Female pelvic congenital malformations

Female pelvic congenital malformations

European Journal of Obstetrics & Gynecology and Reproductive Biology 159 (2011) 35–39 Contents lists available at ScienceDirect European Journal of ...

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European Journal of Obstetrics & Gynecology and Reproductive Biology 159 (2011) 35–39

Contents lists available at ScienceDirect

European Journal of Obstetrics & Gynecology and Reproductive Biology journal homepage: www.elsevier.com/locate/ejogrb

Review

Female pelvic congenital malformations Part II: sexuality, reproductive outcomes and psychological impact Rosa M. Laterza a,*, Mario De Gennaro b, Andrea Tubaro c, Heinz Koelbl a a

Department of Obstetrics and Gynaecology, Johannes Gutenberg University, Mainz, Germany Department of Nephrology and Urology, Bambino Gesu` Children’s Hospital, Rome, Italy c Department of Urology, Sant’Andrea Hospital 2nd School of Medicine, ‘‘La Sapienza’’ University, Rome, Italy b

A R T I C L E I N F O

A B S T R A C T

Article history: Received 26 January 2011 Received in revised form 30 April 2011 Accepted 9 June 2011

The second part of this review deals with the quality of life of patients with congenital pelvic malformations, focusing on the sexuality, reproductive outcomes and overall psychological impact of the women affected. The presence of deformed pelvic anatomy, congenital or iatrogenic, and therefore of altered urinary, anal or sexual functions, are not only a physical limitation but seriously compromise psychological health from childhood. These difficulties jeopardise the thorny path from childhood to adult life through adolescence, and if neglected, could be responsible for seriously impairing quality of life in adulthood, in terms of mental health and psychosocial functions. If, in the 1970s, the main objective was to save the lives of newborns/infants, nowadays the therapy concept looks beyond that, focusing on quality of life and aiming to establish a satisfactory sexual life, allow the possibility of becoming a parent and enable the successful psychosocial integration of the patient. Ensuring urinary and fecal continence as well as the possibility of normal sexual activity, are the basis for allowing a normal psychological growth during adolescence, which leads to a satisfactory life later on. ß 2011 Elsevier Ireland Ltd. All rights reserved.

Keywords: Sexuality Fertility Pregnancy Psychological impact Female pelvic congenital malformations

Contents 1. 2. 3. 4.

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Sexuality, fertility and pregnancy in women with congenital pelvic anomalies. Psychological involvement: beyond the function . . . . . . . . . . . . . . . . . . . . . . . . . Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

1. Introduction The aim of the second part of this review is to consider current scientific evidence regarding the sexuality, fertility and pregnancy of women affected by congenital pelvic anomalies and to evaluate the psychosocial effects of such anomalies.

2. Sexuality, fertility and pregnancy in women with congenital pelvic anomalies Female adult patients with uncorrected anatomical sexual features due to bladder exstrophy are not usually able to have

* Corresponding author at: Department of Obstetrics and Gynaecology, Urogynaecology Unit, Johannes Gutenberg University, Langenbeckstrasse 1, 55131 Mainz, Germany. Tel.: +49 6131 17 7064; fax: +49 6131 173415. E-mail address: [email protected] (R.M. Laterza). 0301-2115/$ – see front matter ß 2011 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ejogrb.2011.06.031

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normal vaginal intercourse. Introital stenosis requires episiotomy or vaginoplasty before intercourse is possible, and sometimes despite this, the abnormal vaginal position and its shortness causes dyspareunia. Clitoral sensitivity may also be reduced as a result of anatomical defects. Urinary incontinence and genital prolapse can contribute to making sexual activity difficult. Besides the anatomical and functional aspects, psychological impact plays an important role in the sexuality of exstrophy patients. The genital anomaly itself has a significant impact on the psychological status of patients, particularly during the adolescent period. The aesthetic appearance of the genitalia (absence of the mons pubis, irregular distribution of pubic hair) makes it difficult to have a normal body image and particularly during adolescence, this can be made worse due to parental overprotection and bullying by peers. It is therefore preferable to perform reconstructive surgery before puberty, giving the patient a more natural genital appearance during their school years.

