Xanthogranulomatous appendicitis as a cause of intussusception in the adult - Case Report

Xanthogranulomatous appendicitis as a cause of intussusception in the adult - A case report

António Rivero1, Jose Parra2, Daniel Cartucho1, António Lourenço1

1 . Serviço de Cirurgia II - Centro Hospitalar Universitário Algarve 2 . Serviço de Anatomia Patológica - Centro Hospitalar Universitário Algarve

tonidelgado@gmail.com

Abstract

In adults, intussusception is a rare cause of bowel obstruction. For the diagnosis, we must have a high index of suspicion, a complete anamnestic recall, a physical examination, and imaging modalities. Nonoperative reduction is first tried, but treatment of intussusception in adults is usually surgical, with a segmental bowel resection that includes the nonviable bowel as well as the leading point of the intussusception. The authors present a case of a 42-year-old male patient with a xanthogranulomatous appendicitis, as the etiology of a ileo-cecal intussusception, followed by a discussion of the pathological entity. The authors review cases with xanthogranulomatous appendicitis, as as cause for right hemicolectomy. Far as we know, this is the first case of an Xanthogranulomatous appendicitis as a lead point causing an adult intussusception.

Keywords:

Xanthogranulomatous appendicitis; intussusception; case report

Introduction

Telescoping (prolapse) of a portion of the intestine within another immediately adjacent portion of intestine, an intussusception, is a rare cause of intestinal occlusion in adults. The proximal segment, or intussusceptum, is carried by progressive smooth muscle contractions into the distal segment, or intussuscipiens 1. Most cases of intussusception occur in children between five months and one year of age. The intussusception in adults accounts for about 2%–3% of bowel obstructions and occurs in 2-3 per hundred thousand inhabitants year2 .

In adults 90% of adult intussusceptions will have a lead point, while the remaining 10% are idiopathic. The intussusceptum is typically the result of a mucosal, intramural, or extrinsic lead point that acts as a focal area of traction pulling the proximal portion of bowel into the peristalsing distal portion 1-8 .

Xanthogranulomatous inflammation of the tissues is a chronic destructive process that could involve several organs more frequent in kidney and gallbladder and a very rare pathology in appendicitis. We have found in a internet survey thirty clinical cases reported.

Xanthogranulomatous appendicitis as a leading point of the intussusception, as far as we know is the first case published.

Case Report

A 42 year old male, arrives in the Emergency Department, complaining of moderate abdominal pain, located on the LRQ / right flank, which began 72 hours previously. He denied vomiting and other symptoms related to the gastrointestinal tract. He had had another episode 7 days before with similar characteristics, although of lesser intensity. He had complained of fever the day before. On physical examination, abdominal pain to deep palpation was revealed in the LRQ where a palpable mass / tenderness of 3-5 cm diameter was felt. There were no signs of peritoneal reaction. Lab tests demonstrated a leukocytosis (13,900) with neutrophilia (84.3%) and CRP (195 mg / L) was high. Abdominal ultrasonography revealed distension of the right colon with images related to a tubular invagination of ileal loops through the ileo-cecal valve and extending to the hepatic angle. The vascular wall was preserved (on Doppler).

The patient was admitted for further study and treatment.

An enema showed a stop in the progression of contrast, the middle third of the colon, which defined a threadlike extension of central contrast, probably to the inside of a loop invaginated colon (intussuceptum) cannot exclude the possibility of malignancy, which was ordered by the CT adominal scan whose report was ileal loop invagination inside the colon that excluding lesion formation. During his stay in the Department of Surgery, the patient was practically asymptomatic, , however, with analgesia, he tolerated liquid diet.

He was submitted to laparotomy where the presence of a sticky, yellowish mass in the lower third of the colon was noticed .The contents of the intussusception was the ileo- appendicular - cecalcolon. Several lymph nodes where observed that were induratum resecos. A right hemicolectomy with ileotransversostomia was perfomed, as the suspicion of neoplasia could not be excluded. The histology report identified a xanthogranulomatous appendicitis, with nodes of inflammatory nature.

The patient was discharged uneventfully seven days after the surgery. At two years, the follow-up is normal.

Figure 1. Ultrasound images with several layers / concentric interfaces at the right flank level Ultrasound - In cross-sectional view multiple concentric rings, related to the invaginated intestinal layers. “Target” / “donut” appearance. In longitudinal view it can have the appearance of a “fork”. In both projections, the mesenteric fat invaginated with the intussusceptum demonstrates an eccentric (semi-lunar) area of increased echogenicity. The projections of mesenteric fat invaginated with the intussusceptum demonstrate an eccentric (semi-lunar) area of increased echogenicity.

