PULMONARY LYMPHANGIECTASIA

Abstract

Congenital pulmonary lymphangiectasia(PL) is a rare developmental disorder involvingthe lung and is characterized by pulmonarysubpleural, interlobar, perivascular, and peribronchiallymphatic dilatation. Both frequencyand etiology are unknown. PL presents atbirth with severe respiratory distress, tachypnea,and cyanosis, with a very high mortality rateat or within a few hours of birth.At birth, mechanical ventilation andpleural drainage are nearly always necessaryto obtain a favorable outcome of respiratorydistress. Home supplemental oxygen therapyand symptomatic treatment of recurrent coughand wheeze are often necessary duringchildhood, sometimes associated to prolongedpleural drainage. Recent advances in intensiveneonatal care have changed the previouslynearly fatal outcome of PL at birth.Patients affected by PL who surviveinfancy present medical problems which arecharacteristic of chronic lung disease.