nt J Surg Case Rep
. 2023 Sep 19;111:108845.
doi: 10.1016/j.ijscr.2023.108845. Online ahead of print. https://pubmed.ncbi.nlm.nih.gov/37741077/
A rare case of hepatopulmonary fusion associated with a right congenital diaphragmatic hernia: Case report and review of the literature
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Introduction and significance: Hepatic-pulmonary fusion is a very rare condition that occurs in the right congenital diaphragmatic hernia and appears to affect both sexes equally. There are no specific diagnostic methods before surgery for this anomaly. Most cases are discovered during surgical repair of the right congenital diaphragmatic hernia. This anomaly is considered to have a poor prognosis because of the associated vascular abnormalities and pulmonary hypoplasia.
Case presentation: We present the case of a neonate who suffered from respiratory distress since birth. Later, a congenital right diaphragmatic hernia was diagnosed. At surgery, it was found that hepatic-pulmonary fusion was present, accompanied by an anomaly of pulmonary venous outflow. A complete detachment of the connective tissue was performed and a patch was placed to close the defect in the diaphragm.
Clinical discussion: The few cases of hepatic pulmonary fusion reported in the medical literature make it difficult to develop a clear plan for diagnosis and surgical management. However, this anomaly should be considered when right-sided congenital diaphragmatic hernia is present, and the case should be well investigated and the surgical decision should not be rushed.
Conclusion: Hepatic-pulmonary fusion has a poor prognosis and a high mortality rate. No pathological mechanism for this anomaly has been identified, so we could not identify a specific diagnostic mechanism or a specific surgical method to treat this anomaly.
Keywords: Case report; Hepatic-pulmonary fusion-HPF; Neonate; Pediatric surgery.
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