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Radiol Case Rep. 2018 Nov 3;14(2):171-174. doi: 10.1016/j.radcr.2018.10.007. eCollection 2019 Feb.

Congenital intestinal hypoganglionosis: A radiologic mimic of Hirschsprung's disease.

Author information

1
Department of Radiology, Akron Childrens Hospital, Akron, OH, USA.
2
Department of Surgery, University of Cincinnati, Cincinnati Children's hospital, OH, USA.
3
Department of Pathology, Akron Childrens Hospital, Akron, OH, USA.

Abstract

Intestinal hypoganglionosis or isolated hypoganglionosis is a rare entity with a clinical and radiologic presentation that can mimic Hirschsprung's disease in the neonatal period. The diagnosis of this entity can be challenging with suction rectal biopsies that are standard for diagnosing Hirschsprung's disease. We present this case of congenital intestinal hypoganglionosis detailing the neonatal course, due to its rarity and the conundrums faced before an eventual diagnosis could be rendered. This case also illustrates the role of full thickness rectal biopsy in selected cases such as ours where the radiologic features are typical of Hirschsprung's, despite negative suction biopsies.

KEYWORDS:

Enteric neuropathy; Hirschsprung's disease; Intestinal hypoganglionosis

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