Arianne B Dessens

Erasmus MC, Rotterdam, South Holland, Netherlands

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Publications (29)51.56 Total impact

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    Full-text · Article · Apr 2015 · International Journal of Pediatric Endocrinology
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    ABSTRACT: In most Western countries, clinical management of disorders of sex development (DSD), including ambiguous genitalia, begins at diagnosis soon after birth. For many Indonesian patients born with ambiguous genitalia, limited medical treatment is available. Consequently, affected individuals are raised with ambiguous genitalia and atypical secondary sex characteristics. We investigated gender identity and gender role behavior in 118 Indonesian subjects (77 males, 41 females) with different types of DSD in comparison with 118 healthy controls matched for gender, age, and residential setting (rural, suburban, or urban). In Study 1, we report on methodological aspects of the investigation, including scale adaptation, pilot testing, and determining reliability and validity of measures. In Study 2, we report on gender development in 60 children (42 boys, 18 girls), 24 adolescents (15 boys, 9 girls), and 34 adults (19 men, 15 women) with DSD. The majority of participants with DSD never received any medical or surgical treatment prior to this study. We observed a gender change in all age groups, with the greatest incidence in adults. Among patients who changed, most changed from female to male, possessed a 46,XY karyotype, and had experienced significant masculinization during life. Gender identity confusion and cross-gender behavior was more frequently observed in children with DSD raised as girls compared to boys. Puberty and associated masculinization were related to gender problems in individuals with 46,XY DSD raised female. An integrated clinical and psychological follow-up on gender outcome is necessary prior to puberty and adulthood.
    No preview · Article · Mar 2015 · Archives of Sexual Behavior
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    ABSTRACT: Objective: The aim of this study is to investigate emotional and behavioral problems among Indonesian patients with disorders of sex development (DSD) who recently came under clinical management. As diagnostic procedures and treatment had been delayed, patients progressively developed ambiguous bodies, difficult to conceal from outsiders. Method: We compared 118 Indonesian patients with DSD aged 6-41 years (60 children, 24 adolescents, 34 adults) and 118 healthy control subjects matched for age, gender, and residential settings. We used the Child Behavioral Checklist (CBCL), Youth Self-Report (YSR), and Adult Self-Report (ASR) to examine differences between patient and control groups as well as differences within patients groups. Results: On the CBCL, parents of young children with DSD reported significantly more emotional and behavioral problems than parents of matched control. Parents of daughters with CAH reported that their daughters withdrew themselves from social interactions. On the ASR, adults with DSD reported significantly more internalizing problems than controls, particularly anxiety and depression. No other differences in emotional functioning were found across different diagnostic groups. Conclusions: Indonesian patients with DSD who were untreated for most of their lives suffered more emotional and behavioral problems than matched controls. Differences and similarities between our findings and observations in patients from Western countries will be discussed.
    Full-text · Article · Dec 2014 · Journal of Psychosomatic Research
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    ABSTRACT: Sex hormones, androgens in particular, are hypothesized to play a key role in the sexual differentiation of the human brain. However, possible direct effects of the sex chromosomes, that is, XX or XY, have not been well studied in humans. Individuals with complete androgen insensitivity syndrome (CAIS), who have a 46,XY karyotype but a female phenotype due to a complete androgen resistance, enable us to study the separate effects of gonadal hormones versus sex chromosomes on neural sex differences. Therefore, in the present study, we compared 46,XY men (n = 30) and 46,XX women (n = 29) to 46,XY individuals with CAIS (n = 21) on a mental rotation task using functional magnetic resonance imaging. Previously reported sex differences in neural activation during mental rotation were replicated in the control groups, with control men showing more activation in the inferior parietal lobe than control women. Individuals with CAIS showed a female-like neural activation pattern in the parietal lobe, indicating feminization of the brain in CAIS. Furthermore, this first neuroimaging study in individuals with CAIS provides evidence that sex differences in regional brain function during mental rotation are most likely not directly driven by genetic sex, but rather reflect gonadal hormone exposure. © The Author 2014. Published by Oxford University Press. All rights reserved. For Permissions, please e-mail: journals.permissions@oup.com.
