgery is superior to conventional microlaryngeal techniques in treating vocal polyps as collateral tissue damage and scarring is minimal. Setting: Dept. of ENT, Head and Neck Surgery, KVG Medical College and Hospital, Sullia, Karnataka, India. Material and methods: Thirty-six patients with hoarseness and diagnosed vocal polyps on laryngoscopic examination were included in the study. All other causes of hoarseness and other benign vocal cord lesions were excluded from the study. Results: All the 36 cases (23 males, 13 females) operated had very good improvement in hoarseness and were followed for 3-12 months. No complications and recurrence were reported. Conclusion: The surgical outcome depends on accurate diagnosis of the vocal polyps with comprehensive voice evaluation. Patient selection and counseling, adequate pre- and postoperative care and meticulous microflap technique determine the success of the surgery.

Key words: Vocal polyp, phonomicrosurgery, hoarseness, microflap technique

V

ocal polyps are one of the most common benign lesions of the true vocal folds.1 The incidence of vocal polyps range from 11 to 51% among the benign lesions of the vocal folds.2 Intermittent, severe voice abuse is the most common etiology and incidence are more among men in 3-6th decade.3,4 The exact pathogenesis of vocal polyp formation is not known but voice abuse seems the prime etiology.5,6 Vocal abuse leads to vibratory trauma to the membranous vocal folds which cause disturbance in their microcirculation and damage the superficial vessels inducing vocal fold hemorrhages.5,6 They result in inflammatory reactions which result in malformed neovascularized masses called vocal polyps.5,6 The polyp typically occurs as a unilateral mass at the free edge of the mid-membranous vocal fold, but it may also be bilateral.7 Sometimes the polyps are bilateral and histopathological and stroboscopic examination should be done to differentiate it from vocal nodules.7,8 Small polyps can be managed by conservative voice

* Dept. of ENT, Head and Neck Surgery **Dept. of Anesthesia KVG Medical College, Sullia, Karnataka

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therapy only and moderate- to large-sized polyps should be managed by phonomicrosurgery.4 Also, small polyps unresolved with voice therapy should be treated surgically to restore normal vocal cord appearance and vibratory function.4 We report 36 cases of vocal polyps managed by phonomicrolaryngeal surgery techniques.

Material and Methods This is a retrospective study of 36 cases of vocal polyps managed in Dept. of ENT, Head and Neck Surgery, KVG Medical College and Hospital, Sullia, Karnataka from December 2006 to December 2010.

Thirty-six cases of vocal polyps were managed by phonomicrolaryngeal techniques during the 49 months study period. The patients were screened for hoarseness by indirect laryngoscopic examination and rigid 90째 laryngoscopic examination in the OPD. All other benign lesions were excluded from the study.

All patients were advised phonomicrosurgery, but seven patients wanted to delay the surgery. These seven patients were put on medical line of treatment which included two weeks of tapering doses of prednisolone 10 mg tablets, 20 mg rabeprazole and antihistamines.

All were advised steam inhalation. But, maximal 27

CASE REPORT benefit was not seen with medical line and so later phonosurgery was done in all the 36 patients.

Results All the patients were in the 4-6th decade with the youngest patient being 36-year-old female and the oldest being 71-year-old male. There were 23 males and 13 females in the study group and the average group age was 52 years. Mean duration of hoarseness was 2.5 months and all the 23 males gave history of smoking and voice abuse for a period of more than 10 years.

Most of the patients were manual laborers (29), two school teachers and five housewives. Hoarseness was seen in all cases with half of them having insidious onset

Figure 1. Small vocal polyp on the left anterior one-third of vocal cord.

Figure 3. Phonomicrolaryngeal surgery being done.

28

and the rest faster in onset. The duration of symptoms varied from 2 to 15 months. Twenty-three patients were cigarette or bidi smokers, 11 were occasional alcoholics and four complained of recent upper respiratory tract infection flaring their hoarseness.

All of our patients had unilateral vocal cord lesions.

Size of the polyp varied from small to moderate and most of them were sessile. None of the patients had improvement with voice rest and medical line of therapy. All the patients were operated under general anesthesia with endotracheal intubation. All the postoperative specimen were sent for histopathological examination to rule out malignant changes in the polyps. Polyps were of fibroepithelial variety in 15 cases and were fibrovascular variety in 21 cases on histopathological examination.

Figure 2. Vocal cord after phonomicrosurgery.

Figure 4. Microscope with 200 mm lens with suspension laryngoscope.

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CASE REPORT education among the masses help detect these laryngeal lesions at an earlier stage.1 On histopathological examination, various stages of hyperplasia and atrophy is seen in the epithelium.1,5 Exudate with basophils, hyaline degeneration, angiectatic vessels and fibrosis are seen in the subepithelial tissue.1,5 Vocal abuse and co-incident upper respiratory infections causes vasodilatation which increases capillary permeability and interstitial edema.2,5 The protein rich content in the exudates leads to degeneration and fibrosis.1,5 Figure 5. Instruments used in phonomicrolaryngeal surgery.

Postoperative period was uneventful in all cases and the patients were discharged on the third day. The follow-up was done for 3-12 months and all the patients had phenomenal voice improvement without complications.

Discussion Vocal polyps are usually unilateral pedunculated or sessile outgrowths of different sizes which appear on the anterior two-third of the vocal cords.9,10 The polyp affects glottal closure and causes aperiodic vocalfold vibrations thus producing clinical features like hoarseness, voice breaks and diplophonia.9,10 Vocal overuse, abuse and misuse leads to excessive mechanical stress and trauma in the mid-membranous vocal fold, resulting in inflammation.9,10 Voice abuse causes acute and chronic trauma to the microvasculature of the superficial lamina propria.11 The hyperfunctioning glottis during voice abuse induces shearing stresses in the superficial lamina propria.11 These stress leads to hemorrhagic inflammation and neovascularization.11 Healing leads to remodeling of the superficial layer of the lamina propria which results in the vocal polyp formation.9,10 Tobacco smoking and chewing does not have any documented association with polyp formation.4 Vocal polyps are well-defined benign lesions of the laryngeal mucosa, with typical histological characteristics.1 Dynamic changes in the way of life and diverse unfavorable ecological changes caused by pollution and industrialization have increased the incidence of polyps.1 Increase in level of medical Journal of Applied Medicine & Surgery, Vol. 2, Issue 4 April-June 2013

As the pathogenesis of polyp formation is not clearly understood, two main theories have been proposed.12 One theory suggest that a local increase in pressure is induced on the epithelium by edema and infiltration in the early stages causing a rupture of epithelium with granulation tissue formation.12 This granulation tissue will be later lined by pseudostratified columnar or squamous epithelium leading to polyp formation.12 The other suggests that local pressure on the epithelium may increase the inflammation and allergy resulting in subepithelial mass herniation and polyp formation.12 The polyps are divided into fibrous polyps, telangiectatic polyps and hyaline polyps depending on their microscopic features.13 Molecular changes in the extracellular matrix in the vocal polyps show an up-regulation of gene procollagen 1 which is a marker of new collagen formation.14 Matrix metalloproteinases 12 and 1, responsible for elastin breakdown and enzyme degradation, are downregulated in the polyps.14 The up-regulated expression of fibroprotein in polyps is associated with increased stiffness in the lamina propria on videostroboscopy.14 The patient presents with persistent hoarseness which is insidious in onset, progressive and unremitting in nature with lowered voice intensity and frequency range.4 Pedunculated polyps hanging in the subglottic space may have normal voice but may suddenly have hoarseness if it impacts the glottis.4 The degree of vocal disability varies with the size, site, nature and pedunculation of the polyps.4 The first-line treatment for vocal polyps is behavioral intervention in form of voice rest and medical therapy, 29

CASE REPORT with the goal of reducing tine vibratory trauma underlying and exacerbating these masses.15

minimal tissue damage, minimal scarring in dysphonic patients particularly in voice professionals.

Contributory factors of dysphonia such as laryngopharyngeal reflex and poor vocal hygiene should also be treated.15 Larger polyps and polyps showing no improvement to maximal behavior therapy, voice rest and medical management should be considered for phonomicrosurgery.15

The outcome depends on accurate diagnosis of the vocal polyps with comprehensive voice evaluation.

Patient selection and counseling, adequate pre- and postoperative care and meticulous microflap technique determine the success of the technique.

Small hemorrhagic polyps in their early stages sometimes resolve spontaneously with low-dose steroid therapy and voice rest, although surgical removal is typically required to return the vocal fold to its normal appearance and vibratory functions.3,16 Phonomicrosurgery using suspension microlaryngoscopy and fine microlaryngeal instrumentation under general anesthesia has led to precession surgery with stable magnified surgical field.17 Surgical technique of microflaps is possible as the vocal polyp pathology is limited to the superficial lamina propria only.17 Its a microsurgery to remove pathology without scar formation on the surface of the vocal folds, the principle of which lies in maximal preservation of Layered microstructure of the vocal fold, that is the epithelium and superficial lamina propria.17 It avoids injury to the deeper layers of the lamina propria and stimulates the fibroblasts resulting in minimal scar with proper voice quality.17 Use of videostroboscopy in analyzing the vocal fold oscillations have led to substantial improvements in microlaryngeal vocal fold surgery.17 Phonomicrosurgery using microflaps techniques has revolutionized the voice surgery.17 These procedures are designed to improve aerodynamic efficiency and voice quality by creating a smooth vocal fold edge that is not excavated with overlying flexible epithelium.17 The surgery is a physiological based approach for vocal polyps and has evolved as a result of convergence of microlaryngeal surgery techniques and mucosal wave theory of laryngeal sound production.17 Laser is most commonly used for more vascular and larger polyps.17 The collateral thermal trauma to the delicately loosely arranged elastic tissue of superficial lamina propria outweigh its advantage in smaller polyps.17 Conclusion Phonomicrosurgery has proved its superiority over the conventional microlaryngeal techniques with its 30

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2. Dikkers FG. Nodules, polyps, Reinke edema, metabolic deposits and foreign body granulomas. In: Diseases of the Larynx. Ferlito A (Ed.), Arnold: London 2000:287-93.

3. Bastian RW. Benign vocal fold mucosal disorders. In: Otolaryngology Head and Neck Surg. 3rd edition Cumming CW, Fredrickson JM, Harker LA, Krause CJ, Richardson MA, Schuller DE (Eds.), Mosby: St Louis 1998:2096-129.

4. Benjamin B. Vocal cord polyps. In: Endolaryngeal Surgery. Benjamin B (Ed.), Martin Dunitz: London 1998:237-40.

5. Kleinsasser O. Pathogenesis of vocal cord polyps. Ann Otol Rhinol Laryngol 1982;91(4Pt 1):378-81.

6. Kambic V, Gale M. Epithelial hyperplastic lesions of the larynx. Elsevier, Amsterdam 1995:197-208.

7. Wallis L, Jackson-Menaldi C, Holland W, Giraldo A. Vocal fold nodule vs. vocal fold polyp: answer from surgical pathologist and voice pathologist point of view.

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8. Dikkers FG, Nikkels PG. Benign lesions of the vocal folds: histopathology and phonotrauma. Ann Otol Rhinol Laryngol 1995;104(9 Pt 1):698-703.

9. Titze IR. Mechanical stress in phonation. J Voice 1994;8(2):99-105.

10. Steinberg BM, Abramson AL, Kahn LB, Hirschfield L, Freiberger I. Vocal cord polyps: biochemical and histologic evaluation. Laryngoscope 1985;95(11):1327-31.

11. MilutinoviÄ&#x2020; Z. Phonosurgical treatment of vocal cord polyps. Srp Arh Celok Lek 1996;124(11-12):311-3.

12. Tos M, Mogensen C. Pathogenesis of nasal polyps.

Rhinology 1977;15(2):87-95.

