Challenges in Diagnosis and Management of Pneumoperitoneum Associated with Pneumatosis Cystoides Intestinalis in
Christina Siouli1, Konstantina Dimopoulou2, Dimitra Dimopoulou3
1Department of Pediatric Surgery, Children's General Hospital "Aghia Sophia", 115 27 Athens, Greece.
Insights
Pneumatosis cystoides intestinalis (PCI) with pneumoperitoneum in children is rare but can mimic perforation. Conservative management is often effective, avoiding unnecessary surgery in most pediatric cases.
Area of Science:
- Pediatric Gastroenterology
- Rare Diseases
- Surgical Emergencies
Background:
- Pneumatosis cystoides intestinalis (PCI) involves gas-filled cysts in the intestinal wall.
- Pneumoperitoneum in children with PCI presents diagnostic and therapeutic challenges, often mistaken for gastrointestinal perforation.
Purpose of the Study:
- To systematically review and summarize evidence on pediatric pneumatosis cystoides intestinalis-associated pneumoperitoneum.
- To analyze clinical presentation, diagnosis, treatment, and outcomes in affected children.
Main Methods:
- Systematic literature search of PubMed/Medline (1972-2025).
- Inclusion of studies on patients ≤16 years with PCI-related pneumoperitoneum.
- Data extraction and selection by two independent reviewers following PRISMA guidelines.
Main Results:
- 23 studies with 95 pediatric cases (4 months-14 years) were included.
- Most patients had underlying conditions (malignancies, autoimmune disorders) and received immunosuppressants.
- Conservative treatment (85%) was favored over surgery (11%); only 3 cases had confirmed perforation.
Conclusions:
- PCI-related pneumoperitoneum in children is uncommon, especially in immunocompromised patients, and can lead to misdiagnosis.
- Conservative management is effective in most cases, guiding treatment based on clinical findings is crucial.
- Increased awareness is vital for pediatricians and surgeons to prevent overtreatment and misdiagnosis.
Abstract:
Background/Objectives: Pneumatosis cystoides intestinalis (PCI) is a rare condition in children characterized by gas-filled cysts in the intestinal wall. The presence of pneumoperitoneum poses significant diagnostic and therapeutic challenges, often mimicking gastrointestinal perforation. This systematic review aims to summarize the existing evidence on PCI-associated pneumoperitoneum in children. Methods: A systematic literature search was conducted in PubMed/Medline for articles published from January 1972 to March 2025. Studies involving patients ≤16 years old with PCI-related pneumoperitoneum and providing information on clinical presentation, diagnosis, treatment, and outcomes were included. Data extraction and study selection were independently performed by two reviewers in accordance with PRISMA guidelines. Results: Out of 209 articles initially identified, 23 studies comprising 95 pediatric cases (age range: 4 months-14 years) were included. The majority had underlying conditions such as malignancies, autoimmune disorders, or gastrointestinal motility issues and were often treated with corticosteroids and/or immunosuppressants. Most cases were incidentally diagnosed through radiographic imaging. Conservative treatment (bowel rest, antibiotics, parenteral nutrition) was applied in 85% of cases, while surgical intervention occurred in 11%. Only three cases had confirmed intestinal perforation. All patients experienced resolution of PCI; however, 20 died due to unrelated underlying diseases. Conclusion: Despite the case heterogeneity of this review, PCI-related pneumoperitoneum in children is an uncommon but clinically important entity, particularly in immunocompromised patients, that may lead to misdiagnosis and unnecessary surgery. Conservative management is effective in most cases, and clinical findings should guide treatment decisions. Increased awareness among pediatricians and surgeons is crucial to avoid overtreatment.
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