title: Unilateral pulmonary agenesis: a report of four cases, two diagnosed antenatally and literature review.
abstract: Pulmonary agenesis is a rare congenital malformation of lung development defined as complete absence of lung tissues, bronchi, and pulmonary vessels; it may be uni- or bilateral. The right-sided form carries the poorest prognosis due to severity of co-existent anomalies. Its diagnostic circumstances are variables: first reported cases were diagnosed at autopsy, but early postnatal as well as fortuitous discovery have been reported. In recent years, progress in obstetrical imaging has made antenatal diagnosis possible so that fetal ultrasound and MRI allow early diagnosis and refinement by permitting the elimination of differential diagnoses (diaphragmatic hernia, cystic adenomatoid malformation of the lung, giant lobar emphysema, and situs inversus). This anomaly is compatible with normal life provided co-existent malformations are thoroughly investigated and managed in a multidisciplinary setting. We report four cases of lung agenesis two of which were diagnosed antenatally at 23rd and 30th weeks of gestation respectively. Our aim is to describe the circumstances having led to diagnosis and report both follow-up and outcome of our patients.
authors.affiliation: Neonatal Medicine Section, Service de Pédiatrie & Médecine Néonatale, Victor Jousselin Hospital, Dreux, France.
authors.forename: S
authors.initials: S
authors.lastname: Kayemba-Kay's
authors.forename: V
authors.initials: V
authors.lastname: Couvrat-Carcauzon
authors.forename: V
authors.initials: V
authors.lastname: Goua
authors.forename: G
authors.initials: G
authors.lastname: Podevin
authors.forename: M
authors.initials: M
authors.lastname: Marteau
authors.forename: E
authors.initials: E
authors.lastname: Sapin
authors.forename: G
authors.initials: G
authors.lastname: Levard
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis / Q000000981:diagnostic imaging*; D000328:Adult; D003025:Clubfoot / Q000175:diagnosis / Q000000981:diagnostic imaging*; D003914:Dextrocardia / Q000175:diagnosis / Q000000981:diagnostic imaging*; D005260:Female; D006801:Humans; D021621:Imaging, Three-Dimensional; D007231:Infant, Newborn; D008168:Lung / Q000002:abnormalities* / Q000000981:diagnostic imaging; D008171:Lung Diseases / Q000175:diagnosis / Q000000981:diagnostic imaging*; D008279:Magnetic Resonance Imaging; D008297:Male; D011296:Prenatal Diagnosis; D013904:Thoracic Vertebrae / Q000002:abnormalities* / Q000000981:diagnostic imaging; D016216:Ultrasonography, Prenatal; D055815:Young Adult
keywords: antenatal diagnosis; co-existent anomalies; fetal MRI; prognosis; pulmonary agenesis