13. Mossallam L, Kotby MN, Ghaly AF, et al.

Histopathological aspects of benign vocal fold lesions associated with dysphonia. In: Vocal Fold Histopathology. Col-Hill Press: San Diego; 1986.

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CASE REPORT 14. Thibeault SL, Gray SD, Li W, Ford CN, Smith ME, Davis RK. Genotypic and phenotypic expression of vocal fold polyps and Reinkeâ&#x20AC;&#x2122;s edema: a preliminary study.

Ann Otol Rhinol Laryngol 2002;111(4):302-9.

16. Sataloff RT. Structural abnormalities of the larynx. In: Professional Voice: The Science and Art of Clinical Care.

2nd edition, Sataloff RT (Ed.), Singular Publishing Group: San Diego 1997:p.509-40.

15. Johns MM. Update on the etiology, diagnosis, and treatment of vocal fold nodules, polyps, and cysts. Curr Opin Otolaryngol Head Neck Surg 2003;11(6):456-61.

17. Kumar P, Murthy S, Kumar R. Phonomicrosurgery for benign vocal fold lesions. Indian J Otolaryngol Head Neck Surg 1970;55(3)184-6.

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CASE REPORT

Wireless Capsule Endoscopy Requiring Surgical Intervention Neha Kantawala*, JS Rajkumar**, S Yuvaraja*, T Perungo†

ABSTRACT Wireless capsule endoscopy is a relatively new diagnostic technique for the detection of small bowel disease. Currently represents an increasingly used method for diagnostic evaluation of the small intestine. It is important for surgeons to be familiar with its uses and complication that may arise. We report an interesting case of spontaneous video capsule entrapment in small bowel Crohn’s disease in which diagnosis was made radiologically.

Key words: Wireless capsule endoscopy, minimally invasive, capsule battery, intestinal strictures

W

ireless capsule endoscopy is a relatively new diagnostic technique for the detection of small bowel disease. This minimally invasive procedure generally poses few risks to the patients and reported complications typically are related to capsule retention. The retrospective analysis of some series indicates that the incidence of capsule retention depends on the indication for the capsule examination 0% in healthy controls, 1.4% in obscure gastrointestinal bleeding, 1.48% in suspected Crohn’s disease, 5-13% in known Crohn’s disease and 21% in suspected small bowel obstruction. We report an interesting case of spontaneous video capsule entrapment in small bowel Crohn’s disease in which diagnosis was made radiologically.

Case Report A 35-year-old woman was evaluated for persistent fever and weight loss in a known case of Crohn’s disease. She had no gastrointestinal symptoms. CT scan of abdomen was normal and colonoscopy revealed terminal ileal Crohn’s disease. Small intestine evaluation was thus pursued and wireless capsule endoscopy was *Consultant Surgeon **Chief Surgeon † Surgical Registrar Dept. of Surgical Gastroenterology and Minimal Access Surgery Lifeline Group of Hospitals, Chennai Address for correspondence Dr JS Rajkumar Chief Surgeon and Chairman Lifeline Multi Speciality Hospital 5/639, Old Mahabalipuram Road Kandanchavadi, Perungudi, Chennai - 600 096

32

performed. Unfortunately, as the endoscopic images revealed, the capsule became lodged in small intestine segment containing stagnant contents and eventually lost battery power. As patient had no symptoms of obstruction, he was called for review after 15 days. An abdominal radiograph showed video capsule in the right lower quadrant. CT of the abdomen revealed that the video capsule was caught in the ileum. As patient had persistent weight loss and persistent fever with capsule retention, it was planned to proceed with diagnostic laparoscopy, which revealed multiple strictures in terminal ileum with capsule retained in proximal stricture. There were five complete strictures in terminal ileum so we decided to proceed with resection with ileotranseverse anastomosis. Postoperatively, the patient recovered well.

Discussion Wireless capsule endoscopy was approved for clinical use in United States in 2001 and currently represents an increasingly used method for diagnostic evaluation of the small intestine. It is important for surgeons to be familiar with its uses and complication that may arise. In our case, CT scan of the abdomen was done to rule out strictures. The endoscopic capsule examination begins when the patient swallows the 1.1 × 2.6 cm, 4 g capsule, which contains a camera, light source, batteries and transmitter. As the capsule transverses the small intestine, it relays images to a recorder wore on a belt around the patient’s waist. The capsule battery has a life span of about eight hours, typically enough time for it to image the entire small bowel as it passes through.

Recorded images are downloaded and analyzed later.

Journal of Applied Medicine & Surgery, Vol. 2, Issue 4 April-June 2013

CASE REPORT Suggested Reading 1. Delaney CP, Fazio VW. Crohnâ&#x20AC;&#x2122;s disease of the small bowel. Surg Clin North Am 2001;81(1):137-58, ix.

2. Iddan G, Meron G, Glukhovsky A, Swain P. Wireless capsule endoscopy. Nature 2000;405(6785):417.

3. Gong F, Swain P, Mills T. Wireless endoscopy.

Gastrointest Endosc 2000;51(6):725-9.

4. Swain P. Wireless capsule endoscopy. Gut 2003;52 Suppl 4:iv48-50.

5. Fritscher-Ravens A, Swain CP. The wireless capsule: new light in the darkness. Dig Dis 2002;20(2):127-33.

6. Bar-Meir S, Bardan E. Wireless capsule endoscopy - pros and cons. Isr Med Assoc J 2002;4(9):726.

Figure 1.

Figure 2.

Most capsules are naturally expelled within 72 hours of ingestion. Current imaging techniques can show long or medium stenosis, with great reduction of lumen size; however, short stenosis usually cannot be detected by standard methods. This fact explains that in most of the reported cases of capsule retention, the previous performance of the usual radiological studies was not capable of diagnosing the intestinal strictures which the capsule clearly showed. It is therefore, proven that the lack of findings in radiological techniques does not rule out the existence of bowel stenosis.

Journal of Applied Medicine & Surgery, Vol. 2, Issue 4 April-June 2013

7. Fireman Z, Glukhovsky A, Jacob H, Lavy A, Lewkowicz S, Scapa E. Wireless capsule endoscopy. Isr Med Assoc J 2002;4(9):717-9.

8. Ge ZZ, Hu YB, Gao YJ, Xiao SD. Clinical application of wireless capsule endoscopy. Zhonghua Xiaohua Zazhi 2003;23:7-10.

9. Ang TL, Fock KM, Ng TM, Teo EK, Tan YL. Clinical utility, safety and tolerability of capsule endoscopy in urban Southeast Asian population. World J Gastroenterol 2003;9(10):2313-6.

10. Appleyard M, Fireman Z, Glukhovsky A, Jacob H, Shreiver R, Kadirkamanathan S, et al. A randomized trial comparing wireless capsule endoscopy with push enteroscopy for the detection of small-bowel lesions.

Gastroenterology 2000;119(6):1431-8.

11. Costamagna G, Shah SK, Riccioni ME, Foschia F, Mutignani M, Perri V, et al. A prospective trial comparing small bowel radiographs and video capsule endoscopy for suspected small bowel disease. Gastroenterology 2002;123(4):999-1005.

12. Bardan E, Nadler M, Chowers Y, Fidder H, Bar-Meir S. Capsule endoscopy for the evaluation of patients with chronic abdominal pain. Endoscopy 2003;35(8):688-9.

13. Schreyer AG, GĂślder S, Seitz J, Herfarth H. New diagnostic avenues in inflammatory bowel diseases.

Capsule endoscopy, magnetic resonance imaging and virtual enteroscopy. Dig Dis 2003;21(2):129-37.

14. Tibble JA, Bjarnason I. Non-invasive investigation of inflammatory bowel disease. World J Gastroenterol 2001;7(4):460-5.

15. Lewis BS. Radiology versus endoscopy of the small bowel. Gastrointest Endosc Clin N Am 1999;9(1):13-27.

16. Chong AK. Comments regarding article comparing small bowel radiographs and video capsule endoscopy.

Gastroenterology 2003;125(1):276.

17. Liangpunsakul S, Chadalawada V, Rex DK, Maglinte D, Lappas J. Wireless capsule endoscopy

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CASE REPORT detects small bowel ulcers in patients with normal results from state of the art enteroclysis. Am J Gastroenterol 2003;98(6):1295-8.

18. MacKenzie J. Push enteroscopy. Gastrointest Endosc Clin N Am 1999;9(1):29-36.

19. Gay GJ, Delmotte JS. Enteroscopy in small intestinal inflammatory diseases. Gastrointest Endosc Clin N Am 1999;9(1):115-23.

20. Seensalu R. The sonde exam. Gastrointest Endosc Clin N Am 1999;9(1):37-59.

21. Delmotte JS, Gay GJ, Houcke PH, Mesnard Y. Intraoperative endoscopy. Gastrointest Endosc Clin N Am 1999;9(1):61-9.

22. Ge ZZ, Xiao SD. Prospects for wireless capsule endoscopy. Wei Chang Bing Xue 2002;7:326-30.

23. Barkin J, Friedman S. Wireless capsule endoscopy (WCE) requiring surgical intervention: The worldâ&#x20AC;&#x2122;s experience. 2nd Conference on Capsule Endoscopy, Berlin 2003:171.

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CASE REPORT

Acute Fatty Liver of Pregnancy with Overlapping HELLP Syndrome Associated with Transient Proximal Myopathy R Vedamanickam*, TKV Sharavanan**, N Dinakaran†, R Ramasubramanian‡

ABSTRACT Acute fatty liver of pregnancy (AFLP) is a specific cause of liver disease in pregnancy of unclear pathogenesis. Clinical syndrome at presentation is usually variable. At times it overlaps with eclampsia of pregnancy and/or Hemolysis, Elevated Liver enzymes, Low Platelets (HELLP) syndrome. If not recognized early, it can result in both maternal and fetal mortality.

Key words: Acute fatty liver of pregnancy, eclampsia, HELLP syndrome, proximal myopathy

A

cute fatty liver of pregnancy (AFLP) is a rare disorder of human gestation which, if

not recognized early, can result in both maternal and fetal mortality. This syndrome is a true form of hepatic failure with coagulopathy and often encephalopathy. Clinical criteria for diagnosis of AFLP include an elevation in the prothrombin time with a low level of fibrinogen in women in the secondhalf of gestation. There may be some overlap of this syndrome with the syndrome of pre-eclampsia and there are some reports of patients with both AFLP and Hemolysis, Elevated Liver enzymes, Low Platelets (HELLP) syndrome.

AFLP with overlapping pre-eclampsia and HELLP syndrome frequently coexist but are less frequently reported. In this article, we present a case of AFLP with coexisting pre-eclampsia and HELLP syndrome.

Early diagnosis and prompt delivery have dramatically improved both maternal and fetal prognosis.

Case History A 21-year-old primigravida with nine months amenorrhea presented with history of nausea,

*Assistant Professor Dept. of Medicine Sri Balaji Medical College and Hospital Chromept, Chennai, Tamil Nadu **Assistant Professor Dept. of Medicine † Professor Dept. of Medical Gastroenterology Melmaruvathur Adhiparasakthi Institute of Medical Sciences and Research, Melmaruvathur, Tamil Nadu ‡ Professor Dept. of Medical Gastroenterology Thoothukudi Medical College and Hospital, Thoothukudi, Tamil Nadu

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exhaustion, giddiness since three days. She had vomited about 150 ml of fresh blood followed by melena at the time of reporting. She was found to have systemic hypertension from 32nd week of pregnancy with albuminuria. On examination, she was drowsy with mild encephalopathy and mild icterus; blood pressure (BP) was 104/60 mmHg with pulse 84/mt.

Per abdominal examination showed full-term gravid uterus; no hepatomegaly.

