Abdominal Cocoon Syndrome: A rare Cause of Acute Abdomen Syndrome

Abstract

BACKGROUND: A rare cause of acute abdomen or intestinal obstruction, the abdominal cocoon syndrome is also described in the literature as sclerosing peritonitis or sclerosing encapsulating peritonitis. Abdominal cocoon is characterized by the total or partial wrapping of the abdominal organs by a fibrous membrane. Although it is usually observed in young women, the etiology is unknown. The diagnosis is usually made during laparotomy. In this case series, we aimed to present seven patients diagnosed with abdominal cocoon syndrome during operation. METHODS: The records of patients who underwent laparotomy for abdominal pain and/or intestinal obstruction in our hospital and diagnosed as abdominal cocoon during operation between January 2012 and November 2018 were retrospectively reviewed. The demographic characteristics of the patients, etiologic factors, surgical procedures, operative findings and follow-up of the patients were recorded. RESULTS: Four out of seven patients who were operated for abdominal cocoon were male and 3 of them were female. The median age of patients was 61 (57-63) years in male and 39.6 (28-49) years in female. Six of the patients were operated in emergency conditions with the diagnosis of an acute abdomen or ileus. One of the patients was operated with the diagnosis of an intra-abdominal mass in elective conditions. In five out of seven patients, all of the small intestines were wrapped with a fibrous collagen capsule, while two of the patient intestines were partially wrapped with a fibrous collagen capsule. Four of the patients had no underlying disease, while one of the patients had Familial Mediterranean Fever (FMF), one had Endometriosis and one had beta-blocker medication. One patient who had small bowel necrosis and septic peritonitis were observed during the operation and died post operative 6 th days. Postoperative complications were not observed in the follow-up of other patients and reoperation was not required due to recurrence. CONCLUSION: Abdominal cocoon is a condition that is usually diagnosed during operation in patients that were operated for reasons, such as the acute abdomen or intestinal obstruction. When the diagnose delayed, death can be seen due to small bowel necrosis and septic complications. High clinical suspicion and radiological imaging are important in the preoperative diagnosis. Treatment is required adhesiolysis and excision of the fibrous membranes.

A Diagnostic Dilemma for Acute Abdomen: Sclerosing Encapsulating Peritonitis (Abdominal Cocoon Syndrome); A Retrospective Cohort Study

“…This difference cannot change the knowledge about the disorder but it can warn us to be more careful about secondary forms and male patients. 1,3 The secondary form may result from different etiological pathologies. After a report by Gandhi et al introduced ambulatory peritoneal dialysis catheter as a cause of secondary SEP, different etiologic pathologies have been reported in case reports and case series.…”

“…SEP can be identified as the primary form without any underlying cause (abdominal cocoon syndrome, ACS) or the secondary form which may be related to abdominal surgical interventions, intraabdominal infections, chronic peritoneal dialysis, liver transplantation, ventriculoperitoneal shunt, and some drugs. 3,4 The primary form is more common than the secondary form. 5 The idiopathic/primary SEP was first described at the end of the nineteenth century and as primary SEP in the beginning of the 20th century, while the secondary SEP was first described by Gandhi et al in 1980.…”

Abdominal Cocoon Syndrome in a 13-Weeks Pregnant Woman

“…Differential diagnosis should include peritoneal encapsulation, which is a development anomaly where the whole of the small bowel is encased [13]. Other differential diagnosis includes perforated viscus or internal herniation and other causes of abdominal obstruction [14] Treatment. Therapeutic options vary from conservative to open or laparoscopic surgical intervention [4,15,11].…”

“…Abdominal ascites might be detected in longstanding cases [1]. Infrequently present chronic constipation, anorexia, weight loss, intrabdominal masses, perforation or ischemia-related necrosis [14].…”

“…Non-mobile intestines may cause asymmetric abdominal distention. Besides, in palpation of the abdominal wall, enlarged bowel in the proximal of the adhered section is palpated soft, while the obstructed zone is palpated rigid, flat and painless due to fibrous tissue [14,19] Complications.…”