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Chapter 3: Psychological Aspects of Hypospadias (part 3)

 

  1. Synthesis of the study and links with the literature

The qualitative study on on the experience of living with hypospadias carried out by Walker (1998) contains many items of interest.

First, the researcher who undertook it has hypospadias, and this certainly helped him to better understand the implications of this condition for those affected. Recently, Berenbaum (2003) has underlined the methodological importance of being able to review the consequences of congenital conditions of the external genital organs in longitudinal studies of the experience of affected individuals

Second, the themes which emerged from the study were able to depict the experience of hypospadias from a perspective which was not only all-encompassing (physical, psychological, sexual, etc), but which also took into account the individual journeys of people throughout their lives.

Third, this method had the merit of broadening out certain data which appeared in the quantitative studies published on the subject of hypospadias. For example, the doubts regarding masculinity which were observed in the study by Berg & Berg (1983b) could be resolved in the light of these results [54]. Other studies related to the development of gender in children born with hypospadias (Sandberg, Meyer-Bahlburg, Yager et al., 1995; see also Sandberg, Meyer-Bahlburg, Aranoff et al., 1989), could also be enhanced by a consideration of the psychosocial aspects of hypospadias as well as the psycho-endocrinal. Equally, I think that the ‘decrease of self-esteem’ reported by Berg et al. (1982) in patients with hypospadias could be linked to the stigmatization related to their penile deficiency. One finding which is certainly clarified by Walker’s research (1998) is that the feelings of embarrassment stemming from the consciousness of having a genital difference – observed in the studies of Mureau et al. (1995c) – can be blamed largely on the lack of discussion about hypospadias in the family and with the medical profession.  As in other earlier studies (Robertson & Walker, 1975), the issue of hypospadias being guarded as ‘secret information’ by family members was also predominant. The fears of sexual intimacy which were discovered by the Dutch team in their work with children, adolescents and adults operated upon for hypospadias (Mureau et al., 1995a, 1995b), may in the same way be understood through a developmental perspective.

Fourth, I would underline that Walker’s research (1998) brought a more positive outlook than that of other earlier research, stressing as it did the capacity for recovery on the part of people affected by hypospadias, following a deep crisis at some moment in their lives.

Fifth, there was also the fundamental concept of being able to meet other men affected by hypospadias. I would stress here, in passing, that the need to meet other similar people, via peer support groups, holds an importance similar to that for individuals born with other congenital conditions affecting the genital organs, such as intersexual conditions (Diamond, 1997 ; Diamond & Sigmundson, 1997; Preeves, 1999).

Sixth, Walker (1998) has emphasized through his research various responsibilities which apply not only to professionals in the fields of mental health and medicine, but also to members of the family. He proposed that these people should be more open to questions from those affected by hypospadias, and should encourage discussion about the condition.  He calls also for change in the protocols surrounding treatment for hypospadias with a view to including a psychological support service throughout the development of the individuals concerned (see Berenbaum, 2003).

  1. Conclusion

The literature on psychological, psychosexual and psychosocial consequences of hypospadias is relatively restricted. Nevertheless, I believe that it offers numerous keys to a better understanding of the problems and challenges faced by individuals affected by this anatomical variation of the urethra.

The first series of studies carried out by the Swedish research team presented the experiences of men who had been operated on for hypospadias. These authors clearly showed the existence, even after surgery, of psychological effects which impacted on the later development of the individuals concerned. They also highlighted the role of psychosocial factors in some of the difficulties which may be encountered (e.g., influence of the family and peers, and symbolic importance of the penis in Western society).

The second series of studies, carried out by the Dutch research team, made it clear that there were some differences in the way in which people with hypospadias viewed their penis. They were also able to show that a number of problems could arise for such people when circumstances risked their condition being discovered (e.g., seeking sexual interactions, or needing to undress in front of others).

In both these studies, it became clear that there was a growing need for psychological or sexual support of a professional kind.

