Abstract
Unilateral lung agenesis initially misdiagnosed as unilateral massive pleural effusion with collapse of lung, and after several investigations, including ultrasonography or CT scan of thorax, fibre-optic bronchoscopy or bronchography, a final diagnosis of unilateral absence of lung is made. An anomaly scan may also reveal associated renal, cardiac and vertebral defects. Here we report a 7 months old female who presented with respiratory distress since birth and after a thorough investigation, she was found to have this rare association of pulmonary-renal-cardiacvertebral defect. DOI: http://dx.doi.org/10.3126/jnps.v34i1.8898 J Nepal Paediatr Soc 2014;34(1):77-79
Highlights
Pulmonary agenesis is an extremely rare condi on with a reported prevalence of 34 per million live births[1]
Le sided pulmonary agenesis is more common and they have a longer life expectancy than those with right sided pulmonary agenesis[2], which is more commonly associated with other congenital abnormali es like cardio-vascular, skeletal, gastrointes nal or genitor-urinary system
There was no history of choking during feeding, foreign body inhala on, vomi ng, suck-rest-suck cycle and/or cyanosis
Summary
Pulmonary agenesis is an extremely rare condi on with a reported prevalence of 34 per million live births[1]. The CXR – PA view done outside showed right upper lobe consolida on with diffuse opacity of le chest and medias nal shi towards le. On examina on, he was ac ve, alert with normal anthropometry but had severe respiratory distress. General physical examina on was unremarkable except a mild hypotonia of all four limbs He had tachycardia (heart rate-180/min), tachypnea (respiratory rate-94/min), grun ng with intercostal and subcostal retrac ons but no cyanosis. Diagnosis of complete agenesis of le lung was made In echocardiography, he was found to have small (6 mm) Os um secondum atrial septal defect with significant le to right shun ng, absent le pulmonary artery, mild pulmonary artery hypertension but normal sized cardiac chambers. Pa ent improved on intra venous an bio cs and conserva ve treatment in seven days and discharged home in clinically stable condi on with an advice of three monthly follow-up
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