Pediatr Gastroenterol Hepatol Nutr.  2017 Mar;20(1):55-60. 10.5223/pghn.2017.20.1.55.

Intestinal Hypoganglionosis Leading to Intestinal Failure and the Compassionate Use of Omegavenâ„¢

• Khalaf R
• Karjoo S
• Danielson
• Wilsey M
• Shakeel F

• KMID: 2375279
• DOI: http://doi.org/10.5223/pghn.2017.20.1.55

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 Omegavenâ„¢, a fat emulsion comprised of omega-3 fatty acids. Omegavenâ„¢ has been shown to be beneficial in the management of cholestatic liver injury. Clinical success with Omegavenâ„¢ 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 Omegavenâ„¢. 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 Omegavenâ„¢.

Keyword

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

MeSH Terms

Alanine Transaminase
Alkaline Phosphatase
Aspartate Aminotransferases
Bilirubin
Cholestasis
Compassionate Use Trials*
Diagnosis
Empathy*
Emulsions
Fatty Acids, Omega-3
Hirschsprung Disease
Humans
Infant, Newborn
Liver
Liver Diseases
Parenteral Nutrition, Total
United States Food and Drug Administration
Alanine Transaminase
Alkaline Phosphatase
Aspartate Aminotransferases
Bilirubin
Emulsions
Fatty Acids, Omega-3

Figure

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  Fig. 1 Contrast enema obtained on day of life four. The colon is diffusely small in caliber. Microcolon is more prominent in the transverse colon to rectum, and somewhat less so in the cecum right colon.

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  Fig. 2 Full thickness section of ileum. Limited numbers and small clusters of ganglion cells (arrows) seen in submucosal and myenteric plexuses. No fibrosis, nerve proliferation, neuronal dysplasia or myopathic changes identified. Note absence of ganglia in adjacent myenteric zone (H&E, ×100).

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  Fig. 3 Clinical measures indicative of intestinal failure associated liver disease. Pertinent laboratory values prior to and post administration of Omegaven™ and concurrent discontinuation of intravenous lipids. ALT: alanine aminotransferase, AST: aspartate aminotransferase, AP: alkaline phosphatase.

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