    No preview · Article · Dec 2014 · Cerebral Cortex
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    ABSTRACT: In Indonesia, disorders of sex development (DSDs) are not well recognized and medical care for affected individuals is scarce. Consequently, many patients live with ambiguous genitalia and appearance. We compared reported outcomes on body image, sexual functioning, and sexual orientation of 39 adults with DSDs (aged 18 to 41) and 39 healthy controls matched for gender, age, and residential setting (urban, suburban, rural). Differences in gender and treatment status (treated or untreated) were also explored. On body image, adults with DSDs reported dissatisfaction with sex-related body parts. Compared to the matched controls, women with DSDs reported greater sexual distress, and men with DSDs reported lower erectile and ejaculation frequencies, and more dissatisfaction with sexual life but not with sexual desire and activities. Men with DSDs who had undergone genital surgery reported higher erectile and ejaculation frequencies than untreated men. More women than men in the DSDs group reported a nonexclusive heterosexual orientation. DSDs and infertility had a great impact on sexuality. Fear of ostracism complicated DSD acceptance. Findings were compared to those of Western studies. Based on these results, education about DSDs and their psychosexual consequences may help reduce the sexual distress and problems in adults with DSDs and improve quality of life.
    No preview · Article · Oct 2013 · The Journal of Sex Research
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    ABSTRACT: Virilization due to hyperandrogenism in women causes male signs and symptoms such as swelling of the clitoris, deepening of the voice, facial hair and increase in body hair. Virilization is caused by less than 0.5% of all ovarian tumors. Here we report a case of virilizing Leydig cell tumor of the left ovary in a 36 year old woman. Misinterpretation of symptoms, conflicting medical information and advice from previous doctors had confused the patient. We performed a diagnostic evaluation including clinical, hormonal parameters, imaging, anatomical pathology examinations, and psychological assessment. Blood analysis showed a high testosterone level. The presence of an ovarian tumor was confirmed by laparoscopy. Since the patient refused ovariectomy, a biopsy of the left ovary was performed. Pathology showed a Leydig cell tumor without histological signs of malignancy. In spite of extensive explanation and psychological counseling, cultural barriers prevented appropriate treatment. An ovarian Leydig cell tumor should always be considered for a woman in the reproductive age with symptoms of virilization. The diagnosis is suspected on the basis of an ovarian mass on examination and further investigation and should be proven by biopsy.
    No preview · Article · Apr 2013 · Acta medica Indonesiana
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    ABSTRACT: Purpose: Indications that the prenatal action of testosterone in the brain is an important determinant of gender development and improved reconstructive techniques have caused a shift in male gender assignments in patients with 46XY disorders of sex development. We report long-term outcome data on psychosexual development and sexual function of these individuals in a cross-sectional study. Materials and methods: Physical status of 14 men with a mean age of 25 years with disorders of sex development was assessed by structured interview and physical examination. Psychosexual outcome was evaluated by questionnaires and compared to a control group of 46 healthy, age matched men. Results: A total of 13 men underwent 1 to 6 (mean 2) genital surgeries. Mean age at first surgery was 2.7 years. Mean penile length was 6.6 cm. All men reported erections and were able to experience orgasms. Ejaculatory dysfunction was reported by 7 men. Mean penile length was 7.9 cm in patients who were able to achieve penetrative intercourse and 4.9 cm in those who were not. Meatus was glanular in 5 patients, coronal in 7 and at the distal shaft in 1. Compared to controls, men with disorders of sex development were less satisfied with the appearance of the penis and scrotum but not with total body image. These patients reported decreased sexual desire and activities. Conclusions: Outcome in this group of men with disorders of sex development was poor regarding penile length, ejaculation, satisfaction with external genitalia and frequency of sexual activity. Other aspects, such as overall body image and psychosexual functioning, showed no difference from controls.
    No preview · Article · Mar 2013 · The Journal of urology
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    ABSTRACT: Introduction. Women with the classical form of congenital adrenal hyperplasia (CAH) are born with different degrees of virilization of the external genitalia. Feminizing surgery is often performed in childhood to change the appearance of the genitalia and to enable penile–vaginal intercourse later in life. There are suggestions that this affects sexual functioning. Aims. The aim is to study the anatomical, surgical, cosmetic, and psychosexual outcomes in women with CAH. Methods. Forty women with CAH, aged over 15 years, from two referral centers for management of Disorders of Sex Development in the Netherlands were included. Physical and functional status were assessed by a gynecological interview and examination. Sexual functioning was assessed with the Female Sexual Function Index and Female Sexual Distress Scale—Revised scales and compared with a reference group. Mean Outcome Measures. Surgery performed, anatomy, cosmetic score, sexual function and distress. Results. Thirty-six of the 40 women had undergone feminizing surgery; 25 women (69%) underwent more than one operation. Resurgery was performed in seven of the 13 (54%) women who had had a single-stage procedure. Anatomical assessment showed reasonable outcomes. Multiple linear regression showed that only level of confluence had a significant effect on cosmetic outcome, the impact depending on the number of surgeries performed. Cosmetic evaluations did not differ between the women and the gynecologists. Only 20 women had experience of intercourse. Eight women reported dyspareunia; seven women reported urinary incontinence. The women's perceived sexual functioning was less satisfactory than in the reference group, and they reported more sexual distress. Conclusion. The level of confluence was the major determinant for cosmetic outcome; the impact depended on the number of surgeries performed. Fifty-four percent of the women required resurgery after a single-stage procedure in childhood. Anatomical assessment showed reasonable outcomes. The women evaluated their sexual functioning and functional outcome less favorable than the reference group, and they experienced less often sexual intercourse. van der Zwan YG, Janssen EHCC, Callens N, Wolffenbuttel KP, Cohen-Kettenis PT, van den Berg M, Drop SLS, Dessens AB, Beerendonk C. Severity of virilization is associated with cosmetic appearance and sexual function in women with congenital adrenal hyperplasia: A cross-sectional study. J Sex Med 2013;10:866–875.
