Ruggeri Giovanni, Destro Francesca, Maffi Michela, Gregori Giulio, Lima Mario
Department of Pediatric Surgery, S. Orsola-Malpighi Hospital, Bologna, Italy.
European J Pediatr Surg Rep. 2013 Jun;1(1):18-20. doi: 10.1055/s-0033-1337111. Epub 2013 Mar 19.
Pulmonary lymphangiectasia (PL) is a rare condition characterized by dilatation of the lymphatic vessels. Post-neonatal PL is usually associated with pleural effusion and should therefore be suspected in the presence of chylothorax. We describe a post-neonatal manifestation of PL in a 7-year-old boy presenting chylothorax. Radiological examinations included thorax X-ray, ultrasound, and computed tomography scans. After the failure of conservative management (maintenance of the chest tube, total parenteral nutrition, administration of somatostatin synthetic analogues) we performed a thoracoscopic massive ligation of the thoracic duct's collateral along with a lung biopsy. Histology was compatible with type 1 congenital pulmonary lymphangectasia. One month after surgery a thoracoscopic pleurodesis was required for persistent chylothorax. The boy is now doing well 1 year after surgery.
肺淋巴管扩张症（PL）是一种罕见疾病，其特征为淋巴管扩张。新生儿期后的PL通常与胸腔积液相关，因此在出现乳糜胸时应怀疑此病。我们描述了一名7岁男孩出现乳糜胸时PL的新生儿期后表现。影像学检查包括胸部X线、超声和计算机断层扫描。在保守治疗（维持胸管引流、全胃肠外营养、给予生长抑素合成类似物）失败后，我们进行了胸腔镜下胸导管侧支的大量结扎并取了肺活检。组织学检查结果符合1型先天性肺淋巴管扩张症。术后1个月，因持续性乳糜胸需要进行胸腔镜胸膜固定术。该男孩术后1年情况良好。
