Myectomy-myotomy for long segment Hirschsprung's disease in a patient with Haddad syndrome.
Hirschsprung's disease is a rare entity with an incidence of 1 in 5000 live born infants. Long segment Hirschsprung's disease occurs in approximately 5% to 10% of this patient population and is defined as a transition zone proximal to the sigmoid colon (Bodian M, Carter CO, Ward BC. Hirschsprung's disease. Lancet. 1951;1:302-309). The association of congenital central hypoventilation syndrome (also known as Ondine's curse) and Hirschsprung's disease is termed Haddad syndrome.
Otabor
Iyore A
IA
Department of Pediatric Surgery, Nationwide Children's Hospital and The Ohio State University College of Medicine, Columbus, OH 43205, USA.
Balint
Jane P
JP
Besner
Gail E
GE
D001663:Bilirubin; D003106:Colon / Q000601:surgery; D015897:Comorbidity; D013505:Digestive System Surgical Procedures / Q000379:methods*; D005217:Fat Emulsions, Intravenous / Q000008:administration & dosage; D006627:Hirschsprung Disease / Q000453:epidemiology* / Q000601:surgery*; D006801:Humans; D006932:Hyperbilirubinemia / Q000188:drug therapy / Q000453:epidemiology; D007082:Ileum / Q000601:surgery*; D007223:Infant; D007412:Intestinal Fistula / Q000601:surgery; D008297:Male; D010289:Parenteral Nutrition, Total; D011183:Postoperative Complications / Q000601:surgery; D020182:Sleep Apnea, Central / Q000453:epidemiology*; D013577:Syndrome
D005217:Fat Emulsions, Intravenous; D001663:Bilirubin