| Abstract: | Bilateral renal agenesis (or dysplasia without any functioning kidney tissue) is almost constantly associated with a characteristic facial appearance. Extra-uterine existence is limited to less than 24 hours because of severe hypoplasia of the lungs. This pulmonary anomaly, together with the eventual positional bowing of the legs and feet and hands and perhaps some features of the "Potter face" may be attributed to the oligohydramnios which is a regular observation, but certain other findings like the obligatory epicanthic fold, swinging outwards to form a most peculiar "prominent" semi-circle below the orbital space, the dysplasia and low slanted position of the ears, the predominance of the boys, and the very high frequency of additional malformations and even total defects of the internal organs (especially of the urogenital and intestinal tract) can hardly be explained as being secondary resp. consequent upon the "foetal compression", resulting from the amniotic fluid deficit (due to anuria). Ten children, including a case of hermaphroditismus verus, with the full-scale Potter syndrome have been seen in this clinic during a six years period and are described and discussed in detail. |
