Abdominal Cocoon Syndrome: Two Cases of an Anatomical Abnormality.

Apostolos Sovatzidis,Eirini Nikolaidou,Anastasios Katsourakis,George Noussios,Iosif Chatzis

doi:10.1155/2019/3276919

Abstract

Introduction Idiopathic sclerosing encapsulating peritonitis or abdominal cocoon syndrome (ACS) is a rare anatomical deformity characterized by the partial or complete encasement of the small intestine with fibrotic peritoneum. 193 cases have been described worldwide. The aim of this study is to present two cases of ACS successfully treated at the Surgical Clinic of the Agios Dimitrios General Hospital in Thessaloniki, Greece. Presentation of Cases Two men (55 and 54 years old) presented to the emergency department complaining of abdominal pain, distension, constipation, nausea, and vomiting. Neither of these patients had any previous operations. The computed tomography scan of the first patient showed considerable distension of the small bowel, suggestive of internal herniation. The second case showed distention of the jejunum with no obvious cause. Both patients underwent emergency surgery. Intraoperatively, it was found that a fibrous membrane had completely covered the small intestine of the first patient and the jejunum and part of the large intestine of the second patient. Adhesiolysis and partial excision of the membrane were performed in both cases. Discussion ACS is a rare cause of small bowel obstruction. Although conservative management with immunosuppressants and steroids has been described, surgical treatment is the gold standard. Conclusion Preoperative clinical suspicion of this disease can help determine the diagnosis and protect surgeons from intraoperative “surprises”.

Highlights

Idiopathic sclerosing encapsulating peritonitis or abdominal cocoon syndrome (ACS) is a rare anatomical deformity characterized by the partial or complete encasement of the small intestine with fibrotic peritoneum. 193 cases have been described worldwide
Idiopathic sclerosing encapsulating peritonitis is a rare cause of intestinal obstruction
It was first described in 1908 by Owtschinnikow and it was defined in 1978 by Foo et al [1]. It is characterized by the partial or complete encasement mainly of the small intestine with the peritoneum, leading to chronic inflammation and fibrosis. This rare clinical entity has been described by various names, including peritonitis chronica fibrosa incapsulata, primary sclerosing peritonitis, and abdominal cocoon syndrome (ACS) [2]

Summary

Introduction

Idiopathic sclerosing encapsulating peritonitis is a rare cause of intestinal obstruction. It is characterized by the partial or complete encasement mainly of the small intestine with the peritoneum, leading to chronic inflammation and fibrosis This rare clinical entity has been described by various names, including peritonitis chronica fibrosa incapsulata, primary sclerosing peritonitis, and abdominal cocoon syndrome (ACS) [2]. The countries that reported the greatest number of cases were China, India, Turkey, and Nigeria [3] Patients with this syndrome present themselves typically with small bowel obstruction. The aim of this article was to present two cases of ACS that were successfully treated at the Surgical Clinic of the Saint Dimitrios General Hospital in Thessaloniki, Greece Both cases were concerning middle-aged males with no previous abdominal operations

Presentation of Cases

Discussion

Conclusion

Conflicts of Interest