Early abdomino-perineal pull-through vaginoplasty

Abstract

Vaginal agenesis is known as one of the Müllerian anomalies. Müllerian 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 Müllerian 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.

Key words: Con genital, hydrometrocolpos, McKusick–Kaufman syndrome, neonate, polydactyly, pull-through, vaginoplasty