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ACG Case Rep J ; 8(5): e00610, 2021 May.
Artículo en Inglés | MEDLINE | ID: mdl-34549071

RESUMEN

Hirschsprung disease (HD) is a congenital bowel innervation disorder characterized by the absence of ganglion cells in the neural plexus of the colorectal wall. Variant HD describes a heterogeneous group of intestinal innervation disorders in which clinical presentation resembles HD despite the presence of ganglion cells seen in rectal biopsies. We present the first reported case of a rare variant HD, hypoganglionosis isolated in the anorectal canal, diagnosed in an adult who presented with a long history of constipation and treated with proctosigmoidectomy with coloanal anastomosis. Histopathology showed rare ganglion cells isolated in the anorectal canal.

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