Idiopathic pneumatosis cystoides intestinalis of the small bowel with perforation and congenital duodenal membranous stenosis and malrotation of intestine. A case report.

INTRODUCTION: Pneumatosis cystoides intestinalis (PCI) in children is a disorder characterized by gas-filled cystic lesions located in the submucosa and subserosa of any segment of the gastrointestinal tract, with pneumoperitoneum occurring in partial cases. This disease is well recognized as an early imaging sign of necrotizing enterocolitis secondary to intestinal ischemia in preterm neonates, yet PCI is rare in older children. Unlike pneumatosis intestinalis associated with life-threatening conditions such as neonatal necrotizing enterocolitis and mesenteric ischemia, PCI is generally regarded as a benign disorder. Conservative non-surgical management is indicated for PCI patients without critical life-threatening complications. CASE PRESENTATION: We present the case of a 11-year-old female patient presenting with recurrent vomiting and abdominal distension for 9 years. She was diagnosed with congenital congenital duodenal membranous stenosis and underwent laparoscopic surgery at our hospital. Intraoperative findings included congenital duodenal membranous stenosis, extensive pneumatosis involving the lesser curvature of the stomach and ileal wall with alveolar air cysts of variable morphology, as well as intestinal contents extravasated secondary to intestinal perforation. Congenital malrotation of the intestine was also identified. The patient received laparoscopic resection of duodenal web combined with duodenal longitudinal incision and transverse plasty, plus reduction of intestinal malrotation; no specific intervention was performed for the intestinal gas cysts. Postoperative diagnoses: congenital duodenal membranous stenosis, pneumatosis cystoides intestinalis, congenital intestinal malrotation. The patient was followed up for 29 months postoperatively. At present, she has no special discomforts such as abdominal pain or vomiting, with markedly relieved abdominal distension, and remains under regular follow-up. DISCUSSION: We discussed whether the lesion distribution of pediatric PCI is correlated with elevated intraluminal gastrointestinal pressure. Pediatric PCI carries a risk of intestinal perforation; non-surgical management can be adopted after exclusion of life-threatening critical conditions. CONCLUSION: The diagnosis and management of pediatric pci are clinically challenging due to the potential risk of perforation, while most concurrent pneumoperitoneum is benign. its pathogenesis may be associated with partial gastrointestinal obstruction and increased intraluminal pressure. regular follow-up and reexamination are recommended for children without life-threatening manifestations, and non-surgical treatment is preferred in the absence of acute abdomen.