Investigations showed serum bilirubin 4.7 mg/dl, direct 2.4 mg/dl and indirect 2.3 mg/dl, aspartate aminotransferase (AST) 87 IU/l, alanine aminotransferase (ALT) 65 IU/l, serum alkaline phosphatase (ALP) 207 IU/l. Urine examination showed: Albumin ++, bile salt and bile pigment present and acetone negative.

Hepatitis B surface antigen (HBsAg) was negative, antinuclear antibody and smooth muscle antibody titers were less than 1:40. Hemoglobin (Hb) was 9.8 g/dl, WBC count 9,000 cells/mm3 (P 68%, L 28%, E 4%), erythrocyte sedimentation rate (ESR) 25 mm/hour, PCV 21% and platelet count 2.86 lakhs/ mm3. Ultrasonogram of abdomen showed a full-term fetus with normal liver, gallbladder, extrahepatic biliary radicles, pancreas and spleen. The portal vein, hepatic vein, inferior vena cava (retrohepatic and suprahepatic) were within normal limits. No free fluid was seen in the abdomen.

The patient was put on conservative treatment with parenteral proton pump inhibitor, liquid antacids, lactulose 10 ml orally twice-daily, ursodeoxycholic acid (UDCA) 150 mg b.i.d. and silymarin 70 mg b.i.d. She was given 2 units of fresh blood. Upper gastrointestinal 35

CASE REPORT Blood investigation showed blood sugar 84 mg/dl, serum potassium 4.1 mEq/l, sodium 139 mEq/l, bicarbonate 24 mEq/l, chloride 102 mEq/l, urea 18 mg/dl, creatinine 0.82 mg/dl, serum bilirubin was 3.1 mg/dl, serum total protein 6.1 g/dl, albumin 4.2 g/dl, globulin 1.9 g/dl and creatinine kinase 128 U/l (normal 10-70 U/l).

Figure 1. Histopathology of liver (H&E 400x).

endoscopy showed Grade III esophagitis with moderate erosive pangastritis; no esophageal varices were seen and duodenum appeared normal.

Three days later, the serum bilirubin increased from 4.7 to 12.5 mg/dl, AST increased from 87 to 165 IU/l and ALT from 65 to 124 IU with normal SAP 214 IU/l. Prothrombin time was 28 second (control 12 second), activated partial thromboplastin time (APTT) was 45 second (control 38 second), platelet count 1.15 lakh/mm3 and serum fibrinogen was 150 mg% (normal 200-400 mg%). There was no fresh upper gastrointestinal bleeding. On 5th day of admission, she delivered a stillborn male baby vaginally. On the 6th day, she developed ascites with Grade I encephalopathy.

Hb dropped to 6.8 g/dl. Further investigation revealed HBsAg, anti-HCV were negative. Serology for HAV and HEV was negative.

She had prolonged vaginal bleeding after vaginal delivery.

She was on supportive line of treatment for acute hepatic decompensation along with injection Stronger Neo-Minophagen-C, which contains monoammonium glycyrrhizinate 53 mg, glycine 400 mg and L-cysteine hydrochloride 20 mg/20 ml injection, 60 ml IV given daily. Injection vitamin K 10 mg IV was given daily with 2 units of platelet transfusion.

Patient showed gradual improvement clinically. Icterus gradually subsided. AST levels dropped to 84 IU/l, ALT to 90 IU/l, platelet count increased to 1.58 lakh/mm3.

Ascites subsided gradually. Patient showed gradual improvement in general condition. On 6th day after delivery, patient noticed difficulty in getting up from bed due to weakness of lower limbs and hip joints.

Weakness was not associated with sensory disturbances or bowel or bladder disturbances. These were no signs of cranial nerve involvement. Upper limb movements and higher functions were normal.

36

Neurological examination revealed that patient was alert, conscious and oriented. Optic fundi were normal. Cranial nerves were normal. Motor system examination revealed features of proximal myopathy; sensory systems were normal and there was no muscle tenderness. Nerve conduction velocity studies were normal in ulnar nerve.

Prothrombin time was 14 seconds (control 12 seconds). Liver biopsy was done with Surecut needle.

Supportive treatment continued for another one week.

Jaundice gradually improved and encephalopathy also improved. Liver biopsy showed following histological changes. zz Moderate amount of microvesicular steatosis. zz Minimal ballooning dilatation of hepatocytes. zz Centrilobular liver cell necrosis. zz Mononuclear cell infiltration in the lobule. zz Few areas of hemorrhage in the space of Disse. zz Patient was discharged on 21st day of delivery.

Summary This patient had pregnancy-induced hypertension, jaundice in the third trimester, higher levels of liver enzyme, low platelet count with fatty liver and proximal myopathy clinically: An overlap syndrome of pre-eclampsia with HELLP syndrome with AFLP. This case is presented for its rarity.

Discussion Liver disease in pregnancy can be considered in two categories. The first group consists of all the acute and chronic diseases of the liver that can affect women of childbearing age including viral hepatitis, gallstone disease, drug-induced hepatitis, autoimmune hepatitis and metabolic syndrome. The second group of diseases are particular to pregnancy and comprise hyperemesis gravidarum, benign recurrent cholestasis, AFLP and HELLP syndrome. Table 1 summarizes some of the salient features of liver disease seen during pregnancy.1 Journal of Applied Medicine & Surgery, Vol. 2, Issue 4 April-June 2013

CASE REPORT Table 1. Usual Clinical Features of Liver Disease During Pregnancy1 Viral hepatitis

HELLP

AFLP

Cholestasis

Hyperemesis

Trimester of onset

Any

Third

Third

Second or third

First

Abdominal pain

Rare

Common

Common

No

Possible

No

Common

Can occur

No

No

Usually > 1000

>100

>100

Usually < 300

>100

Jaundice

Common

Common

Common

Unusual

Unusual

Pruritus

Rare

None

None

Always

None

Family history

None

Occasional

Occasional

Often

Not usually

Increased BP Aminotransferase levels (IU)

Recurrence possible Effect on the fetus

No

Yes

Yes

Yes

??

Usually none

Poor

Poor

Poor

?? Good outcome

HELLP = Hemolysis, Elevated Liver enzymes, Low Platelets syndrome; AFLP = Acute fatty liver of pregnancy.

AFLP is not a rare disorder but often needs recognition.

It was first described by Sheehan in the year 1940.

The syndrome usually presents late during the course of pregnancy commonly between 34 and 37 weeks; rarely patient may present after delivery. The incidence of this syndrome ranges from one in 6,692 to one in 10,000 births.2 Subclinical cases or overlap syndromes of AFLP probably exist.3 The course of affected patients are quite variable.4 The pathogenesis of AFLP is still unclear.5,6 Inherited defect in β-oxidation of fatty acid has been seen. Specific defect of long-chain 3-hydroxyacyl coenzyme A dehydrogenase (LCHAD) in the fetus is associated with development of AFLP if the mother is heterozygote. Two mutations (g 1528c and C1132t) have been observed in the gene coding for LCHAD. Male gender of the fetus seems to be a risk factor.

HELLP syndrome is a term that was coined by Weinstein in 1982 and represents the hepatic expression of the preeclampsia syndrome.7,8 HELLP syndrome is regarded as hepatic microangiopathy with fibrin deposition and Abnormal implantation Poor placental perfusion Blood-borne toxins Sensitivity to pressors

Activated endothelial cells

Vasospasm Perfusion

Injury

Figure 2. Mechanism of pathogenesis of pre-eclampsia to HELLP syndrome.

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platelet consumption causing areas of hemorrhage and necrosis causing endothelial damage.9,10 These changes are responsible for hypertension and proteinuria as well as manifestations.

Conclusion AFLP is a rare disorder of human gestation. If not recognized early, it can result in both maternal and fetal mortality. The syndrome usually presents late during the course of pregnancy; male gender of the fetus seems to be a risk factor.11 Recurrence of this syndrome in subsequent pregnancies is rare but has been reported with varying clinical symptoms.12 Diagnosis is often difficult and sometimes requires liver biopsy. The differential diagnoses of patient with AFLP include viral hepatitis and drug-induced hepatitis.

Clinical symptoms at presentation are usually variable and may be nonspecific. However, the pathogenesis of this disorder is still unclear. There are recent reports of infants born to pregnancies complicated by ALFP, who were found to be deficient in one of the mitochondrial enzymes responsible for oxidation of LCHAD coenzyme a dehydrogenase.13,14 References 1. Norman G. The liver and systemic disease, Table 14.1, Churchill Livingstone 1997:p.267.

2. Castro MA, Goodwin TM, Shaw KJ, Ouzounian JG, McGehee WG. Disseminated intravascular coagulation and antithrombin III depression in acute fatty liver of pregnancy. Am J Obstet Gynecol 1996;174(1 Pt 1):211-6.

3. Riely CA, Latham PS, Romero R, Duffy TP. Acute fatty liver of pregnancy. A reassessment based on observations in nine patients. Ann Intern Med 1987;106(5):703-6.

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CASE REPORT 4. Burroughs AK. Liver Disease and Pregnancy. In: Oxford Textbook of Hepatology. In: Bircher J, Benhamou JP, McIntyre M, Risetto M, Rodes J (Eds.) 1991:p.1321-32.

5. Riely CA. Acute fatty liver of pregnancy. Semin Liver Dis 1987;7(1):47-54.

6. Dani R, Mendes GS, Medeiros Jde L, PĂŠret FJ, Nunes A. Study of the liver changes occurring in preeclampsia and their possible pathogenetic connection with acute fatty liver of pregnancy. Am J Gastroenterol 1996;91(2):292-4.

7. Weinstein L. Syndrome of hemolysis, elevated liver enzymes, and low platelet count: a severe consequence of hypertension in pregnancy. Am J Obstet Gynecol 1982;142(2):159-67.

8. Sheila Sherlock, James S. Dooley. Diseases of the Liver and Biliary System. 10th edition, Wiley-Blackwell 1997:p.477.

9. Hay JE. Liver disease in pregnancy. Hepatology 2008;47(3):1067-76.

10. Hepburn IS, Schade RR. Pregnancy-associated liver disorders. Dig Dis Sci 2008;53(9):2334-58.

11. James WH. Sex ratios of offspring and the causes of placental pathology. Hum Reprod 1995;10(6):1403-6.

12.

Barton JR, Sibai BM, Mabie WC, Shanklin DR. Recurrent acute fatty liver of pregnancy. Am J Obstet Gynecol 1990;163(2):534-8.

13. Treem WR, Rinaldo P, Hale DE, Stanley CA, Millington DS, Hyams JS, et al. Acute fatty liver of pregnancy and long-chain 3-hydroxyacyl-coenzyme: a dehydrogenase deficiency. Hepatology 1994;19(2):339-45.

14. Wilcken B, Leung KC, Hammond J, Kamath R, Leonard JV. Pregnancy and fetal long-chain 3-hydroxyacyl coenzyme A dehydrogenase deficiency. Lancet 1993;341(8842):407-8.

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CASE REPORT

Ruptured Pulmonary Hydatid Cyst: The Camalote Sign Manjot Kaur*, Rakendra Singh**

ABSTRACT Echinococcosis or hydatid disease is caused by larvae of the tapeworm Echinococcus. It remains an important health problem, especially in regions with inadequate hygienic environments and poor veterinarian control. Liver is the most common organ of involvement in adults and lungs are commonly involved in younger people. The cysts are characteristically seen as solitary or multiple circumscribed or oval masses on imaging. The characteristic imaging appearance changes with rupture of cyst.

Surgery is the recommended form of treatment. We present a case of ruptured pulmonary hydatid cyst with communicating rupture into the bronchus, which is not very common.

Key words: Hydatid cyst, rupture, computed tomography

T

he vast majority of infestations in humans are caused by Echinococcus granulosus. It causes cystic echinococcosis, which has a worldwide distribution.1 Humans are exposed less frequently to E. multilocularis, which causes alveolar echinococcosis.

