Campobasso P, Salano F, Cimaglia M L, Cappellari F, Scalabrin U, Belloli G
Pediatr Med Chir. 1986 Sep-Oct;8(5):659-64.
The Authors report their experience in the treatment of 86 patients, in pediatric age, with Hirschsprung's disease: 40 with congenital rectosigmoid or subtotal megacolon, 8 with total colic or ileocolic aganglionosis and 38 with ultrashort aganglionic segment. Out of the patients 29% had necrotizing enterocolitis with 40% mortality rate in rectosigmoid or subtotal forms and with 75% mortality rate in total colic or ileocolic forms. In the last group the mortality was higher related also to difficulties and complications of surgical treatment. Necrotizing enterocolitis is the major cause of death in infants with Hirschsprung's disease. Early diagnosis and treatment can reduce the mortality rate.
作者报告了他们治疗86例小儿先天性巨结肠病患者的经验:40例为先天性直肠乙状结肠或次全巨结肠,8例为全结肠或回结肠无神经节细胞症,38例为超短无神经节细胞段。患者中29%发生坏死性小肠结肠炎,直肠乙状结肠或次全型死亡率为40%,全结肠或回结肠型死亡率为75%。在最后一组中,死亡率较高还与手术治疗的困难和并发症有关。坏死性小肠结肠炎是先天性巨结肠病婴儿死亡的主要原因。早期诊断和治疗可降低死亡率。