肛门闭锁短结肠联合征的诊断和治疗

Association of Imperforate Anus with Short Colon (AIASC):diagnosis and treatment

探讨肛门闭锁短结肠联合征诊治中的若干问题。方法作者对1986年5月以后诊断治疗的14例患儿的临床资料进行回顾性分析，观察发现12例患儿存在肠乐膜下血管，并对其采用了短结肠的管状成形术和袖套状翻转结肠肌层的肛门成形术。结果手术病人7／13（54％）存活，新生儿2／7（28．6％）存活，幼儿患者5／6（83％）存活。新生儿病人，术式未改进前病人死亡率高，效果差。幼儿及术式改良后死亡率明显下降，效果明显改善。结论及时正确诊断，新生儿期结肠造口、幼儿期行短结肠管状成形及末端肌层袖状翻转肛门成形术能明显改善患儿预后。

To assess the experience of diagnosis and treatment of Association of Imperforate Anus with Short Colon in children.Methods From May, 1986 to May, 1998. 14 children (male 6.female 8) with AIASC were reviewed. It was found that the blood supply of one side of short colon in 12 patients was from the inferior mesenteric vessels, they were treated by 1-stage or 2-stage coloplasty (to reform the saccular colon in to Tubular one) and anoplasty procedure (by overding the muscle ular cuff of the stump of colon to form an internal sphincter).Results Seven cases of 13 were alive (54 % ). Among 7 operated newborn patients. 2 were alive (28. 6% ). 5 of six infants patients, were alive (83% ). All of the 7 living cases were followedup 1-12 years (mean 7. 3 years). One died of unrelated disease. and six cases were living well.Conclusions Earlier diagnosis and colostomy in newborn, and tubular coloplasty plus internal sphineter plastic procedure in infants are important in improving the cure rate and reducing the mortality of AIASC.