Sclerosing encapsulating peritonitis with perforation of the gastrointestinal tract: a serious complication of continuous peritoneal dialysis

To the Editor: Sclerosing encapsulating peritonitis (SEP) is one of the most serious complications of peritoneal dialysis. It is characterized by partial or intermittent bowel obstruction and survives subsequent high morbidity and mortality. ¹ A 36-year-old female patient was admitted to the nephrology department with a 24-hour history of abdominal pain and intermittent vomiting. The patient had had chronic renal failure with right renal agenesis for 13 years. SEP was established 8 months previously, and peritoneal dialysis, performed for a long period, was stopped. The general surgery department was consulted. On physical examination, a tender lump was palpated in the umblical region of the abdomen with mild muscle guarding and rebound tenderness. Plain abdominal x-ray showed dilated loops of small bowel with air-fluid levels and free gas under the diaphragm. Abdominal ultrasonography revealed dilated bowel loops, especially in the center of the abdomen. There were localized fluid collections in whole recesses. At laparotomy, 400 mL of purulent fluid was aspirated. A fibrous capsule covering all abdominal viscera was revealed (Figures 1 and 2). The liver, stomach, appendix, small bowel loops, as well as the whole colon were also covered with this fibrous capsule, which appeared like a cocoon. Dissection of adhesions from other was impossible. Although we tried to find the perforation, we could not find it because of extensive adhesions and the fragility of the tissues. Histology of the peritoneal membrane showed thickened fibrocollagenous tissue with chronic non-specific inflammation. An enterocutaneous fistula developed on the fifth postoperative day. The patient died from sepsis 3 weeks after the operation.

Figure 1.

The fibrous capsule covering all abdominal viscera.

Figure 2.

The fibrous capsule covering all abdominal viscera.

SEP is rare. The idiopathic form (also known as abdominal cocoon) was first described by Foo et al in 1978.^2,3 It is characterized by a thick grayish-white fibrotic membrane, partially or totally encasing the small bowel.⁴ The fibrocollagenic cocoon sometimes extends to involve other organs such as the colon, liver and stomach. Clinically, it presents with recurrent episodes of acute, subacute or chronic small bowel obstruction, weight loss, nausea and anorexia, and sometimes with a palpable abdominal mass, but some patients may be asymptomatic.⁵ SEP can be classified as idiopathic or secondary. The secondary form of SEP has been reported to occur in association with peritoneal dialysis. Other rare causes include use of beta-blockers, abdominal tuberculosis, ventriculoperitoneal and peritoneovenous shunts, liver transplantation, systemic lupus erythematosus, cirrhosis of the liver, carcinoid syndrome, familial Mediterranean fever, asbestos exposure and recurrent peritonitis. The initial symptoms directly depend on disorders of the gastrointestinal tract. Despite the distinctive clinical findings strongly supporting a diagnosis of SEP, a radiologic examination is required to establish the clinical diagnosis of SEP. Laparotomy or laparoscopy is required to confirm the diagnosis. The key points in conservative treatment are early diagnosis, cessation of peritoneal dialysis with transfer to hemodialysis, sustained bowel rest with total parenteral nutrition, and corticosteroids. In the literature, there is no consensus for the treatment of SEP.^1,5

It is very important to establish diagnosis before development of life-threatening complications of SEP such as intestinal obstruction and perforation because of high morbidity and mortality rates. Prevention and early diagnosis become even more important.