肝移植治疗儿童Abernethy畸形合并肝肺综合征一例并文献复习

doi:10.3877/cma.j.issn.2095-3232.2022.03.015

目的

探讨儿童Abernethy畸形合并肝肺综合征的诊治。

方法

回顾性分析2020年6月5日在上海交通大学医学院附属新华医院行肝移植治疗的1例儿童Abernethy畸形合并肝肺综合征患者临床资料，并复习相关文献。患儿男，12岁，因"口唇发绀2年，发现肝硬化3个月"入院。肝脏三维重建门静脉示门静脉左、右支及主干近左、右支段严重狭窄。超声心动图示气泡试验阳性。术前诊断：Abernethy畸形，肝纤维化4期，门静脉高压症，肝肺综合征。患儿监护人已签署知情同意书，符合医学伦理学规定。

结果

完善术前准备后行亲体肝移植+脾动脉结扎术。术后采用他克莫司+糖皮质激素免疫抑制治疗。患儿手术成功，肝功能顺利恢复。术后高流量吸氧仪治疗，逐渐下调氧流量及氧浓度。术后2周更换为鼻导管吸氧。术后3周出院，出院后在家鼻导管吸氧，术后5个月脱离吸氧。肝脏病理学检查示弥漫性肝纤维化伴静脉异常改变。

结论

肝移植是治疗儿童Abernethy畸形合并肝肺综合征的有效手段，术前应进行全面评估，术后进行高流量吸氧恢复治疗。

关键词: Abernethy畸形, 肝肺综合征, 肝移植, 诊断, 治疗, 儿童

Objective

To investigate the diagnosis and treatments of Abernethy malformation complicated with hepatopulmonary syndrome in children.

Methods

Clinical data of 1 child with Abernethy malformation complicated with hepatopulmonary syndrome who underwent liver transplantation in Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine on June 5, 2020 were retrospectively analyzed. Relevant literature review was performed. A 12-year-old boy was admitted to our hospital due to "perioral cyanosis for 2 years and liver cirrhosis for 3 months". Three-dimensional reconstruction of portal vein showed severe stenosis of the left and right branches of hepatic portal vein and the left and right branches adjacent to the common trunk. Echocardiography showed a positive result for the bubble test. Preoperative diagnosis: Abernethy malformation, stage Ⅳ hepatic fibrosis, portal hypertension and hepatopulmonary syndrome. The informed consents of the guardians of this child were obtained and the local ethical committee approval was received.

Results

After comprehensive preoperative preparations, living related liver transplantation combined with splenic artery ligation were performed. Postoperatively, tacrolimus and glucocorticoid were adopted for immunosuppressive therapy. The child successfully completed the surgery and liver function recovered properly. High-flow oxygen inhalation was performed postoperatively, with gradually declining oxygen flow and concentration, and was changed to nasal cannula oxygen inhalation at 2 weeks after surgery. The child was discharged at postoperative 3 weeks and treated with nasal cannula oxygen inhalation at home, which was removed at 5 months after surgery. Pathological examination of the liver revealed diffuse hepatic fibrosis complicated with abnormal venous changes.

Conclusions

Liver transplantation is an effective treatment for pediatric Abernethy malformation complicated with hepatopulmonary syndrome. Comprehensive preoperative evaluation should be delivered, and high-flow oxygen inhalation can be adoptd after surgery.

Key words: Abernethy malformation, Hepatopulmonary syndrome, Liver transplantation, Diagnosis, Treatment, Child

图1 一例儿童Abernethy畸形合并肝肺综合征肝脏三维重建注：基于肝脏增强CT采用IQQA-3D软件完成三维重建，示门静脉左、右支及近左、右支的主干段严重狭窄

图2 一例儿童Abernethy畸形合并肝肺综合征患儿母亲供肝三维重建注：基于肝脏增强CT使用IQQA-3D软件完成，示带肝中静脉左半肝供肝，供肝体积468 ml

表1 一例儿童Abernethy畸形合并肝肺综合征患者肝移植术前及脱氧期动脉血气分析结果

指标	移植术前（吸氧，8 L/min）	移植术前（不吸氧）	脱氧期（不吸氧）
pH	7.35	7.49	7.46
PaO₂（mmHg）	134.2	41.2	86.6
PaCO₂（mmHg）	37.0	27.4	38.2
SaO₂（%）	96.6	74.8	92.5
BE	-3.3	-3.9	-2.8

表1 一例儿童Abernethy畸形合并肝肺综合征患者肝移植术前及脱氧期动脉血气分析结果

指标	移植术前（吸氧，8 L/min）	移植术前（不吸氧）	脱氧期（不吸氧）
pH	7.35	7.49	7.46
PaO₂（mmHg）	134.2	41.2	86.6
PaCO₂（mmHg）	37.0	27.4	38.2
SaO₂（%）	96.6	74.8	92.5
BE	-3.3	-3.9	-2.8

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Liver transplantation for Abernethy malformation complicated with hepatopulmonary syndrome in children: report of one case and literature review

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Liver transplantation for Abernethy malformation complicated with hepatopulmonary syndrome in children: report of one case and literature review