目的探讨儿童先天性肠系膜裂孔疝的早期诊断和治疗。方法回顾性分析2例经手术探查明确诊断的肠系膜裂孔疝患儿的临床资料,总结对该病的诊治体会。结果 2例患儿均表现为不明原因的呕吐,无明显腹痛、腹胀、肌紧张及压痛性腹部肿块,早期实验室及影像学表现不典型,均手术探查后确诊。1例并发感染性休克、多器官功能衰竭死亡,1例及时手术,预后良好。结论小儿肠系膜裂孔疝术前很难明确诊断,早期识别、早期手术可预防肠坏死、中毒性休克等严重并发症。 Objective To investigate the early diagnosis and treatment of congenital hiatus hernia in children. Methods The clinical data of 2 children with mesenteric hernia with hiatal hernia diagnosed by surgical exploration were retrospectively analyzed. The diagnosis and treatment of the disease were summarized. Results Both of the 2 cases showed unexplained vomiting, no obvious abdominal pain, abdominal distension, muscular tension and tender abdominal mass. The early laboratory and radiographic findings were not typical. All cases were confirmed by surgical exploration. One patient was complicated by septic shock, died of multiple organ failure, and one patient had timely operation. The prognosis was good. Conclusions It is very difficult to confirm the diagnosis, early identification and early operation of children with mesenteric hiatal hernia before surgery to prevent serious complications such as intestinal necrosis and toxic shock.