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Congenital pulmonary airway malformation (CPAM) is a rare congenital lung mass of the foetus that can present as solid or cystic. The incidence of CPAM lesions is between 1:25,000-1:35,000 live births. This is often diagnosed prenatally with sonography and routinely followed through the term of the pregnancy. CPAM is classified into five different types. CPAM is predominant in males and may affect any lobe of the lung. The lesion is unilobar in 80%-95% of cases and bilateral in fewer. Outcome depends on CVR at presentation, if >1.6 there is 80% chance of developing hydrops.
This is a case of 24 years old primigravida referred from private hospital at 22week of gestation with her obstetric ultrasound suggestive of congenital airway malformation with marked mediastinal shift with hypoplastic right lungs (type 3 CPAM) and CVR 2.1. Neonatologist and paediatric surgeon opinion were taken and follow up ultrasonography was done. A term alive female baby was delivered by emergency caesarean section and was shifted to NICU due to respiratory distress (grunt with tachypnoea ) and was on CPAP.CT thorax showed a thinned wall cystic lesion in anterior segment of left middle lobe with air fluid level within it and communicating with a mildly dilated bronchiolar airway adjacent to it .Baby was reviewed in paediatric surgery department after 1 month and was advised for conservative management .
Early diagnosis and management of prenatally diagnosed CPAM can prevent both prenatal and postnatal complication with favourable outcome.