    Preview · Article · Dec 2012 · Journal of Sexual Medicine
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    ABSTRACT: In patients with disorders of sex development requiring creation of a neovagina, a number of techniques are available, including surgical vaginoplasty and self-dilation therapy. Vaginal dilation therapy has been recommended as a first-line treatment because of its less invasive character and high success rate. However, no data exist on long-term psychosexual functioning after vaginal dilation as compared with that after vaginal surgery. The aim of this study is to compare the psychosexual and anatomical outcome of women with congenital vaginal hypoplasia followed in the same clinical setting after vaginoplasty with that after vaginal dilation. The sexual quality of life of 35 women at least 2 years after vaginoplasty (N = 15), vaginal dilation therapy (N = 8), or coital dilation/no treatment (N = 12) was investigated and compared with the Dutch test validation population (as control). Psychosexual functioning was assessed with the female sexual Function index, the female sexual distress scale-revised, and a semi-structured interview. A gynecological examination was performed to determine the anatomical outcome after both vaginal treatment regimens. After either treatment, 26% of these women had a shortened vaginal length of less than 6.6 cm, i.e., more than two standard deviations below the published mean value (9.6 ± 1.5 cm). Irrespective of the treatment, 47% of the patients had (a) sexual dysfunction(s) and experienced sexual distress. However, after vaginoplasty, patients reported significantly more problems with lubrication (P = 0.025) than after self-dilation therapy. Both psychological and physical factors are predisposing for sexual difficulties. To optimize psychosexual comfort, the clinical management of women with vaginal hypoplasia needs to be multidisciplinary and individually tailored. With high success rates reported, vaginal dilation should remain the cornerstone of treatment.
    Preview · Article · Apr 2012 · Journal of Sexual Medicine
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    ABSTRACT: Clinical practice developed to promote psychosexual well-being in DSD is under scrutiny. Although techniques for genital surgery have much improved lately, long-term studies on psychosexual functioning and cosmetic outcome on which to base treatment and counseling are scarce. We studied 91 women with a DSD. Feminizing surgery was performed in 64% of the women; in 60% of them, resurgery in puberty was needed after a single-stage procedure. Both patients and gynecologists were satisfied with the cosmetic appearance of the genitalia. However, forty percent of these females experienced sexuality-related distress and 66% was at risk for developing a sexual dysfunction, whether they had surgery or not. Recognizing the difficulty of accurate assessment, our data indicate that feminizing surgery does not seem to improve nor hamper psychosexual outcome, especially in patients with severe virilization.
    Full-text · Article · Mar 2012

  • No preview · Article · Apr 2010 · The Journal of Urology
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    ABSTRACT: This chapter provides an outline of what is known about ovarian and female development. Specifically, the question is addressed if there are any known genes which are specifically required for differentiation of the bipotential gonads to become ovaries. In addition, defects in female differentiation as a result of sex chromosomal mosaicism are discussed. Subsequently, various causes of maternal and fetal androgen excess syndromes are reviewed as well as agenesis syndromes of uterus and vagina forming a separate entity within the 46,XX disorders of sex development category. Finally, relevant points regarding the independent effects of genetic factors and androgens on brain and behavior are summarized.
