Gökhan Güzide Ayşe, Ozbilim Gülay, Bozova Sevgi, Güra Aşkin, Ongun Hakan, Mihci Ercan, Arslan Gökhan
Department of Pathology, Akdeniz University Faculty of Medicine, Antalya, Turkey.
Turk J Pediatr. 2005 Jul-Sep;47(3):295-7.
Unilateral pulmonary agenesis is a very rare developmental malformation that is often associated with other anomalies. It can be asymptomatic or present with respiratory symptoms. Our case is a female newborn infant who had been taken to the hospital suffering from difficulty in breathing at the first day of birth. The baby died at the age of three days due to respiratory failure. On autopsy examination and its histopathological evaluation, we detected right pulmonary agenesis and colloidal goiter. According to the literature, pulmonary agenesis is associated with other anomalies including esophageal atresia, tracheal stenosis, musculoskeletal anomalies, DiGeorge syndrome and cardiovascular malformations such as septal defects, patent ductus arteriosus and total anomalous pulmonary venous return. To our knowledge, this is the first case of pulmonary agenesis associated with colloidal goiter.
单侧肺发育不全是一种非常罕见的发育畸形，常与其他异常情况相关联。它可以是无症状的，也可能表现出呼吸道症状。我们的病例是一名女性新生儿，出生第一天因呼吸困难被送往医院。婴儿在三天大时因呼吸衰竭死亡。经尸检及其组织病理学评估，我们发现了右肺发育不全和胶样甲状腺肿。据文献报道，肺发育不全与其他异常情况有关，包括食管闭锁、气管狭窄、肌肉骨骼异常、迪格奥尔格综合征以及心血管畸形，如房间隔缺损、动脉导管未闭和完全性肺静脉异位引流。据我们所知，这是首例与胶样甲状腺肿相关的肺发育不全病例。
