Alomar Khaled, Alghazal Linah Kaf, Alloun Maher, Dawarah Methad, Alkhayer Ghuroub, Alkader Mohammed Abd
Damascus University - University pediatrics' Hospital, Syria.
Damascus University - University pediatrics' Hospital, Syria.
Int J Surg Case Rep. 2023 Oct;111:108845. doi: 10.1016/j.ijscr.2023.108845. Epub 2023 Sep 19.
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.
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.
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.
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.
肝肺融合是一种非常罕见的情况，发生于右侧先天性膈疝，且似乎对两性影响相同。对于这种异常情况，术前尚无特定的诊断方法。大多数病例是在右侧先天性膈疝的手术修复过程中发现的。由于相关的血管异常和肺发育不全，这种异常被认为预后不良。
我们报告一例自出生即患有呼吸窘迫的新生儿病例。后来，诊断为先天性右侧膈疝。手术时发现存在肝肺融合，并伴有肺静脉流出异常。进行了结缔组织的完全分离，并放置补片以封闭膈肌缺损。
医学文献中报道的肝肺融合病例很少，因此难以制定明确的诊断和手术管理计划。然而，当存在右侧先天性膈疝时，应考虑这种异常情况，应对病例进行充分调查，不应仓促做出手术决定。
肝肺融合预后不良，死亡率高。尚未确定这种异常的病理机制，因此我们无法确定针对这种异常的特定诊断机制或特定手术方法。