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In adult life, those affected appear to have a normal sexual identity and a normal level of sexual desire: Woodhouse et al. [1] reported a very high libido in 73 exstrophy patients (52 men and 21 women) between the ages of 18 and 44. Ben-Chaim et al. [2] evaluated urinary continence, sexual function, fertility and psychosocial integration in 20 exstrophy patients (16 men and 4 women, average age 24), and concluded that sexual function and psychosocial integration appeared to be adequate in the female patients. Psychosexual developmental issues would appear to be more of a problem for men than for women. In a recent case series of 25 bladder exstrophy patients (average age 30.5), Wittmeyer et al. [3] reported that 66% of 9 women (9 submitted to female external genitoplasty at an average age of 2 years, 7 submitted to introitus enlargement at an average age of 17.5 years) were found to be sexually active; 2 (33%) of them complained of dyspareunia during sexual intercourse, which, in one patient, was associated with genital prolapse. After surgical enlargement of a tight introitus, female exstrophy patients appear to have normal fertility: Woodhouse [4] described 32 pregnancies in 22 patients, with 27 (84%) live births. In a historical review carried out between 1722 and 1958, Clemston [5] reported 64 pregnancies in exstrophy patients with 49 live births (76%). Considering the level of obstetric care in this period, this serves as a confirmation that exstrophy patients have a good chance of giving birth. Greenwell et al. [6] reviewed a series of 20 patients, 7 exstrophy-epispadias with reconstructive procedures for congenital urological abnormalities, who had 29 live babies. Their results showed that pregnancy has no long-term effect on renal function and does not compromise the reconstruction. Despite this finding, Tomezsko et al. [7] reported that the rate of upper tract obstruction during pregnancy that requires intervention, is far higher in exstrophy patients (10%) than in the general population (<1%). Upper urinary tract obstruction during pregnancy in patients with reconstructions may simply result from increased fetal pressure on the less physically robust enterocystoplasty or neobladder. Although there is a substantial complication rate (increased incidence of premature labour and malpresentation) and an increased need for caesarean section, pregnancy in women with a lower urinary tract reconstruction for congenital urological abnormalities is considered safe for both mother and baby [6]. Pelvic organ prolapse is a significant problem during and after pregnancy in exstrophy patients (Fig. 1). It may occur in up to half of patients following pregnancy [4], in many cases leading to procidentia. The development of procidentia is already a possible consequence of pregnancy in 30% of term pregnancies, so monitoring of the pregnancy using an interdisciplinary approach is necessary. Several possible reasons have been proposed to explain the high incidence of uterine prolapse in patients with bladder exstrophy: a deficiency of the pelvic floor due to the wide separation of the pubic symphysis, an inherent deficiency of the cardinal ligament complex or the abnormal axis and short length of the vagina [8]. The presence of a weak pelvic floor in exstrophy patients may make vaginal delivery easier, however vaginal delivery is also likely to make prolapse worse and far harder to treat. Therefore, despite vaginal deliveries in exstrophy patients having been reported, elective caesarean section is highly recommended at 36 weeks [6]. When prolapse occurs, it is hugely disabling and difficult to treat, with a tendency to recur despite the best surgical efforts. Hysterectomy is not the best option as patients often wish to preserve their child-bearing capacity. Moreover, the uterus would appear to play a supporting role in these patients. Sacrospinous ligament fixation of the cervix could be considered, although the

short length of the vagina may cause difficulty in obtaining the suspension without significant suture bridges [8]. Stein et al. [9] suggested prophylactic surgery, consisting of fixing the uterus to the anterior abdominal wall during childhood, which appears to prevent prolapse but still permits a normal pregnancy. Gastaud et al. [10] used the Female Sexual Function Index score to investigate the sexuality of 35 Congenital Adrenal Hyperplasia (CAH) patients (average age 29), who underwent endocrinological care and reconstructive surgery (71% first and 29% additional surgeries, respectively). They reported a severe decrease in every area of sexual function in comparison with healthy women. Nevertheless, none of the patients expressed any doubts about their gender assignment. The most important rule in the sexual function of CAH patients seems to be represented by the status of vaginal introitus and the external genitals, rather than the degree of exposure to androgen hormones. An interesting case-control study performed recently, evaluated genital sensitivity and sexual function in women with CAH [11]: women who had undergone feminizing genital surgery demonstrated a significant impairment to clitoris sensitivity in comparison with normal controls. Four CAH patients, who had not undergone surgery, had a similar level of sensitivity to the controls. Sexual problems were more severe in women who had undergone surgery. Where feminizing genital surgery has been performed, genital sensitivity is impaired and a decrease in sensitivity is linearly related to difficulties in sexual function. Chronic exposition to an adrenal androgen excess certainly contributes to sexual function being worse in CAH patients, who develop general somatic virilisation with muscle hypertrophy, acne and hirsutism, together with secondary effects of suppressive glucocorticoid therapy, such as fat accumulation and impaired growth. A recent study [12] identified increased gender-atypical behaviour in women with CAH, that could be correlated to the CYP21A2 genotype. This supports the hypothesis of the dosedependent effects of prenatal androgens on the development of higher brain function. All these factors contribute to the impairment of body image and physical attractiveness to partners, in particular during adolescence. In patients with CAH, the fertility rate depends on several factors: compliance with therapy, irregular periods, amenorrhoea, early menopause, adrenal overproduction of androgens and gestagens (17-hydroxyprogesterone and progesterone), ovarian hyperandrogenism, polycystic ovary syndrome, ovarian adrenal rest tumours and neuroendocrine factors, as well as psychological factors such as low levels of maternal feeling. A Swedish case-control study [13] showed that pregnancy and delivery rates were significantly lower in 62 CAH women, but the outcome of the children did not differ when compared with the controls. Compared to a non-CAH female population, pregnancy and live birth rates are severely reduced in salt-wasting (the most severe form) patients, mildly reduced in patients with virilisation and normal in non-classical patients. This has been demonstrated by Mulaikal et al. [14], who reported that 15 of 25 patients with simple virilising form had 25 pregnancies, which resulted in 20 normal children, whereas only 1 of 15 women with salt-losing form became pregnant. Furthermore, Gastaud et al. [10] reported that only 1 of 14 Prader IV–V women became pregnant, although they do not specify the outcomes of pregnancy or newborns. 77% of cloaca patients have associated genital tract anomalies, with a septate system in 33–50%. They usually remain dormant in the neonatal period and childhood, but may cause serious problems at the onset of puberty with hematocolpos, hematometra, hematosalpinx or hemoperitoneum developing due to partial or complete obstruction of menstrual flow. Levitt et al. [15] reported on 22 patients who had reached puberty and were operated on for

