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KMID : 1038820170200010055
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Pediatric Gastroenterology, Hepatology & Nutrition
2017 Volume.20 No. 1 p.55 ~ p.60
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Intestinal Hypoganglionosis Leading to Intestinal Failure and the Compassionate Use of OmegavenTM
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Khalaf Racha
Karjoo Sara
Danielson Paul
Shakeel Fauzia
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Abstract
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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 OmegavenTM, a fat emulsion comprised of omega-3 fatty acids. OmegavenTM has been shown to be beneficial in the management of cholestatic liver injury. Clinical success with OmegavenTM 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 OmegavenTM. 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 OmegavenTM.
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KEYWORD
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Hirschsprung disease, Omega-3 fatty acids, Liver diseases, Total parenteral nutrition
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