J Islamabad Med Dental Coll 2023 150 Open Access A Case Report on Abdominal Cocoon Syndrome Fahad Akhtar1, Kanza Farrukh 2, Sidra Rauf 3, Aiman Noor 4, Manzer Mehmood 5 1,2,5 Post Graduate Resident, Department of General Surgery, Pakistan Institute of Medical Sciences, Islamabad 3Post Graduate Resident, Department of Gynae and Obstetrics ,Pakistan Institute of Medical Sciences, Islamabad 4Post Graduate Resident, Department of Anesthesia, Pakistan Institute of Medical Sciences, Islamabad A B S T R A C T Abdominal cocoon syndrome (ACS) is encapsulation of the abdominal organs by thick fibro-collagenous material forming cocoons. On the basis of the part of abdominal organ encapsulated, ACS is classified into three types. Patients with this condition usually present with intestinal obstruction. We present a case who presented to us with features of intestinal obstruction and was diagnosed as a rare case of abdominal cocoon syndrome on CT scan. He was managed operatively where adhenolysis was performed and patient had uneventful recovery. CT scan is found to be the investigation of choice. Laparotomy with excision of membrane overlying bowel loops is the treatment most commonly adopted by surgeons and it has satisfactory results. Key words: Cocoon syndrome, Computed tomography, Intestinal obstruction. Cite this article. Akhtar F, Farrukh K, Rauf S, Noor A, Mehmood M. A Case Report on Abdominal Cocoon Syndrome. J Islamabad Med Dental Coll. 2023; 12(2): 150-152 DOI: https://doi.org/10.35787/jimdc.v12i2.988 Funding Source: Nil Conflict of interest: Nil I n t r o d u c t i o n Abdominal cocoon syndrome (ACS) is a encapsulation of the abdominal organs by thick fibro-collagenous membrane forming cocoons. There are two types of abdominal cocoon syndrome i.e., primary and secondary. Primary also known as idiopathic, is the one in which no associated cause has been identified. It is found to be linked with retrograde menstruation and therefore is more common in young females. The secondary type is associated with various conditions like trauma, peritoneal dialysis, peritonitis, previous abdominal surgery, sarcoidosis, tuberculosis, autoimmune disorders, pelvic inflammatory disease, endometriosis, beta-blockers, chemotherapy, hepatitis C, liver transplantation or gastrointestinal malignancy.1 This rare syndrome mostly presents as intestinal obstruction. Computed tomography (Ct scan) being radiological investigation of choice for this condition.2 Diagnosis of this condition is difficult to establish, pre operatively. Therefore, mostly the condition is diagnosed intra operatively when its pathognomonic features of thick fibrous membrane encapsulating abdominal viscera are seen. 3 Here we present a case who presented to us with intestinal obstruction diagnosed as cocoon syndrome. C a s e R e p o r t A 35 years old male man reported to us in emergency with complain of pain in abdomen for the past 10 days associated with vomiting, constipation and abdominal distension from 8 days along with multiple episodes of vomiting, bilious in nature and patient hadn’t pass stool and flatus from 8 days. Following these complaints patient developed abdominal distension as well. On C a s e R e p o r t J Islamabad Med Dental Coll 2023 151 examination his GCS was 15/15, with pulse of 110/min, blood pressure of 95/60, and was maintaining SpO2 at room air. Chest auscultation showed B/L equal air entry. On abdominal inspection his whole abdomen was distended with symmetrical appearance and central umbilicus. Palpation showed generalized tense abdomen and was resonant on percussion with sluggish bowel sounds. Rest of the systemic examination was unremarkable. Patient was initially managed conservatively. Work up was done that showed all baseline investigations, GeneXpert MTB and AFB direct smear were negative. Erect Abdominal X- revealed multiple air fluid levels. Abdominal Ultrasound showed matted fluid filled gut loops with sluggish peristalsis in central abdomen. CT scan abdomen with IV contrast was carried out that reported dilated encapsulated thick walled jejunal loops in left upper