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Fig. 1. Pelvic organ prolapse in 43 years old patient after surgery for bladder exstrophy and after pregnancy (A); abdominal hysterosacrocolpopexy (B) and outcomes of perineal and external genitalia reconstruction (C).

persistent cloaca: 7 (32%) had normal menstruation, 6 (27%) had primary amenorrhoea due to an absent or atretic uterus, 9 (41%) had severe abdominal pain as well as cystic abdominal masses and required surgical resection of inflamed collections of old blood in uteri, hemiuterus, tubes, blind vaginas, hemivaginas or in the peritoneum. When managing a newborn with cloaca, the surgeon often debated over whether to try and repair the abnormal structures or to resect them. If an obstructed tract (vagina, uterus, cervix or tube) is identified, it is possible to repair the obstructed system, to resect the uterus and the tube, or to monitor the patient until puberty. The most common genital malformation in a cloaca patient is a bilateral hemiuterus, which has the best obstetrical outcome [16–19]. To our knowledge, very little data on the obstetric outcomes of cloaca patients have been described. Hendren [20] reported on 2

women who conceived and delivered children via caesarean section after cloacal repair, but he did not describe their original genital anatomy. The first clinical signal of MRKH syndrome is generally primary amenorrhoea in spite of the presence of normal female sexual characteristics (pubic hair and breast development), and a 46, XX karyotype, accompanied by the inability to have penile–vaginal intercourse. Khen-Dunlop et al. [21] reported 23 cases of MRKH syndrome whereby sigmoidal vaginoplasty was performed at an average age of 16 years: 39% of the patients had an active sexual life 3 years after surgery. Infertility is the most difficult aspect of the disorder for the patient to accept. Nowadays, medical technology allows women in many countries to opt for in vitro fertilization of their own eggs and to use a surrogate for the pregnancy. However, the risk of transmission of the disease cannot be accurately evaluated,

