Mesenterico-left portal vein bypass in children with congenital extrahepatic portal vein thrombosis: a unique curative approach.
OBJECTIVE
Current management of extrahepatic portal vein thrombosis (EPVT) comprises endoscopic eradication therapy of esophageal varices and conventional shunt surgery. The authors have used the novel technique of mesenterico-left portal bypass (Rex shunt) in seven children with symptomatic EPVT, and report their results here.


METHODS
Median age of the children was 12 years (range, 2-16 years). All children had portal hypertension with hypersplenism and recurrent bleeding from esophageal varices. Furthermore, one patient suffered from a severe hepatopulmonary syndrome. Preoperative evaluation included liver function tests, liver biopsy, hepatic duplex ultrasonography, and radiologic evaluation of the intrahepatic and extrahepatic vascular anatomy. The internal jugular vein was used as vein graft in all patients.


RESULTS
Median follow-up period was 15 months (range, 3-28 months). Ultrasound scans revealed sufficient perfusion in all shunts (median, 35 cm/s; range, 28-60 cm/s). The intrahepatic portal perfusion in segment 4 improved from a median of 6 cm/s before surgery to 18 cm/s postoperatively. The platelet count increased within 3 months from a mean of 50,625/microL to 137,750/microL. The clinical signs of hypoxemia in the child with hepatopulmonary syndrome disappeared within 6 months.


CONCLUSIONS
In accordance with the limited experience published by others, the authors' data confirmed the mesenterico-portal Rex shunt as the therapy of choice for children with EPVT. Furthermore, this report is the first to show that a hepatopulmonary syndrome can be abolished by mesenterico-portal Rex shunt.
Fuchs
Jörg
J
Department of Pediatric Surgery, University Hospital, University of Tübingen, Hoppe-Seyler-Strasse 3, 72076 Tübingen, Germany. fuchs.joerg@med.uni-tuebingen.de
Warmann
Steven
S
Kardorff
Rüdiger
R
Rosenthal
Herbert
H
Rodeck
Burkhard
B
Ure
Benno
B
Melter
Michael
M
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000601:surgery; D007601:Jugular Veins / Q000637:transplantation; D008099:Liver / Q000000981:diagnostic imaging / Q000473:pathology; D008111:Liver Function Tests; D008297:Male; D008642:Mesenteric Veins / Q000601:surgery*; D011169:Portal Vein / Q000601:surgery*; D011170:Portasystemic Shunt, Surgical / Q000379:methods*; D011859:Radiography; D016896:Treatment Outcome; D014463:Ultrasonography; D020246:Venous Thrombosis / Q000151:congenital / Q000601:surgery*