Rare Association of Extended Total Colonic Aganglionosis and Intestinal Malrotation
Keywords:Total colonic aganglionosis, Hirschsprung’s disease, Malrotation, Intestinal obstruction, Neonate
Total colonic aganglionosis occurring together with malrotation is a rare occurrence and may pose diagnostic and management dilemmas for the paediatric surgeon. We report a case of a neonate that presented with extended total colonic aganglionosis and malrotation, along with a spectrum of central nervous system and renal abnormalities. The clinical and radiological features and potential diagnostic and management pitfalls are discussed along with a literature review of this exceptionally infrequent association.
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