True hermaphroditism: 10 years' experience

Zd, Krstić; Smoljanic, Zeljko; Vukanic, Dragan; Varinac, D; Janjić, G.

doi:10.1007/s003830000415

Cited by 38 publications

(19 citation statements)

References 6 publications

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“…These tissues may be co-existent in the same gonad (ovotestis) or independently the ovary on one side and the testicle on the other). The incidence is rare, accounting for nearly 3 to 10% of DSD cases [2]. The external genitalia show variable phenotypes, ranging from a normal male to a normal female presentation.…”

Section: Introductionmentioning

confidence: 99%

Evaluation and treatment for ovotesticular disorder of sex development (OT-DSD) - experience based on a Chinese series

Chen

Wang

et al. 2017

BMC Urol

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BackgroundThe aim of this study is to review and present the clinical features and process of evaluation and treatment for OT-DSD in a single center in recent years in China.MethodsSixteen patients with OT-DSD during the past 4 years underwent the evaluation and treatment in a single center. The clinical characteristics and outcomes of surgery were analyzed.ResultsThe surgical age ranged from 17 months to 66 months with a mean age of 20 months, and the mean follow-up was 30 months (4 months to 56 months). The presentation in 11 patients was ambiguous genitalia, and the rest 5 patients were suspected to have DSD in preoperative examination before hypospadias repair. The karyotypes were 46, XX in 11 patients, 46, XX/46, XY in 3, 46, XX/47, XXY in 1, and 46, XY in 1. Initial reared sex was male in 14 patients, female in 1, and undetermined in 1. After surgery, genders were reassigned in 3 patients, while 15 patients were raised as male with testicular tissue left. Only 1 patient with ovarian tissue left was raised as female. Repair was completed in 11 males and 1 female, and stage I urethroplasty was done in 4 males. No further surgery to remove the gonads was needed for inconsonance of gender assignment. No gonadal tumors were detected.ConclusionsOT-DSD is a rare and complex deformity with few systematic reports in China. It’s important to establish a regular algorithm for evaluation and treatment of OT-DSD.

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Section: Introductionmentioning

confidence: 99%

Evaluation and treatment for ovotesticular disorder of sex development (OT-DSD) - experience based on a Chinese series

Chen

Wang

et al. 2017

BMC Urol

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“…The risk of gonadal malignancy is low unless a Y chromosome is present [Looijenga et al, 2007]. Female sex of rearing, which preserves the chance of fertility, has been the preferred option in many cases and in these, the testicular component is removed to prevent unwanted testosterone secretion and reduce the risk of malignancy [Damiani et al, 1997Krstić et al, 2000;Verkauskas et al, 2007].…”

Section: XX Ovotesticular Dsdmentioning

confidence: 99%

Long-Term Outcome of Disorders of Sex Development

Warne

2008

Sex Dev

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The management of disorders of sex development (DSD) has been a problem area for years, partly because clinicians have started to see that not all of their patients grow up to be happy adults content with the gender assigned to them at birth, and partly because of the vigorous activities of patient advocacy organizations who have publicized their unhappiness and disagreement about current practices to the world at large and to politicians in particular. Results from a large number of long-term outcome studies have been published in the last decade and this paper attempts to give an overview of what we now know and what we still do not know about how to obtain a good outcome for our patients. Many studies have focused on a particular disorder and there have been more about congenital adrenal hyperplasia and complete androgen insensitivity (CAIS) than any of the other conditions, even though mixed gonadal dysgenesis is probably more common than CAIS. This is because researchers have wanted to know about the effects of hormones on the brain. There have been studies from a number of different countries, and cultural differences come to the fore in disorders affecting sex and gender. Very few studies have been done in Africa or East Asia so far. Long-term outcome should be studied in every treatment center, but there is a great need for study instruments to be developed that would be robust enough to use in a range of different cultural settings and languages. The studies show that while many patients fare well and are leading productive lives, gender dysphoria has been underestimated in the past and that gender counseling as well as sexual counseling should be part of the multi-disciplinary service available to patients with DSD. More emphasis is also needed on strategies to prevent the development of germ cell cancers. Urological problems in both males and females with DSD have been underestimated and deserve more attention.

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“…Ovotesticular disorder of sex differentiation (true hermaphroditism) is an uncommon cause of disorder of sex differentiation (DSD) and has an estimated world incidence of 4% [1]. With an unusually high incidence of this condition among South African patients investigated for ambiguous genitalia (51%), several authors have shown that true hermaphroditism in Southern Africa is different in several respects [2,3].…”

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confidence: 99%