    No preview · Article · Jan 2010
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    ABSTRACT: Purpose Although Frank already described vaginal dilation therapy for treatment of a short vagina in 1938 patient selection criteria, treatment protocols and outcome parameters are still poorly defined. Since 2000 vaginal dilation is our first-line treatment in 46 XY-DSD with female gender. We present data of 15 consecutive patients. Material and Methods In 15 adolescents (mean age 16 years) vaginal length was measured under anesthesia and the compliance of the vaginal vault was assessed. Diagnosis was Androgen Insensitivity Syndrome in 9 (group A) and other cause, like T-synthesis disorder, in 6 (group B). In 5 patients from group A the vagina was considered sufficient. Four/9 from group A and all 6 from group B were considered candidates for selfdilation therapy. After psychological counseling and instruction patients were advised to dilate the vagina daily with a Hegar sound during 3-5 minutes. Results Mean vaginal length in group A was 5 (range 3-10) cm and in group B 3 (range 2-5) cm. Mean vaginal length in the 5/15 who did not require therapy was 7 (range 5-10) cm. Mean vaginal length in the therapy group was 3 (range 2-5) cm. Three/10 completed dilation therapy after 12-24 months with an average increase in vaginal length of 5,7 cm; 1/10 discontinued selfdilation after successful coïtus. Three/10 are not yet ready for treatment; 3/10 are lost to follow-up. Conclusions Although follow-up is short and information on sexual function is lacking in most patients to allow definitive conclusions we consider vaginal self-dilation therapy as treatment of first choice in 46 XY-DSD patients with a short vagina. Patient motivation and a delicate approach in a multidisciplinary setting are keystones to a successful result of dilation treatment.
    No preview · Article · Apr 2009 · Journal of Pediatric Urology
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    ABSTRACT: Het fenotype van jongens met een gestoorde geslachtsontwikkeling (DSD) varieert van (ernstige) hypospadie tot ambigu genitaal, vaak met enkel- of dubbelzijdig cryptorchisme. Müllerse structuren kunnen persisteren, zowel op het niveau van de - meestal cryptorche - gonade, in de vorm van een tuba en hemi-uterus, als in de vorm van een vergrote utriculus of mannelijke vagina. Persisterende Müllerse structuren bij een ondergeviriliseerde jongen moet nadrukkelijk onderscheiden worden van het zeldzame persistent müllerian duct syndrome (PMDS), waarbij een jongen met een normale penis naast intra-abdominaal gelegen testes, epididymides en vasa deferentia ook tubae en een uterus heeft. In dit artikel worden huidige behandelingsmogelijkheden toegelicht, inclusief de falloplastiek, een ingreep die momenteel niet in Nederland maar wel in België beschikbaar is. Langdurige follow-up van deze kinderen, inclusief evaluatie van psychoseksuele aspecten na de puberteit, is onderdeel van de multidisciplinaire behandeling. The phenotype of boys with disorders of sex development (DSD) varies between proximal hypospadias and ambiguity, often with cryptorchidism. Müllerian derivatives may persist at the level of the cryptorchid gonad like fallopian tube or hemi-uterus, or as an enlarged utricle or male vagina. Persisting Müllerian structures in the undervirilized boy should be distinguished from the persistent müllerian duct syndrome (PMDS), a rare anomaly in which a normal virilized male has, besides intra-abdominal testes, epididymes and deferential ducts also fallopian tubes and a uterus. This article summarizes present treatment modalities, including indications and techniques of phalloplasty. Long-term follow-up until after puberty, with special attention to psychosexual aspects, is part of the multidisciplinary treatment of these children.
    No preview · Article · Jun 2008 · Tijdschrift voor kindergeneeskunde
  • A. B. Dessens · P. T. Cohen-Kettenis
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    ABSTRACT: Bij de meeste patiënten met een stoornis in de geslachtsdifferentiatie (DSD) ontwikkelen genderrolgedrag en genderidentiteit zich conform de toegewezen gender bij geboorte. Toch komen genderidentiteitsproblemen relatief vaak voor. Onderzoek geeft bovendien aan dat bij sommige vormen van DSD het risico van genderidentiteitsproblemen groter is dan bij andere vormen. In dit artikel wordt een overzicht gegeven van genderrolgedrag en genderidentiteit bij patiënten met DSD en wordt besproken hoe deze in de psychologische begeleiding van ouders en kinderen kunnen worden aangepakt. In most patients with disorders of sex development (DSD) gender role behaviour and gender identity develop in accordance with the assigned gender. Yet, gender identity problems are relatively frequent. Research indicates that the risk of developing gender identity problems varies between conditions. In this article a review is given of gender role behaviour and gender identity in patients with DSD and is discussed how these problems may be addressed in the psychological counselling of parents and children.
    No preview · Article · Jun 2008 · Tijdschrift voor kindergeneeskunde
  • A.B. Dessens · P.T. Cohen-Kettenis
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    ABSTRACT: In most patients with disorders of sex development (DSD) gender role behaviour and gender identity develop in accordance with the assigned gender. Yet, gender identity problems are relatively frequent. Research indicates that the risk of developing gender identity problems varies between conditions. In this article a review is given of gender role behaviour and gender identity in patients with DSD and is discussed how these problems may be addressed in the psychological counselling of parents and children.
