Efficacia del trattamento con octreotide in un caso di linfangectasia polmonare congenita

Abstract

Congenital pulmonary lymphangiectasia is a rare condition that may present antenatally with pleural effusions and hydrops. This condition is associated with congenital chylothorax, that is the accumulation of lymphatic fluid within the pleural space. We report the use of the octreotide (a somatostatin analogue) in the treatment of congenital chylothorax in a newborn with severe non-immune hydrops who had previously failed conservative medical therapy. The patient improved rapidly after initiation of octreotide with complete resolution after 13 days of continuous therapy (7 microg/kg per hour).