159例小婴儿先天性巨结肠的临床特点分析

Clinical features of Hirschsprung’s disease in infants:An analysis of 159 cases

目的:总结小婴儿先天性巨结肠(Hirschsprung’s disease,HD)临床特点及预后,指导临床早期诊断及治疗。方法:回顾性分析2011至2016年在我科手术治疗的159例小婴儿(0~90 d)HD患儿的临床资料及术后随访情况。结果:(1)本组病例159例小婴儿HD,男女比例为8.35∶1;主要以腹胀(94.3%)、呕吐(76.1%)、胎便排出延迟(71.4%)、便秘(44.0%)为起病表现。(2)钡剂灌肠(barium enema,BE)、肛管直肠测压(anorectal manometry,ARM)、直肠黏膜活检(rectal suction biopsy,RSB)的诊断准确率分别为84.8%、93.2%、96.6%,各辅助检查诊断准确率有统计学差异(χ^2=13.872,P=0.001);各病理分组间BE(χ^2=2.273,P=0.518)、ARM(χ^2=1.297,P=0.730)及RSB(χ^2=1.355,P=0.716)检查诊断准确率无统计学差异。(3)本组病例均经手术治愈出院,总体术后平均住院时间为9.42 d;术后并发症主要有小肠结肠炎(10.7%)、切口感染(2.5%)、切口裂开(1.3%)。结论:小婴儿HD患儿的临床表现以腹胀、呕吐为主,临床表现缺乏特异性,结合ARM、BE及RSB有助于早期诊断。早期手术总体疗效明确好,术后并发症少,术后恢复快。

Objective:To investigate the clinical features and prognosis of Hirschsprung’s disease(HD)in infants,and to guide early clinical diagnosis and treatment.Methods:A retrospective analysis was performed for the clinical data and postoperative follow-up data of 159 infants(aged 0-90 days)with HD who underwent surgical treatment in our department from 2011 to 2016.Results:The male/female ratio was 8.35∶1 among all 159 infants with HD;as for the manifestation of disease onset,94.3%had abdominal distension,76.1%had vomiting,71.4%had delayed discharge of meconium,and 44.0%had constipation.The diagnostic accuracies of barium enema(BE),anorectal manometry(ARM),and rectal suction biopsy(RSB)were 84.8%,93.2%,and 96.6%,respectively,and there was a significant difference in diagnostic accuracy between these auxiliary examinations(χ^2=13.872,P=0.001).There were no significant differences between pathological groups in the diagnostic accuracies of BE(χ^2=2.273,P=0.518),ARM(χ^2=1.297,P=0.730),and RSB(χ^2=1.355,P=0.716).All infants were cured after surgical treatment and were then discharged.The mean postoperative hospital stay was 9.42 days,and major postoperative complications included enterocolitis(10.7%),incision infection(2.5%),and rupture of incision(1.3%).Conclusion:Major clinical manifestations of HD in infants include abdominal distension and vomiting.Since theseclinical manifestations lack specificity,ARM,BE,and RSB help with early diagnosis.Early surgery has a good clinical effect with few complications and rapid recovery.