Congenital pulmonary lymphangiectasis presenting as a unilateral hyperlucent lung

Abstract

Congenital pulmonary lymphangiectasis can be a cause of respiratory distress of the newborn infant. We present a case of congenital pulmonary lymphangiectasis presenting as a unilateral hyperlucent lung. Such a presentation has only once been previously described.

Congenital unilobar pulmonary lymphangiectasis

“…One patient died postoperatively from septicemia, 9 and one patient developed intractable pulmonary hypertension. 10 Clinical manifestation of the condition may occur shortly after birth, 6,8 but the diagnosis can be delayed until 4 weeks, 10 as infants may appear clinically stable. Our patient was in excellent clinical condition and breathing normally from the time of extubation on day 5 until elective surgery almost 4 weeks later.…”

“…Until now only five newborns with congenital unilobar pulmonary lymphangiectasis have been published. [6][7][8][9][10] In most cases, a cystic malformation of the left lung was found on chest X-rays, suggesting congenital lobar emphysema or cystic adenomatoid malformation. In only one report did the malformation appear radiologically as a fluid density.…”

High-Resolution CT of the Lung in Children: Clinical Applications

“…Pleural effusion and pneumothorax may be associated. Unilateral or lobar involvement has been reported (Verlaat et al 1994;Li et al 1985;Rettwitz-Volk et al 1999).…”

Linfangiectasia pulmonar congénita unilateral en un neonato prematuro

“…Autopsy studies suggest that approximately 0.5-1% of newborns who died in the neonatal period had CPL [6][7][8] . Generally, in the CPL both lungs are affected and have a poor prognosis with a mortality rate of 50-98% 8,9 . Unilateral presentation is extremely rare, only few cases have been reported, it has a better prognosis and sometimes it has a spontaneous resolution [10][11][12] .…”