Impact of Portal Flow on the Prognosis of Children With Congenital Portosystemic Shunt: A Multicentric Observation Study in Japan

被引:1
作者
Uchida, Hajime [1 ]
Shinkai, Masato [2 ]
Okuyama, Hiroomi [3 ]
Ueno, Takehisa [3 ]
Inoue, Mikihiro [4 ]
Yasui, Toshihiro [4 ]
Hiyama, Eiso [5 ]
Kurihara, Sho [5 ]
Sakuma, Yasunaru [6 ]
Sanada, Yukihiro [6 ]
Taketomi, Akinobu [7 ]
Honda, Shohei [7 ]
Wada, Motoshi [8 ]
Ando, Ryo [8 ]
Fujishiro, Jun [9 ]
Yoshida, Mariko [9 ]
Yamada, Yohei [10 ]
Uchida, Hiroo [11 ]
Tainaka, Takahisa [11 ]
Kasahara, Mureo [1 ]
机构
[1] Natl Ctr Child Hlth & Dev, Organ Transplantat Ctr, 2-10-1 Okura,Setagaya Ku, Tokyo 1578535, Japan
[2] Kanagawa Childrens Med Ctr, Dept Surg, Yokohama, Japan
[3] Osaka Univ, Grad Sch Med, Dept Pediat Surg, Suita, Osaka, Japan
[4] Fujita Hlth Univ, Dept Pediat Surg, Toyoake, Aichi, Japan
[5] Hiroshima Univ Hosp, Dept Pediat Surg, Hiroshima, Japan
[6] Jichi Med Univ, Dept Surg, Div Gastroenterol Gen & Transplant Surg, Shimotsuke, Japan
[7] Hokkaido Univ, Grad Sch Med, Dept Gastroenterol Surg 1, Sapporo, Japan
[8] Tohoku Univ, Grad Sch Med, Dept Pediat Surg, Sendai, Japan
[9] Univ Tokyo, Fac Med, Dept Pediat Surg, Tokyo, Japan
[10] Keio Univ, Sch Med, Dept Pediat Surg, Tokyo, Japan
[11] Nagoya Univ, Dept Pediat Surg, Grad Sch Med, Nagoya, Aichi, Japan
关键词
Liver transplantation; Portal hypertension; Portal vein; Portopulmonary hypertension; Portosystemic shunt; DONOR LIVER-TRANSPLANTATION; HEPATOCELLULAR LESIONS; PULMONARY-HYPERTENSION; ABSENCE; HEPATOBLASTOMA; PATIENT;
D O I
10.1016/j.jpedsurg.2024.05.008
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
Background: Although congenital portosystemic shunts (CPSSs) are increasingly being recognized, the optimal treatment strategies and natural prognosis remain unclear, as individual CPSSs show different phenotypes. Methods: The medical records of 122 patients who were diagnosed with CPSSs at 15 participating hospitals in Japan between 2000 and 2019 were collected for a retrospective analysis based on the state of portal vein (PV) visualization on imaging. Results: Among the 122 patients, 75 (61.5%) showed PV on imaging. The median age at the diagnosis was 5 months. The main complications related to CPSS were hyperammonemia (85.2%), liver masses (25.4%), hepatopulmonary shunts (13.9%), and pulmonary hypertension (11.5%). The prevalence of complications was significantly higher in patients without PV visualization than in those with PV visualization (P < 0.001). Overall, 91 patients (74.6%) received treatment, including shunt closure by surgery or interventional radiology (n = 82) and liver transplantation (LT) or liver resection (n = 9). Over the past 20 years, there has been a decrease in the number of patients undergoing LT. Although most patients showed improvement or reduced progression of symptoms, liver masses and pulmonary hypertension were less likely to improve after shunt closure. Complications related to shunt closure were more likely to occur in patients without PV visualization (P = 0.001). In 25 patients (20.5%) without treatment, those without PV visualization were significantly more likely to develop complications related to CPSS than those with PV visualization (P = 0.011). Conclusion: Patients without PV visualization develop CPSS-related complications and, early treatment using prophylactic approaches should be considered, even if they are asymptomatic. (c) 2024 Elsevier Inc. All rights are reserved, including those for text and data mining, AI training, and similar technologies.
引用
收藏
页码:1791 / 1797
页数:7
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