摘要
对胡尔勒综合征患者的先天性脐疝(黏多糖贮积症I型),一般都采取保守治疗,因为像箝闭等并发症是很少见的,同时外科修补术的危险性很高。
Congenital umbilical hernias in Hurler's syndrome (mucopolysaccharidosis Ⅰ) are generally treated conservatively, because complications such as incarceration are, rare, and risks involved in surgical correction are high. This case report describes the surgical management of a ruptured umbilical hernia in a 3 - yearold child with Hurler's syndrome. Emergency repair of the hernia was performed with primary closure of the fascia; hernia recurrence 6 months later was treated laparoscopically using a PTFE mesh graftwith no evidence of re-recurrence. In selected cases of Hurler's syndrome (warning signs of rupture) elective surgical hernia repair may be indicated.
