Portosystemic shunt in children: Outcomes from a pediatric referral center

Thanh Tri Tran , Nguyen Ha Vi Trinh , Phi Duy Ho , Nguyen Nhat Thang Tran , Nguyen An Thuan Luu , Hai Trung Bui , Tuan Kiet Phan
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Abstract

Background

Congenital portosystemic shunts are rare vascular malformations in children that can result in complications such as abnormal liver function, liver masses, pulmonary hypertension, and hepatopulmonary syndrome.

Methods

Patients with portosystemic shunts were managed according to the protocol established at the center. The characteristics and treatment outcomes of patients over a 3-year period (from January 2021 to December 2023) were retrospectively reviewed and reported.

Results

There were 21 pediatric patients, including 13 intrahepatic cases and 8 extrahepatic cases. All intrahepatic cases were detected at birth. The extrahepatic cases were diagnosed at an average age of 6 years. Among the 13 intrahepatic cases, clinical presentations included cholestatic jaundice (7/13) and hyperammonemia (8/13). During follow-up, 10 out of 11 intrahepatic shunts spontaneously closed after 2–10 months of monitoring, while 1/11 shunt remained open after 18 months. Two patients died due to severe pneumonia and heart defects. Among the 8 extrahepatic cases, clinical presentations included liver masses in 3/8 cases, hepatopulmonary syndrome in 1/8 case, hyperammonemia in 5/8 cases, and pulmonary hypertension in 1/8 case. Four cases without intrahepatic portal system were monitored regularly, awaiting liver transplantation if necessary. One case was lost to follow-up. Three cases underwent surgery or endovascular interventions, performed in one or two stages to close the shunt, all resulting in positive outcomes (1 case with hepatopulmonary syndrome fully recovered, 1 case with hyperammonemia and coagulation disorder returned to normal, 1 case with pulmonary hypertension and hyperammonemia did not show improvement in pulmonary hypertension after 6 months, but NH3 levels returned to normal).

Conclusions

Intrahepatic portosystemic shunts detected at birth often close spontaneously. In contrast, extrahepatic shunts are often detected in older children with a variety of symptoms. Surgical or endovascular interventions to close the shunt are the preferred treatment options when possible, yielding good outcomes.
儿童门静脉系统分流:来自儿科转诊中心的结果
背景先天性门系统分流是罕见的儿童血管畸形,可导致肝功能异常、肝肿块、肺动脉高压和肝肺综合征等并发症。方法根据中心制定的方案对门静脉分流患者进行管理。回顾性回顾并报道了3年期间(2021年1月至2023年12月)患者的特征和治疗结果。结果患儿21例,其中肝内13例,肝外8例。所有肝内病例均在出生时发现。肝外病例的平均诊断年龄为6岁。13例肝内病例中,临床表现为胆汁淤积性黄疸(7/13)和高氨血症(8/13)。在随访中,11个肝内分流管中有10个在监测2-10个月后自发关闭,而1/11的分流管在监测18个月后仍保持开放。两名患者死于严重的肺炎和心脏缺陷。8例肝外病例中,临床表现为3/8例肝脏肿块,1/8例肝肺综合征,5/8例高氨血症,1/8例肺动脉高压。4例无肝内门静脉系统,定期监测,必要时等待肝移植。1例失访。3例患者均行手术或血管内介入治疗,分一期或二期关闭分流,均取得阳性结果(1例肝肺综合征完全恢复,1例高氨血症和凝血功能障碍恢复正常,1例肺动脉高压和高氨血症6个月后肺动脉高压无改善,但NH3水平恢复正常)。结论出生时发现的肝门静脉分流常自发关闭。相反,肝外分流常在有各种症状的大龄儿童中发现。手术或血管内介入关闭分流是最好的治疗选择,如果可能,产生良好的结果。
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