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

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

Abstract

Abstract:

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

Shuai Zhao, Jian Wang, Bing Han, Yichi Zhang, Han Ding, Hao Cai, Jinyang Gu. Liver transplantation for Abernethy malformation complicated with hepatopulmonary syndrome in children: report of one case and literature review[J]. Chinese Journal of Hepatic Surgery(Electronic Edition), 2022, 11(03): 288-293.

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References 24

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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

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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