Hirschsprung's disease : one surgeon's experience in one institution.
Pacific Association of Pediatric Surgeons. Annual meeting. Kagoshima JPN, 1994/05/23.
The clinical experience of 137 patients treated by one pediatric surgeon at HanYang University Hospital over 12 years was analyzed.
The sex ratio was 3.6 : 1 with male predominance.
Neonatal Hirschsprung's disease (HD) occurred in 70 cases (51.1%). One hundred fourteen cases (83.2%) were short-segment and 23 cases (16.8%) were long-segment disease, of which 4 cases were total colon aganglionosis.
Symptoms were abdominal distension, vomiting, constipation, and delayed passage of meconium.
Family history was noted in 4 cases : 3 involved siblings and 1 involved relatives.
All patients were male and had short-segment diseases.
There were 3 pairs of monozygotic twins discordant for HD.
Primary Duhamel operations were performed on 30 children.
Anorectal myectomies were performed on 12 cases of short rectal aganglionosis.
The incidence of enterocolitis was 25.5% preoperatively and 10.1% postoperatively.
Complications after the Duhamel operation were intestinal obstruction (3), wound disruption (4), and septum reformation (3) with fecaloma formation in the blind rectal pouch.
Duhamel operation with a GIA stapler after preliminary colostomy could be done at the age of 104 days on average with good functional results and with acceptable morbidity.
Primary Duhamel operation could be done safely on the patients whose conditions were diagnosed after 90 days of life with good results.
There was no mortality after Duhamel operation.
Mots-clés Pascal : Mégacôlon, Congénital, Enfant, Homme, Epidémiologie, Diagnostic, Traitement, Pronostic, Corée, Asie, Côlon, Aganglionose, Chirurgie, Appareil digestif pathologie, Intestin pathologie, Maladie congénitale, Malformation, Système nerveux autonome pathologie, Système nerveux pathologie, Hirschsprung maladie, Malformation intestinale neuronale
Mots-clés Pascal anglais : Megacolon, Congenital, Child, Human, Epidemiology, Diagnosis, Treatment, Prognosis, Korea, Asia, Colon, Aganglionosis, Surgery, Digestive diseases, Intestinal disease, Congenital disease, Malformation, Diseases of the autonomic nervous system, Nervous system diseases, Neuronal intestinal malformation
Notice produite par :
Inist-CNRS - Institut de l'Information Scientifique et Technique
Cote : 95-0418411
Code Inist : 002B13B03. Création : 01/03/1996.