Department of Pediatrics and Child Health, Jimma University, Ethiopia.
Department of Surgery, Jimma University, Ethiopia.
Ethiop J Health Sci. 2021 Jul;31(4):907-910. doi: 10.4314/ejhs.v31i4.27.
Congenital pulmonary airway malformation is a very rare congenital cystic lung disease that presents in 0.004% of all pregnancies and constitutes <25% of all congenital pulmonary anomalies in children. Respiratory distress is a major concern in these patients.
Here in, we report an 8 month old girl presenting with exacerbation of fast breathing of three days duration. Chest X-ray showed hyper lucent right lung with significant shift of mediastinum to the left side, flattening of the diaphragm on the right side and compression of the left lung. Computed tomography scan of the chest revealed multiseptated cystic mass on the right lung measuring 8.9cm by 6.9cm. After receiving treatment for pneumonia, surgical excision of the mass was performed and biopsy showed congenital pulmonary airway malformation type1. The infant died on 40 postoperative day from uncontrolled hospital acquired infection.
When a child has respiratory distress, congenital pulmonary airway malformation could be considered after common pathologies are ruled out. Surgical excision, which is the treatment of choice, is recommended to make a definite diagnosis and exclude hidden malignancies.
先天性肺气道畸形是一种非常罕见的先天性囊性肺部疾病，在所有妊娠中的发病率为 0.004%，占儿童所有先天性肺部异常的<25%。这些患者的主要关注点是呼吸窘迫。
在此，我们报告了一例 8 个月大的女孩，因呼吸急促加重就诊，病程为 3 天。胸部 X 线片显示右侧肺部透亮影，纵隔明显向左侧移位，右侧膈肌变平，左侧肺部受压。胸部 CT 扫描显示右侧肺部有一个多分隔的囊性肿块，大小为 8.9cm×6.9cm。在接受肺炎治疗后，对肿块进行了手术切除，活检显示为 1 型先天性肺气道畸形。患儿术后第 40 天死于无法控制的医院获得性感染。
当患儿出现呼吸窘迫时，在排除常见疾病后可考虑先天性肺气道畸形。建议进行手术切除以明确诊断并排除隐匿性恶性肿瘤，这是首选的治疗方法。
