A Case of Encapsulating Peritoneal Sclerosis Associated With Ventriculo-Peritoneal Shunt

Introduction: Encapsulating peritoneal sclerosis (EPS) is a rare complication commonly associated with long term peritoneal dialysis and is characterized by intraperitoneal inflammation and fibrosis, resulting in the encasement of bowel loops. EPS is associated with significant morbidity and high mortality. We report a case of a 37-year-old man with this rare condition. Case Description/Methods: A 37-year-old man with a history of Gilbert’s syndrome and hydrocephalus status post ventricloperitoneal shunt (VP shunt) at birth with 7 revisions, was referred to the GI clinic for evaluation of a 2-year history of intermittent abdominal pain, nausea, and vomiting. He denied any aggravating or relieving factors, weight loss or bowel changes. His vital signs were normal and physical exam was unremarkable. A CT scan of abdomen/pelvis and MRI revealed a thin membrane-like structure encapsulating multiple centrally located small bowel loops, consistent with encapsulating peritoneal sclerosis (Figure 1). The VP shunt was in a stable position terminating in the left mid-abdomen and there was no evidence of bowel obstruction. Laboratory parameters including complete blood count, liver and kidney function tests, CRP and QuantiFERON were unremarkable except for elevated indirect bilirubin, in keeping with Gilbert’s syndrome. EGD only showed LA grade A esophagitis. Patient is planned for corticosteroid therapy. Discussion: Encapsulating peritoneal sclerosis is a debilitating condition. The exact cause is unknown but medications, peritoneal infection, and systemic inflammatory disorders have been implicated. EPS is a rare complication of VP shunting and only a few cases have been reported. Diagnosis is often delayed given the rarity of the condition and many patients undergo exploratory laparotomy for diagnosis. EPS causes bowel obstruction and is associated with significant morbidity and a high mortality approaching 50% 1-year after diagnosis. Our patient’s symptoms were most likely due to encapsulating peritoneal sclerosis, a complication of his VP shunt. He may have had recurrent partial small bowel obstructions over the years, contributing to his intermittent abdominal pain. Treatment for EPS involves corticosteroid in the inflammatory phase and tamoxifen in the fibrotic stage. In clinical practice, it may be difficult to differentiate between the 2 stages, hence both medications may be used together. Peritonectomy and enterolysis, may be considered for patients who fail medical treatment.Figure 1.: Contrast-enhanced CT images of the abdomen and pelvis in the Coronal (A) and Axial (B) plane demonstrate a thin, membrane-like structure (yellow arrowheads) encapsulating centrally located small bowel loops (orange asterisk), with a small volume of fluid in between bowel loops (white asterisk). Note the ventriculoperitoneal shunt catheter within the fluid (white arrowhead). T2 weighted images MR images of the abdomen performed 6 months later in the Coronal (C) and Axial (D) plane demonstrated the similar position of the small bowel loops (orange asterisk) encapsulated by a thin hypointense membrane (yellow arrowheads).