Division of Pediatric Surgery, Department of Surgery, Columbia University College of Physicians and Surgeons, Morgan Stanley Children's Hospital of New York-Presbyterian, Columbia University Medical Center, New York, NY 10032, USA.
J Pediatr Surg. 2010 Jan;45(1):265-8. doi: 10.1016/j.jpedsurg.2009.10.090.
Hepatic pulmonary fusion is extremely rare with only 9 previous cases reported in the literature. In typical cases, the clinician should be alerted to the possibility of hepatic pulmonary fusion if the chest radiograph shows a large opacity on the right side without a contralateral mediastinal shift. The authors present a case of right-sided diaphragmatic hernia and hepatic pulmonary fusion with associated contralateral mediastinal shift discovered beyond the neonatal period. The 9 previous cases were retrospectively reviewed with special attention to mediastinal shift on preoperative chest radiograph, operative procedure, and mortality. Only one previous case demonstrated a contralateral mediastinal shift. The most common procedure performed was partial separation of the hepatic pulmonary fusion and approximation of the diaphragmatic defect. Four of the previous 9 patients died. In our case, reduction of bowel and approximation of the diaphragmatic defect around the fused liver and lung have been successful.
肝肺融合非常罕见,文献中仅报道过 9 例。在典型病例中,如果胸部 X 线片显示右侧大片不透明影且无对侧纵隔移位,临床医生应警惕肝肺融合的可能性。作者报告了一例右侧膈疝和肝肺融合伴对侧纵隔移位的病例,该病例在新生儿期后发现。对 9 例既往病例进行了回顾性分析,特别关注术前胸部 X 线片上纵隔移位、手术过程和死亡率。只有 1 例既往病例显示对侧纵隔移位。最常见的手术方式是部分分离肝肺融合和接近膈肌缺陷。9 例患者中有 4 例死亡。在我们的病例中,成功地减少了肠腔并接近融合的肝和肺周围的膈肌缺陷。