Abstract
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.
Keywords
pulmonary lymphangiectasia - chylothorax - thoracoscopy