The hydatid tapeworm (E. granulosus) requires two hosts to complete its life cycle. Dogs (and other canines) are the definitive hosts and a variety of species of warm-blooded vertebrates (sheep, cattle, goats, horses, pigs, camels and humans) are the intermediate hosts. Humans are accidental hosts and do not play a role in the biological cycle. As two mammalian species are required for completion of the life cycle, direct transmission of echinococcosis from human-to-human does not occur.

Case Report A 70-year-old male came to the Dept. of Medicine of our hospital with complaints of dyspnea, productive cough and pain chest following a mild chest trauma.

He mentioned that his expectoration was salty in taste. On general examination, patient was hemodynamically stable and had mild respiratory distress. Patient was afebrile and had no cyanosis

or pallor. A previous computed tomography (CT) scan done about a month back at outside multislice scanner, showed a well-defined cystic lesion measuring 10.1 × 7.2 cm in size in posterior basal segment of left lower lobe (Fig. 1) and provisional diagnosis of intrapulmonary hydatid cyst was given. On presentation at our institute a CT scan was planned.

CT scanning of the lungs with CT/e scanner (GE; Milwaukee, USA) using 130 mA, 120 kV, 7-s scan time, 512 × 512 matrix, and 7 mm section thickness was done. Anteroposterior (AP) scout film from CT showed a well-defined oval cavitating lesion with air-fluid level in the lower zone of left lung abutting the left dome of diaphragm (Fig. 2). On CT axial sections, a large spherical air-filled cavity with collapsed membranes was seen in the posterior basal segment of left lung (Figs. 3 and 4). Surrounding consolidation was seen. Left pleural effusion was noted. Well-defined cysts were also seen in the liver and gastrophrenic ligament on CT (Fig. 5). Diagnosis of findings suggestive of pulmonary hydatid cyst with communicating rupture into bronchus was given. The Casoni and indirect hemagglutination tests were found to be positive. Diagnosis was confirmed at surgery.

Discussion

*2nd Year Resident **3rd Year Resident † Associate Professor Dept. of Medicine, Jawaharlal Nehru Medical College, Ajmer Address for correspondence Dr Tarachand Saini 20, Gaurav Enclave, Near City Palace, Civil Line, Ajmer, Rajasthan E-mail: drtcsaini20@gmail.com

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Hydatid disease is one of the most important helminthic diseases. The lung is the second most common involved organ, but in children it is the commonest site. The lung may be affected when the liver is bypassed via the lymphatic system.2 Pulmonary 39

CASE REPORT

Figure 1. Axial CT of chest (mediastinal window) showing well-defined cystic lesion in the posterior basal segment of left lung lower lobe.

Figure 2. CT scout film showing a cavitating lesion in the left lower zone abutting the left dome of diaphragm.

Figure 4. Axial CT of chest (mediastinal window) shows a cystic lung lesion (HC) with dependent wavy contour consistent with germinative membranes (GM) - the camalote or the water lily sign. Minimal left pleural effusion seen.

Figure 5. Axial CT of upper abdomen showing a welldefined cystic lesion in the gastrophrenic ligament proven to be hydatid cyst at surgery.

hydatid cyst may rupture into pleural cavity, pericardium or the bronchial tree leading to cough, chest pain and hemoptysis. The patients define this as a salty or peppery water expectoration, indicating spring-water expectoration, and it may even be an expectoration of membrane particles.3

Figure 3. Axial CT of chest (lung window) shows spherical cystic lesion (HC) with air-fluid level and surrounding consolidation in the posterior basal segment of left lung lower lobe.

40

The most frequent complication of pulmonary hydatid disease is the rupture of the cyst into a bronchus, which is also regarded as a complicated cyst.4 The rupture of echinococcal cysts is of three types: Contained, communicating and direct. Contained rupture occurs when only the parasitic endocyst ruptures and the cyst contents are confined within the host-derived pericyst. When cyst contents escape via bronchial or

Journal of Applied Medicine & Surgery, Vol. 2, Issue 4 April-June 2013

CASE REPORT biliary radicles that are incorporated in the pericyst, the rupture is communicating. Direct rupture occurs when both the endocyst and the pericyst tear, spilling cyst contents directly into the peritoneal or pleural cavities or occasionally into other structures.

Communicating and direct forms have more serious clinical implications than contained rupture, but even contained rupture needs prompt surgical attention to prevent it from developing into one of the other forms.5 In our case, a cavity with air-fluid level and collapsed germinative layers consistent with direct rupture of hydatid cyst into left lower bronchus was seen. Surrounding consolidation and mild pleural effusion was likely due to inflammatory response to endobronchial spread of cyst contents as no larvae or membranes could be elucidated in the pleural fluid.

Conventional chest radiography has been the mainstay investigation for diagnosis of hydatid disease of the lungs, often coupled with Casoni skin testing and serologic testing for antibodies. A variety of descriptive have been given to plain film findings if rupture of cyst occurs with the air around or within the endocyst, an air-fluid level and the collapsed, crumpled membranes floating in the fluid.

The ‘camalote or water lily’ sign is described when endocyst membrane float on top of remaining fluid due on collapse of endocyst and partial evacuation of fluid. This was demonstrated in our case.

The other signs named are the ‘meniscus/crescent’ sign, the sign of the ‘rising sun’, the ‘serpent’ sign, the ‘whirl’ sign, the ‘onion peel’ sign and the ‘cumbo’ sign.6-8 Sometimes, after rupture of a cyst into the bronchus, an inflammatory reaction may close the draining bronchus and imprison the membrane (incarcerated membrane). In these cases, the definite regular outline is lost and there may be a blurred shadow that can easily be mistaken for tuberculous focus or carcinoma (Ivanissevich sign).3 CT scan is useful to confirm the diagnosis and it is also able to demonstrate cysts not identified with plain radiographs.8 Apart from the classically described

features of pulmonary hydatid disease, a crescentshaped rim of air at the lower end of the cyst (inverse crescent sign) and a bleb of air in the wall of as-yet unruptured cysts (signet ring sign) have also been described on CT scan.9 Conclusion CT scan helps to elucidate the cystic nature of the lung mass, its accurate localization and evidence of any form of rupture so that surgical treatment can be planned at the first opportunity to avoid potentially fatal complications.

References 1. Kurt Y, Sücüllü I, Filiz AI, Urhan M, Akin ML. Pulmonary echinococcosis mimicking multiple lung metastasis of breast cancer: the role of fluoro-deoxyglucose positron emission tomography. World J Surg Oncol 2008;6:7.

2. Rebhandl W, Turnbull J, Felberbauer FX, Tasci E, Puig S, Auer H, et al. Pulmonary echinococcosis (hydatidosis) in children: results of surgical treatment. Pediatr Pulmonol 1999;27(5):336-40.

3. Xanthakis D, Efthimiadis M, Papadakis G, Primikirios N, Chassapakis G, Roussaki A, et al. Hydatid disease of the chest Report of 91 patients surgically treated. Thorax 1972;27(5): 517-28.

4. Doğan R, Yüksel M, Cetin G, Süzer K, Alp M, Kaya S, et al. Surgical treatment of hydatid cysts of the lung: report on 1055 patients. Thorax 1989;44(3):192-9.

5. Lewall DB, McCorkell SJ. Rupture of echinococcal cysts: diagnosis, classification, and clinical implications.

AJR Am J Roentgenol 1986;146(2):391-4.

6. Pedrosa I, Saíz A, Arrazola J, Ferreirós J, Pedrosa CS. Hydatid disease: radiologic and pathologic features and complications. Radiographics 2000;20(3):795-817.

7. Haliloglu M, Saatci I, Akhan O, Ozmen MN, Besim A. Spectrum of imaging findings in pediatric hydatid disease. AJR Am J Roentgenol 1997;169(6):1627-31.

8. Celik M, Senol C, Keles M, Halezeroglu S, Urek S, Haciibrahimoglu G, et al. Surgical treatment of pulmonary hydatid disease in children: report of 122 cases. J Pediatr Surg 2000;35(12):1710-3.

9. Koul PA, Koul AN, Wahid A, Mir FA. CT in pulmonary hydatid disease: unusual appearances. Chest 2000;118(6):1645-7.

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CASE REPORT

Glomus Tumor: A Case Report with Review of Literature Monika Gupta*, Ravindra Srivas**, Prabhu†, Jayakar Thomas‡

ABSTRACT Glomus tumor is a rare benign neoplasm that arises from neuroarterial structure called a glomus body and accounts for 1-2% of soft tissue tumor in the hand. We report the case of a 34-year-old female who presented with a painful swelling of distal digit of the right middle finger. Clinically, slightly bluish nail plate and extreme tenderness over the nail were observed. Hildreth’s sign and Love’s pin test were positive. The mass was excised and histopathological examination confirmed glomus tumor.

Key words: Glomus tumor, distal digit

T

he normal glomus body is an arteriovenous shunt related to thermoregulation, located mostly in digits. The glomus tumor is an uncommon hamartoma composed of endothelium lined vascular spaces (the Sucquet-Hoyer canal) surrounded by glomus cells1,2 arising from the arterial end of the glomus body. Glomus tumors are relatively rare vascular tumors with a reported incidence of 1.6% among all soft tissue tumors.3 A solitary glomus tumor is a pink or purple nodule with a classic triad of pain, cold sensitivity and point tendernerss.4 Glomus tumors are most commonly found in distal extremities in the nail bed and subcutaneous tissue of the distal phalanx.

About 75% of these tumors occur in the hand and 60% are subungual.5,6 Other sites include wrist, forearm and foot7 but they can occur anywhere in the body.

Case Report

no family history of glomus tumor. On examination, she had an 6 × 5 mm reddish blue mass in the right middle finger with extreme tenderness over the nail.

A positive Love’s sign was observed with maximal tenderness, localized to a small pinhead area confined to the swelling. Hildreth’s sign was also positive. Plain X-ray of the affected finger did not reveal any abnormality.

Magnetic resonance imaging (MRI) of the affected finger was planned but the patient could not afford the cost.

The lesion was excised and sent for histopathological examination. We received few irregular soft tissue pieces aggregate measuring 0.2 cm all processed. Microscopic examination showed branching vascular channels lined by endothelial cells, interspersed by uniformly round to ovoid glomus cells forming nests and sheets. The tumor cells had slightly eosinophilic cytoplasm with welldemarcated borders (Fig. 1).

A 34-year-old female presented with painful mass on her right middle finger, which had been present for six years and had slightly increased in size. She had no significant past medical or surgical history. There was

*Associate Professor, Dept. of Pathology Adesh Institute of Medical Science and Research, Bathinda, Punjab **Assistant Professor, Dept. of Pathology Major SD Singh Medical College and Hospital Fatehgarh, Farrukhabad, Uttar Pradesh † Assistant Professor, Dept. of Anatomy Sree Balaji Medical College and Hospital, Chromepet, Chennai ‡ Professor and Head, Dept. of Dermatology Sree Balaji Medical College and Hospital, Chromepet, Chennai Address for correspondence Dr Monika Gupta 38/1 New Lawrence Lines Bathinda Cantt, Punjab E-mail: monikagupta0703@gmail.com

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Figure 1. Shows uniform round to ovoid glomous cells forming sheets interspersed with vascular channels.

Journal of Applied Medicine & Surgery, Vol. 2, Issue 4 April-June 2013

CASE REPORT Discussion Glomus tumor is a rare and benign vascular tumor8 and constitutes 1-2% of the soft tissue tumors in hand.

