Congenital hepatopulmonary fusion (HPF) is a rare anomaly characterized by a fusion between the liver and lung parenchyma. HPF cases have been scarcely reported in the literature. An extensive search of publications was performed in the PubMed and Google Scholar databases without a time limit. In total, 34 clinical case reports were found in the literature, and a study by the Congenital Diaphragmatic Hernia (CDH) Study Group reported data on 10 patients with HPF. Of these 44 infants, 20 were male, 20 were female, and four were reported without gender specification. Nineteen (43.2%) patients required intubation on the first day of life. Six (13.6%) patients had their clinical presentation during the first year of life, and four (9%) clinically presented with HPF between 2.5 and 11 years of age. In these patients, cough, asthma-like symptoms, dyspnea, hemoptysis, right-side chest pain, respiratory infections, and pneumonia were the relevant clinical signs. Right-lung vascular anomalies were present in 18 (40.9%) patients. A complete liver and lung separation was successful in 17 (38.6%) patients. The overall survival was 56.8%. Congenital HPF has no gender predominance. In most cases, it behaves similar to a right CDH in need of resuscitation and intubation after birth. The majority of the cases are discovered during the surgical procedure for CDH. The best surgical approach has not been established and depends on the degree of fusion and vascular anomalies. An advanced imaging assessment is necessary before a surgical approach is attempted. The prognosis is ominous.

先天性肝肺融合 (HPF) 是一种罕见的异常，其特征是肝脏和肺实质之间的融合。HPF病例在文献中几乎没有报道。在 PubMed 和 Google Scholar 数据库中对出版物进行了广泛的搜索，没有时间限制。文献中总共发现了 34 份临床病例报告，先天性膈疝 (CDH) 研究组的一项研究报告了 10 名 HPF 患者的数据。在这 44 名婴儿中，20 名男性，20 名女性，还有 4 名没有明确性别说明。19 名 (43.2%) 患者在出生后第一天需要插管。六名 (13.6%) 患者在出生后第一年出现临床表现，四名 (9%) 患者在 2.5 至 11 岁之间出现 HPF 临床表现。在这些患者中，咳嗽、哮喘样症状，呼吸困难、咯血、右侧胸痛、呼吸道感染和肺炎是相关的临床体征。18 名 (40.9%) 患者存在右肺血管异常。17 名 (38.6%) 患者成功实现肝肺完全分离。总生存率为 56.8%。先天性 HPF 没有性别优势。在大多数情况下，它的表现类似于出生后需要复苏和插管的右侧 CDH。大多数病例是在 CDH 的手术过程中发现的。最佳手术方法尚未确定，取决于融合程度和血管异常情况。在尝试手术方法之前，需要进行高级影像学评估。预示不祥。18 名 (40.9%) 患者存在右肺血管异常。17 名 (38.6%) 患者成功实现肝肺完全分离。总生存率为 56.8%。先天性 HPF 没有性别优势。在大多数情况下，它的表现类似于出生后需要复苏和插管的右侧 CDH。大多数病例是在 CDH 的手术过程中发现的。最佳手术方法尚未确定，取决于融合程度和血管异常情况。在尝试手术方法之前，需要进行高级影像学评估。预示不祥。18 名 (40.9%) 患者存在右肺血管异常。17 名 (38.6%) 患者成功实现肝肺完全分离。总生存率为 56.8%。先天性 HPF 没有性别优势。在大多数情况下，它的表现类似于出生后需要复苏和插管的右侧 CDH。大多数病例是在 CDH 的手术过程中发现的。最佳手术方法尚未确定，取决于融合程度和血管异常情况。在尝试手术方法之前，需要进行高级影像学评估。预示不祥。它的行为类似于出生后需要复苏和插管的右侧 CDH。大多数病例是在 CDH 的手术过程中发现的。最佳手术方法尚未确定，取决于融合程度和血管异常情况。在尝试手术方法之前，需要进行高级影像学评估。预示不祥。它的行为类似于出生后需要复苏和插管的右侧 CDH。大多数病例是在 CDH 的手术过程中发现的。最佳手术方法尚未确定，取决于融合程度和血管异常情况。在尝试手术方法之前，需要进行高级影像学评估。预示不祥。