Rare Association of Extended Total Colonic Aganglionosis and Intestinal Malrotation

Iulia Stratulat-Chiriac, Danielle Mc Laughlin, Brice Antao

Abstract


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.


Keywords


Total colonic aganglionosis; Hirschsprung’s disease; Malrotation; Intestinal obstruction; Neonate

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DOI: http://dx.doi.org/10.21699/jns.v5i4.468

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