Successful redo pull-through for Hirschsprung's disease in a Haddad syndrome patient.
نویسندگان
چکیده
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.
منابع مشابه
Long-term follow-up of redo pull-through procedures for Hirschsprung's disease: efficacy of the endorectal pull-through.
BACKGROUND/PURPOSE The purpose of this study was to review the authors' 25-year experience with redo pull-through procedures for Hirschsprung's disease including surgical technique and long-term outcome. METHODS From 1974 to now, over 325 patients with Hirschsprung's disease have been treated at C.S. Mott Children's Hospital. This includes 30 patients referred after an unsuccessful pull-throu...
متن کاملCombined abdominal and posterior sagittal approach for redo pull-through operation in Hirschsprung's disease.
BACKGROUND/PURPOSE In Hirschsprung's disease (HD) redo pull-through (PT) is indicated for anastomotic complications and retained aganglionosis after a previous operation. Duhamel or Swenson method is used commonly for redo operations. The pelvic dissection may be difficult, especially in Swenson's type of operation, because of fibrosis resulting from previous surgery or its complications. To ov...
متن کاملEffectiveness of various surgical methods in treatment of Hirschsprung's disease in children.
BACKGROUND/AIM Hirschsprung's disease is the most common identifiable developmental disorder of the enteric nervous system, characterized by a failure of its formation in a variable segment of distal bowel. Currently available surgical therapies for Hirschsprung's disease, although lifesaving, are associated with numerous complications. The aim of our study was to evaluate the effectiveness of ...
متن کاملA new modification of transanal Swenson pull-through procedure for Hirschsprung's disease.
BACKGROUND The one-stage pull-through procedures for Hirschsprung's disease (HD) have become popular because it is well accepted by surgeons and mothers with no visible scar and a short hospital stay. It represents the latest development in the concept of a minimally invasive surgery for HD. We introduce a new method of transanal one-stage pull-through for Hirschsprung's disease, different from...
متن کاملSingle Stage Transanal Pull-Through for Hirschsprung’s Disease in Neonates: Our Early Experience
OBJECTIVE Hirschsprung's disease is one of the common causes of intestinal obstruction in neonates. Transanal endorectal pull-through represents the latest development in the concept of the minimally invasive surgery for Hirschsprung's disease. In this study, we present our early experience with single stage transanal pull through in neonates. DESIGN Retrospective study of neonates with singl...
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عنوان ژورنال:
- Journal of the Chinese Medical Association : JCMA
دوره 73 8 شماره
صفحات -
تاریخ انتشار 2010