|LETTER TO EDITOR
|Year : 2015 | Volume
| Issue : 1 | Page : 22-23
Pneumatosis cystoides intestinalis
R Amita, S Sandhyamani
Department of Pathology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India
|Date of Web Publication||20-Jul-2015|
Department of Pathology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram - 695 011, Kerala
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Amita R, Sandhyamani S. Pneumatosis cystoides intestinalis. Niger J Surg Sci 2015;25:22-3
Pneumatosis cystoides intestinalis (PCI) is a rare gastrointestinal complication related to a variety of causes ranging from benign to life-threatening, such as connective tissue diseases (scleroderma, dermatomyositis), inflammatory diseases (inflammatory bowel disease), infectious diseases (clostridium difficile, retroviral infections, chronic obstructive pulmonary disease [COPD], drugs (corticosteroids, immunosuppressive therapy, acarbose), trauma (blunt abdominal trauma, endoscopy), bowel ischemia, and intestinal neoplasms. ,, The small intestine is the most commonly involved site (42%) followed by colon (36%) while both may be involved in 22%.  We present the pathological findings of PCI in an elderly patient and analyze the various etiologies in this context.
Our patient was a 70-year-old male, smoker, with a history of COPD, diabetes, and hypertension, who presented with fever and cough of 2 days duration. This was associated with profuse sweating and dyspnea on exertion. On examination, he was dyspneic with profuse sweating and blood pressure was 200/100 mm Hg, with pulse rate of 76/mm. There were bilateral crepitations in the basal lung fields. Investigations showed a non-ST elevation myocardial infarction with pulmonary edema. He was first managed conservatively and later taken up for emergency coronary artery bypass grafting. Postoperative period, he developed atrial fibrillation and respiratory distress for which he was mechanically ventilated. On the 3 rd postoperative day, he developed abdominal pain, for which an emergency laparotomy was done due to the clinical suspicion of ischemic colitis. Intraoperatively, the bowel was gangrenous up to mid ascending colon with absent pulsations in the ileocolic artery. Middle colic artery pulsations were well felt. The gangrenous bowel was resected. The patient continued to deteriorate in the postoperative period and succumbed on the 6 th postoperative day following laparotomy.
The gangrenous bowel measured 7 m in length. There was diffuse thinning of the bowel wall with presence of multiple thin-walled translucent air filled pockets along the mesenteric border [Figure 1]a, b. Microscopy showed a necrotic mucosa and congested vessels, some containing layered thrombi. Multiple submucosal and subserosal cystic spaces devoid of lining [Figure 1]c. suggesting their recent origin were seen, confirming the gross diagnosis of PCI.
|Figure 1: Resected segment of intestine showing multiple air-filled pockets along the mesenteric border (a), closer view of the air filled pockets (b) and microscopy shows necrotic mucosa with multiple submucosal cystic spaces, separated by fibrin strands (*) devoid of lining. Arrow shows muscularis mucosae (c, H and E, ×100)|
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Pneumatosis cystoides intestinalis was first described in a cadaveric dissection by Du Vernoi. It is characterized by the presence of multiple gas-filled cysts in the submucosa and or subserosa of the intestine.  The subserous cysts are most frequently located in the small intestine while the submucous ones are predominantly seen in the colonic wall.
Several mechanisms have been postulated for the development of PCI, including bacterial, mechanical, and pulmonary.  Bacterial theory proposes that fermentation of carbohydrates by bacteria leads to excessive gas formation, leading to increased intraluminal pressure, forcing the gas to penetrate the muscularis mucosae, resulting in PCI,  while mechanical theory postulates that increased intraluminal pressure as in bowel obstruction or dysmotility due to trauma, surgery or colonoscopy may lead to accumulation of gas within cystic spaces of submucosa and subserosa.  In patients with COPD, air may leak from the ruptured alveoli through the mediastinum into the retroperitoneal space and into the intestinal wall along perivascular spaces through the mesentery. 
In our patient, combined pathologies of COPD, bowel ischemia due to microangiopathy of hypertension and diabetes, impaired intestinal motility in the immediate postoperative period together with bacterial proliferation and increased intraluminal pressure lead to the development of PCI.
To conclude, a multitude of factors may act together in the pathogenesis of PCI as seen in our patient.
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