A primigravida presented with severe oligohydramnios and breech presentation with fetal distress. She underwent emergency cesarean section. The baby had multiple anomalies including Potter’s facies, phocomelia, mesomelia, renal dysplasia and anomalies of external genitalia. Baby died immediately after birth. MRI and foetal dissection showed findings correlating with Ulbright-Hodes syndrome or Renal dysplasia – limb defects syndrome (RL Syndrome). This is a very rare autosomal recessive congenital disorder. Here we report a case of Ulbright-Hodes syndrome as only four cases have been reported so far.
Prof. Dr. Bilal BİLGİN