    No preview · Article · Jun 2008 · Tijdschrift voor kindergeneeskunde
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    ABSTRACT: Een stoornis van de geslachtelijke ontwikkeling met onduidelijk geslacht na de geboorte is een ingrijpende situatie en eist multidisciplinaire behandeling door een gespecialiseerd team. Aan de hand van de karyotypering kan een eerste onderscheid gemaakt worden tussen virilisatie van een genotypisch meisje en ondervirilisatie van een genotypische jongen. Belangrijk in het diagnostische traject is de hormonale evaluatie op het juiste tijdstip. Echografisch onderzoek van de genitalia interna helpt bij verdere evaluatie van de genitalia interna en de positie van de gonaden. Geslachtstoewijzing wordt uitgesteld totdat een definitieve diagnose is gesteld. Naast snelle diagnostiek naar de onderliggende oorzaak is psychologische begeleiding van de ouders een belangrijk doel. A disorder in the sexual development with ambiguous genitalia in the neonatal period is a stressful situation for parents and requires early diagnostic procedures by a multidisciplinary team. The first step in the evaluation of a newborn child with ambiguous genitalia is karyotyping which gives a first difference between virilised 46,XX girls and 46,XY undervirilised males. Ultrasonography of the internal genitalia gives additional information about the presence and location of the gonads as well as the presence of Müllerian structures. It is important to perform hormonal evaluation at the right time. Gender assignment has to be made only after the diagnostic path is completed. Beside laboratory and radiological evaluation psychological support of the parents by experienced psychologists is important.
    No preview · Article · Jun 2008 · Tijdschrift voor kindergeneeskunde
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    ABSTRACT: A disorder in the sexual development with ambiguous genitalia in the neonatal period is a stressful situation for parents and requires early diagnostic procedures by a multidisciplinary team. The first step in the evaluation of a newborn child with ambiguous genitalia is karyotyping which gives a first difference between virilised 46,XX girls and 46,XY undervirilised males. Ultrasonography of the internal genitalia gives additional information about the presence and location of the gonads as well as the presence of Müllerian structures. It is important to perform hormonal evaluation at the right time. Gender assignment has to be made only after the diagnostic path is completed. Beside laboratory and radiological evaluation psychological support of the parents by experienced psychologists is important.
    No preview · Article · Jun 2008 · Tijdschrift voor kindergeneeskunde
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    ABSTRACT: The phenotype of boys with disorders of sex development (DSD) varies between proximal hypospadias and ambiguity, often with cryptorchidism. Müllerian derivatives may persist at the level of the cryptorchid gonad like fallopian tube or hemi-uterus, or as an enlarged utricle or male vagina. Persisting Müllerian structures in the undervirilized boy should be distinguished from the persistent müllerian duct syndrome (PMDS), a rare anomaly in which a normal virilized male has, besides intra-abdominal testes, epididymes and deferential ducts also fallopian tubes and a uterus. This article summarizes present treatment modalities, including indications and techniques of phalloplasty. Long-term follow-up until after puberty, with special attention to psychosexual aspects, is part of the multidisciplinary treatment of these children.
    No preview · Article · Jun 2008 · Tijdschrift voor kindergeneeskunde
  • Arianne B Dessens · Froukje M E Slijper · Stenvert L S Drop
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    ABSTRACT: This article reviews the literature on studies and case reports on gender identity and gender identity problems, gender dysphoria, and gender change in chromosomal females with congenital adrenal hyperplasia, raised male or female. The large majority (94.8%) of the patients raised female (N= 250) later developed a gender identity as girls and women and did not feel gender dysphoric. But 13 (5.2%) patients had serious problems with their gender identity. This percentage is higher than the prevalence of female-to-male transsexuals in the general population of chromosomal females. Among patients raised male, serious gender identity problems were reported in 4 (12.1%) out of 33 patients. From these observations, we conclude that the assignment to the female gender as a general policy for 46,XX patients with CAH appears justified, even in severely masculinized 46,XX newborns with CAH (Prader stage IV or V).
    No preview · Article · Sep 2005 · Archives of Sexual Behavior

Publication Stats

457 Citations
51.56 Total Impact Points

Institutions

  • 2005-2015
    • Erasmus MC
      • • Department of Endocrinology
      • • Department of Child and Adolescent Psychiatry / Psychology
      • • Department of Pediatrics
      Rotterdam, South Holland, Netherlands
  • 1994-1999
    • University of Amsterdam
      • Department of Obstetrics and Gynaecology
      Amsterdamo, North Holland, Netherlands