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Pediatr Neurol ; 18(5): 452-5, 1998 May.
Article in English | MEDLINE | ID: mdl-9650691

ABSTRACT

A 2-year-old boy with hemimegalencephaly and Hirschsprung's disease is reported. The unique association of these two entities is considered to be the presence of a common insult or insults that affect the innervation of the bowel and the formation of the cerebral cortex. Short-segment subtype of Hirschsprung's disease may suggest that this effect occurred between the eighth and twelfth weeks of gestation. Although there is a well-known coexistence of Hirschsprung's disease with the malformations that share a common neurocristopathic origin (abnormalities of neural crest cell growth, migration, or differentiation), a few extremely rare cases, as in this case, might reflect the coexistence of Hirschsprung's disease with a cerebral malformation (i.e., hemimegalencephaly) that is a nonneurocristopathic entity by itself.


Subject(s)
Abnormalities, Multiple , Brain/abnormalities , Hirschsprung Disease , Nervous System Malformations , Abnormalities, Multiple/embryology , Abnormalities, Multiple/pathology , Brain/embryology , Brain/pathology , Brain/physiopathology , Child, Preschool , Hirschsprung Disease/embryology , Hirschsprung Disease/pathology , Humans , Male , Nervous System Malformations/embryology , Nervous System Malformations/pathology , Spasms, Infantile/drug therapy , Spasms, Infantile/etiology
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