Clinical Study
Abernethy Malformations: Evaluation and Management of Congenital Portosystemic Shunts

https://doi.org/10.1016/j.jvir.2019.08.007Get rights and content

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.

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

References (18)

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    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.

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None of the authors have identified a conflict of interest.

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