Early abdomino-perineal pull-through vaginoplasty

Abstract

Vaginal agenesis is known as one of the Mόllerian anomalies. Mόllerian anomalies occur during gonadal development and differentiation, and may lead to complex outcomes. McKusick-Kaufman syndrome (MKS) is an autosomal recessive multiple malformation syndrome characterized by Mόllerian anomalies with hydrometrocolpos (HMC) and postaxial polydactyly (PAP). We report a case of a female newborn that developed hydronephrosis and respiratory distress due to compression of the cystic mass and underwent surgery on postpartum day 3. Pull-through vaginoplasty was performed with an abdomino-perineal approach. We report the unique treatment approach in this case.

Keywords

• Congenital
• hydrometrocolpos
• McKusick-Kaufman syndrome
• neonate
• polydactyly
• pull-through
• vaginoplasty