The coincidence of Abernethy malformation and blue rubber bleb nevus syndrome: A case report

Authors

  • Thipsumon Tangsiwong Buddhachinaraj Phitsanulok Hospital, Phitsanulok, Thailand
  • Siriwan Wananukul Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand
  • Panruethai Trinavarat Faculty of Medicine, Chulalongkorn University and King Chulalongkorn Memorial Hospital, Bangkok, Thailand

Keywords:

Abernethy malformation, blue rubber bleb nevus syndrome, congenital absence of portal vein, vascular malformations

Abstract

Abernethy malformation was demonstrated in a 10-year-old boy, whose underlying disease of blue rubber bleb nevus syndrome (BRBNS) was achieved first-time-right diagnosis by cutaneous stigmata, presenting with persistent gross hematuria and hemodynamic instability secondary to vesical venous malformation. Abdominal computed tomography (CT) imaging was obtained to explore identifiable and treatable causes of the active bleeding, revealing Abernathy malformation and unusual visceral slow-flow vascular malformations including venous varicosities and splenic lymphangiomatosis. This is a first case report of the coincidence of Abernathy malformation and BRBNS. Imaging played an important role in detection of clinically occult congenital portal venous anomalies and visceral vascular malformations.

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Published

2023-07-18

How to Cite

1.
Tangsiwong T, Wananukul S, Trinavarat P. The coincidence of Abernethy malformation and blue rubber bleb nevus syndrome: A case report. Chula Med J [Internet]. 2023 Jul. 18 [cited 2024 Oct. 12];66(4). Available from: https://he05.tci-thaijo.org/index.php/CMJ/article/view/108