Pediatric Gastroenterology, Hepatology & Nutrition  2017;20(1):55-60

doi:10.5223/pghn.2017.20.1.55

Intestinal Hypoganglionosis Leading to Intestinal Failure and the Compassionate Use of Omegaven™.

Racha KHALAF 1 ; Sara KARJOO ; Paul DANIELSON ; Michael WILSEY ; Fauzia SHAKEEL

Affiliations

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Keywords

Hirschsprung disease; Omega-3 fatty acids; Liver diseases; Total parenteral nutrition

Country

Republic of Korea

Language

English

Abstract

Intestinal hypoganglionosis is a rare innervation disorder that provides numerous nutritional, medical and surgical challenges. In this case report, we present a case of a newborn with intestinal hypoganglionosis leading to intestinal failure and intestinal failure-associated liver disease who responded to Omegaven™, a fat emulsion comprised of omega-3 fatty acids. Omegaven™ has been shown to be beneficial in the management of cholestatic liver injury. Clinical success with Omegaven™ was seen in this patient with a clear decrease in aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase and complete resolution of cholestasis with a direct bilirubin of zero within two weeks of initiation of Omegaven™. No current guidelines for the diagnosis and management of hypoganglionosis are available. We recommend a multidisciplinary approach and the use of novel therapies such as fat emulsions composed of omega-3 fatty acids for improved patient outcomes. Appropriate compassionate use protocols should be obtained from the Food and Drug Administration prior to initiation of Omegaven™.