Unilateral lung hypoplasia: A rare cause of unilateral opaque hemithorax in chest X-ray in a young boy

Abstract

Congenital abnormalities of lung are very rare entity, and very often under or misdiagnosed by physicians. The present case, a 12-year boy, who was initially diagnosed as unilateral massive pleural effusion with collapse of lung, and after thorough investigation, including CT scan of thorax, fiber-optic bronchoscopy, and echocardiography, a final diagnosis of unilateral lung hypoplasia was made. So if a teenager present with a unilateral opaque hemithorax in chest X-ray, this entity may be a differential diagnosis.

Congenital thoracic malformations in pediatric patients: two decades of experience

“…The following malformations were found: congenital lobar emphysema, in 6; pulmonary agenesis, in 5; congenital pulmonary airway malformations, in 4; pulmonary hypoplasia, in 3; cystic disease, in 2; congenital diaphragmatic hernia, in 2; bronchogenic cyst, in 1; lobar agenesis, in 1; pulmonary vascular malformations, in 1; and diaphragmatic eventration, in 1. Pulmonary agenesis is very rare, having occurred in 5 patients; of those, 4 had right lung agenesis, which is consistent with the literature, ( 5 ) with only 1 asymptomatic child. None of the patients had been diagnosed with pulmonary sequestration.…”

“…Abnormal development of the aortic arch during embryogenesis-which was not observed in the present study-results in pulmonary hypoplasia, ( 5 ) which in turn is a consequence of congenital diaphragmatic hernia. One patient had decreased pulmonary vascularization.…”

“…Pulmonary agenesis (particularly right lung agenesis) can be associated with other congenital abnormalities. ( 5 ) Of the 26 patients studied, 11 had other malformations, including facial dysmorphism, heart disease, clubfoot, bilateral deafness, umbilical hernia, and inguinal hernia. All of the patients with pulmonary agenesis had other malformations, one of the patients having multiple birth defects and an incidental diagnosis of congenital thoracic malformation at the age of 7 months.…”

Late presentation of unilateral lung agenesis in adulthood

“…The heart usually lies flush with the chest wall. There is variable degree of proliferation of fat on the affected side [ 12 ].…”

Left Lung Agenesis with Bicuspid Aortic Valve

“…M ajor congenital anomalies of lung such as hypoplasia and agenesis are very rare en ty and seldom being reported 1 . They have varied clinical presenta on ranging from totally asymptoma c to severe respiratory insuffi ciency.…”