In 1812, glomus tumor was described by Wood as a painful subcutaneous ‘tubercle’.9 Fifty percent of these occur under the finger nail, usually between the ages of 30 and 50 years, twice as often in women than in men.8-10 Multiple tumors are present in 1-3% of cases.11 Glomus tumors are extremely painful and present early. Clinically, solitary lesions are characterized by a reddish-purple discoloration of the lesion, paroxysmal pain, tenderness and cold sensitivity. The lesion is usually located on extremities especially the nail bed; pain can be of severe nature, leading to disuse atrophy of the limb. Occasional glomus tumors may show unusual clinical features such as large size, deep soft tissue or, visceral locations infiltrative growth patten or multicentricity.12-14 There are very few case reports of clinically malignant glomus tumors.12 They present as a classic triad of severe pain, point tenderness and cold sensitivity.15-17 In a series of 51 patients with glomus tumors of the hand by Van Geertruyden et al,15 spontaneous pain was seen in 80% of patients, sensitivity to touch was present in 100% of patients and cold sensitivity in 63%. Bhaskaranand and Navadgi17 reported in their series of 18 hand tumors, that 14 patients with glomus tumor presented with severe pain. Four patients with other types of tumors presented with dull aching pain. In the series, 100% of patients had point tenderness and 78% had cold sensitivity. Carroll and Berman18 state that excruciating, paroxysmal pain is pathognomonic for glomus tumors.

Two clinical findings are described, particularly in relation to painful subungual solitary glomus tumor, they are the Hildreth’s sign and Love’s test. zz Hildreth’s sign is disappearance of pain after application of a tourniquet proximally. The test is positive when releasing the cuff causes sudden onset of pain and tenderness in the tumor area. zz In one study of 24 patients with hand tumors, a positive Hildreth’s test was noted in 13 patients.

Twelve of these had glomus tumors and one had a hemangiopericytoma with a sensitivity of 92% and specificity of 91% with a positive predictive value of 92% and negative predictive value of 91%.19 Journal of Applied Medicine & Surgery, Vol. 2, Issue 4 April-June 2013

zz

Love test20 is positive when severe tenderness is elicited by pressing the tip of pin over the suspicious region of nail bed. In one study17 comprising of 18 patients, cold sensitivity and Hildreth’s test had sensitivity of 100% and 77.4%, respectively and specificity of 100%. Love’s test had a sensitivity of 100%.

Routine laboratory studies have no role in diagnosis of glomus tumor. MRI can be used to detect glomus tumor, but high resolution MRI can assess tumor characteristics more accurately.

Histologically the tumor has variable quantities of glomus cells, blood vessels and smooth muscle. The glomus tumor shows numerous small, vascular lumina lined by a single layer of flattened endothelial cells.

The vascular spaces were surrounded by clusters of cells characterized by a faintly eosinophilic cytoplasm and a large pale nucleus. Immunohistochemical studies have shown that glomus tumor cells stained positively for smooth muscle actin. In some cases, the glomus tumor cells weakly expressed desmin. They were not reactive with Ulex europaeus agglutinin I and antifactor VIII related antigen, where only the endothelial cells were reactive with them. The tumor cells expressed CD 34 in few cases.21 Subungual glomus tumors are treated by surgical excision. The tumor may be approached by subungual or lateral approach. In either method, the nail plate is reflected and the tumor is exposed and excised along with capsule.

References 1. Dawber RP, Baran R. Disorders of Nails. In: Rook/ Wilkinson/Ebling Textbook of Dermatology. Champion RH, Burton JL, Ebling FJG (Eds.), 5th edition, Oxford: Blackwell Science 1992:p.2497-532.

2. Heys SD, Brittenden J, Atkinson P, Eremin O. Glomus tumour: an analysis of 43 patients and review of the literature. Br J Surg 1992;79(4):345-7.

3. Shugart RR, Soule EH, Johnson EW Jr. Glomus tumor.

Surg Gynecol Obstet 1963;117:334-40.

4. Walsh JJ 4th, Eady JL. Vascular tumors. Hand Clin 2004;20(3):261-8, v-vi.

5. Nazerani S, Motamedi MH, Keramati MR. Diagnosis and management of glomus tumors of the hand. Tech Hand Up Extrem Surg 2010;14(1):8-13.

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CASE REPORT 6. Lee IJ, Park DH, Park MC, Pae NS. Subungual glomus tumours of the hand: diagnosis and outcome of the transungual approach. J Hand Surg Eur Vol 2009;34(5):685-8.

7. Miyamoto H, Wada H. Localized multiple glomangiomas on the foot. J Dermatol 2009;36(11):604-7.

8. Theumann NH, Goettmann S, Le Viet D, Resnick D, Chung CB, Bittoun J, et al. Recurrent glomus tumors of fingertips: MR imaging evaluation. Radiology 2002;223(1):143-51.

9. Wood W. Further observations on painful subcutaneous tubercle. Edinburg Medical and Surgical Journal 1812;8:429-35.

10. Van Ruyssevelt CE, Vranckx P. Subungual glomus tumor: emphasis on MR angiography. AJR Am J Roentgenol 2004;182(1):263-4.

11. Heys SD, Brittenden J, Atkinson P, Eremin O. Glomus tumour: an analysis of 43 patients and review of the literature. Br J Surg 1992;79(4):345-7.

12. Gould EW, Manivel JC, Albores-Saavedra J, Monforte H. Locally infiltrative glomus tumors and glomangiosarcomas. A clinical, ultrastructural, and immunohistochemical study. Cancer 1990;65(2):310-8.

13. Kiyosawa T, Umebayashi Y, Nakayama Y, Soeda S. Hereditary multiple glomus tumors involving the

glans penis. A case report and review of the literature.

Dermatol Surg 1995;21(10):895-9.

14. Lumley JS, Stansfeld AG. Infiltrating glomus tumour of lower limb. Br Med J 1972;1(5798):484-5.

15. Van Geertruyden J, Lorea P, Goldschmidt D, de Fontaine S, Schuind F, Kinnen L, et al. Glomus tumours of the hand. A retrospective study of 51 cases. J Hand Surg Br 1996;21(2):257-60.

16. Tomak Y, Akcay I, Dabak N, Eroglu L. Subungual glomus tumours of the hand: diagnosis and treatment of 14 cases. Scand J Plast Reconstr Surg Hand Surg 2003;37(2):121-4.

17. Bhaskaranand K, Navadgi BC. Glomus tumour of the hand. J Hand Surg Br 2002;27(3):229-31.

18. Carroll RE, Berman AT. Glomus tumors of the hand: review of the literature and report on twenty-eight cases.

J Bone Joint Surg Am 1972;54(4):691-703.

19. Giele H. Hildrethâ&#x20AC;&#x2122;s test is a reliable clinical sign for the diagnosis of glomus tumours. J Hand Surg Br 2002;27(2):157-8.

20. Love JG. Glomus tumors: diagnosis and treatment. Proc Staff Meet, Mayo Clin 1944;19:113-6.

21. Hatori M, Aiba S, Kato M, Kamiya N, Kokubun S. Expression of CD34 in glomus tumors. Tohoku J Exp Med 1997;182(3):241-7.

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CASE REPORT

Unusually Large Functional Adrenal Adenoma: A Rare Case Report with Review of Literature Monika Gupta*, Ravindra Srivas**, Prabhu†, Jayakar Thomas‡

ABSTRACT Functional adrenal adenomas of >4 cm in size are rare. We report a case of adrenal adenoma of 12 cm in size, presenting with Cushing’s syndrome.

Key words: Adrenal adenoma, large, functional

P

rimary diseases of adrenal gland are responsible for upto 20% of cases of Cushing’s syndrome.

Cortical adrenal adenomas account for 15% of cases. These benign tumors are usually smaller than 4 cm and secrete cortisol.1 Large adrenal adenoma producing Cushing’s syndrome is a rare finding.

The presence of large adrenal mass in a patient with Cushing’s syndrome is practically always indicative of carcinoma. We present a case of a large adrenal adenoma, measuring 12 cm in size, along with discussion on the clinical, radiological and biochemical features of the case and relevant review of literature.

Case Report A 30-year-old female presented with complaints of swelling all over the body for last one year, along with increased body hair and presence of abdominal striae.

There was no history of headache, vomiting, visual difficulty, polyuria and polydypsia. Her past medical, personal and family histories were unremarkable. Her blood pressure was 140/100 mm of mercury. On examination, patient had moon-facies, increased body hair, proximal muscle weakness, especially involving

*Associate Professor, Dept. of Pathology Adesh Institute of Medical Science and Research, Bathinda, Punjab **Assistant Professor, Dept. of Pathology Major SD Singh Medical College and Hospital Fatehgarh, Farrukhabad, Uttar Pradesh † Assistant Professor, Dept. of Anatomy Sree Balaji Medical College and Hospital, Chromepet, Chennai ‡ Professor and Head, Dept. of Dermatology Sree Balaji Medical College and Hospital, Chromepet, Chennai Address for correspondence Dr Monika Gupta 38/1 New Laurence Lines Bathinda Cantt, Punjab E-mail: monikagupta0703@gmail.com

Journal of Applied Medicine & Surgery, Vol. 2, Issue 4 April-June 2013

the pelvic girdle and nonpitting pedal edema. Per abdominal as well as external genitalia examination was within normal limits. No abnormalities were detected in cardiovascular and respiratory system. Her hemogram, serum electrolytes, urea and creatinine values were within normal limits. Routine urine analysis, chest X-ray and electrocardiogram (ECG) revealed no abnormalities. Skeletal survey did not show any symptoms of osteoporosis and there was no seller pathology on skull X-ray. Urinary free cortisol concentration, measured as cortisol/creatinine ratio on two successive 24-hour urine collections were found to be raised to 90 and 169 mol/millimole (reference range 5-55 mol/millimole). On high dose dexamethasone suppression test, (dexamethasone 2 mg orally every 6 hours for 48 hours), her basal plasma cortisol level was 515 nanomole/liter that failed to suppress after 48 hours and remained raised at 510 nanomole/ liter. Computed tomography (CT) scan of abdomen showed a large mass arising from adrenal gland, suggestive of adrenal neoplasm. Right adrenelectomy of patient was done and resected specimen was sent for histopathological examination. On gross examination, (Fig. 1) the operated tumor mass measured 12 cm in size, yellow to brown in color, homogeneous, and partly encapsulated. No evidence of necrosis or hemorrhage was apparent. Various sections from the tumor showed tumor cells disposed in nests, cords and alveolar arrangements (Fig. 2). Cells had vacuolated, clear cytoplasm and variable number of compact type of cells. The nuclear-cytoplasmic ratio was low, although a few cells showed enlarged hyperchromatic nuclei. Scarce mitotic activity was seen, with no abnormal mitotic figures. No necrosis, capsular, venous or sinusoidal invasion was seen. Weiss scoring2 of the 45

CASE REPORT few (≤10%) of them are functional with production of glucorticoides (Cushing’s syndrome), mineralocorticoides or sex hormones. Approximately 80% of patients are females, and in only 20% of cases, the syndrome occurs before puberty. The adrenal adenomas are usually unilateral although bilateral cases are on record.3

Figure 1. Resected specimen of adrenal adenoma.

Figure 2. Microscopic section of adrenal adenoma showing tumor cells in sheets and trabeculae (H&E × 400).

tumor was calculated to be 2. Postoperative period was uneventful and the patient fully recovered after the surgery.

Discussion Cushing’s syndrome is a group of clinical and metabolic disorders characterized by adrenocortical hyperfunction. It is associated with excess production of glucocorticoides. In clinical practice, most cases of Cushing’s syndrome are the result of the administration of exogenous glucocorticoides. The endogenous Cushing’s syndrome may be associated with primary hypothalamic or pituitary disease associated (70-80%), hypersecretion of adrenocorticotrophic hormone (ACTH) or cortisol by an adrenal adenoma, carcinoma or nodular hyperplasia (10%) or secretion of ectopic ACTH by a nonendocrinal source (10%).

