title: 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.
authors.affiliation: Department of Pediatric Surgery, Selcuk University, Medical Faculty, Konya, Turkey. driciftci@yahoo.com
authors.forename: Ilhan
authors.initials: I
authors.lastname: Ciftci
authors.forename: Ayhan
authors.initials: A
authors.lastname: Tastekin
authors.forename: Ali
authors.initials: A
authors.lastname: Annagur
authors.forename: Mustafa
authors.initials: M
authors.lastname: Koplay
mesh_terms: D000005:Abdomen / Q000601:surgery*; D000015:Abnormalities, Multiple / Q000175:diagnosis* / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D013509:Gynecologic Surgical Procedures / Q000379:methods*; D006330:Heart Defects, Congenital / Q000175:diagnosis* / Q000601:surgery; D006801:Humans; D052202:Hydrocolpos / Q000175:diagnosis* / Q000601:surgery; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D010502:Perineum / Q000601:surgery*; D017689:Polydactyly / Q000175:diagnosis* / Q000601:surgery; D019651:Plastic Surgery Procedures / Q000379:methods*; D014591:Uterine Diseases / Q000175:diagnosis* / Q000601:surgery; D014621:Vagina / Q000002:abnormalities / Q000601:surgery*