PURPOSE: We present the followup of a large cohort of patients with ovotesticular disorder of sex development treated at a single tertiary center. MATERIALS AND METHODS: We reviewed the records of 20 patients with ovotesticular disorder of sex development. We retrospectively evaluated clinical and surgical characteristics. A prospective study was also performed, including evaluation of surgical results, gonadal function, sexual activity and voiding symptoms of these patients during adulthood. RESULTS: All patients had ambiguous genitalia, including 18 with a 46,XX karyotype and 2 with a 46,XX/46,XY karyotype. Gender assignment at birth was male in 13 patients and female in 7. Three females were later reassigned to the male gender. Bilateral gonadectomy was performed in 10 patients. Testicular tissue was preserved in 8 males and ovarian tissue was preserved in 2 females. Average followup was 25 years (range 4 to 46). Puberty started spontaneously in 14 patients between ages 11 and 14 years. Seven patients showed spontaneous puberty after conservative gonadal surgery and 4 required hormonal replacement during adulthood. The most frequent complications in males were urethral fistula in 6 and late urethral stenosis in 3. Two patients with urethral stenosis had symptoms 10 years postoperatively. One female presented with temporary dyspareunia. In adulthood 8 males and 2 females reported sexual activity. All male patients reported orgasm and 2 reported ejaculation. CONCLUSIONS: Male gender assignment was more prevalent. Long-term followup revealed adequate pubertal development and sexual activity. Complications involving the urethra developed frequently in male patients.
PURPOSE: We present the followup of a large cohort of patients with ovotesticular disorder of sex development treated at a single tertiary center. MATERIALS AND METHODS: We reviewed the records of 20 patients with ovotesticular disorder of sex development. We retrospectively evaluated clinical and surgical characteristics. A prospective study was also performed, including evaluation of surgical results, gonadal function, sexual activity and voiding symptoms of these patients during adulthood. RESULTS: All patients had ambiguous genitalia, including 18 with a 46,XX karyotype and 2 with a 46,XX/46,XY karyotype. Gender assignment at birth was male in 13 patients and female in 7. Three females were later reassigned to the male gender. Bilateral gonadectomy was performed in 10 patients. Testicular tissue was preserved in 8 males and ovarian tissue was preserved in 2 females. Average followup was 25 years (range 4 to 46). Puberty started spontaneously in 14 patients between ages 11 and 14 years. Seven patients showed spontaneous puberty after conservative gonadal surgery and 4 required hormonal replacement during adulthood. The most frequent complications in males were urethral fistula in 6 and late urethral stenosis in 3. Two patients with urethral stenosis had symptoms 10 years postoperatively. One female presented with temporary dyspareunia. In adulthood 8 males and 2 females reported sexual activity. All male patients reported orgasm and 2 reported ejaculation. CONCLUSIONS: Male gender assignment was more prevalent. Long-term followup revealed adequate pubertal development and sexual activity. Complications involving the urethra developed frequently in male patients.
Authors: Jyoti D Chouhan; David I Chu; Antoinette Birs; Louise C Pyle; Jason P Van Batavia; Rebecca L Linn; Susan J Back; Pierre Russo; Kassa Darge; Thomas F Kolon; Arun K Srinivasan Journal: Urology Date: 2017-04-13 Impact factor: 2.649
Authors: Georgette Beatriz De Paula; Beatriz Amstalden Barros; Stela Carpini; Bruna Jordan Tincani; Tais Nitsch Mazzola; Mara Sanches Guaragna; Cristiane Santos da Cruz Piveta; Laurione Candido de Oliveira; Juliana Gabriel Ribeiro Andrade; Guilherme Guaragna-Filho; Pedro Perez Barbieri; Nathalia Montibeler Ferreira; Marcio Lopes Miranda; Ezequiel Moreira Gonçalves; Andre Moreno Morcillo; Nilma Lucia Viguetti-Campos; Sofia Helena Valente Lemos-Marini; Roberto Benedito de Paiva Silva; Antonia Paula Marques-de-Faria; Maricilda Palandi De Mello; Andrea Trevas Maciel-Guerra; Gil Guerra-Junior Journal: Int J Endocrinol Date: 2016-11-28 Impact factor: 3.257