Hepatic pulmonary fusion (HPF) is a very rare congenital disease which is characterized by a fibrous connection between the liver and lung tissues. It is commonly associated with congenital diaphragmatic hernia (CDH), pulmonary sequestration, congenital heart disease and other diseases. Surgical operation has been reported to be the only option for the treatment of this disease. The most sophisticated point lies in how to define the dividing line between liver and lung fusion tissues. And the postoperative mortality is high. At present, the etiology and pathogenesis of HPF are not completely clear. In this study, we reported a case of a 4-month-old male infant presented with cough and shortness of breath and intraoperatively found to have HPF associated with atrial septal defect and scimitar syndrome. Staged surgery was performed to avoid the simultaneous involvement of multiple organs such as heart, lung and liver, and shorten the operation and anesthesia time to a certain extent, improving the success rate of the operation. We only separated the fusion tissues and repaired the diaphragmatic hernia in the first operation, and in the second surgery, we conducted intra-cardiac repair of cardiac malformations. The follow-up results showed that the right lung gradually developed and there were no significant abnormalities in liver. This experience can provide a useful reference for future cases.
Keywords: Hepatic pulmonary fusion (HPF); case report; congenital diaphragmatic hernia (CDH); congenital heart malformation; staged surgery.
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