Siouli Christina, Dimopoulou Konstantina, Dimopoulou Dimitra, Krikri Aggeliki, Kelaidi Natalia, Zavras Nikolaos, Dimopoulou Anastasia
Department of Pediatric Surgery, Children's General Hospital "Aghia Sophia", 115 27 Athens, Greece.
Department of Gastroenterology, Korgialenio-Benakio Red Cross Hospital, 115 26 Athens, Greece.
J Clin Med. 2025 Sep 14;14(18):6479. doi: 10.3390/jcm14186479.
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. 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. 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. 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.
小肠气囊肿（PCI）是一种儿童罕见病，其特征为肠壁内有充满气体的囊肿。气腹的存在带来了重大的诊断和治疗挑战，常酷似胃肠道穿孔。本系统评价旨在总结关于儿童PCI相关气腹的现有证据。在PubMed/Medline中对1972年1月至2025年3月发表的文章进行了系统文献检索。纳入了涉及≤16岁患有PCI相关气腹的患者且提供临床表现、诊断、治疗及结局信息的研究。两名评审员根据PRISMA指南独立进行数据提取和研究选择。在最初识别的209篇文章中，纳入了23项研究，共95例儿科病例（年龄范围：4个月至14岁）。大多数病例有潜在疾病，如恶性肿瘤、自身免疫性疾病或胃肠动力问题，且常接受皮质类固醇和/或免疫抑制剂治疗。大多数病例通过影像学检查偶然诊断。85%的病例采用保守治疗（肠道休息、抗生素、肠外营养），11%的病例进行了手术干预。仅3例确诊为肠穿孔。所有患者的PCI均得到缓解；然而，20例因无关的基础疾病死亡。尽管本评价的病例存在异质性，但儿童PCI相关气腹是一种罕见但临床上重要的情况，尤其是在免疫功能低下的患者中，可能导致误诊和不必要的手术。大多数情况下保守治疗有效，临床发现应指导治疗决策。提高儿科医生和外科医生的认识对于避免过度治疗至关重要。
