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Idiopathic Peritoneal Sclerosis: Case Presentation, And Literature Review

1. Abstract 1.1. 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. 1.2. Case Presentation: 54 years 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. 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 TB. Patient was referred to tertiary center for further management and was discussed internationally with peritoneal sclerosis expertise. He recovered on conservative measure and tapering doses of steroids. He is still experiencing recurrent small bowel obstruction which usually resolve using steroids and conservative measures. 1.3. Conclusion: Idiopathic Peritoneal Sclerosis is a rare condition with high mortality and morbidity. It poses a very challenging management dilemma. 2. Introduction Most of the literature regarding Encapsulated Peritoneal Sclerosis (EPS) is derived from nephrology literature surrounding peritoneal dialysis as the main and primary cause of this very rare and devastating disorder [1]. The primary aim of this abstract is to present a case of idiopathic peritoneal sclerosis and elaborate further on this rare condition with exceptionally low incidence. 3. Case Presentation 54 years old male patient with background medical history of traumatic brain injury and alcoholic liver disease, presented with 9months history of intermittent progressive vomiting, constipation and weight loss in 2014. Clinically, his abdominal examination was unremarkable. Blood investigations showed elevated inflammatory markers. CT abdomen and pelvis was reported as subacute distal small bowel obstruction (Figure 2). He didn’t respond to conservative management. Subsequently, He underwent an exploratory laparotomy that revealed a cocooned matted small bowel loops resulting in a frozen abdomen. The small bowel was thickened and had a leathery feel. All structures at the root of the mesentery were covered by a thick sheet of fibrous tissue including the mesentery (Figure 1). Peritoneal specimens were taken for microbiology and histology, report of the latter was consistent with encapsulating peritoneal sclerosis. Notably, it was negative for TB (Figure 3).