摘要目的 探讨胎粪性肠梗阻的临床特点与治疗.方法 对4例胎粪性肠梗阻患儿的发病病因、临床症状、诊断及治疗进行回顾性分析.结果 4例胎粪性肠梗阻患儿以肠梗阻就诊3例,术前均怀疑为全结肠型无神经节细胞症,以消化道穿孔为首诊症状者1例,术前怀疑为新生儿坏死性小肠结肠炎.3例为新生儿期就诊,1例生后4个月就诊.4例患儿均进行手术治疗,其中2例行肠腔冲洗、末端回肠造瘘术,1例行回盲部狭窄肠管切除肠吻合术,1例行穿孔肠管修补术及腹腔冲洗引流术.3例新生儿患者术中均发现胎类极度黏稠、阻塞回盲部远端肠腔,4例患儿均在术中取病理证实肠壁存在神经节细胞.随访中1例患儿出院1个月后反复发生呼吸道感染、重症肺炎、呼吸衰竭而死亡:1例患儿X线胸片持续存在肺部阴影,但呼吸正常;其他2例患儿生长发育正常.结论 新生儿期便秘患儿在怀疑全结肠型无神经节细胞症的同时,应考虑到本病的可能,术中病理活检对诊断该病十分重要,术后随访应密切注意肺等其他器官系统的相应合并症.

abstractsObjective To discuss the clinical features and treatment of meconium ileus. Methods Four cases of children with meconium ileus were analyzed about the pathogenesis, etiology, clinical symptoms, diagnosis and treatment. Results Three out of 4 cases presented with intestinal obstruction, they were all suspected to be total colonic aganglionosis firstly. One patient presented with digestive tract perforation, who was suspected to be neonatal necrotizing enterocolitis. Three cases were admitted to hospital in neonatal period ,one preterm infant came to hospital 4 months after birth. Four patients were all treated with operation, the operation methods were ileostomy and bowel irrigation in 2 patients, narrowed bowel resection and anastomosis in 1, perforation repair and peritoneal cavity irrigation in 1. Meconium was discovered to be extremely viscous and obstructed ileocecal distal bowel in 3 cases. Ganglion cells were found in distal intestinal wall in all 4 patients. In follow-up, 1 case died of repeatedly respiratory infection, serious pneumonia and respiratory failure 1 month after discharging, 1 patient was well otherwise besides consistently pulmonary shadow,2 cases thrived well. Conclusion Meconium ileus should be borne in the diagnosis of neonatal intestinal obstruction besides total colonic aganglionosis, intraoperative biopsy is very important. Early diagnosis and proper treatment are very important for the survival of the patients. However,in postoperative follow-up,one should pay attention to pulmonary and other complications.