Literature DB >> 32107126

Abernethy Malformations: Evaluation and Management of Congenital Portosystemic Shunts.

Shankar Rajeswaran1, Andrew Johnston2, Jared Green3, Ahsun Riaz4, Bartley Thornburg4, Samdeep Mouli4, Timothy Lautz5, Caroline Lemoine5, Riccardo Superina5, James Donaldson3.   

Abstract

PURPOSE: To assess the utility of preoperative venography in evaluating and managing patients with congenital portosystemic shunts (CPSSs).
MATERIALS AND METHODS: A retrospective study was performed of 42 patients (62% female; median age, 4.1 years) diagnosed with a CPSS from 2005 to 2018. Preoperative venography (n = 39) and balloon occlusive pressure measurements (n = 33) within the mesenteric venous system guided treatment. Primary outcome was serum ammonia levels at 1 month after shunt closure. Management strategies included single (n = 12) or staged (n = 18) operative ligation, endovascular occlusion (n = 8), combined surgical and endovascular closure (n = 2), and observation (n = 2).
RESULTS: At 1 month, serum ammonia levels decreased from 82.5 ± 10.3 μmol/L to 38.4 ± 4.6 μmol/L (P < .001). No difference was observed in the decrease between patients treated surgically or endovascularly (P = .91). Mean occluded to non-occluded pressure gradients were significantly lower for endovascular closure (5.3 ± 1.8 mmHg) than for surgical closure (12.3 ± 3.3 mmHg, P = .02). Shunts were classified as extrahepatic in 29 patients and as intrahepatic in 13 patients; all shunts demonstrated filling of the portal system with occlusive venography. Broad and short shunts were closed surgically; narrow and long shunts were closed endovascularly. Shunts were closed in a single session (n = 20) if the pressure gradient was less than 10 mmHg and the occluded mesenteric pressure was less than 25 mmHg.
CONCLUSIONS: Preoperative venography delineates shunt morphology, and balloon occlusion simulates closure hemodynamics. This information is necessary to determine whether definitive closure should be performed through endovascular or surgical methods and whether closure should be performed in a single or staged setting.
Copyright © 2019 SIR. Published by Elsevier Inc. All rights reserved.

Entities:  

Year:  2020        PMID: 32107126     DOI: 10.1016/j.jvir.2019.08.007

Source DB:  PubMed          Journal:  J Vasc Interv Radiol        ISSN: 1051-0443            Impact factor:   3.464


  5 in total

Review 1.  Physiological fetal vascular shunts and failure to regress: what the radiologist needs to know.

Authors:  Michael A Leshen; Rajiv Devanagondi; David Saul; Apeksha Chaturvedi
Journal:  Pediatr Radiol       Date:  2022-02-15

2.  A case of two shunts in the endovascular treatment of type II Abernethy syndrome.

Authors:  Brenden Bombardier; Adam Alli; Aaron Rohr; Zachary Collins; Kavi Raval
Journal:  CVIR Endovasc       Date:  2022-01-05

3.  Congenital Porto-Azygous Shunt (Abernethy Malformation Type II) in an Elderly Patient: A Too-Often-Forgotten Occult Abnormality.

Authors:  Steven Tessier; Firas Ido; Thomas Zanders; Santo Longo; Sudip Nanda
Journal:  Cureus       Date:  2022-04-25

4.  Surgical shunt ligation for a congenital extrahepatic portosystemic shunt with pulmonary hypertension: A case report.

Authors:  Kensuke Yamada; Satoshi Matsukuma; Yukio Tokumitsu; Yoshitaro Shindo; Yasuhiro Ikeda; Hiroaki Nagano
Journal:  Int J Surg Case Rep       Date:  2022-04-02

5.  Percutaneous transhepatic treatment of a unique portal vein malformation with portal hypertension in a pediatric patient.

Authors:  Paolo Marra; Ludovico Dulcetta; Claudia Pellegrinelli; Lorenzo D'Antiga; Sandro Sironi
Journal:  CVIR Endovasc       Date:  2021-06-07
  5 in total

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