Novel mutation among two sisters with 17β hydroxysteroid dehydrogenase type 3 deficiency.

The clinical presentations of 17β hydroxysteroid dehydrogenase type 3 (17β-HSD3) deficiency, 5α-reductase type 2 deficiency, and complete androgen insensitivity syndrome can be similar. However, those with 17β-HSD3 deficiency and 5α-reductase type 2 deficiency will develop virilization and should undergo gonadectomy after genetic testing before the age of puberty if reared in the female sex. Two sisters were initially diagnosed with complete androgen insensitivity syndrome as young children after testes were discovered during hernia surgery.

Radial forearm free flap phalloplasty for penile inadequacy in patients with exstrophy.

Purpose: Male patients with bladder and cloacal exstrophy are born with demure genitalia and often desire phalloplasty during late adolescence or early adulthood. Radial forearm free flap phalloplasty was used successfully for congenital aphallia in a few small series. We present a series of patients treated with phalloplasty using a radial forearm free flap after the repair of bladder and cloacal exstrophy.

Persistent vesicocutaneous fistula after repair of classic bladder exstrophy: a sign of failure?

Objective: To describe the potential predisposing clinical characteristics at initial closure, the treatments of and outcomes associated with vesicocutaneous fistula (VCF) after primary bladder closure.

Materials And Methods: Eighteen patients were referred for VCF after primary bladder exstrophy closure. Aspects from the primary closure such as gender, timing of diagnosis and repair, osteotomies, pelvic immobilization, layers of closure, use of tissue adjuncts, and complications in addition to details from their required treatment of VCF were retrospectively extracted from the medical record.