Abdominal Imaging - Letter to the Editor

Less known but important complications and associated anomalies of Abernethy malformation


  • Ayşe Rüksan Ütebey
  • Furkan Ufuk
  • Muhammed Raşid Aykota

Received Date: 11.03.2021 Accepted Date: 09.01.2022 Diagn Interv Radiol 2023;29(2):410-411 PMID: 36987909

Keywords: Cirrhosis, congenital, diagnosis, liver, portal hypertension, portal vein

Dear Editor,

We read with interest the article “Abernethy malformation: a comprehensive review” by Kumar et al.1, published in Diagnostic and Interventional Radiology. We congratulate the authors for their comprehensive and instructive article on this unusual anomaly. Abernethy malformation is an extremely rare anomaly characterized by portal venous blood passing into the systemic circulation bypassing the liver.1,2 Abernethy malformation can cause pulmonary hypertension, hepatic encephalopathy, hepatopulmonary syndrome, and heart failure.2 Moreover, Abernethy malformation is associated with multiple congenital anomalies (such as cardiovascular and skeletal anomalies) and acquired complications (such as benign and malignant primary hepatic tumors). Therefore, it is essential to recognize this anomaly and follow up regarding these complications.1,2 Although complications and abnormalities accompanying Abernethy malformation are described in detail in Kumar et al.’s1 article, we would like to make a contribution to this article.

According to the pathophysiology, Abernethy malformation can also cause the following clinical consequences: the decrease in hepatic glucose uptake may increase the blood’s glucose level and cause hyperinsulinism and, consequently, hypoglycemia attacks, especially in the pediatric population.3,4 Moreover, there are accompanying osteoporosis cases in patients with Abernethy malformation, primarily due to liver enzymes’ role in vitamin D metabolism.3,5 In addition to the anomalies related to Abernethy malformation stated by Kumar et al.1 it has been shown in the literature that visceral arterial and venous aneurysms can be found (Figure 1).2,6 The awareness of this unusual entity is crucial for the prevention and close monitoring of possible complications, such as abdominal hemorrhage.

Conflict of interest disclosure

The authors declared no conflicts of interest.


  1. Kumar P, Bhatia M, Garg A, Jain S, Kumar K. Abernethy malformation: a comprehensive review. Diagn Interv Radiol. 2022;28(1):21-28.
  2. Ogul H, Bayraktutan U, Yalcin A, et al. Congenital absence of the portal vein in a patient with multiple vascular anomalies. Surg Radiol Anat. 2013;35(6):529-534.
  3. Bas S, Guran T, Atay Z, et al. Premature pubarche, hyperinsulinemia and hypothyroxinemia: novel manifestations of congenital portosystemic shunts (Abernethy malformation) in children. Horm Res Paediatr. 2015;83(4):282-287.
  4. Senniappan S, Pitt K, Shah P, et al. Postprandial hyperinsulinaemic hypoglycaemia secondary to a congenital portosystemic shunt. Horm Res Paediatr. 2015;83(3):217-220.
  5. Peček J, Fister P, Homan M. Abernethy syndrome in Slovenian children: Five case reports and review of literature. World J Gastroenterol. 2020;26(37):5731-5744.
  6. Kong Y, Zhang H, Liu C, et al. Abernethy malformation with multiple aneurysms: incidentally found in an adult woman with Caroli’s disease. Ann Hepatol. 2013;12(2):327-331.