Division of Pediatric Surgery, Department of Surgery, Taipei Veterans General Hospital and National Yang-Ming University School of Medicine, Taipei, Taiwan, R.O.C.
J Chin Med Assoc. 2010 Aug;73(8):438-40. doi: 10.1016/S1726-4901(10)70094-0.
Haddad syndrome is characterized by congenital central hypoventilation syndrome (Ondine's curse) associated with segmental distal gut aganglionosis (Hirschsprung's disease). The prognosis of Haddad syndrome is very poor, and survival is often less than 2 years. Treatment of Hirschsprung's disease is usually influenced by the association with Ondine's curse. We report the case of a girl with Haddad syndrome who underwent redo pull-through with Duhamel's method because of persistent obstruction after primary transanal pull-through surgery. After 7 years of follow-up, the patient is alive and does not suffer from recurrent partial intestinal obstruction. She performs her daily activities with the support of a portable ventilator. Thus, aggressive surgical treatment for Hirschsprung's disease could have good outcome in terms of long-term survival in Haddad syndrome patients.
哈达德综合征的特征是先天性中枢性通气不足综合征（翁迪恩诅咒）合并节段性远端肠道无神经节细胞症（先天性巨结肠）。哈达德综合征的预后非常差，生存时间通常少于 2 年。对于合并翁迪恩诅咒的先天性巨结肠，其治疗通常会受到影响。我们报告了 1 例哈达德综合征女孩的病例，她在初次经肛门拖出术（transanal pull-through surgery）后因持续梗阻而接受了杜厄梅尔（Duhamel）法的再次拖出术。经过 7 年的随访，患者存活，没有发生复发性部分肠梗阻。她在便携式呼吸机的支持下进行日常活动。因此，对于哈达德综合征患者，积极的先天性巨结肠手术治疗可能会获得长期生存的良好结果。