Adrenal adenomas are uncommon in patients younger than 30 years. Most of them are small in size. Only a 46

The most significant point in an adrenocortical neoplasm is the differentiation between adrenocortical adenoma and carcinoma. Truly speaking, there is no single histological criterion by which we can reliably differentiate the two. Several studies have been done in past to reach a convincing guideline to solve the puzzle. The most relevant and widely used one was given by Weiss2 in 1984 who proposed nine histopathological criteria to distinguish adenomas from adrenal cortical tumors that had metastasized or locally recurred. Malignant ones met four or more of these histological criteria.2 The threshold for malignancy was lowered in 19894 and the presence of three or more of these of histological pathologic criteria was suggestive for a malignant clinical behavior.

However, Weiss criteria are exclusively histopathological in nature, which can be assessed only after the tumor has been resected. In practice, the clinicians need to know before surgical intervention if the lesion they are dealing with is benign or a malignant one as adrenocortical carcinomas are usually associated with a dismal prognosis. Both CT and magnetic resonance imaging (MRI) are useful in evaluation, CT is currently regarded as the most accurate imaging modality for the preoperative localization of these tumors.5 Besides signs of local invasion, lymphadenopathy, distant metastasis and few other imaging parameters, size of the tumor remains to be one of the most important preoperative predictor in making such differentiation.

Several studies have been done in past to evaluate size of the tumor as a marker of malignancy. Barnett et al6 in their retrospective study evaluated the value of tumor size and other clinical parameters in the prediction of the adrenal malignancy. They found size of the tumor to be the single best predictor of malignancy. In their study, the median size of the adrenal cortical adenomas was found to be 3 cm, while the median size of the adrenal cortical carcinomas was 9.2 cm. In their study, Lumachi et al7 found that there was a significant difference in size between benign neoplasms (range, 3-6 cm, median, 4 cm) and malignant neoplasms Journal of Applied Medicine & Surgery, Vol. 2, Issue 4 April-June 2013

CASE REPORT (range, 3-12 cm, median, 6 cm), while no benign lesion exceeded 6 cm in size in their study. In another study

by Jain et al,8 the mean size and weight of the malignant tumors were found to be statistically significantly higher than that of the benign tumors, with no adenoma >5 cm and no carcinoma < 6 cm. According to Fassnacht et al9 the probability of malignancy is clearly related to tumor size as almost all lesions < 3 cm are benign, whereas diameters >3 cm indicate a high-risk of malignancy.

Thus, the previous studies show that the adrenal adenomas are mostly smaller in size, that is, <5-6 cm and adrenal carcinomas often >5 cm, though smaller carcinomatous lesions might be detected.

However, the problem in suggesting the larger lesions to be the malignant ones is the fact that rarely, large histopathologically proven (as per the Weiss criteria) adenomas do also occur, as in our case where gross size was 12 cm. Barnett et al6 too concluded that although size remains a good predictor of the histologic features and clinical behavior of adrenal neoplasm, both small and large benign adrenal cortical tumors occur with measurable frequency. A subgroup of adrenal adenomas are larger, more heterogeneous, and more frequently calcified than those with the usual imaging findings. Central necrosis, hemorrhage or both are responsible for many of the imaging features.

Differentiation of these lesions from other large adrenal masses, including adrenal carcinoma, cannot be made by means of imaging alone; resection is required for the definitive diagnosis. Adrenal adenomas may occasionally undergo intratumoral hemorrhagic degeneration with development of avascular and cystic internal regions and subsequent fibrosis. They then become much larger and display focal liquefaction, central or peripheral calcification and fibrosis, internal soft-tissue nodules, and patchy heterogeneous contrast enhancement, features that are commonly associated with adrenocortical carcinomas and other lesions.

These lesions can be misdiagnosed histologically also if the criteria for malignancy, which include mitoses, a trabecular growth pattern, and an increased nuclearcytoplasmic ratio, are not applied correctly.10 To conclude, we suggest that tumor size determined by imaging techniques does help in deciding malignant potential of the tumor but is not infallible.

High-quality imaging studies (CT and MRI), imagingguided fine needle aspiration cytology (FNAC)7 and diagnostic laparoscopy may be useful in establishing a preoperative diagnosis. Ultimately, histopathological examination continues to be the benchmark for diagnosis.

References 1. Sturgeon C, Kebebew E. Laparoscopic adrenalectomy for malignancy. Surg Clin North Am 2004;84(3):755-74.

2. Weiss LM. Comparative histologic study of 43 metastasizing and nonmetastasizing adrenocortical tumors. Am J Surg Pathol 1984;8(3):163-9.

3. Aiba M, Kawakami M, Ito Y, Fujimoto Y, Suda T, Demura H. Bilateral adrenocortical adenomas causing Cushingâ&#x20AC;&#x2122;s syndrome. Report of two cases with enzyme histochemical and ultrastructural studies and a review of the literature. Arch Pathol Lab Med 1992;116(2):146-50.

4. Weiss LM, Medeiros LJ, Vickery AL Jr. Pathologic features of prognostic significance in adrenocortical carcinoma. Am J Surg Pathol 1989;13(3):202-6.

5. Porte HL, Ernst OJ, Delebecq T, MĂŠtois D, Lemaitre LG, Wurtz AJ. Is computed tomography guided biopsy still necessary for the diagnosis of adrenal masses in patients with resectable non-small-cell lung cancer? Eur J Cardiothorac Surg 1999;15(5):597-601.

6. Barnett CC Jr, Varma DG, El-Naggar AK, Dackiw AP, Porter GA, Pearson AS, et al. Limitations of size as a criterion in the evaluation of adrenal tumors. Surgery 2000;128(6):973-82;discussion 982-3.

7. Lumachi F, Borsato S, Brandes AA, Boccagni P, Tregnaghi A, Angelini F, et al. Fine-needle aspiration cytology of adrenal masses in noncancer patients: clinicoradiologic and histologic correlations in functioning and nonfunctioning tumors. Cancer 2001;93(5):323-9.

8. Jain M, Kapoor S, Mishra A, Gupta S, Agarwal A. Weiss criteria in large adrenocortical tumors: a validation study.

Indian J Pathol Microbiol 2010;53(2):222-6.

9. Fassnacht M, Kenn W, Allolio B. Adrenal tumors: how to establish malignancy? J Endocrinol Invest 2004;27(4):387-99.

10. Newhouse JH, Heffess CS, Wagner BJ, Imray TJ, Adair CF, Davidson AJ. Large degenerated adrenal adenomas: radiologic-pathologic correlation. Radiology 1999;210(2):385-91.

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CASE REPORT

Hirschsprung’s Enterocolitis Presenting as Fulminant Sepsis in A Neonate Rahul Gandhi*, Monika Gupta**

ABSTRACT Hirschsprung’s disease (congenital megacolon) is caused by the failed migration of colonic ganglion cells during gestation.

Varying lengths of the distal colon are unable to relax, causing functional colonic obstruction. Hirschsprung’s disease most commonly involves the rectosigmoid region of the colon but can affect the entire colon and, rarely the small intestine. Here we report a case of late-onset sepsis with pneumonia and necrotizing enterocolitis at first instance in case of Hirschsprung’s disease. This case suggests that Hirschsprung’s disease may have unusual early roentgenographic presentation within the first few days of life. Suction biopsies are crucial for further diagnosis.

Key words: Hirschsprung’s disease, necrotizing enterocolitis

H

irschsprung’s disease usually presents in infancy, although some patients present with persistent, severe constipation later in life. Symptoms include difficult bowel movements, poor feeding, poor weight gain and progressive abdominal distension. Early diagnosis is important to prevent complications (e.g., enterocolitis, colonic rupture). A rectal suction biopsy can detect hypertrophic nerve trunks and the absence of ganglion cells in the colonic submucosa, confirming the diagnosis. Upto one-third of patients develop Hirschsprung’s-associated enterocolitis, a significant cause of mortality. Patients should be monitored closely for enterocolitis. With proper treatment, most patients will not have long-term adverse effects and can live normally.

Case History We report a case of a 5-day male child, who presented to us with history of abdominal distension progressively increasing since last three days of life, vomiting and lethargy two days, breathing difficulty one day. Child was born to a primigravidae mother at home. He cried

*Assistant Professor Dept. of Pediatrics **Associate Professor Dept. of Pathology Adesh Institute of Medical Science and Research, Bathinda, Punjab Address for correspondence Dr Rahul Gandhi 1280, Huda Sec. 1, Shahbad M, Kurukshetra, Haryana E-mail: drrahul05@sify.com

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immediately after birth, birth weight was 2.6 kg. He was brought to emergency department on Day 5, with admission vitals heart rate 148/mt, respiratory rate 68/mt, pulse oximetry SpO2 76%, (capillary refilling time [CRT]) <3 second. On systemic examination of chest bilateral crepts were present, per abdomen examination revealed dilated, tense and shiny abdomen with no periumbilical redness, bowel sounds were not audible. Child was intubated immediately in view of impending respiratory failure and put on P-SIMV mode of ventilation. He was suspected to have late-onset sepsis with pneumonia and necrotizing entercolitis at first instance and sepsis screen was sent for confirmation.

Investigations revealed hemoglobin (Hb) 16.5 g/dl, total leukocyte count (TLC) 32,000/mm3, polymorphs 82%, lymphocytes 16%, basophils 2%, platelet count 60,000/mm3. C-reactive protein (CRP) quantitative 5.6 (0-6), blood culture sensitivity showed no growth.

Abdominal X-ray showed dilated small and large bowel loops with no air under diaphragm. Chest X-ray showed bilateral infiltrates in lung fields. Child was put on IV antibiotics. He was made nil per oral and nasogastric feeding tube was inserted, which revealed bilious aspirates approx 4 ml. A per rectal tube was inserted, which led to passage of meconium. This was followed by massive decrease in abdominal distension.

A possibility of meconium plug was kept with other differential diagnosis of Hirschsprung’s disease with enterocolitis. The child again developed abdominal distension. Multiple rectal irrigations decreased the Journal of Applied Medicine & Surgery, Vol. 2, Issue 4 April-June 2013

CASE REPORT distension. After being weaned from ventilation for five days he was planned for barium enema. Barium enema revealed dilated rectum with rectal sigmoid ratio 2:1, a sign of Hirschsprung’s disease. A intraoperative biopsy was taken from rectum, which revealed no ganglion cells. Child underwent colostomy and is presently passing stools via colostomy at normal frequency and planned for surgical repair.

have revealed its varied manifestations at varied age groups but it is extremely rare to manifest at Day 5.

The clinical presentation of Hirschsprung’s enterocolitis can mimic necrotizing enterocolitis secondary to septicemia but Hirschsprung’s enterocolitis if undiagnosed can itself can lead to fulminant septicemia and deaths secondary to invasion of pathogens from intestinal epithelium.

Discussion

Suggested Reading

Hirschsprung’s disease occurs in one out of 5,000 births. The disease is caused by the failure of ganglion cells to migrate cephalocaudally through the neural crest during Weeks 4-12 of gestation. The aganglionic segment usually begins at the anus and extends proximally. Short-segment disease is most common and is confined to the rectosigmoid region of the colon. Long-segment disease extends past this region and can affect the entire colon symptoms range from neonatal intestinal obstruction to chronic progressive constipation in older children. Approximately 80% of patients present in the first few months of life with difficult bowel movements, poor feeding and progressive abdominal distension. Upto 90% of infants with Hirschsprung’s disease fail to pass meconium in the first 24 hours of life. Disease is common in Indian population with incidence about 1:4,000/live births.

Incidence of Hirschsprung’s enterocolitis at Day 5 is extremely uncommon. Enterocolitis occurs in 17-50% of infants with Hirschsprung’s disease. Case reports

1. Amiel J, Lyonnet S. Hirschsprung disease, associated syndromes, and genetics: a review. J Med Genet 2001;38(11):729-39.

