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Congenital portosystemic shunts in children: a new anatomical classification correlated with surgical strategy
Thomas Blanc1, Florent Guerin, Stéphanie Franchi-Abella
1*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.
Insights
A new anatomical classification for congenital portosystemic shunts (CPSs) aids surgical planning. This classification helps determine the best surgical approach for successful closure and improved patient outcomes.
Area of Science:
- Vascular Surgery
- Pediatric Surgery
- Anatomy
Background:
- Congenital portosystemic shunts (CPSs) pose life-threatening risks due to poor portal inflow.
- Current classifications do not effectively guide surgical planning for CPS closure.
- Effective surgical closure of CPSs can prevent or cure serious complications.
Purpose of the Study:
- To introduce a novel anatomical classification for congenital portosystemic shunts.
- To correlate this classification with successful conservative surgical strategies.
- To improve surgical planning and patient outcomes for CPSs.
Main Methods:
- Retrospective analysis of 23 patients undergoing surgical closure of CPSs (1997-2012).
- Development of a classification system based on the shunt's termination point in the caval system.
- Evaluation of surgical outcomes based on the proposed anatomical classification.
Main Results:
- The study identified various CPS types including extrahepatic, portacaval (end-to-side-like, side-to-side-like, H-type), portohepatic, and persistent ductus venosus.
- Successful one-stage ligation was achieved for extrahepatic, H-type, portohepatic, and patent ductus venosus shunts.
- Two-stage closure was effective for end-to-side-like portal-caval shunts, while side-to-side portal-caval shunts benefited from one-stage caval partition.
- All patients survived without requiring liver transplantation.
Conclusions:
- The proposed anatomical classification effectively correlates CPS anatomy with surgical strategy.
- Optimal outcomes are achieved when end-to-side-like portal-caval shunts undergo two-stage closure.
- Side-to-side portal-caval shunts are best treated with one-stage caval partition, and other types with one-stage ligation.
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.
