Congenital Generalized Hypoganglionosis: Clinical Aspect
Congenital generalized hypoganglionosis (CGH) is a rare disorder with a poor prognosis. Both dedicated diagnosis and treatment improve the management of CGH patients. The first step in treating CGH is the creation of an appropriate stoma during the neonatal period. A jejunostomy less than 50 cm from the Treitz ligament is the preferred treatment option in neonatal period, even in advance of a definitive diagnosis. The second step is to preserve intestinal function. The habilitation procedure for the distal intestine avoids unnecessary massive bowel resection and intestinal bacterial overgrowth, which are the main management points for long-term survival. The following steps are to provide enteral caloric intake as soon as possible and to make the successful transition from parenteral to full enteral nutrition. This section focuses on the importance of CGH treatments during the neonatal and early infancy period.
KeywordsCongenital generalized hypoganglionosis Hirschsprung’s allied disorders Upper jejunostomy Diagnosis and treatment
This work was supported by a grant from the Ministry of Health, Labour, and Welfare of Japan (H24-Nanchi-Ippan-037, Health and Labour Sciences Research Grants for Research on intractable diseases).
Disclosures: The authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research reported.
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