Localized pulmonary lymphangiectasia

Congenital unilobar pulmonary lymphangiectasis

“…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.…”

“…In only one report did the malformation appear radiologically as a fluid density. 8 In all patients, congenital pulmonary lymphangiectasis was present in the left upper lobe, and all underwent surgery. One patient died postoperatively from septicemia, 9 and one patient developed intractable pulmonary hypertension.…”

“…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.…”

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).…”