Idiopathic encapsulating peritoneal sclerosis: a case report

INTRODUCTION

Encapsulating peritoneal sclerosis (EPS) is a rare cause of intestinal obstruction. It is characterised by partial and complete encapsulation of small bowel with a dense white fibrotic membrane.^1-5 Depending upon the cause, it is further divided as idiopathic (primary) and secondary. Primary EPS was first observed by Owtschinnikow in 1907 and was called chronica fibrosa encapsulate.⁶ The presentation is nonspecific and the diagnosis is usually made intraoperatively. Medical treatment includes antiinflammatory or antifibrogenic drugs such as tamoxifen, steroids, colchicine, azathioprine and mycophenolate mofetil.⁷ Surgical management includes membrane resection and adhesiolysis with or without bowel resection.

CASE REPORT

A 16-year-old female, presented with the complaint of pain in abdomen, abdominal distension for 5 days; nausea and vomiting with non-passage of faeces and flatus for 2 days. There was history of similar episodes in past and was managed conservatively. There was no history of tuberculosis or Koch’s contact and no other significant medical history. On examination abdomen was distended and diffuse tenderness with no guarding and rigidity was present. X-ray abdomen showed dilated jejunal loops with multiple air fluid levels.

Contrast enhanced computed tomography (CECT) abdomen showed dilated proximal jejunal loops with clumped distal jejunal and ileal loops surrounded in a peritoneal sac. There was no evidence of mesenteric lymphadenopathy and ascites.

Exploratory laparotomy was done. Intraoperatively, proximal 70 cm of jejunum was found to be dilated with remaining jejunum and ileum encased in dense peritoneal sac that was firmly adhered to terminal ileum. There was no ascites or mesenteric lymphadenopathy. Peritoneal sac was excised and adhesiolysis was done. Approximately 180 cm bowel length was regained. Postoperative period was uneventful. Histopathology report showed nonspecific inflammatory changes, with no findings suggestive of tuberculosis. Patient was orally allowed on post-operative day (POD-3) and was discharged on POD-7. On follow up there was no evidence of any post op complication or recurrent obstruction.

DISCUSSION

EPS is rare with a mortality rate of 60-93%.² Depending upon the cause it is divided as primary (idiopathic) and secondary. Incidence of idiopathic EPS is 0.4-5.5%.² It is mostly observed in adolescent girls living in tropical and subtropical countries, although may be seen in children living in temperate regions or adults of advanced age.¹ Exact cause of idiopathic EPS is not known, however many theories to explain the primary aetiology, includes viral peritonitis due to infection from retrograde menstruation, developmental malformations, greater omentum hypoplasia, and malformation of mesentric vessels.^2,3 Most common secondary cause worldwide is chronic peritoneal dialysis (2). 3% of the patients on peritoneal dialysis develop EPS. Abdominal tuberculosis is one of the most common causes of secondary EPS in underdeveloped countries.¹¹ Other causes includes prior abdominal surgeries, prolonged beta blocker therapy, radiation enteritis, sarcoidosis, SLE, ventriculoperitoneal shunts, and malignancy.^8-10

Clinical presentation includes asymptomatic course, acute or recurrent subacute or chronic intestinal obstruction.^1,2,6 X-ray abdomen shows dilated bowel loops with air fluid levels, consistent with intestinal obstruction. CT scan is the most useful radiological investigation for its diagnosis.² Since most of the patients presents to emergency department with acute abdomen, diagnosis is ususally made interaoperatively.⁵

Asymptomatic patients are put on regular follow up.⁶ Mildly symptomatic patients are best managed by conservative management that includes intestinal rest, nasogastric decompression and nutritional support (enteral or parenteral).^3,7 Cases with no regression of symptoms may be treated with anti-inflammatory or antifibrogenic drugs such as tamoxifen, steroids, colchicine, azathioprine and mycophenolate mofetil.⁷ Surgical management is the main stay of treatment for patients not responding to conservative management and those presenting with intestinal obstruction. It includes resection of peritoneal sac, enterolysis with or without bowel resection.

CONCLUSION

High degree of suspicion is fundamental for diagnosis of EPS. Surgical excision and adhesiolysis is usually curative.

Funding: No funding sources

Conflict of interest: None declared

Ethical approval: Not required

REFERENCES

1. 1. Akbulut S, Yagmur Y, Babur M. Coexistence of abdominal cocoon, intestinal perforation and incarcerated Meckel's diverticulum in an inguinal hernia: A troublesome condition. World J Gastrointest Surg. 2014;6(3):51-4.
2. 2. Tannoury JN, Abboud BN. Idiopathic sclerosing encapsulating peritonitis: abdominal cocoon. World J Gastroenterol. 2012;18(17):1999-2004.
3. 3. Li N, Zhu W, Li Y, Gong J, Gu L, Li M, et al. Surgical treatment and perioperative management of idiopathic abdominal cocoon: single-center review of 65 cases. World J Surg. 2014;38(7):1860-7.
4. 4. Solak A, Solak I. Abdominal cocoon syndrome: preoperative diagnostic criteria, good clinical outcome with medical treatment and review of the literature. Turk J Gastroenterol. 2012;23(6):776-9.
5. 5. Serter A, Kocakoc E, Cipe G. Supposed to be rare cause of intestinal obstruction; abdominal cocoon: report of two cases. Clin Imaging. 2013;37(3):586-9.
6. 6. Xu P, Chen LH, Li YM. Idiopathic sclerosing encapsulating peritonitis (or abdominal cocoon): a report of 5 cases. World J Gastroenterol. 2007;13(26):3649-51.
7. 7. Habib SM, Betjes MG, Fieren MW, Boeschoten EW, Abrahams AC, Boer WH, et al. Management of encapsulating peritoneal sclerosis: a guideline on optimal and uniform treatment. Neth J Med. 2011;69(11):500-7.
8. 8. Mann RD. An instructive example of long-latency adverse drug reaction–sclerosing peritonitis due to practolol. Pharmacoepidemiol Drug Saf. 2007;16(11):1211-6.
9. 9. Levine S, Saltzman A. Abdominal cocoon: an animal model for a complication of peritoneal dialysis. Perit Dial Int. 1996;16(6):613-6.
10. 10. Suh WN, Lee SK, Chang H, Hwang HJ, Hyung WJ, Park YN, et al. Sclerosing encapsulating peritonitis (abdominal cocoon) after abdominal hysterectomy. Korean J Intern Med. 2007;22(2):125-9.
11. 11. Singh B, Gupta S. Abdominal cocoon: a case series. Int J Surg. 2013;11(4):325-8.