958 Encapsulating Peritoneal Sclerosis with Pericardial Tamponade: A Case Report

Abstract

Abstract Aim Encapsulating peritoneal sclerosis (EPS) is a rare morbidity associated primarily with peritoneal dialysis. Definitive diagnosis and management can often be prolonged and complicated. The pathogenesis is a two-hit hypothesis of inflammation and myofibroblast differentiation. This report aims to demonstrate the pathogenesis of EPS and provide insight into management. Method We present a unique case of a patient with EPS who follows a ‘classical’ disease course before developing a concomitant pericardial sclerosis, presenting with cardiac tamponade. We explore the proposed pathophysiology and provide a hypothesis for both uncommon pathologies presenting in the same patient. Results A 45-year-old male was treated for EPS and went on to develop a concomitant pericardial sclerosis. Following referral to our centre, the patient underwent a semi-elective surgical enterolysis and peritonectomy for EPS, with excision of all sclerotic and obstructing peritoneal membrane. Two weeks following the surgery, he developed a pericardial tamponade with cardiovascular compromise, unresolved by two separate episodes of pericardiocentesis, leading to surgical intervention. A pericardial pleural window was created via open thoracotomy and 800ml of thick clotted blood was removed from the pericardial cavity. Histology demonstrated pericardial sclerosis. Conclusions This case of EPS is unique given the concomitant pericardial sclerosis. Uraemia may be a common mediator of inflammation in the peritoneum and pericardium, predisposing to sclerosis of both membranes of identical embryological origin. Pericardial sclerosis may be present in EPS patients but may not manifest itself clinically by and large. It also demonstrates that effective surgical intervention can lead to excellent patient outcomes.