S. Zielsdorf1,3, S. Kantymyr2, L. Narayan2, C. Weaver3, R. Kohli3, K. Etesami1,3, Y. Genyk1,3, J. Emamaullee1,3 1University Of Southern California,Department Of Surgery,Los Angeles, CA, USA 2University Of Southern California,Keck School Of Medicine,Los Angeles, CA, USA 3Children’s Hospital Los Angeles,Pediatric Liver Transplant Program,Los Angeles, CA, USA
Introduction:
Extrahepatic portal vein obstruction (EHPVO) occurs following obstruction of the extrahepatic portal vein (PV) and is one of the main causes of portal hypertension (PHT) in children. Multiple surgical treatment options have been described, including a variety of portosystemic shunt (PSS) techniques as well as the Meso-Rex shunt (MRS). There is some evidence that MRS results in better outcomes due to restoration of the native physiologic PV outflow, but small series have described post-operative complications including stenosis and thrombosis. In an effort to understand post-operative complication rates and long-term outcomes following surgical interventions for EHPVO, we reviewed our institutional experience and performed a systematic review.
Methods:
Case series– The billing records for the Children’s Hospital of Los Angeles (CHLA) were reviewed from 1/2/2000-5/15/2019 for CPT codes associated with classic portal decompression surgery, including: open portocaval, renoportal, mesocaval, proximal splenorenal, and distal splenorenal anastomoses. Pre-, intra-, and post-operative data on 25 pediatric patients were analyzed retrospectively. Systematic review– PubMed and Google Scholar were searched for articles published in English between 1992 and February 2019. After multiple rounds of filtering, which also involved the exclusion of intrahepatic causes of PHT and patients >18 years old, 25 articles were included in our study, consisting of 276 pediatric patients.
Results:
In our single institution, we identified 25 patients who had undergone PSS procedures. The overall post-shunt thrombosis rate was 4% (N=1/25) over a mean follow-up time of 7.2 ± 4.1 years. There were no post-operative stenoses, gastrointestinal bleeds, or other serious complications. Long-term follow-up also revealed normal growth and development, seen by decreasing z-scores over time. In the systematic review of 276 pediatric patients, there were no differences in patient demographics when MRS (N=184) were compared to PSS (N=92). While the overall post-shunt thrombosis rate was 10.9% (N=30/276), post-operative thrombosis following MRS was significantly higher than PSS (13.6% vs 5.4%, p=0.04).
Conclusion:
This case series and systemic review are the first comprehensive analysis of surgical interventions for EHPVO in children. These data suggest that both MRS and PSS can achieve good results; however, the more technically demanding MRS procedure is associated with significantly higher post-operative shunt thrombosis, which often requires further procedural intervention. Our case series demonstrates that PSS, which may be less physiologic than the MRS, has very low post-operative stenosis and thrombosis rates and did not impact growth in these pediatric patients. This study suggests that the risk of the MRS may outweigh the benefit, when compared to other surgical shunts for pediatric patients with EHPVO.