Figure 2. Cecum in high location, with molding of its inferior and internal aspect, with visualization of a long central canal of reduced calibre terminating in a beak represented by the imprisoned contrast medium in the invaginated area. Eventual distension may existe above the invagination. Images of concentric rings (“coiled-spring”/ “espiral”) – within the invaginated segment which is stretched and whose pleats are perpendicular to the invginated segment.

Figure 3. TC – perpendicular to the invagination demonstrating 3 concentric rings formed by 1) the canal the wall of the intussusceptum (internal layer); 2) mesenteric fat (middle layer); 3)intussuscipiens as the outer layer. Images in “trident”, as a “dome”, as a “crescent”, as“lobster pincer”.

Discussion

Xanthogranulomatous inflammation of the tissues is a chronic destructive process and infrequently, characterized by yellowish nodular masses, consisting of foamy macrophages, plasma cells, lymphocytes and polymorphonuclear cells and occasionally giant cells of foreign bodies and areas of fibrosis.

It affects mainly the kidney and gallbladder. The kidney is the organ where it is primarily found. Other infrequent locations are the endometrium, epididymis, prostate, rectum, bladder, ovary, bone, thyroid and adrenal gland. The differential diagnosis should be made with malakoplakia, a chronic granulomatous entity of unknown etiology and specific clinical manifestations, whose histopathology is characterized by the infiltration of large macrophages (cells Hansenmann) and bodies of MichaelisGutmann (remnants of phagosomes mineralized by iron and calcium deposits) both pathgnomonic of the entity.

There are several theories that attempt to justify the pathology, the appendiceal lumenal obstruction, mucosal ulceration and bacterial infections (gram-negative and less frequently in gram-positive cocci). The release of lipids to the extracellular space, repeated changes in the immune and inflammatory processes are some of the mechanisms that appear to be involved in the genesis. Often presented as non-specific abdominal pain, this was the most common symptom 14. Cramping pain is the single most prevalent symptom manifesting from 41% to 90% of all patients according to different series, with an average time course ranging from 2 months to 5 years . Vomiting and rectal blood, followed in order of frequency. Intestinal obstruction (70%) is most prominent, but less frequent in presentation then acute abdomen (7%). Palpation of an abdominal mass is detected in 24 to 42% of all cases, most associated with pain. The classical triad of vomiting, rectal bleeding and abdominal pain is present only in between 15-20% of cases. The clinical difference between a malignant or benign presentation is that the first is more associated to melaena and occult blood in stools whereas the latter is usually an occlusive disease 2 .

Sonography images on transversal or longitudinal view are described as a target, donut-eye beef. The diagnostic accuracy may decrease with air in the bowel loops if occlusive. CT imaging is the choice for preoperative diagnosis in some 78% of cases by showing a typical image of the target (concentric rings), fat attenuation of the bowel segment involved and / or an intraluminal mass of soft consistency 2. Reported by Ahn 15 CT was considered the most effective and accurate diagnostic tool. Enemas are used only in cases of doubtful diagnosis.

Figure 4. Xanthogranulomatous inflammation is characterized histologically by a collection of lipid-laden macrophages admixed with lymphocytes, plasma cells, neutrophils, and often multinucleated giant cells with or without cholesterol clefts

Figure 5. On gross appearance there is presence of golden yellow mass with abscesses. On microscopy the lesion shows recruitment of acute and chronic inflammatory cells, lipid laden macrophages and foam cells16,17 .

Colonoscopy and flexible sigmoidoscopy are useful in those of colonic origin and suspect of malignancy. Because of the variety of symptoms and different etiologies, it is not possible to define a diagnostic algorithm. However, in clinical practice to diagnose an intussusception it might be a good choice to do an abdominal x-ray and a CT scan.

The lead point can be a malignant neoplasm (intestinal carcinoma, lymphoma, polyps), Meckel’s diverticulum, ectopic pancreatic tissue, endometriosis, inflammatory bowel disease and adhesions. Even an appendix has also been reported as a lead point 13 .

Half of the patients are diagnosed at surgery. The treatment of choice is surgical (laparotomy), but it remains controversial, advocating that reduction if it is suspected benign, should be attempted before resection and resection without reduction, if the lesion is a suspected malignancy, due to possible spread of malignant cells. The technique of choice would be en bloc resection.

We found in an Internet survey, eight clinical cases reported of Xanthogranulomatous appendicitis that led to colon resection 17-24. With 5 males and 3 female, all of the cases were adults (ages 39 - 75, in the fourth decade of life 4 cases, medium 53 years), with the presence of a mass in 8 cases (mass 4; acute appendicitis 2; mucocelo 1; colitis of cecum 1) a right hemicolectomy was done, with an associated procedure in 3.

