长段型先天性巨结肠的临床诊治进展  

Progress in Diagnosis and Treatment of Long-segment Hirschsprung′s Disease

作　　者：赵绪稳[1] 胡博[1] ZHAO Xuwen;HU Bo(Department of Neonatal Surgery,Tianjin Children′s Hospital,Tianjin 300074,China)

机构地区：[1]天津市儿童医院新生儿外科,天津300074

出　　处：《医学综述》2022年第16期3259-3263,共5页Medical Recapitulate

摘　　要：先天性巨结肠又称希尔施普龙病(HD),是儿童常见的消化道发育畸形疾病,有家族遗传倾向,常影响患儿生长发育,也是造成新生儿及小婴儿急性肠梗阻的常见原因之一。尤其是长段型HD,由于狭窄段肠管较长,生后多有胎便排出延迟病史,并且早期即可出现排便困难、腹胀甚至呕吐等症状,伴随病情进展,并对消化系统造成渐进性损害,可诱发巨结肠相关小肠结肠炎,也是导致患儿死亡的重要原因。手术是唯一有效的根治方法,虽然腹腔镜下根治术明显减少了开腹手术的并发症,手术安全性有所提高并取得巨大进步,但以常见型巨结肠的报道居多,对于手术方式仍有争议。Congenital megacolon,also known as Hirschsprung′s disease(HD),is a common deformity disease in children′s digestive tract,which has familial hereditary tendency and affects growth and development of the children.It is also one of the common causes of acute neonatal intestinal obstruction.Children with long-segment HD often have a history of meconium delay,and have early symptoms such as difficulty defecating,abdominal distension,and even vomiting due to the long narrow segment of intestine.With the progression of the disease,it causes progressive damage to the digestive system and can induce Hirschsprung-associated enterocolitis,which is also an important cause of death in children.Surgery is the only effective treatment.Although laparoscopic HD radical surgery significantly reduces the complications of open surgery,and thus the surgical safety has been significantly improved,yet more common type have been reported,and there are still controversies about the surgical method.

关 键 词：长段型先天性巨结肠 肠梗阻 腹腔镜 

分 类 号：R574[医药卫生—消化系统]