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目的 提高对小儿巨结肠类缘病的认识。方法 对小儿巨结肠类缘病合并畸形的 4例病例临床与病理特征加以总结。结果 4例术后病理活检均证实为巨结肠类缘病 ,其中 2例同时伴有先天性肛门直肠畸形 ,1例伴有先天性脊柱侧弯 ,1例伴有先天性白内障。 2例行升结肠造瘘 ,1例行Duhamel改良根治术 ,1例全消化道神经节细胞发育不良者行空肠造瘘。结论 巨结肠类缘病病因复杂 ,诊断困难 ,可同时存在其他先天性畸形。营养不良、电解质紊乱及顽固性肠道感染是该病常见的并发症 ,预后不良。
Objective To raise awareness of pediatric patients with Galeria. Methods The clinical and pathological features of 4 cases of Hirschsprung disease with malformation in children were summarized. Results Four cases of pathological biopsy were confirmed as Hirschsprung’s disease, of which 2 cases were accompanied by congenital anorectal malformations, 1 case with congenital scoliosis and 1 case with congenital cataract. 2 cases of ascending colostomy, 1 case of Duhamel modified radical mastectomy, 1 case of total gastrointestinal ganglion cell dysplasia were jejunal fistula. Conclusion The cause of Hirschsprung’s disease is complex and difficult to diagnose. Other congenital malformations may exist at the same time. Malnutrition, electrolyte imbalance and intractable intestinal infections are common complications of the disease, the prognosis is poor.