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. Author manuscript; available in PMC: 2022 Mar 15.
Published in final edited form as: J Pediatr. 2021 Oct 21;241:261–262. doi: 10.1016/j.jpeds.2021.10.010

Neonatal Presentation of Congenital Portosystemic Shunt

Rebecca L Shay 1, Alyssa Goldberg 2, Shikha S Sundaram 3, Lorna P Browne 4, Clyde J Wright 5,*, Aparna Annam 6,*
PMCID: PMC8923355  NIHMSID: NIHMS1781969  PMID: 34687689

Agrowth-restricted neonate was born at 34 weeks gestation with a birth weight of 1.28 kg to a 33-year-old woman with unremarkable prenatal laboratory test results. During his first 6 weeks of life in the neonatal intensive care unit, he demonstrated persistent hypoglycemia requiring continuous enteral feeding, oral feeding immaturity, respiratory insufficiency, cholestasis, elevated liver enzymes, and hyperammonemia. In addition, he had a history of anemia, thrombocytopenia, and coagulopathy, requiring packed red blood cells, platelets, fresh frozen plasma, and cryoprecipitate transfusions. He was transferred to a quaternary care neonatal intensive care unit for further diagnostic and subspecialty evaluation on day of life 44. An abdominal ultrasound with doppler and an abdominal computed tomography venogram confirmed an intrahepatic portosystemic shunt from the middle hepatic vein to the left portal vein (Figure; Video available at www.jpeds.com).

Figure.

Figure.

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A, Ultrasound and B, computed tomography venogram demonstrating a connection between dilated hepatic and portal venous systems. LPV, left portal vein; MHV, middle hepatic vein.

The shunt was embolized using detachable coils from a jugular approach at 43 weeks postconceptual age with a weight of 5 kg. After coil embolization of the shunt, the patient had rapid normalization of all markers of portosystemic shunting, including resolution of cholestasis, hyperammonemia, and normalization of his alpha fetoprotein levels. He was able to tolerate consolidated gastric feeds while maintaining euglycemia. He required less respiratory support and was ultimately discharged home on low oxygen via nasal cannula (1/8 L/min); he was able to be weaned to room air within 4 weeks of discharge.

Congenital portosystemic shunts (CPSS) are rare malformations with a variety of clinical presentations. Owing to improvements in imaging modalities, there are increasing reports of children with documented CPSS; these are described anatomically owing to differences in outcomes related to the vessels involved.1–3 The clinical presentation of CPSS can include cholestasis, hepatopulmonary syndrome, pulmonary hypertension, encephalopathy, hypoglycemia, coagulopathy, heart failure, liver failure, hyperandrogenism, hepatic nodules, and hepatocellular carcinoma.1,2,4

Some studies report the clinical option of following CPSS over time to assess for spontaneous shunt closure, but alternatives to the observational approach should be considered in neonatal patients with any complications related to the shunt other than cholestasis.1,5 Techniques for closure depending on the anatomy of the shunt include percutaneous Amplatzer devices or coils, surgical placement of a covered stent to exclude the shunt from systemic circulation with subsequent anticoagulation therapy, and surgical banding followed by complete closure to avoid portal hypertension.1

This case reveals a variety of clinical comorbidities that can be related to a CPSS in the neonate and demonstrates the value of review of early postnatal ultrasound examinations obtained in the setting of a workup for neonatal cholestasis.

Contributor Information

Rebecca L. Shay, Department of Pediatrics, University of Colorado Hospital, Section of Neonatology, Children’s Hospital Colorado.

Alyssa Goldberg, Department of Pediatrics, University of Colorado Hospital, Section of Gastroenterology, Hepatology and Nutrition, Children’s Hospital Colorado.

Shikha S. Sundaram, Department of Pediatrics, University of Colorado Hospital, Section of Gastroenterology, Hepatology and Nutrition, Children’s Hospital Colorado.

Lorna P. Browne, Department of Radiology, University of Colorado Hospital, Section of Radiology, Children’s Hospital Colorado.

Clyde J. Wright, Department of Pediatrics, University of Colorado Hospital, Section of Neonatology, Children’s Hospital Colorado.

Aparna Annam, Department of Radiology, University of Colorado Hospital, Section of Radiology, Children’s Hospital Colorado, Aurora, Colorado.

References

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