Surgical treatment of intersex disorders

True hermaphroditism (TH) is the rarest variant of intersex malformations. By definition, these patients have both ovarian and testicular tissue. Most present due to ambiguous genitalia and/or gynecomastia. From 1986 to 1996, we treated 97 patients with intersex disorders; there were 4 with TH. In all cases testicular and ovarian tissue was separate. In 3 patients the external genitalia were ambiguous and 1 had hypoplastic male genitalia. Three patients had a 46, XY and 1 a 46, XX karyotype. Three patients had been listed as males and 1 as a female. The number of operations required varied from 3 to 9 (mean 5.7 per patient). Two patients who had been raised as males finished puberty with hypogenitalism and hypoplastic testes. One married and could have erections, but no ejaculation. Two male patients have been on supplementary androgen therapy. The only patient who had been raised as a female after clitorovaginoplasty had a 6-cm-deep, blind-ending vagina that was deepened to 12 cm with bougienage, so that she was capable of having normal intercourse. It is felt at present that most patients with TH should be raised as females. This was confirmed in our patients as well, which underlines the significance of early diagnosis.

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“…From 1986 to 1996, we treated 97 patients with intersex disorders [6]. There were 4 (4%) patients with TH.…”

Section: Methodsmentioning

confidence: 99%

True hermaphroditism: 10 years' experience

Smoljanic

Vukanic

et al. 2000

Pediatric Surgery International

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“…Studies addressing early feminizing genitoplasty for patients with Prader grade II–V included primary outcome measures of cosmetic result and stricture or stenosis formation [57–62, 69–77, 85–88]. Studies in this category reported mean age at the time of surgical reconstruction as well as at follow-up periods.…”

Section: Discussionmentioning

confidence: 99%

“…Children undergoing feminizing genitoplasty for a variety of DSDs (CAH, mixed gonadal dysgenesis [MGD], ambiguous genitalia, androgen insensitivity syndrome [AIS], ovotestis, true hermaphrodite, intersex, Mayer Rokitansky Kuster Hauser [MRKH], adrenogenital syndrome, etc.) ranged in age from 1 day old to 33 years, with mean follow-up periods of 2 months to 16 years [57–62, 69–77, 85–88]. Although most studies reported patients' satisfaction with regard to cosmesis, stenosis rates were reported as high as 35 percent [69].…”

Section: Discussionmentioning

confidence: 99%

“…Furthermore, studies reviewed demonstrated too many inconsistencies to make direct head-to-head study comparisons. For instance, six studies reported one surgeon and one technique [58, 70–73, 85], five studies reported multiple surgeons ranging from one technique to several surgical techniques [75–77, 87, 88], and seven studies did not specifically list number of surgeons or techniques involved [57, 59–62, 74, 85, 86]. Only three studies reported on pubertal evaluation following primary surgery as infants or toddlers [71, 75, 86].…”

Section: Discussionmentioning

confidence: 99%

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Consensus in Guidelines for Evaluation of DSD by the Texas Children's Hospital Multidisciplinary Gender Medicine Team

Douglas

Axelrad

Brandt

et al. 2010

International Journal of Pediatric Endocrinology

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The Gender Medicine Team (GMT), comprised of members with expertise in endocrinology, ethics, genetics, gynecology, pediatric surgery, psychology, and urology, at Texas Children's Hospital and Baylor College of Medicine formed a task force to formulate a consensus statement on practice guidelines for managing disorders of sexual differentiation (DSD) and for making sex assignments. The GMT task force reviewed published evidence and incorporated findings from clinical experience. Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) was used to assess the quality of evidence presented in the literature for establishing evidence-based guidelines. The task force presents a consensus statement regarding specific diagnostic and therapeutic issues in the management of individuals who present with DSD. The consensus statement includes recommendations for (1) laboratory workup, (2) acute management, (3) sex assignment in an ethical framework that includes education and involvement of the parents, and (4) surgical management.

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“…The most common disorder of sexual differentiation is male pseudohermaphroditism that comprises about 55–60% of all cases [9]. However, some series from endocrine centres have the largest number of cases as congenital adrenal hyperplasia [10].…”

Section: Discussionmentioning

confidence: 99%

Male Genitoplasty for Intersex Disorders

Sharma

Gupta

2008

Advances in Urology

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Aim. To evaluate surgical procedures adopted for male genitoplasty in intersex disorders. Patients and Methods. Case records of intersex patients undergoing male genitoplasty from Pediatric Intersex clinic were studied. Results. Of 356 intersex cases undergoing urethroplasty from 1989–2007, the hypospadias was penoscrotal (68%), scrotal (17%) and perineal (15%). 351 patients underwent chordee correction for mild: moderate: severe chordee in 24 : 136 : 191 cases. Byars flaps were fixed upto the corona in 267 cases. Urethroplasty performed was Theirsch duplay in 335 cases, Snodgrass in 16 cases and Ducketts onlay graft in 5 cases that did not require chordee correction. Age at urethroplasty was 2.5 years—22 years (mean 11.5 years, median—5.6 years). Penoscrotal transposition correction and testicular prosthesis insertion were performed independently. Complications included fistula (45), recurrent fistula (11), stricture (12), baggy urethra (8) and recurrent infection due to persistent vaginal pouch (5). Additional distal urethroplasty was required in 15 patients for previous urethroplasty done upto the corona 5–15 years earlier. Conclusion. Hypospadias in intersex disorders is associated with severe chordee in most cases and requires an early chordee correction to allow phallic growth, staged urethroplasty and multiple surgeries to achieve good cosmetic and functional results.

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Surgical treatment of intersex disorders

Cited by 16 publications

References 14 publications

True hermaphroditism: 10 years' experience

True hermaphroditism: 10 years' experience

Consensus in Guidelines for Evaluation of DSD by the Texas Children's Hospital Multidisciplinary Gender Medicine Team

Male Genitoplasty for Intersex Disorders

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