Sadiqi Jamshid, Hamidi Hidayatullah
Department of Radiology, French Medical Institute for Mothers & Children (FMIC), behind Kabul Medical University, Jamal mina, P.O. Box: 472, Kabul, Afghanistan.
BMC Med Imaging. 2018 Oct 30;18(1):37. doi: 10.1186/s12880-018-0281-5.
Lung agenesis is a rare congenital anomaly. The main etiology of the disease is unknown whereas genetic, iatrogenic and viral factors as well as vitamin A deficiency during early pregnancy may result in developmental failure of primitive lung bud causing unilateral pulmonary agenesis. Affected patients usually present with variable respiratory symptoms and recurrent chest infection at any age. Plain film demonstrates opaque unilateral lung while chest CT scan can definitely diagnosis the disease. The anomaly has three types. Type I is pulmonary agenesis, type II is called pulmonary aplasia and type III is pulmonary hypoplasia.
CASES' PRESENTATION: Six patients with main complaint of dyspnea underwent contrast enhanced chest CT in radiology department of French Medical Institute for Mothers and children, Kabul and were diagnosed lung agenesis. Three patients were categorized as type II pulmonary agenesis (aplasia). Two patients, three months old boy and a seven year- old girl demonstrated right lung aplasia. Another patient boy of eighteen years old presented with left lung aplasia. Two boys of four and seven months of age were classified as type I pulmonary agenesis (agenesis). A boy of one year old was diagnosed pulmonary agenesis type III, right lung hypoplasia.
Six patients were diagnosed with pulmonary agenesis by Chest CT scan. The clinicians should consider possibility of congenital pulmonary agenesis in dyspneic patients with opaque unilateral hemithorax in plain film.
肺不发育是一种罕见的先天性异常。该病的主要病因尚不清楚,而遗传、医源性和病毒因素以及孕早期维生素A缺乏可能导致原始肺芽发育失败,从而引起单侧肺不发育。受影响的患者通常在任何年龄都有不同的呼吸道症状和反复的胸部感染。X线平片显示单侧肺不透明,而胸部CT扫描可以明确诊断该病。该异常有三种类型。I型是肺不发育,II型称为肺发育不全,III型是肺发育不良。
6例以呼吸困难为主诉的患者在喀布尔法国母婴医学研究所放射科接受了增强胸部CT检查,被诊断为肺不发育。3例患者被归类为II型肺不发育(发育不全)。2例患者,一名3个月大的男孩和一名7岁女孩表现为右肺发育不全。另一名18岁的男性患者表现为左肺发育不全。2名4个月和7个月大的男孩被归类为I型肺不发育(不发育)。一名1岁男孩被诊断为III型肺不发育,右肺发育不良。
6例患者经胸部CT扫描诊断为肺不发育。临床医生对于X线平片显示单侧半胸不透明的呼吸困难患者应考虑先天性肺不发育的可能性。