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since very little is currently known about the genetics of MRKH syndrome [22]. 3. Psychological involvement: beyond the function Genital malformations appear to be directly related to the mental problems and psychological dysfunction of the patients affected. From the neonatal age, an early risk-benefit analysis to balance the psychological effects of surgical correction against possible complications is important and the long-term psychological repercussions of iatrogenic interventions must be taken into account. During childhood, it is necessary to pay attention to the quality of life and the psychological environment of these patients. Some studies [23,24] have reported that compared with healthy controls, children with congenital defects appear to have twice the risk of developing a secondary emotional handicap or psychosocial maladjustment. Steinhausen et al. [25] compared handicapped, chronically ill and healthy children, where the first group lacked emotional integration into their environment without conflict. In a series of 29 exstrophy children with an average age of 7.8 years, Montagnino et al. [26] demonstrated that there was no evidence of clinical psychopathology but that problems occurred in adaptive and acting-out behaviour. During adolescence, the impact of pelvic malformations on psychological status amplifies. Body image as a sexual human being, the start of interpersonal relationships and emotional life, as well as the growth of self-esteem, mean that this period is more vulnerable when pelvic defects are present. In 22 adolescents aged between 11 and 20 years old and with bladder exstrophy or epyspadia, Diseth et al. [27] demonstrated that 50% of them met the criteria for psychiatric diagnosis and 36% had scores indicating significant psychosocial dysfunction. Parental warmth, the patient’s genital appraisal and urinary continence function seem to represent the main predictors of mental health. Fecal incontinence and worries about future sexual relationships are risk factors for psychosocial dysfunction. Some studies have evaluated the psychological impact of hypospadia in children and adolescents. Mureau et al. [24] reported that boys aged between 9 and 18 with hypospadia have a more negative genital appraisal, and are anxious and shy towards their partners. Berg et al. [28–30] compared men operated on for hypospadia with normal controls, and found that the former were less secure in their sense of masculinity, that they had an uncertain gender identity but that the two groups were similar in terms of sexual orientation. These patients show more neurotic constrictions, a lower ego, a lower use of resources, a distorted body image, anxiety and hostility, lower self-esteem and less capacity for interpersonal relations, features that are compatible with the psychoanalytic theory on the role of castration anxiety in the formation of neuroses. Different stages of life face different developmental tasks with regards to the anomalies. Diseth et al. [27] showed that in the youngest group (11–14 years), genital appearance was the only somatic predictor of mental health problems, whereas prolonged urinary continence function was the only somatic predictor of mental health in the older group (15–20 years). Another important problem currently, is when to perform surgical reconstruction of the ambiguous genitalia. Early genitoplasty is a procedure essentially cosmetic in childhood and whilst there is no obvious benefit for young girls or their parents, it is a mutilating and irreversible surgery. Although published reports suggest that the incidence of CAH girls who opt to change gender in later life is low, the question is if it is better to delay the feminising genitoplasty until the onset of

menstruation or beyond, when it is possible to obtain the fullyinformed consent of the patient. However, as yet there is no evidence regarding the long-term outcome of the effect of uncorrected severe anatomical virilisation. Further studies are needed in order to evaluate whether the assignment of gender must be on an individual basis and the decision may need to include how aware the patient is as well as cultural considerations [31]. Young women diagnosed with MRKH syndrome suffer from extreme anxiety and very high levels of psychological distress when they are told they were born with neither a uterus nor a vagina. It can induce feelings of being different from other women, leading to a sense of inferiority and doubts about their female role and femininity. Women with MRKH syndrome may feel ‘incomplete’: they cannot menstruate, have intercourse or become pregnant and give birth to a child. Even when a woman has an iatrogenic vagina, she still has to cope with genital malformation and sterility. It has been demonstrated that a group programme is helpful to support women with MRKH syndrome in dealing with psychological distress. Weijenborg et al. [32] demonstrated that 17 MRKH women participating in a semi-structured programme where they could ask questions and share their experiences and feelings, significantly reduced their psychological distress. The huge impact of pelvic congenital malformations, with their iatrogenic consequences on psychological and mental health, caused some authors to pose the question of whether the quality of life in patients with severe genitor-urinary malformations is so questionable that a termination of pregnancy should be considered. Feitz et al. [33] evaluated the psychosexual and socioeconomic development of 22 adult exstrophy patients and concluded that the quality of life of these patients is hardly ever considered to be so poor that a termination of pregnancy would be seen as a realistic alternative. The aim of the surgeon is thus to ensure urinary-fecal continence and genital reconstruction, not only to restore function but also to give the patient a solid foundation for emotional and social growth, leading to a satisfactory adult life.

4. Conclusion If, 40 years ago, the first aim of clinicians was to save the life of patients with pelvic congenital malformations and achieve anatomical normality, nowadays functional reconstruction is considered to be equally fundamental. The pelvic floor is responsible for urinary and fecal continence and is essential for a normal sexual life, pregnancy and delivery. It is then easy to understand to what extent its morphological and functional integrity is of primary importance for a satisfactory quality of life. An active interaction between specialists (paediatric urologists, urogynaecologists, neurourologists, paediatric endocrinologists and neonatologists) whereby they exchange knowledge and expertise, is therefore necessary for the correct management of those patients. Prenatal diagnosis techniques and treatments involve obstetricians in the diagnostic–therapeutic process, and the development of imaging (MRI, ultrasound) in the investigation of pelvic floor disorders has also lead to a co-operation with radiologists that helps surgeons to have a better understanding of the anatomical anomalies prior to surgery. The aim for the future is to improve the care of patients with congenital malformations as follows:  with a multi-professional, interactive collaboration between specific teams, covering the entire period from birth to adulthood,  by improving surgical techniques, and results constantly being sought by multi-centre collaborations,

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 professional mental health guidance with a developmental and family perspective being offered routinely to both the patient and the parents,  with the establishment of patient and parent support groups, as well as family information services and seminars,  with prospective, multicentric, longitudinal studies based on semi-structured interviews, using validated and specific questionnaires.

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