TY - JOUR
T1 - Surgical strategy according to the anatomical types of congenital portosystemic shunts in children
AU - Matsuura, Toshiharu
AU - Takahashi, Yoshiaki
AU - Yanagi, Yusuke
AU - Yoshimaru, Koichiro
AU - Yamamura, Kenichiro
AU - Morihana, Eiji
AU - Nagata, Hazumu
AU - Uike, Kiyoshi
AU - Takada, Hidetoshi
AU - Taguchi, Tomoaki
N1 - Funding Information:
The authors thank Mr. Brian Quinn for help in the preparation of the manuscript. This research was partly supported by JSPS KAKENHI Grant number 15K10029 .
Publisher Copyright:
© 2016 Elsevier Inc.
PY - 2016/12/1
Y1 - 2016/12/1
N2 - Background Congenital portosystemic shunts (CPSS) with intrahepatic portal vein (IHPV) hypoplasia or absence cause encephalopathy or pulmonary hypertension (PH). Acute shunt closure may result in postoperative portal hypertension. The aim of this study was to propose a surgical strategy according to the anatomical types of CPSS and IHPV. Methods Twenty-three CPSS patients were diagnosed from1990 to 2015. All patients were evaluated by computed tomography, angiography, and PV pressure monitoring under a shunt occlusion test. CPSS were categorized into 5 types according to the anatomical shunt location. Results The median age at diagnosis was 34 months. Three of 23 total patients, who had an extrahepatic portosystemic shunt with a hypoplastic IHPV, died before treatment initiation because of severe PH. Fourteen cases received surgical or interventional treatment at the median age of 5 years. A total of 6 cases received surgical therapy, including liver transplants for 2 absent IHPV cases. The remaining 8 cases received interventional coiling. All shunt ligations were successfully accomplished in 1-stage ligation without any complications. After the treatment, the hypoplastic IHPV gradually enlarged with an efficient portal inflow. Conclusion A precise pretreatment anatomical evaluation of CPSS and IHPV types is mandatory for the selection of surgical treatment. Level of evidence Diagnostic study – level II and treatment study – level III.
AB - Background Congenital portosystemic shunts (CPSS) with intrahepatic portal vein (IHPV) hypoplasia or absence cause encephalopathy or pulmonary hypertension (PH). Acute shunt closure may result in postoperative portal hypertension. The aim of this study was to propose a surgical strategy according to the anatomical types of CPSS and IHPV. Methods Twenty-three CPSS patients were diagnosed from1990 to 2015. All patients were evaluated by computed tomography, angiography, and PV pressure monitoring under a shunt occlusion test. CPSS were categorized into 5 types according to the anatomical shunt location. Results The median age at diagnosis was 34 months. Three of 23 total patients, who had an extrahepatic portosystemic shunt with a hypoplastic IHPV, died before treatment initiation because of severe PH. Fourteen cases received surgical or interventional treatment at the median age of 5 years. A total of 6 cases received surgical therapy, including liver transplants for 2 absent IHPV cases. The remaining 8 cases received interventional coiling. All shunt ligations were successfully accomplished in 1-stage ligation without any complications. After the treatment, the hypoplastic IHPV gradually enlarged with an efficient portal inflow. Conclusion A precise pretreatment anatomical evaluation of CPSS and IHPV types is mandatory for the selection of surgical treatment. Level of evidence Diagnostic study – level II and treatment study – level III.
UR - http://www.scopus.com/inward/record.url?scp=85000350864&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85000350864&partnerID=8YFLogxK
U2 - 10.1016/j.jpedsurg.2016.09.046
DO - 10.1016/j.jpedsurg.2016.09.046
M3 - Article
C2 - 27697318
AN - SCOPUS:85000350864
SN - 0022-3468
VL - 51
SP - 2099
EP - 2104
JO - Journal of Pediatric Surgery
JF - Journal of Pediatric Surgery
IS - 12
ER -