2025 Clinician Corner - A rare cause of neonatal cholestasis: congenital portosystemic shunt

Abstract 

Neonatal cholestasis is a pathological condition characterized by impaired bile flow in infants, typically presenting within the first 3 months of life. While common etiologies include biliary atresia, metabolic disorders, and infections, rare causes such as congenital portosystemic shunts must also be considered in the differential diagnosis. We present the case of a term infant with a congenital portosystemic shunt who presented with cholestasis and hyperammonemia. Bilirubin and liver function enzymes gradually decreased after initiation of ursodeoxycholic acid therapy. In newborns presenting with hypoglycemia, cholestasis, and thrombocytopenia, initial investigations are usually performed for metabolic diseases, congenital infections (toxoplasmosis, cytomegalovirus, rubella, herpes simplex virus), and genetical causes. As these tests may take time to return, early radiological imaging should be pursued to evaluate for anatomical abnormalities.   

Keywords:  Cholestasis; congenital portosystemic shunt; liver; newborn

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Faculty credentials/disclosure 

The planners and faculty for this activity have no relevant financial relationships to disclose. The patient consented to the publication of this report.

Process

Click the "add to cart/begin" button, pay any relevant fee, take the quiz, complete the evaluation, and claim your CME credit.  You must achieve 100% on the quiz with unlimited attempts available.

  • By completing this process, you are attesting that you have read the journal article.

Expiration date:

Credit eligibility for this article is set to expire on November 1, 2026.

Learning Objectives

After completing the article, the learner should be able to:

  • Understand the diagnostic approach to neonatal cholestasis, emphasizing the need for advanced imaging (e.g., Doppler ultrasound, MRI) when initial metabolic and infectious workups are negative. 

  • Recognize congenital portosystemic shunts as a rare but important cause of neonatal cholestasis, describe the appropriate diagnostic workup including early imaging strategies, and outline management considerations for affected infants. 

Key Points 

  • Congenital portosystemic shunts are vascular malformations caused by aberrant portal vein development or persistent fetal shunts (e.g., ductus venosus), resulting in liver bypass and metabolic/systemic complications. 

  • In newborns presenting with hypoglycemia, thrombocytopenia, and cholestasis, imaging plays a critical diagnostic role. Since portosystemic shunts may  escape detection on initial screening, repeated imaging or abdominal computed tomography should be considered. 

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Course summary
Available credit: 
  • 1.00 American Board of Internal Medicine (ABIM) MOC Part 2
    Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to 1.00 MOC points in the American Board of Medicine’s (ABIM) Maintenance of Certification (MOC) program. It is the CME activity provider’s responsibility to submit participant completion information to ACCME for the purpose of granting ABIM MOC credit.
  • 1.00 American Board of Surgery (ABS) Accredited CME
    Successful completion of this CME activity enables the learner to earn credit toward the CME requirement of the American Board of Surgery’s Continuous Certification program. It is the CME activity provider's responsibility to submit learner completion information to ACCME for the purpose of granting ABS credit.
  • 1.00 AMA PRA Category 1 Credit™
    The A. Webb Roberts Center for Continuing Medical Education of Baylor Scott & White Health is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to provide continuing medical education for physicians.
  • 1.00 Attendance
Course opens: 
11/01/2025
Course expires: 
11/01/2026
Cost:
$25.00
Rating: 
0

 

Rıdvan Turhan, MD1, Nezihe Köker, MD, PhD1, Seda Aydoğan, MD2, Asuman Nur Karhan, MD3, and Hasibe Gökçe Çınar, MD4 

 

1 Pediatrics, Inonu University Faculty of Medicine, Malatya, Türkiye  

2 Pediatrics, Newborn Unit, Ankara Etlik City Hospital, Ankara, Türkiye  

3 Pediatrics, Pediatric Gastroenterology, Ankara Etlik City Hospital, Ankara, Türkiye  

4 Radiology, Pediatric Radiology Clinic, Ankara Etlik City Hospital, Ankara, Türkiye  

 

Corresponding author: Nezihe Köker, MD, PhD, Pediatrics, Inonu University Faculty of Medicine, Malatya, Türkiye (e-mail: [email protected]) 

 

Accreditation

The A. Webb Roberts Center for Continuing Medical Education of Baylor Scott & White Health is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to provide continuing medical education for physicians.

Designation

AMA PRA Category 1 Credit™

The A. Webb Roberts Center for Continuing Medical Education of Baylor Scott & White Health designates this Journal-based CME activity for a maximum of 1.0 AMA PRA Category 1 Credit™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

ABS CC

Successful completion of this CME activity enables the learner to earn credit toward the CME requirement of the American Board of Surgery’s Continuous Certification program. It is the CME activity provider's responsibility to submit learner completion information to ACCME for the purpose of granting ABS credit.

ABIM MOC

 Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to 1.0 MOC points in the American Board of Internal Medicine’s (ABIM) Maintenance of Certification (MOC) program. It is the CME activity provider’s responsibility to submit participant completion information to ACCME for the purpose of granting ABIM MOC credit.

 

Available Credit

  • 1.00 American Board of Internal Medicine (ABIM) MOC Part 2
    Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to 1.00 MOC points in the American Board of Medicine’s (ABIM) Maintenance of Certification (MOC) program. It is the CME activity provider’s responsibility to submit participant completion information to ACCME for the purpose of granting ABIM MOC credit.
  • 1.00 American Board of Surgery (ABS) Accredited CME
    Successful completion of this CME activity enables the learner to earn credit toward the CME requirement of the American Board of Surgery’s Continuous Certification program. It is the CME activity provider's responsibility to submit learner completion information to ACCME for the purpose of granting ABS credit.
  • 1.00 AMA PRA Category 1 Credit™
    The A. Webb Roberts Center for Continuing Medical Education of Baylor Scott & White Health is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to provide continuing medical education for physicians.
  • 1.00 Attendance

Price

Cost:
$25.00
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