[Clinical study of multiple zonal aganglionosis in long segment Hirschsprung's disease].
OBJECTIVE
To discuss pathogeny and diagnosis and management of multiple zonal aganglionosis in Hirschsprung's disease.


METHODS
Records of 3 children, aging 5 days, 29 days and 18 months, 3 boys, with multiple zonal aganglionosis in long segment Hirschsprung's disease between 1987-2005 were reviewed retrospectively. Total colectomy and Soave's operations were performed.


RESULTS
3 children were diagnosed HD before surgery, but the convulsive stenosis in distal ileum and proximal ascending colon were detected during surgery. The aganglionic cells in the stenosis gut were confirmed by pathologic diagnosis.


CONCLUSIONS
The total colon and ileum should be detected carefully during surgery in children with long segment Hirschsprung's disease. It is believed not sound that the neuroblastic cells stop moving from neuroectoderm to gut in early gestation in HD, but it is also believed that some other causes in the course of gestation might interfere the normal growth of the ganglionic cells.
Yang
He-ying
HY
Department of Pediatric Surgery, First Affiliated Hospital, Henan Clinical Medical Key Laborator, Zheng Zhou University, Zhengzhou 450052, China.
Liu
Qiu-liang
QL
Wang
Jia-xiang
JX
Xu
Hua-feng
HF
D003106:Colon / Q000473:pathology*; D006627:Hirschsprung Disease / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery*; D006801:Humans; D007082:Ileum / Q000473:pathology*; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012189:Retrospective Studies