Clinical StudyAbernethy Malformations: Evaluation and Management of Congenital Portosystemic Shunts
Section snippets
Materials and Methods
After obtaining approval from the institutional review board, a retrospective review was performed of the electronic medical records and diagnostic imaging reports of a database of 42 patients identified with a CPSS at a single children’s hospital from 2005 through 2018. Figure 2 illustrates the approach. Patients with a confirmed diagnosis of CPSS by ultrasound, computed tomography (CT), or magnetic resonance imaging (MRI) were included. No patients were excluded from the study. Clinical
Results
Hyperammonemia was diagnosed on initial presentation in 85% of patients (n = 33), with a mean serum ammonia level of 82.5 ± 10.3 μmol/L (median, 82; range, 20–166). Preoperative serum ammonia levels were 78.7 ± 25.9 μmol/L in patients who underwent endovascular closure and 83.3 ± 11.8 μmol/L in patients who underwent surgical closure (P = .73), with postoperative levels of 35.4 ± 11.1 μmol/L and 39.0 ± 5.3 μmol/L (P = .54), respectively. Ammonia levels in the entire patient group decreased from
Discussion
The anatomic and physiologic information obtained by direct catheterization of a CPSS is central to the evaluation of congenital portosystemic shunts and directs treatment decisions. Diagnostic venography with portal pressure measurement, including temporary balloon occlusion of the shunt if technically feasible, simulates the physiology of shunt closure and defines portal system anatomy. Measurements obtained during this procedure help determine whether shunt closure may be safely performed in
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Cited by (19)
Percutaneous sequential closure of an Abernethy malformation: A case report
2023, Radiology Case ReportsHepatic Vascular Disorders: From Diagnosis to Interventional Radiology
2022, Seminars in Ultrasound, CT and MRICitation Excerpt :To determine portal pressure after occlusion a venography is performed and the shunt temporarily occluded with a balloon. Portal pressure during occlusion above 24 mmHg and a portal pressure gradient before/after occlusion above 9 mmHg indicates high risk for portal hypertension and thus precludes a single session shunt closure (Fig. 19).29 Traditionally endovascular treatment is performed in shunts with an appropriate size for safe embolization with coils or plugs that can be occluded in a single, while the remaining are surgically approached.27
Surgical shunt ligation for a congenital extrahepatic portosystemic shunt with pulmonary hypertension: A case report
2022, International Journal of Surgery Case ReportsCitation Excerpt :This is because the intrahepatic portal vein is diminutive, and the portal venous system may not adequately accommodate the increased blood flow. The criteria for safe shunt occlusion to avoid acute portal hypertension have been reported [8,9]. Primary shunt ligation is indicated if the change in portal venous pressure before and after shunt occlusion is less than 10 mmHg and the portal venous pressure after occlusion is less than 25 mmHg.
Endovascular treatment of a portosystemic shunt presenting with hypoglycemia; case presentation and review of literature
2022, Clinical ImagingCitation Excerpt :These shunts are divided into two categories: Type I (complete) which are complete shunts with no perfusion to liver parenchyma and type II (partial) with some perfusion to the liver.1 Other classifications are based on location of the shunt in relation to the liver as well as presence or absence of the portal vein,2 origin of the shunt,3 severity of the shunt,4 systemic drainage site5 or combination of number and pattern of communications, shunt origin, and systemic communication.6 Current recommendations for treatment of extrahepatic shunts is early closure before one year of age to avoid complications.7
Congenital extrahepatic portosystemic shunt type II occluded with cardiac closure device
2021, Radiology Case ReportsCitation Excerpt :A two-step approach, with reduction of the shunt size months before complete closure, is advocated when a high portal pressure is recorded during shunt occlusion (cut-off level described in the literature ranging from 25 to 32 mmHg) or when there is a pressure increase of at least 10 mmHg after shunt occlusion. When the occlusion pressure remains less than 25 mmHg and the pressure gradient less than 10 mmHg, the shunt can be safely closed in one session [9,11]. This was the case in this patient and the procedure took place without complications.
Abernethy Malformation: An Unusual Extrathoracic Cause of Chronic Hypoxemia in Pediatrics
2021, Archivos de Bronconeumologia
None of the authors have identified a conflict of interest.