Variation in clinical and genitourinary lesions associated with pulmonary hypoplasia in Potter's syndrome--two autopsy reports.

ABSTRACT

Potter's syndrome is a rare entity with an incidence of 1 in 2000 to 1 in 5000 live births. All babies born with this condition are either stillborn or die very early within neonatal period. We present two autopsy cases which presented with abnormal facies, oligohydramnios, pulmonary hypoplasia and genitourinary abnormality. One case presented with infantile polycystic kidney whereas in the other case both the kidneys were normal but had adenomatoid tumour of left testis. Both the children died few hours after birth. In both the cases pulmonary hypoplasia was the cause of death rather than genitourinary abnormality.