Pneumatosis cystoides intestinalis

Case contributed by Jeffrey Hocking
Diagnosis almost certain

Presentation

Pain, generalized guarding, previous bowel obstructions/perforations with resections.

Patient Data

Age: 85 years
Gender: Male

Large hiatus hernia.

A grossly dilated loop of large bowel lies over the left side of the abdomen up to a diameter of 11cm with an air-fluid level extending up as high as the left upper quadrant subdiaphragmatic position. Possible beaked appearance to the right of midline in the central abdomen suggesting this is a point of transition/torsion.

Diffuse septated/loculated gas throughout the abdomen surrounding loops of small bowel. Occasional small locules of peritoneal gas, such as adjacent to the medial liver.

Pulmonary fibrosis with bilateral subpleural reticulation, ground-glass opacity and honeycombing. Associated traction bronchiectasis. Bilateral calcified pleural plaques.

IMPRESSION:

Cecal volvulus with diffuse small bowel and mesenteric pneumatosis cystoides intestinalis.

PROCEDURE:

Laparotomy. Pertinent findings as follows:

  • Blood stained fluid
  • Impressive pneumotosis throughout small bowel
  • Cecal volvulus and ischemia with imminent perforation
  • Extensive omental adhesions from previous laparotomies with distorted layout of bowel, tight band to pelvis and right flank likely to be the cause of volvulus.

Case Discussion

Diffuse pneumatosis cystoides intestinalis is a relatively uncommon finding and is often asymptomatic. When present, especially to this degree, an underlying cause is often present. This case is very similar to that discussed in the below case report 1, including the presence of a sigmoid volvulus. Due to the patients repeated bowel resections an intestinal hypermobility resulting in PCI (and the volvulus) is a possibility. 

Small volume pneumoperitoneum is noted in the patient, adjacent to the liver edge. This may be due to 'benign' rupture of one of the cysts, and as such was not noted during laparotomy.

The cause of PCI is debated, with some sources suggesting an association with chronic pulmonary diseases. This patient does appear to have an interstitial fibrosis, likely induced by previous asbestos exposure, as evidenced by the dense pleural plaques - although a separate/concurrent interstitial process cannot be excluded.

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