Congenital cystic adenomatoid malformation in bilateral renal agenesis. Its mitigation of Potter's syndrome.
Potter's syndrome develops secondary to a deficiency of amniotic fluid, such as occurs in renal agenesis. Congenital cystic adenomatoid malformation (CCAM), on the other hand, is frequently accompanied by polyhydramnios. We describe a newborn with both renal agenesis and CCAM who had only mild features of Potter's syndrome. The pathogenesis of polyhydramnios in CCAM is discussed with regard to the ultrastructural findings of numerous type 2 pneumocytes lining the cysts. The association between CCAM and bilateral renal anomalies is emphasized.
D000015:Abnormalities, Multiple; D000328:Adult; D005145:Face / Q000002:abnormalities; D006801:Humans; D007231:Infant, Newborn; D007668:Kidney / Q000002:abnormalities*; D008168:Lung / Q000002:abnormalities* / Q000473:pathology; D008297:Male; D013577:Syndrome; D014513:Ureter / Q000002:abnormalities