Bilateral Type 2 Congenital Pulmonary Airway Malformation with Multicystic Dysplastic Kidney: A Fetal Autopsy Case Report
DOI:
https://doi.org/10.21276/apalm.3884Keywords:
congenital pulmonary airway malformation, congenital cystic adenomatoid malformation, multicystic dysplastic kidney, fetal autopsy, congenital anomaliesAbstract
Congenital pulmonary airway malformation (CPAM), previously known as congenital cystic adenomatoid malformation, is a rare developmental anomaly of the lower respiratory tract characterized by cystic proliferation of terminal bronchioles. It is commonly diagnosed during antenatal ultrasonography in the second trimester (anomaly scan). Multicystic dysplastic kidney (MCDK) is a developmental anomaly resulting from abnormal interaction between the ureteric bud and metanephric mesenchyme during renal embryogenesis.
We report a rare case of bilateral CPAM type 2 associated with unilateral MCDK detected in a fetus at 19 weeks and 6 days of gestation. A 33-year-old gravida 3 para 2 woman presented with absent perception of fetal movements, and ultrasonography revealed multiple fetal anomalies. The pregnancy was medically terminated, and a female fetus weighing 340 g was received for autopsy. Gross examination showed multiple cystic lesions in both lungs and an enlarged cystic left kidney. Histopathological examination confirmed bilateral CPAM Type 2 and multicystic dysplastic kidney.
The coexistence of pulmonary and renal developmental anomalies is rarely reported and likely reflects a shared disturbance in early embryogenesis. Recognition of such associations is important for accurate prenatal diagnosis and counselling.
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