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Congenital Portosystemic Shunts in Children: A New Anatomical Classification Correlated With Surgical Strategy

Blanc, Thomas MD*,†; Guerin, Florent MD*; Franchi-Abella, Stéphanie MD; Jacquemin, Emmanuel PhD§; Pariente, Danièle MD; Soubrane, Olivier PhD; Branchereau, Sophie MD*; Gauthier, Frédéric MD*

doi: 10.1097/SLA.0000000000000266
Original Articles

Objective: To propose an anatomical classification of congenital portosystemic shunts (CPSs) correlating with conservative surgery.

Background: CPSs entail a risk of life-threatening complications because of poor portal inflow, which may be prevented or cured by their closure. Current classifications based on portal origin of the shunt are not helpful for planning conservative surgery.

Methods: Twenty-three patients who underwent at least 1 surgical procedure to close the CPSs were included in this retrospective study (1997–2012). We designed a classification according to the ending of the shunt in the caval system. We analyzed the results and outcomes of surgery according to this classification.

Results: Two patients had an extrahepatic portosystemic shunt, 17 had a portacaval shunt [subdivided in 5 end-to-side–like portal-caval, 7 side-to-side–like portal-caval, and 5 H-shaped (H-type portal-caval)], 2 had portal-to-hepatic vein shunts (portohepatic), and 2 had a persistent ductus venosus. All extrahepatic portosystemic shunts, H-type portal-caval, portohepatic, and patent ductus venosus patients had a successful 1-stage ligation. All 5 end-to-side–like portal-caval patients had a threadlike intrahepatic portal venous system; a 2-stage complete closure was successfully achieved for 4 and a partial closure for 1. The first 2 side-to-side–like portal-caval patients had a successful 2-stage closure whereas the 5 others had a 1-stage longitudinal caval partition. All patients are alive and none needed a liver transplantation.

Conclusions: Our classification correlates the anatomy of CPSs and the surgical strategy: outcomes are good provided end-to-side–like portal-caval shunts patients have a 2-stage closure, side-to-side portal-caval shunts patients have a 1-stage caval partition, and the others have a 1-stage ligation.

Supplemental Digital Content is Available in the Text.A new anatomical classification of congenital portosystemic shunt (CPS), based on caval ending of CPS, has been developed during a 15-year experience with 23 patients to facilitate a proactive policy of conservative surgical management. Complete closure of CPS was achieved in 22 patients after a 2-stage procedure in 4.

*Department of Pediatric Surgery, Univ Paris Sud, Hôpitaux Universitaires Paris Sud, APHP, Le Kremlin Bicêtre France

Department of Pediatric Surgery, Univ Paris Descartes, Sorbonne Paris Cité, Hôpital Necker Enfants Malades, APHP, Paris, France

Department of Pediatric Radiology, Univ Paris Sud, Hôpitaux Universitaires Paris Sud, APHP, Le Kremlin Bicêtre France

§Department of Pediatric Hepatology, Univ Paris Sud, Hôpitaux Universitaires Paris Sud, APHP, Le Kremlin Bicêtre France

Department of Hepato-biliary Surgery and Liver Transplantation, Univ Pierre et Marie Curie, Hôpital Saint Antoine, APHP, Paris, France.

Reprints: Frédéric Gauthier, MD, Service de Chirurgie Pédiatrique, Centre Hospitalier Universitaire Bicêtre, 78 rue du Général Leclerc, F-94270, Le Kremlin Bicêtre, France. E-mail:

Disclosure: The research was done without any funding. The authors declare no conflicts of interest.

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© 2014 by Lippincott Williams & Wilkins.