title: Severe primary pulmonary lymphangiectasis in a premature infant: management and follow up to early childhood.
abstract: Primary pulmonary lymphangiectasis (PPL) is a rare congenital developmental abnormality of the lung with a generally poor prognosis. Only a limited number of patients with neonatal-onset PPL have been reported to survive. We present the case of a male preterm infant (gestational age 34 weeks 6 days) with histologically confirmed PPL, complicated by hydrops fetalis, bilateral hydrothorax (treated in utero with pleuro-amniotic shunts), and immediate respiratory distress at birth. He survived after extensive neonatal intensive care therapy and was discharged home at the age of 7 months. At last follow up he was 3 years 7 months old, still requiring assisted ventilation via tracheostomy, having recurrent episodes of wheezing and had mild global developmental delay. This case demonstrates that survival beyond the neonatal period is possible even with severe PPL but long-term morbidity may be relevant, and multidisciplinary management and close follow up are essential.
authors.affiliation: Division of Neonatology, Medical University of Graz, Graz, Austria.
authors.forename: Friedrich
authors.initials: F
authors.lastname: Reiterer
authors.forename: Karin
authors.initials: K
authors.lastname: Grossauer
authors.forename: Andreas
authors.initials: A
authors.lastname: Pfleger
authors.forename: Martin
authors.initials: M
authors.lastname: Häusler
authors.forename: Bernhard
authors.initials: B
authors.lastname: Resch
authors.forename: Ernst
authors.initials: E
authors.lastname: Eber
authors.forename: Helmut
authors.initials: H
authors.lastname: Popper
authors.forename: Berndt
authors.initials: B
authors.lastname: Urlesberger
mesh_terms: D000328:Adult; D001706:Biopsy; D019468:Disease Management; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D007234:Infant, Premature; D007235:Infant, Premature, Diseases / Q000175:diagnosis* / Q000628:therapy; D008171:Lung Diseases / Q000151:congenital* / Q000175:diagnosis / Q000628:therapy; D008200:Lymphangiectasis / Q000151:congenital* / Q000175:diagnosis / Q000628:therapy; D008297:Male; D011247:Pregnancy; D014057:Tomography, X-Ray Computed
keywords: follow up; hydro-chylothorax; pleuro-amniotic shunt; preterm infant; primary pulmonary lymphangiectasis