Congenital Acinar Dysplasia: Report of a Case and Review of Literature

• Published in: American journal of perinatology reports Vol. 3; no. 1; pp. 009 - 012
• Main Authors: Langenstroer, Mary, Carlan, S.J., Fanaian, Naim, Attia, Suzanna
• Format: Journal Article
• Language: English
• Published: 333 Seventh Avenue, New York, NY 10001, USA Thieme Medical Publishers 01.05.2013; Thieme Medical Publishers, Inc
• Subjects: congenital anomaly; lung maldevelopment; pulmonary hypoplasia; respiratory insufficiency; lung maldevelopment; congenital anomaly; pulmonary hypoplasia; respiratory insufficiency
• ISSN: 2157-6998; 2157-7005
• DOI: 10.1055/s-0032-1329126

Abstract Objective  Describe a case of congenital acinar dysplasia and review the literature. Study Design  Retrospective chart review and literature search. Results  Congenital acinar dysplasia is a rare malformation of growth arrest of the lower respiratory tract resulting in critical respiratory insufficiency at birth. It is a form of pulmonary hypoplasia that is characterized by diffuse maldevelopment and derangement of the acinar and alveolar architecture of the lungs, resulting in the complete absence of gas exchanging units. The growth-arrested lung tissue resembles the pseudoglandular phase of 16 weeks' gestation. The etiology is unknown. It is diagnosed by exclusion of all other causes of pulmonary hypoplasia and a summation of clinical, imaging, and histopathologic findings. Conclusion  There is no cure and clinical treatment is supportive until death of the infant. We present a case of congenital acinar dysplasia in a male infant who lived 20 days with intensive support.