title: Pulmonary agenesis--vascular airway compression and gastroesophageal reflux influence outcome.
abstract: Pulmonary agenesis (PA) is a rare malformation that can be isolated or associated with other anomalies. We report 3 cases of left-sided PA having ipsilateral renal agenesis, facial, and radial ray anomalies. Patients presented in infancy with nonspecific respiratory symptoms and were diagnosed to have PA on chest radiograph and computed tomographic scan. Bronchial compression, by dilated pulmonary artery and associated severe gastroesophageal reflux, aggravated respiratory symptoms and required surgical intervention. The relevant literature is briefly reviewed.
authors.affiliation: Department of Surgery, The Aga Khan University Hospital, Karachi 74800, Pakistan. zafar.nazir@aku.edu
authors.forename: Zafar
authors.initials: Z
authors.lastname: Nazir
authors.forename: Saqib Hamid
authors.initials: SH
authors.lastname: Qazi
authors.forename: Nausheen
authors.initials: N
authors.lastname: Ahmed
authors.forename: Mehnaz
authors.initials: M
authors.lastname: Atiq
authors.forename: Abdul Gaffer
authors.initials: AG
authors.lastname: Billoo
mesh_terms: D000015:Abnormalities, Multiple; D000402:Airway Obstruction / Q000209:etiology*; D001982:Bronchial Diseases / Q000209:etiology*; D004108:Dilatation, Pathologic / Q000150:complications; D018662:Fundoplication; D005764:Gastroesophageal Reflux / Q000209:etiology* / Q000601:surgery; D005774:Gastrostomy; D006801:Humans; D007223:Infant; D007668:Kidney / Q000002:abnormalities; D008168:Lung / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000503:physiopathology; D008297:Male; D011651:Pulmonary Artery / Q000002:abnormalities*; D013902:Radiography, Thoracic; D014057:Tomography, X-Ray Computed