1642 Pelvic Congestion Syndrome: An Unusual Manifestation of Sigmoid Pneumatosis Cystoides Intestinalis

Abstract

INTRODUCTION: Pneumatosis cystoides intestinalis (PCI) is a rare condition characterized by gas-filled cysts in the intestinal subserosa and submucosa. We present a rare case of primary PCI affecting sigmoid colon complicated by pelvic congestion syndrome (PCS). CASE DESCRIPTION/METHODS: A 34-year-old morbidly obese female presented with diffuse lower abdominal pain and intermittent foamy diarrhea without blood or mucus for the past 5 months. CT abdomen showed small bubbles of gas in the peritoneum in the left upper quadrant and large amount of cystic pneumatosis in a loop of sigmoid colon. Lab work was unremarkable. She was given one week course of antibiotics. A repeat CT scan 5 months later showed interval resolution of free intraperitoneal air but pneumatosis bubbles had enlarged in size. A colonoscopy was performed which showed large cystic-appearing air-filled bullae in sigmoid colon causing some luminal narrowing and the scope could not be safely advanced beyond mid-transverse colon. Patient underwent laparoscopic sigmoid colectomy and intraoperatively noted to have dilated and tortuous bilateral gonadal veins. Surgical pathology showed multiple clear fluid-filled cysts throughout the length of the specimen with viable and unremarkable proximal and distal resection margins. Patient recovered well from surgery and was discharged with no further complaints. DISCUSSION: PCI is an unusual condition caused by a myriad of underlying pathophysiological processes that range from benign to life-threatening conditions. Primary PCI is rare (15%), while underlying conditions for secondary PCI can be divided into several groups; traumatic, mechanical, inflammatory, autoimmune, infectious, pulmonary and drug (steroids and chemotherapeutic agents) induced. Conservative management is preferable except in cases of clinical deterioration. This case is unique because even after the resolution of free intraperitoneal air the patient continued to have lower abdominal and pelvic pain with worsening of pneumatosis on repeat CT scan several months later. It is possible that sigmoid anatomic changes due to pneumatosis resulted in chronic intermittent venous obstruction of pelvic veins leading to gonadal veins dilation and tortuosity and hence secondary PCS which resulted in ongoing lower abdominal pain. This case highlights the fact that persistent PCI, with ongoing abdominal symptoms even in the absence of any obvious etiology warrants laparoscopic exploration to rule out any underlying disease process.