2. Parisi MA, Kapur RP. Genetics of Hirschsprung disease.

Curr Opin Pediatr 2000;12(6):610-7.

3. Feldman M, Friedman LS, Sleisenger MH. Hirschsprung’s disease: congenital megacolon. In: Sleisenger & Fordtran’s Gastrointestinal and Liver Disease: Pathophysiology, Diagnosis, Management. 7th edition, P Saunders: Philadelphia, Pa 2002:2131-5.

4. Stewart DR, von Allmen D. The genetics of Hirschsprung disease. Gastroenterol Clin North Am 2003;32(3):81937, vi.

5. Holschneider AM, Puri P. Hirschsprung’s Disease and Allied Disorders. 2nd edition, Harwood Academic Publishers: Amsterdam, 2000.

6. Coran AG, Teitelbaum DH. Recent advances in the management of Hirschsprung’s disease. Am J Surg 2000;180(5):382-7.

7. Parsons SJ, Fenton E, Dargaville P. Clostridium difficile associated severe enterocolitis: a feature of Hirschsprung’s disease in a neonate presenting late. J Paediatr Child Health 2005;41(12):689-90.

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CASE REPORT

Laurence-Moon-Biedl Syndrome NS Saradha, K Saraswathy, L Nandini

ABSTRACT A 23-year-old woman presented to our gynecology OPD with amenorrhea, reduced vision since childhood, obesity, hypogonadism and polydactyly. Retinitis pigmentosa was seen on fundoscopy. With typical primary features she was diagnosed to have Laurence-Moon-Biedl syndrome. It is a rare autosomal recessive disorder with mutation in six loci identified so far.

Itâ&#x20AC;&#x2122;s common among communities with consanguineous marriage. There is no specific treatment available yet.

Key words: Laurence-Moon-Biedl syndrome, autosomal recessive disorder, consanguineous marriages, amenorrhea,

visual impairment

L

aurence-Moon-Biedl syndrome is a rare autosomal recessive disorder. Two copies of the defective gene are required to be born with the disorder. Fourteen genes have been found to be responsible on four different sites of chromosomes 16, 11, 3, 15. Hence, the disorder is found in communities with consanguineous marriages. It has ill-defined complex symptoms usually presenting with signs of obesity, hypogonadism, polydactyly, retinitis pigmentosa and mental deficiency.

developed with minimal hair growth. P/v could not be done. All baseline investigations were done, which showed no significance except a mild rise in folliclestimulating hormone (FSH) values.

Case Report A 23-year-old, unmarried, nulliparous woman presented with amenorrhea since three months and reduced vision since childhood. Detailed history was taken. Though she attained menarche spontaneously at 16 years of age she never got her spontaneous bleeding, for which she takes progesterone tablets for withdrawal bleeding once in three months. She was diagnosed to have retinitis pigmentosa on fundoscopy by an ophthalmologist.

There was second degree consanguineous marriage in her parents and her elder sister aged 24 years had similar complaints. She was short statured with central obesity wearing spectacles, her body mass index (BMI) - 45.5. She had polydactyly in both upper and lower limbs (Fig. 1). Muscle tone was normal. Mental IQaverage. Breast development was Tannerâ&#x20AC;&#x2122;s Grade 2, sparse axillary hair, external genitalia was not well-

Dept. of Obstetrics and Gynecology Sree Balaji Medical College and Hospital, Chromepet, Chennai

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Figure 1. Laurence-Moon-Biedl syndrome showing polydactyly.

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CASE REPORT Discussion Though, there is wide variation of symptoms, there should be at least four symptoms to confirm diagnosis. Primary features include visual impairment, obesity, hypogonadism, renal anomalies, polydactyly and learning difficulties. Secondary features are developmental delays, increased blood pressure, speech disorders, dental anomalies, anosmia, flat wide feet, thyroid problems and short stature. Our patient had four primary features and two secondary features. Hence, Laurence-Moon-Biedl syndrome was diagnosed. It is hypogonadotrophic hypogonadism hence if endometrial thickness is more then a progesterone challenge can be given, if it is less then combined oral contraceptives can regularize periods. Studies have shown that there are chances of pregnancy, if there are no structural abnormalities. Regular visits to ophthalmologist are important to make use of the remaining vision by wearing proper corrective lenses. Specialized diet plan and exercise is needed for these patients. Yearly check-up for diabetes, hypertension, renal parameters is a must.

Conclusion Clinical features, though appear from childhood, diagnosis can be made only in puberty. There is no definite treatment but early diagnosis with symptomatic, supportive and rehabilitative measures can go a long way for a better living.

References 1. Laurence JZ, Moon RC. Four cases of “retinitis pigmentosa” occurring in the same family, and accompanied by general imperfections of development.

Obes Res 1995;3(4):400-3.

2. Hutchinson J. Slowly progressive paraplegia and disease of the choroids with defective intellect and arrested sexual development. Arch Surg 1900;11:118-22.

3. Bardet G. Sur un syndrome d’obésitécongénitale avec polydactylie et rétinite pigmentaire (contribution àl’etude des formes cliniques de l’obésite hypophysaire).

Thése de Paris 1920 (No 170):107.

4. Biedl A. Ein geschwisterpaar mit adioposo-genitaler dystrophie. Dtsch Med Wschr 1922; 48:1630.

5. Solis-Cohen S, Weiss E. Dystrophia adopogenitalis with atypical retinitis pigmentosa and mental deficiency: the Laurence-Biedl syndrome a report of four cases in one family. Am J Med Sci 1925;169:489-505.

6. Ammann F. Investigations cliniques et génétiques sur le syndrome de Bardet-Biedl en Suisse. J Genet Hum 1970 (Suppl 18):1-310.

7. Schachat AP, Maumenee IH. Bardet-Biedl syndrome and related disorders. Arch Ophthalmol 1982;100(2):285-8.

8. Farag TI, Teebi AS. Bardet-Biedl and Laurence-Moon syndromes in a mixed Arab population. Clin Genet 1988;33(2):78-82.

9. Bauman ML, Hogan GR. Laurence-Moon-Biedl syndrome.

Report of two unrelated children less than 3 years of age.

Am J Dis Child 1973;126(1):119-26.

10. Hurley RM, Dery P, Norady MB, Drummond KN. The renal lesion of the Laurence-Moon-Biedl syndrome. J Pediatr 1975;87(2):206-9.

11. Churchill DN, McManamon P, Hurley RM. Renal disease-a sixth cardinal feature of the Laurence-Moon-Biedl syndrome. Clin Nephrol 1981;16(3):151-4.

12. Linné T, Wikstad I, Zetterström R. Renal involvement in the Laurence-Moon-Biedl syndrome. Functional and radiological studies. Acta Paediatr Scand 1986;75(2):240-4.

13. Bluett NH, Chantler C, Singer JD, Saxton HM. Congenital renal abnormalities in the Laurence-Moon-Biedl syndrome.

Arch Dis Child 1977;52(12):968-70.

14. Tieder M, Levy M, Gubler MC, Gagnadoux MF, Broyer M. Renal abnormalities in the Bardet-Biedl syndrome.

Int J Pediatr Nephrol 1982;3(3):199-203.

15. Price D, Gartner JG, Kaplan BS. Ultrastructural changes in the glomerular basement membrane of patients with Laurence-Moon-Biedl-Bardet syndrome. Clin Nephrol 1981;16(6):283-8.

16. Harnett JD, Green JS, Cramer BC, Johnson G, Chafe L, McManamon P, et al. The spectrum of renal disease in Laurence-Moon-Biedl syndrome. N Engl J Med 1988;319(10):615-8.

17. Hall JG. The value of the study of natural history in genetic disorders and congenital anomaly syndromes. J Med Genet 1988;25(7):434-44.

18. Williams B, Jenkins D, Walls J. Chronic renal failure an important feature of the Laurence-Moon-Biedl syndrome. Postgrad Med J 1988; 64(752):462-4.

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CASE REPORT

Nevus of Ota is a Rare Nevus and Q-switched Laser is the Best Available Option for Successful Treatment OP Patidar*, Rekha Patidar**

ABSTRACT Patients with the nevus of Ota have increased amounts of melanin (pigment) and melanin-producing cells (melanocytes) in and around their eyes. This includes the intraocular blood vessel layer called the uvea (choroid, ciliary body and iris), on the selera (the white part of the eye ball), and in the eyelids. Q-switched laser is the best available option for successful treatment.

Key words: Nevus of Ota, melanin (pigment), melanocytes (melanin-producing cells), Q-switched Nd-YAG laser

T

he nevus of Ota appears as increased pigmentation of one eye and its eyelids. The eyelid is darkened, pigment can be observed on the white of the eye and that iris may be darker (compared to the contralateral iris). Though none of these findings produce physical symptoms, physical asymmetry can have a psychological impact on the patient.

Careful inspection of any patient with two different colored irises (heterochromia irides) may uncover the eyelid and episcleral pigmentation diagnostic of the nevus of Ota. Slit-lamp biomicroscopy is used to distinguish between conjunctival and episcleral pigmentation. Comparative examination of the irises can be used to evaluate coloration and surface characteristics. Dilated ophthalmoscopy similarly demonstrates asymmetric â&#x20AC;&#x2DC;darkerâ&#x20AC;&#x2122; pigmentation of the choroidal vascular layer beneath the retina. Ophthalmic ultrasound imaging will reveal the thickening of the vascular uveal layer of the eye.

Ocular melanosis has the same ocular melanocyte and pigment distribution as well as the risks associated with the nevus of Ota. But, in cases of ocular melanosis there is no eyelid involvement.

*Assistant Professor Dept. of Medicine **Assistant Professor Dept. of General Surgery Jhalawar Medical College, Jhalawar, Rajasthan Address for correspondence Dr OP Patidar Gayatri Colony, NH-12 Jhalawar - 326 001, Rajasthan E-mail: dr.oppatidar.patidar@gmail.com

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Case Report A 22-year-old female student of dental college presented to us with complaints of dark bluish pigmentation over right upper one-third of face along with involvement of right eye. This pigmentation had been present since birth but initially in infantile age, it was a very small crescentic lesion just below the right eye with a very small wedge-shaped lesion in right eye.

This lesion remained stationary for initial few years but with advancing age especially at the age of puberty, the lesion spread gradually and involved the right upper one-third of face including upper and lowers eyelids with involvement of right eye on examination.

She is a healthy young girl, with dark bluish pigmentation over right upper one-third of face along with involvement of bulbar conjunctiva of right eye.

No similar hypo- or hyperpigmentation was seen elsewhere on the body. She was diagnosed as a case of nevus of Ota. Hydroquinone preparation had been applied locally years together but there was no response. Ultimately laser therapy was decided upon. Q-switched Nd-YAG 1,064 laser was used and seven treatments were given with satisfactory results. Over 90% of pigment has disappeared from the affected site. Hydroquinone and sunscreen lotion are used daily.

Discussion Nevus of Ota is a form of congenital dermal melanosis, a rare entity in Indians and common in Japanese as compared to others, the incidence rate was about 0.4%.1 Journal of Applied Medicine & Surgery, Vol. 2, Issue 4 April-June 2013

CASE REPORT

Figure 1. Pre-treatment.

Figure 2. Post-treatment.

Figure 3. Pre-treatment.

Figure 4. Post-treatment.

No exact incidence of nevus of Ota in Indians is available so far. Although there have been reports of nevus of Ota in three successive generation, most patient have no family history.2

Female sex hormones also have an important role in the development of other melanocytic conditions such as melasma; other stimuli, such as infection, trauma or ultraviolet light exposure have also been reported to trigger onset of the nevus.4-6 In this case also, similar is true as the lesion spread at puberty.

