Congenital pulmonary airway malformation – hybrid lesion – a case report
Journal Title: MedPulse -International Medical Journal - Year 2014, Vol 1, Issue 9
Abstract
Introduction: Congenital pulmonary airway malformation (CPAM) is a fetal condition usually diagnosed in the early second trimester. This is a mass of lung tissue which has proliferation of bronchial structures. The space occupying nature of the lesion may result in mediastinal shift, or pleural effusion or hydrops due to increased intra-thoracic pressure. Post-natally, the CPAM tissue is unavailable for gas exchange leading to respiratory compromise. The condition is known to regress in utero but needs to be assessed for progression with evaluation of the CPAM volume ratio (CVR) calculated as CPAM volume divided by head circumference of fetus; to anticipate the occurrence of hydrops. Fetal surgery has been performed in a few centres for fetuses with a high CPAM ratio to alleviate hydrops and improve development of normal lung tissue for perinatal oxygenation. Case report: We describe the diagnosis and fetal management of a hybrid CPAM diagnosed at 20 weeks and monitored to term for an optimal outcome. An increase in CVR was noted at 30 weeks, following which antenatal corticosteroids were administered to the patient following which there was a rapid reduction in the size of the lesion. The use of corticosteroids in cases with CPAM has been reported to reduce the size of the lesion leading to avoidance of fetal compromise, and this has been the case in our patient also Conclusion: CPAM has a natural history of spontaneous regression in utero without any medical intervention. The use of steroids in selected cases with increasing CVR may be an additional tool to delay or avoid fetal surgery by causing regression of the lesion. Further study including randomization of selected cases is required to conclude on the use of antenatal steroids in the fetal management of CPAM.
Authors and Affiliations
Chitra Andrew, Shivani Gopal, Hemalatha Ramachandran, Umamaheshwari Balakrishnan, Usha Vishwanath
Keywords
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