EP221 - Pneumatosis Cystoides Intestinalis: A Case Report Of An Uncommon Diagnosis

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Purpose/Background: Pneumatosis Cystoides Intestinalis (PCI) is a rare condition marked by gas-filled cysts within the submucosa or subserosa of the intestinal wall. First described in 1946, PCI is more prevalent in males, with a male-to-female ratio of 2.4:1, and peak age of onset of 45 years. PCI occurs in about 0.03% of the population, though its true prevalence may be higher due to asymptomatic cases. Disease duration averages six months. PCI can be iatrogenic or linked to conditions such as malignancy, diverticulosis, AIDS, inflammatory bowel disease, and chemotherapy. Its pathophysiology remains unclear but is likely multifactorial. PCI may be asymptomatic but may also present as a severe condition with complications like intestina​​l ischemia and pneumoperitoneum.

Methods/Interventions: A 74-year-old female with a history of hypertension, peripheral arterial disease, lung cancer, and schizoaffective disorder, was transferred to our facility for evaluation of pneumatosis intestinalis and possible pneumoperitoneum. She initially presented to an outside hospital with a month-long history of diffuse abdominal pain and non-bloody diarrhea, which worsened after one day of increased pain, diarrhea, and generalized weakness. Upon arrival at our facility, she was hemodynamically stable with mild abdominal tenderness. An abdominal X-ray was performed and was concerning for pneumoperitoneum. A CT scan revealed extensive intramural gas in the ascending and transverse colon, along with pericolonic cystic and interstitial gas, suggestive of PCI. She was admitted, made NPO, and treated with piperacillin-tazobactam and supplemental oxygen.

Results/Outcomes: Her symptoms gradually improved, and her diet was advanced. By hospital day 5, she was transitioned to oral antibiotics, and a repeat CT on day 6 showed near-complete resolution of PCI. She was discharged on day 7 with a three-month course of metronidazole and plan for follow-up colonoscopy.

Conclusion/Discussion: Multiple mechanisms of PCI have been proposed but pathophysiology remains unclear, with potential causes including mechanical injury secondary to intraluminal pressure, gas migration due to chronic lung disease, and gas-producing bacteria.
Given its relatively low incidence, diagnosis can be challenging and requires experienced interpretation of radiologic images and a high index of suspicion by the evaluating surgical team. Symptoms vary depending on the extent and location of the gas with abdominal pain, nausea, and diarrhea commonly seen. In this case, the patient had a one-month history of non-bloody diarrhea, often associated with PCI in the terminal ileum or colon. Although complications like volvulus or perforation are rare (around 3% of cases), her initial presentation suggested an acute gastrointestinal event. A CT scan confirmed intramural gas but due to her stable condition and absence of peritonitis non-operative management was pursued.
There are no standard treatment guidelines for PCI and management depends on suspected etiology and severity of the condition. Non-operative treatments include hyperbaric oxygen, antibiotics, and endoscopy. In this case, antibiotics, supplemental oxygen, and judicious advancement of diet led to gradual improvement.
This case shows the potential for spontaneous resolution in PCI, particularly in stable patients. Follow-up is essential, and the patient will undergo a colonoscopy to identify any underlying pathology. Given her history of schizoaffective disorder, mental health support will be integrated into her care.