Congenital peritoneal encapsulation: A review and novel classification system

Abstract

Congenital peritoneal encapsulation (CPE) is a very rare, congenital condition characterised by the presence of an accessory peritoneal membrane which encases a variable extent of the small bowel. It is unclear how CPE develops, however it is currently understood to be a result of an aberrant adhesion in the peritoneal lining of the physiological hernia in foetal mid-gut development. The condition was first described in 1868, and subsequently there have been only 45 case reports of the phenomenon. No formal, systematised review of CPE has yet been performed, meaning the condition remains poorly understood, underdiagnosed and mismanaged. Diagnosis of CPE remains clinical with important adjuncts provided by imaging and diagnostic laparoscopy. Two thirds of patients present with abdominal pain, likely secondary to sub-acute bowel obstruction. A fixed, asymmetrical distension of the abdomen and differential consistency on abdominal palpation are more specific clinical features present in approximately 10% of cases. CPE is virtually undetectable on plain imaging, and is only detected on 40% of patients with computed tomography scan. Most patients will undergo diagnostic laparotomy to confirm the diagnosis. Management of CPE includes both medical management of the critically-unstable patient and surgical laparotomy, partial peritonectomy and adhesiolysis. Prognosis following prompt surgical treatment is excellent, with a majority of patients being symptom free at follow up. This review summarises the current literature on the aetiology, diagnosis and treatment of this rare disease. We also introduce a novel classification system for encapsulating bowel diseases, which may distinguish CPE from the commoner, more morbid conditions of abdominal cocoon and encapsulating peritoneal sclerosis.

Congenital peritoneal encapsulation presented with small bowel obstruction

“…CPE is usually asymptomatic, and the preoperative diagnosis is challenging as it is rarely complicated by intestinal obstruction. If small bowel obstruction is evident, plain radiographs probably will be able to detect the dilated small bowel loops or multiple air-fluid levels; however, CT may be able to detect the membranous capsule and the sac, as well as, any underlying complications [1]. Mimics such as paraduodenal hernias can be excluded by their specific CT appearance.…”

“…The two other peritoneal encapsulation conditions that should be differentiated from CPE are abdominal cocoon (AC) and sclerosing encapsulating peritonitis (SEP). Both conditions are acquired but with different pathogenesis and etiology [1].…”

“…Congenital peritoneal encapsulation (CPE) is a rare congenital condition, characterized by the development of an accessory peritoneal layer by the 12th week of gestation which creates an accessory peritoneal sac that partially or completely encapsulates the small bowel [1][2][3]. The majority of the cases are asymptomatic, and few reported cases presented with bowel obstruction.…”

Secondary Abdominal Cocoon Syndrome Due To Chronic Beta-Blocker Use

“…While the pathogenesis of Idiopathic SEP is poorly understood, studies suggest that the fibrosis occurs secondary to unregulated fibroblasts activity and cytokine release. Additional theories postulate that because SEP is often accompanied by embryologic abnormalities, including omental hypoplasia, it may occur as a consequence of a developmental disorder [2][3][4][5][6][7][8][9][10]. While retrograde menstruation with a superimposed viral infection has been thought to cause this condition, the fact that a majority of patients with SEP are men, preadolescent, and postmenopausal women makes this less likely [2].…”

“…SEP or "abdominal cocoon syndrome" is often misidentified as a variant of abdominal encapsulation syndrome called congenital peritoneal encapsulation [4]. These conditions, however, are different.…”