hemiabdomen. Intervening gut loops showed streak of fluid and engorged mesenteric vessels. Overall features suggested intraperitoneal adhesions with small gut encapsulated at multiple levels along with small bowel obstruction (abdominal cocoon) (figure- 1). Figure 1: Per-operative findings showing multiple bands with dilated small bowel. Figure 2: CT Scan Abdomen showing intraperitoneal adhesions encapsulating small gut. As patient didn’t respond to conservative management, he had exploratory laparotomy. Per- op findings were; 500ml ascitic fluid, thick fibrous membrane enclosing whole small bowel with multiple bands and markedly dilated small bowel (figure-2) for which Adhesiolysis was conducted. Post-operatively patient recovered uneventfully. D i s c u s s i o n Abdominal cocoon syndrome is a condition that involves peritoneum.4 It is considered a variation of retroperitoneal fibrosis or Ormond disease. On the basis of the part of abdominal organ encapsulated, ACS is further classified into 3 types. In type 1, only part of the small intestine is encased while in type 2, the entire small intestine is covered by membrane and in last type, abdominal organs like stomach, ovaries, liver or appendix can be encapsulated.2 Patients of this condition have complaints of abdominal pain and constipation associated with vomiting. Abdominal X-ray in erect view shows multiple air- fluid levels, finding being non-specific. Sonography usually reveals dilated bowel loops, and specific trilaminar appearance formed by the gut wall, overlying membrane, and posterior abdominal wall with which bowel loop is adherent.5 The J Islamabad Med Dental Coll 2023 152 findings on CT scan are consistent with features of intestinal obstruction along with of abdominal cocoon in which there are conglomerated, adherent gut loops enclosed in thick peritoneal membrane giving an appearance of cauliflower sign in the center of abdomen. If secondary ACS is suspected then, further investigations like ESR, Gene Xpert for tuberculosis and even biopsies with diagnostic laparoscopy are taken to rule out inflammatory, malignant, autoimmune and gynecological cause.6 Exploratory laparotomy with complete excision of membrane and adhesiolysis is considered the surgical treatment of choice. Abdominal cocoon syndrome must be considered by surgeons to be a cause of intestinal obstruction after other causes been ruled out. Computed tomography of the abdomen is a useful radiological investigation for establishing preoperative diagnosis. Adhesiolysis is the treatment of choice. In general, the outcome of this management showed satisfactory results. R e f e r e n c e s 1. Krishna Dhali G, Akbulut S, Minh Duc N, Alsadery HA. Abdominal cocoon syndrome (idiopathic sclerosing encapsulating peritonitis): An extremely rare cause of small bowel obstruction—Two case reports and a review of literature. https://www.frontiersin.org/journals/medicine. 12 October 2022 DOI 10.3389/fmed.2022.1003775 2. Karona P, Blevrakis E, Kastanaki P, Tzouganakis A, Kastanakis M. Abdominal Cocoon Syndrome: An Extremely Rare Cause of Small Bowel Obstruction. Cureus. 2021 Apr 7; 3. Çolak Ş, Bektaş H. Abdominal cocoon syndrome: A rare cause of acute abdomen syndrome. Ulusal Travma ve Acil Cerrahi Dergisi. 2019;25(6):575–9. 4. Singh AU, Subedi SS, Yadav TN, Gautam S, Pandit N. Abdominal cocoon syndrome with military tuberculosis. Clin J Gastroenterol. 2021 Apr 1;14(2):577–80. 5. Chorti A, Panidis S, Konstantinidis D, Cheva A, Papavramidis T, Michalopoulos A, et al. Abdominal cocoon syndrome: Rare cause of intestinal obstruction - Case report and systematic review of literature. Vol. 101, Medicine (United States). Lippincott Williams and Wilkins; 2022. p. E29837. Chorti et al • Medicine 101(27):p e29837, July 8, 2022. | DOI: 10.1097/MD.0000000000029837 6. Aziz W, Malik Y, Haseeb S, Mirza RT, Aamer S. Abdominal Cocoon Syndrome: A Laparoscopic Approach. Cureus 2021. 13(7): e16787. doi 10.7759/cureus.16787 https://www.frontiersin.org/journals/medicine https://journals.lww.com/md-journal/toc/2022/07080 https://journals.lww.com/md-journal/toc/2022/07080