POTTER SYNDROME, A RARE ENTITY WITH HIGH RECURRENCE RISK IN WOMEN WITH RENAL MALFORMATIONS – CASE REPORT AND A REVIEW OF THE LITERATURE

Abstract

Potter syndrome represents an association between a specific phenotype and pulmonary hypoplasia as a result of oligohydramnios that can appear in different pathological conditions. Thus, Potter syndrome type 1 or autosomal recessive polycystic renal disease is a relatively rare pathology and with poor prognosis when it is diagnosed during the intrauterine life. We present the case of a 24-year-old female with an evolving pregnancy, 22/23 gestational weeks, in which the fetal ultrasound revealed oligohydramnios, polycystic renal dysplasia and pulmonary hypoplasia. The personal pathological history revealed the fact that 2 years before this pregnancy, the patient presented a therapeutic abortion at 16 gestational weeks for the same reasons. The maternal ultrasound showed unilateral maternal renal agenesis. Due to the fact that the identified fetal malformation was incompatible with life, we decided to induce the therapeutic abortion. The particularity of the case consists in diagnosing Potter syndrome in two successive pregnancies in a 24-year-old female, without any significant family history, but who is diagnosed with unilateral renal agenesis.

Keywords

• potter syndrome
• autosomal recessive polycystic renal disease
• oligohydramnios