One center experience of pneumatosis cystoides intestinalis

Abstract

Introduction

Pneumatosis cystoides intestinalis (PCI) is a rare benign condition, in which gas is found in a linear or cystic form in the subserosa or submucosa. The subserous cysts are most frequently found in the small bowel while the submucous localizations are predominantly seen in the colonic wall. Peritoneal pneumatosis, abdominal gas cysts, cystic lymphopneumatosis, intestinal emphysema or intestinal gas cysts are terms used to describe the occurrence of multiple, gas-filled cysts, of the gastrointestinal tract. Incidence of PCI was reported to be 0.03% in the general population. It is a radiographic finding and not a diagnosis, as the etiology varies from benign conditions to fulminant gastrointestinal disease.

Case description

A 77-year-old patient was admitted in Pauls Stradins Clinical University Hospital with complains of abdominal discomfort and bloating during two years. Blood laboratory tests revealed no changes in the blood count, CRP (C reactive protein) was 77.6mg/l (N<5mg/l). A colonoscopy showed c.sigmoideum, c.descendens submucosal lesion in 10-15cm zone with submucous cystic formations, visually reminiscent of "a bunch of grapes, which are connected to each other", filled with a whitish, in some areas bluish content, with unchanged superficial mucosae.

Results

Since 2011, lesion was increased in size of 5cm. Endoscopic ultrasound showed formation of submucosal anehogenic mass; 11-17mm thick, blurring, palpable densely, minimally vascularized with no signs of malignancy. A computer tomography (CT) scan of the abdomen and pelvic area, and retroperitoneal space revealed infiltrative mass in the wall of the c.sigmoideum. Colonoscopy was performed for tumor location, followed by laparoscopic resection of the tumor mass. Morphological examination of full specimen revealed pneumatosis cystoides intestinalis in size of 11.5x5.6x4cm, with multicore foreign body type gigantic cells on the inner surface of the cysts, and no signs of malignancy.

Take-home message

Our case, pneumatosis cystoides intestinalisis is a rare disease, which is difficult to diagnose by radiology or endoscopy, even for exclusion of malignancy. PCI is an indication for surgery if the lesion is growing in size and may cause the symptoms of colon obstruction.