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.

A Rare Case of Chylothorax due to Pulmonary Lymphangiectasia in a 7-Year-Old Boy

RUGGERI, GIOVANNI;LIMA, MARIO
2013

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.
2013
Ruggeri G; Destro F; Maffi M; Gregori G; Lima M
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11585/251478
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