Pulmonary agenesis--vascular airway compression and gastroesophageal reflux influence outcome.
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.
Nazir
Zafar
Z
Department of Surgery, The Aga Khan University Hospital, Karachi 74800, Pakistan. zafar.nazir@aku.edu
Qazi
Saqib Hamid
SH
Ahmed
Nausheen
N
Atiq
Mehnaz
M
Billoo
Abdul Gaffer
AG
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