AB187. Idiopathic peritoneal sclerosis: case presentation and literature review

Background: Most of the literature regarding peritoneal sclerosis is derived from nephrology literature surrounding peritoneal dialysis as the main and primary cause of this very rare and devastating disorder. The primary aim of this abstract is to encounter a case presentation of idiopathic peritoneal sclerosis and elaborate further on this rare condition.

Methods: A 54-year-old male patient, presented with 9 months history of intermittent progressive vomiting and significant weight loss in 2014. Clinically his abdomen was soft not tender but distended. His blood tests showed elevated inflammatory markers. Computerized tomography (CT) abdomen and pelvis showed sub-acute distal small bowel obstruction. He didn’t respond to conservative management. He underwent a laparotomy that revealed a frozen, matted small bowel loops which were thickened and leathery feeling. All structures at the root of the mesentery were covered by a thick sheet of fibrous tissue including the mesentery. Peritoneal biopsies were taken for histology and microbiology. The report of which was consistent with encapsulating peritoneal sclerosis. Notably, it was negative for tuberculosis (TB).

Results: Patient was referred to tertiary centre for further management and was discussed internationally with peritoneal sclerosis expertise. He recovered on conservative measure. He is still experiencing recurrent small bowel obstruction which usually resolve using tapering doses of steroids and conservative measures.

Conclusions: Idiopathic Peritoneal Sclerosis is a rare condition with high mortality and morbidity. It poses a very challenging management dilemma.

Keywords: Idiopathic peritoneal sclerosis; steroids; surgery