Contributed by
University Hospital Aachen, Germany
Sabine Schacht, and Martha Dohna.
History
•8 year old girl
•Increasing abdominal pain since weeks, now admitted for severe abdominal pain
•History of recurrent vomiting since birth
Images (Click any image to enlarge)
Question
What is the most likely diagnosis of the patient?
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Correct answer
Pneumatosis cystoides intestinalis
Discussion
Pneumatosis cystoides intestinalis (PCI) is a rare condition in children marked by numerous gas-filled cysts located within the submucosal and/or subserosal layers of the small intestine. It represents a distinct form of pneumatosis with characteristic features and may develop anywhere along the gastrointestinal tract. 85% of cases have underlying malignancies, autoimmune disorders, gastrointestinal motility disorders, and patients often have a history of corticosteroid treatment and/or immunosuppressants. PCI-related pneumoperitoneum in children is uncommon and typically caused by rupture of cysts in the bowel wall causing minor pneumoperitoneum without an intestinal perforation. PCI-associated pneumoperitoneum is a clinically important entity, particularly in immunocompromised patients, that may lead to misdiagnosis and unnecessary surgery. Conservative treatment with bowel rest, antibiotics and parenteral nutrition mostly suffice for complete resolution of symptoms.
Differential diagnosis
The multiple hyperlucent round images localized in the bowel on x-ray images may suggest ingestion of foreign bodies causing bowel obstruction. As immunocompromised patients are especially vulnerable PCI associated pneumoperitoneum may lead to unnecessary action such as surgery for fear of missing a severe complication.
Additional images
References
- Siouli C, Dimopoulou K, Dimopoulou D, Krikri A, Kelaidi N, Zavras N, Dimopoulou A. Challenges in Diagnosis and Management of Pneumoperitoneum Associated with Pneumatosis Cystoides Intestinalis in Children: A Systematic Review. J Clin Med. 2025 Sep 14;14(18):6479. doi: 10.3390/jcm14186479. PMID: 41010683; PMCID: PMC12470710. Aygüneş U, Karagün BŞ, Şaşmaz İ, Tutuş K, Özden Ö, Antmen B. Pneumatosis cystoides intestinalis mimicking free intraabdominal air following chemotherapy for relapsed acute myeloblastic leukemia in a transplanted neutropenic child: a case report. Turk J Pediatr. 2023;65(4):693-697. doi: 10.24953/turkjped.2023.116. PMID: 37661686. D'Agostino S, Fabbro MA, Musi L, Bozzola L. Pneumatosis cystoides intestinalis: a rare cause of nonsurgical pneumoperitoneum in an infant. J Pediatr Surg. 2000 Jul;35(7):1106-8. doi: 10.1053/jpsu.2000.7836. PMID: 10917306.