Hepatopulmonary fusion associated with congenital diaphragmatic hernia: A case series

IF 0.2 Q4 PEDIATRICS
Nicole Chicoine , Carrie Foster , Mihai Puia-Dumitrescu , Jimiane Ashe , Rebecca Stark , Samuel E. Rice-Townsend
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Abstract

Introduction

Hepatopulmonary Fusion (HPF) is an exceedingly rare anomaly associated with right sided congenital diaphragmatic hernia (CDH) and has a high mortality rate.

Case presentations

Three neonates were cared for at our pediatric medical center for management of their CDH and coinciding HPF. Case 1 was a former term female who had a prenatal diagnosis of partial anomalous pulmonary venous return (PAPVR). She developed respiratory distress shortly after birth, which prompted further imaging. She underwent a computed tomography angiography (CTA) that showed a right CDH and abnormal perfusion of the superior aspect of the right hepatic lobe, suggestive of anomalous pulmonary venous return to the right portal system. HPF was suspected based on that finding. At 2 months old she underwent a successful separation of the fusion and a mesh repair of the CDH. The patient survived to the hospital discharge. Case 2 was a late preterm female with a prenatal diagnosis of a right CDH and images on prenatal echocardiogram suggestive of atypical venous return to the liver. HPF was diagnosed incidentally during the repair of the CDH. The fusion was separated with electrocautery and a stapler. The CDH defect was repaired with a mesh. The patient survived to the hospital discharge. Case 3 was a former term female who underwent repair of her right CDH on day of life 5. Her HPF was identified at the time of the CDH repair and was separated using electrocautery and a stapler. Her course was notable for refractory pulmonary hypertension with infectious complications, which eventually resulted in her demise during the index hospitalization.

Conclusion

While HPF is typically encountered incidentally in the operating room during the repair of a right CDH, it should be suspected in patient who have vascular anomalies of the right hepatopulmonary region.
肝肺融合合并先天性膈疝:一个病例系列
肝肺融合(HPF)是一种非常罕见的与右侧先天性膈疝(CDH)相关的异常,死亡率很高。病例介绍:在我们的儿科医疗中心治疗了3例新生儿,以管理他们的CDH和合并HPF。病例1是一位前足月女性,产前诊断为部分肺静脉回流异常(PAPVR)。她出生后不久就出现呼吸窘迫,这促使她进行了进一步的影像学检查。她接受了计算机断层血管造影(CTA),显示右侧CDH和右肝叶上侧异常灌注,提示肺静脉返回右侧门静脉系统异常。HPF是基于这一发现被怀疑的。2个月大时,她接受了成功的融合分离和CDH的补片修复。病人活到出院。病例2是一名晚期早产女性,产前诊断为右侧CDH,产前超声心动图显示非典型静脉回流肝脏。HPF是在CDH修复过程中偶然诊断出来的。用电灼和订书机分离融合。用补片修复CDH缺损。病人活到出院。病例3是一位前足月女性,在出生后第5天接受了右侧CDH的修复。她的HPF在CDH修复时被确定,并使用电灼和订书机分离。她的病程以难治性肺动脉高压伴感染性并发症而著称,最终导致她在第一次住院期间死亡。结论HPF通常在手术室右侧CDH修复过程中偶然发生,但在右肝肺区血管异常的患者中应予以怀疑。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
CiteScore
0.60
自引率
25.00%
发文量
348
审稿时长
15 days
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