Our case was a 42-year-old male Caucasian with a mass within intussusception in the context of xanthogranulomatous appendicitis led to a right hemicolectomy. In an interesting report by Guangming, this author obtained a histopathological, interval study (delayed), of appendectomy specimens and found a strong association with granulomatous and xanthogranulomatous appendicitis 12 .

As far as we know, we have reported the first case of an Xanthogranulomatous appendicitis as a lead point causing an adult intussusception. References:

1. Cera S M. Intestinal Intussusception. Clin Colon Rectal Surg. 2008 May; 21(2): 106–113.

2. Azar T, Berger DL. Adult intussusception. Ann Surg 1997;226(2):134–138

3. Takeuchi K, Tsuzuki Y, Ando T et al. The diagnosis and treatment of adult intussusception. J Clin Gastroenterol 2003;36(1):18–21

4. Erkan N, Haciyanh M, Yildirim M, Sayhan H, Vardar E, Polat AF. Intussusception in adults. Int J Colorectal Dis 2005;20:452–456

5. Barussaud M, Regenet N, Briennon X et al. Clinical spectrum and surgical approach of adult intussusception. Int J Colorectal Dis 2006;21:834–839

6. Goh BKP, Quah HM, Chow PKH, et al. Predictive factors of malignancy in adults with intussusception. World J Surg 2006;30:1300–1304

7. Zubaidi A, Al-Saif F, Silverman R. Adult intussusception: a retrospective review. Dis Colon Rectum 2006;49(10):1546– 155.

8. Wang LT, Wu CC, Yu JC, Hsiao CW, Hsu CC, Jao SW. Clinical entity and treatment strategies for adult intussusceptions: 20 years’ experience. Dis Colon Rectum. 2007 Nov;50(11):1941-9.

12. Guangming G; Greenson J K. Histopathology of interval (delayed) appendectomy specimens: Strong association with granulomatous and xanthogranulomatous appendicitis. The American journal of surgical pathology. 2003, vol. 27, no8, pp. 1147-1151

13. Barussaud M, Regenet N, Briennon X et al. Clinical spectrum and surgical approach of adult intussusceptions: a multicentric study Int J Colorectal Dis. 2006 21:834-839.

14. Zubaidi A, Al-Saif F , Silverman R . A Clinical Overview of a Retrospective Study About Adult Intussusceptions: Focusing on Discrepancies Among Previous Studies. Digestive Diseases and Sciences. 2009 Volume 54: 2643-2649.

15. Wang N, Cui XY, Liu Y, Long J, Xu YH, Guo RX, Guo KJ. Adult intussusception: a retrospective review of 41 cases. World J Gastroenterol. 2009 Jul 14;15(26):3303-8.

16. Anadol AZ, Gonul II, Tezel E. Xanthogranulomatous inflammation of the colon: a rare cause of cecal mass with bleeding. South Med J. 2009;102:196-99.

17. Kochhar G, Saha S, Andley M, Kumar A, Kumar A. Xanthogranulomatous appendicitis with a fulminant course: report of a case. J Clin Diagn Res. 2014;8(12):ND01-ND2.

18. Chuang YF, Cheng TI, Soong TC, Tsou MH. Xanthogranulomatous appendicitis. J Formos Med Assoc 2005; 104: 752-754

19. Omori I, Kohashi T, Matsugu Y, Nakahara H, Nishisaka T. A case of xanthogranulomatous appendicitis difficult to differentiate from appendiceal cancer. J Japan Surgical Assoc 2011; 72: 409- 413

20. Kochhar G, Saha S, Andley M, Kumar A, Kumar A. Xanthogranulomatous appendicitis with a fulminant course: report of a case. J Clin Diagn Res. 2014;8(12):ND01-ND2.

21. Chandanwale SS, Dey I, Kaur S, Nair R, Patil AA. Xanthogranulomatous appendicitis mimicking appendicular lump: An uncommon entity. Clin Cancer Investig J 2015; 4: 769-771

22. Kaushik R, Gulati A, Vedant D, Kaushal V. Cytological diagnosis of xanthogranulomatous appendicitis. J Cytol 2017; 34: 48-50

23. Al-Zaidi RS. Xanthogranulomatous Appendicitis: an Unusual Pattern of Appendiceal Inflammation. Saudi J Pathol Microbiol 2018; 3: 115-120 24. Quadri R, Vasan V, Hester C, Porembka M, Fielding J. Comprehensive review of typical and atypical pathology of the appendix on CT: cases with clinical implications. Clin Imaging 2019; 53: 65-77