Successful Redo Pull-through for Hirschsprung's Disease in a Haddad Syndrome Patient

Abstract

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.