The age of onset is another interesting aspect: It is not uncommon for nevus of Ota to appear in the perinatal period or around puberty. In one study, it was found that 48% developed the nevus at or soon after birth, 11% developed it between 1 and 10 years of age and the remaining 36% developed it between 11 and 20 years of age.3 Nevus of Ota is more common in females; the ratio of male-to-female is 1:4.8. It has been observed that the onset is common at puberty in females so female sex hormones have been suggested as persistent stimuli.

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Patients with the nevus of Ota are at greater risk for development of intraocular and central nervous system malignant melanomas (e.g., choroidal melanoma less than 4%). Patient with the nevus of Ota should be periodically examined by ophthalmologists and neurologists.

As far as treatment of nevus of Ota is concerned, laser therapy is the mode of choice because of excellent results in short duration but cost and availability are the limiting factors.

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CASE REPORT Ideally Q-switched Nd-YAG laser is used and complete removal requires an average of eight treatments, spaced at least a month apart, using different wave lengths for different colored skin lesions. Q-switched lasers will shatter the pigment into particles that are cleared by the lymphatic system of the body.

Q-switched laser is also used to remove tattoos. In this case only seven treatments were given spaced at least six months apart because of her education schedule.

But results are satisfactory.

References 1. Rapini Ronald P, Bolognia Jean L, Jorizzo Joseph L. Dermatology. 2-Volume Set. Mosby: St. Louis 2007:p.

1720-2.

2. James William D, Berger Timothy G, et al. Andrews Diseases of the Skin: Clinical Dermatology. Elsevier: Saunders, 2006.

3. Chan HH, Kono T. Nevus of Ota: clinical aspects and management. Skinmed 2003;2(2):89-96; quiz 97-8.

4. eMedicine-Nevi of Ota and Ito. Article by Harvey Lui.

Acknowledgment

5. Geronemus RG. Q-switched ruby laser therapy of nevus of Ota. Arch Dermatol 1992;128(12):1618-22.

We are highly thankful to Dr Lakshyajit D Dhami, MS MCh (plastic) cosmetics, plastic and laser surgeon Hon. Nanavati Hospital, Mumbai, for laser treatment.

6. Shimbashi T, Hyakusoku H, Okinaga M. Treatment of nevus of Ota by Q-switched ruby laser. Aesthetic Plast Surg 1997;21(2):118-21.

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CASE REPORT

Scorpion Bite causing Acute Severe Myocarditis: A Rare Complication Tarachand Saini*, Shailendar Gupta**, Maniram Kumhar†

ABSTRACT Scorpion bites are common in India as well as in other countries. Usually, these bites are harmless but sometime have serious clinical sequelae, including death. We report herein a case of scorpion bite that presented with acute severe myocarditis - a rare complication.

Key words: Scorpion bite, severe myocarditis, pulmonary edema

T

here are about 1,500 species of scorpions worldwide, out of these 50 are dangerous to human. Among 86 species in India, Mesobuthus tamulus and Palamnaeus swammerdami are of medical importance.1

Almost all lethal scorpions except Hemiscorpius species, belong to the scorpion family called Buthidae.

The lethal member of Buthidae include genera Buthus, Parabuthus, Mesobuthus, Tityus, Leiurus, Androctonus and Centruroides.

Scorpions live in warm dry regions throughout India.

They commonly inhabit the crevices of dwellings, underground burrows, under logs or debris, paddy husk, sugarcane fields, coconut and banana plantations.

Their distribution is more in regions with abundant red soil.2 They hunt during night and hide in crevices and burrow during the day to avoid light. Scorpion stings increase dramatically in summer months and lower in winter.

Scorpion stings causes a wide range of manifestation, from local skin reaction to neurological, respiratory and cardiovascular collapse. Cardiovascular effect are particularly prominent after stings by Indian red scorpion (M. tamulus).3

*2nd Year Resident **3rd Year Resident † Associate Professor Dept. of Medicine, Jawaharlal Nehru Medical College, Ajmer, Rajasthan Address for correspondence Dr Tarachand Saini 20, Gaurav Enclave, Near City Palace, Civil Line, Ajmer, Rajasthan E-mail: drtcsaini20@gmail.com

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Case Report An 18-year-old girl was stung by a scorpion on the distal phalanx of the left index finger and presented with intense pain and swelling on the local site. She admitted with heart rate 140 beats/minute, and blood pressure 70/40 mmHg. Symptomatic treatment was given. But the patient’s condition continued to worsen, and she developed severe respiratory distress. Because of respiratory failure, she was immediately intubated and admitted to the intensive care unit. Bilateral basal rales were heard on auscultation, and cardiovascular examination revealed a loud S3 gallop at the apex.

Laboratory results were as follows: Hemoglobin was 12.6 g/dl, white blood cell count was 24,380 cells/mm3, platelet count was 1,80,000 cells/mm3 and urine microscopy showed hematuria. Blood urea 71 mg/dl (17-43 mg/dl), serum creatinine 1.6 mg/dl (0.5-1.1 mg/ dl), aspartate transaminase 93 U/l (5-38 U/l), alanine transaminase 30 U/l (5-41 U/l), creatine kinase (CK) 743 U/l (26-190 U/l), CK-MB 52 U/l (0-24 U/l), Trop-I 0.02 ng/ml (0.00-0.04 ng/ml).

An electrocardiogram (ECG) revealed sinus tachycardia and ST-T wave changes. Chest X-ray demonstrated bilateral fluffy shadows indicative of pulmonary edema (Fig. 1).

Echocardiography reveal hypokinesia of the basal twothird of the interventricular septum with reduced ejection fraction (Fig. 2).

In accordance with these symptoms and findings, a diagnosis of acute heart failure with pulmonary edema was made. Dopamine, dobutamine, diuretics 55

CASE REPORT of the somatic, sympathetic, and parasympathetic neurons results in autonomic and neuromuscular overexcitation symptoms due to release of excessive neurotransmitters such as epinephrine, norepinephrine, acetylcholine glutamate and aspartate. Meanwhile, the short polypeptide neurotoxin blocks the potassium channels.

Figure 1. Chest X-ray showing bilateral fluffy shadows.

Figure 2. Echocardiography.

and steroids were given. With treatment, patientâ&#x20AC;&#x2122;s status improved. Repeat chest X-ray was clear within 24 hours of initiating this treatment.

Discussion Scorpion venom may contain multiple toxin-like neurotoxin, cardiotoxin, nephrotoxin and hemolytic toxin. The primary targets of scorpion venom are voltage-dependent ion channels. The long-chain polypeptide neurotoxin causes stabilization of voltagedependent sodium channels in the open state leading to continuous, prolonged, repetitive firing 56

Scorpion bites usually have a good prognosis.

However, occasionally potentially fatal complications such as myocarditis, pulmonary edema and shock occur.4 Among these, myocarditis and resultant pulmonary edema are important causes of death.5 Three possible mechanisms might explain cardiac dysfunction, including adrenergic myocarditis, toxics myocarditis and myocardial ischemia. a-receptor stimulation by toxins play a major role in development of tachycardia, myocardial dysfunction and pulmonary edema.6 Unopposed a-receptors stimulation lead to suppression of insulin secretion, hyerglycemia, hyperkalemia, free fatty acid and free radical accumulation that are injurious to myocardium. Bahloul et al4 examined the histopathology of two fatal myocarditis cases resulting from a scorpion bite.

The pathologic conditions revealed a mixed picture of toxic myocarditis and coagulative myocytolysis, similar to catecholamine-induced cardiotoxicity.4 Myocardial ischemia is not only due to the release of catecholamines but also due to effect of cytokines and/or neuropeptide Y on coronary vessels. Cardiac damage might be enhanced by the depressive effect of cytokines upon myocardial cells. Hyperglycemia may also contribute to myocardial injury.7 Valdivia et al8 reported a series of 32 children with scorpion bites who developed cardiac complications. Among these, 50% exhibited myocarditis, 12.5% had subclinical disease, and 63% had observable ECG changes. We observed both ECG changes and myocarditis in this case.

Conclusion We report here the case of an 18-year-old girl who developed life-threatening, acute toxic myocarditis and pulmonary edema, after a scorpion bite. Aggressive medical treatment with inotropic agents, diuretics and parenteral corticosteroids resulted in rapid clinical resolution.

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CASE REPORT References 1. Erfati P. Epidemiology, symptomatology and treatment of buthinae stings. In: Arthpod Venoms. Handbook of Experimental Pharmacology. Bettini S (Ed.), SpringerVerlag: New York 1978:p.312-5.

2. Bawaskar HS. Personal communication. 1998.

3. Bawaskar HS, Bawaskar PH. Indian red scorpion envenoming. Indian J Pediatr 1998;65(3):383-91.

4. Bahloul M, Kallel H, Rekik N, Ben Hamida C, Chelly H, Bouaziz M. Cardiovascular dysfunction following severe scorpion envenomation. Mechanisms and physiopathology. Presse Med 2005;34(2 Pt 1):115-20.

5. Gueron M, Yaron R. Cardiovascular manifestations of severe scorpion sting. Clinicopathologic correlations.

Chest 1970;57(2):156-62.

6. Bawaskar HS, Bawaskar PH. Management of the cardiovascular manifestations of poisoning by the Indian red scorpion (Mesobuthus tamulus). Br Heart J 1992;68(5):478-80.

7. Garg AK, Pimparkar AB, Abraham P, Chikhalikar AA. Myocarditis and pulmonary edema following scorpion bite. (A case report). J Postgrad Med 1983;29(1):46-8.

8. Valdivia HH, Kirby MS, Lederer WJ, Coronado R. Scorpion toxins targeted against the sarcoplasmic reticulum Ca(2+)-release channel of skeletal and cardiac muscle. Proc Natl Acad Sci U S A 1992;89(24):12185-9.

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Journal of Applied MEDICINE & SURGERY

CASE REPORT

Information for Authors

Manuscripts should be prepared in accordance with the ‘Uniform requirements for manuscripts submitted to biomedical journals’ compiled by the International Committee of Medical Journal Editors (Ann Intern Med 1992-96:766-767). The Journal strongly disapproves of the submission of the same articles simultaneously to different journals for consideration as well as duplicate publication and will decline to accept fresh manuscripts submitted by authors who have done so.

The boxed checklist will help authors in preparing their manuscript according to our requirements. Improperly prepared manuscripts may be returned to the author without review. The checklists should accompany each manuscript.

Covering letter: The covering letter should explain if there is any deviation from the standard IMRAD format (Introduction, Methods, Results and Discussion) and should outline the importance of the paper. Principal/ Senior author must sign the covering letter indicating full responsibility for the paper submitted, preferably with signatures of all the authors. Articles must be accompanied by a declaration by all authors stating that the article has not been published in any Journal/Book. Authors should mention complete designation and departments, etc., on the manuscript.

Manuscript: Three complete sets of the manuscript should be submitted and preferably with a CD; typed double spaced throughout (including references, table and legends to figures). The manuscript should be arranged as follow: Covering letter, Checklist, Title page, Abstract, Keywords (for indexing, if required), introduction, Methods, Results, Discussion, References, Tables, Legends to Figures and Figures. All pages should be numbered consecutively beginning with the title page.

Types of Submission: Original Research articles, Review articles, Case reports and Clinical study

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Materials and Methods: Theses should be described in sufficient details to permit evaluation and duplication of the work by others. Ethical guidelines followed by the investigations should be described.

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Results: These should be concise and include only the tables and figures necessary to enhance the understanding of the text.

Prof. Jayakar Thomas, Department of Dermatology and STD, Sree Balaji Medical College & Hospital, #7, Works Road, Chromepet, Chennai - 600 044. Tamil Nadu, India.

Discussion: This should consist of a review of the literature and relate the major findings of the article to other publications on the subject. The particular relevance of the results to healthcare in India should be stressed, e.g., practically and cost.

Journal of Applied Medicine & Surgery, Vol. 2, Issue 4 April-June 2013

It is mandatory to send a soft copy to jamsbharath@gmail.com

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