Oligohydramnios, cause of the nonrenal features of Potter's syndrome, including pulmonary hypoplasia

Abstract

This report strongly supports the following hypothesis: The nonrenal features of Potter's syndrome—the altered facies, aberrant hand and foot positioning, late fetal growth deficiency, and pulmonary hypoplasia—are all secondary manifestation of prolonged fetal compression due to oligohydramnios. The oligohydramnios is usually due to renal agenesis or some other defect in the urinary system such that urine production or flow into the amniotic space is grossly deficient. In support of the above hypothesis examples are cited of infants who had renal agenesis, but for unusual reasons did not have oligohydramnios; they did not have the nonrenal features of Potter's syndrome. Furthermore, instances are cited of infants with normal urinary systems but oligohydramnios secondary to prolonged leakage of amniotic fluid; these infants had all the nonrenal features of Potter's syndrome. The authors recommend the term oligohydramnios tetrad for these secondary features rather than the term Potter's syndrome, since the latter designation as it is commonly employed does not represent a primary diagnosis. The oligohydramnios tetrad may be a nonspecific feature in a number of different disorders. This report strongly supports the following hypothesis: The nonrenal features of Potter's syndrome—the altered facies, aberrant hand and foot positioning, late fetal growth deficiency, and pulmonary hypoplasia—are all secondary manifestation of prolonged fetal compression due to oligohydramnios. The oligohydramnios is usually due to renal agenesis or some other defect in the urinary system such that urine production or flow into the amniotic space is grossly deficient. In support of the above hypothesis examples are cited of infants who had renal agenesis, but for unusual reasons did not have oligohydramnios; they did not have the nonrenal features of Potter's syndrome. Furthermore, instances are cited of infants with normal urinary systems but oligohydramnios secondary to prolonged leakage of amniotic fluid; these infants had all the nonrenal features of Potter's syndrome. The authors recommend the term oligohydramnios tetrad for these secondary features rather than the term Potter's syndrome, since the latter designation as it is commonly employed does not represent a primary diagnosis. The oligohydramnios tetrad may be a nonspecific feature in a number of different disorders.