小儿先天性肠旋转不良——附63例报告

目的：总结小儿先天性肠旋转不良的临床特点。探讨合理的诊断及治疗措施。方法：对63例小儿先天性肠旋转不良的临床资料进行回顾性分析。结果：63例中新生儿40例（63%），非新生儿23例（37%）；合并畸形17例（27%）。49例行传统的拉德（Ladd)手术；11例出现肠坏死，行肠外置或造瘘5例，肠切除肠吻合6例；1例肠反向旋转病儿因结肠梗阻行升结肠与横结肠侧侧吻合；2例盲肠位置正常的保留阑尾，切断松解十二指肠周围粘连的索带，手术中发现肠扭转45例，其中新生儿占31例，63例中56例痊愈，5例死亡，2例病情危重自动出院，7例行2次手术。结论：先天性肠旋转不良是发病率较高，复杂的消化道畸形，其临床表现常因发病年龄而异，出现小肠扭转，肠坏死是死亡的主要原因，早期诊断，掌握诊治要点及合理处理并发畸形是提高治愈率的关键。

Congenital intestinal malrotation in children (report of 63 cases).

Objective: To evaluate clinical characteristics, appropriate diagnosis and management of congenital intestinal malrotation. Methods: 63 cases of children with congenital intestinal malrotation in children were studied retrospectively. Results: Of 63 patients with congenital intestinal malrotation, 40 subjects (63% ) were neonates and 23 cases (37% ) were beyond the neonatal period. 17 patients had associated congenital abnormalities. Ladd's operative method had been used in 49 cases. Eleven subjects presented with intestinal necrosis. Intestinal stoma was performed in 5 patients and bowel resection in 6. Volvulus was found at the time of surgery in 45 patients, 31 of them were neonates. Of 63 cases, 56 were cured, 5 died and 2 refused further treatment because of severe complications. Reoperation occurred in 7 patients. Conclusions: Congenital intestinal malrotation has a high incidence rate in children. Various clinic manifestations occur at different age. Children with intestinal necrosis have a significant risk of death. Early diagnosis and correct management of associated abnormalities is the key to improve the curative rate.