The qualitative research delved deeper into several issues which had already been researched in the past. But above all, it highlighted the lack of support offered to people with hypospadias by either the medical profession or the family. One of the strongest points of this research was its ability to stress the importance of providing good information and education about hypospadias so that this condition is no longer seen as something shameful. Too often, hypospadias has been perceived as something abnormal which it is vital to hide. The author has stressed the necessity to destigmatize the condition through meeting other people affected by hypospadias. Being able to express one’s own feelings and experience of hypospadias to trustworthy people has an extremely beneficial psychological effect.

GENERAL CONCLUSION

This, then, is the end of this work about research on hypospadias. While this account is certainly incomplete, overall I think it helps to foster a better understanding of the possible psychological consequences related to this particular congenital condition.

Although relatively common, hypospadias remains very little discussed today in the field of psychology. However, as I have tried to show, the psychological implications of such a condition are not minor. But the greater part of current research is more centered on the physical aspects of hypospadias than on the impact it may have on the people who experience it.

The principal focus of this paper was to establish whether there were psychological consequences associated with having hypospadias, a question I can now answer in the affirmative. I have shown in this work that surgical operations may be necessary but they are not sufficient: the few studies carried out in this field indicate that every operation requires, to some extent, the support of a psychologist specially trained in this field. Indeed, the influence of both the family and the medical profession have a major role to play in helping the affected boy or man to confront and accept his condition. And better communication and information about hypospadias, as well as simply revealing its existence, will permit better integration of the feelings of difference which can occur through comparison with the images of male genitalia which are usually encountered in our society.

I have drawn my conclusion from research work which had access to representative populations. In this sense, I can see a limitation in my work, since it was not possible for me to verify these conclusions for myself. A way forward which would improve this work would be to conduct further qualitative research, since I believe the information I gathered from my interviews was insufficient to be introduced here in a strictly scientific way. The people with whom I had contact experienced enormous difficulty and huge emotional challenges in speaking about their condition, and in most cases it still remains a secret for them. And, unfortunately, the research framework of a thesis scarcely lends itself to therapy.

Finally, I would like to highlight my desire to continue my research in this field. This is only one paper, and much more could be done. It would be interesting, for example, to ask some new questions; for example, to research why, given the frequency of this condition, it remains still largely unknown today. On this matter, many authors (Dorais, 1999; Dreger, 1999; Kessler, 1998) have shown that the Western society in which we live tends to perceive the sexual organs along strictly divided gender lines. However, with the existence of not only hypospadias but also other congenital anatomical variations of the external sex organs which can occur in both men and women, it’s a fact that human external sexual anatomy can appear in more forms than are normally represented. Perhaps, instead of a binary vision of the sexes, we should rather accept that a ‘continuum’ exists.

[42] Between 1952 and 1963, to be more precise.

[43] The semi-structured interview comprised first a collection of psychiatric symptoms in two versions – one for adults and one for children – and second general questions (e.g., family history, social activities, relations with peers at school), questions related to sexuality (e.g., detailed sexual history) and more specific questions on the medical condition of hypospadias (e.g., family attitudes to the condition, memories of hospitalization). The personality test was done using the Rorschach test (1921) (for details, see Berg et al., 1982, pp. 400-402).

[44] Gender identity was examined through the ‘Franck Drawing Completion Test ‘ (FDCT; Franck & Rosen, 1949) and gender role behavior through the ‘Gough Femininity Scale’ (GFS; Gough, 1952) (for details, see Berg & Berg, 1983b, pp. 156-157).

[45] These questionnaires measured the degree of satisfaction with different aspects of the penis such as, for example,  the size of the penis when flaccid and when erect, the position of the urethral meatus, the shape of the glans and the general appearance of the penis. Two supplementary questions were also asked with the aim of finding out whether the subjects had already received comments on the appearance of their penis, and whether the subjects considered their penis to be similar in appearance to that of other men/boys.

[46] It is notable that relatively similar data has emerged from a study carried out comparatively recently, in Italy, by Mondaini et al. (2002). The available sample was composed of 42 adults, aged over 18, with hypospadias (control group numbered 500). The results indicated that 22% (against 9% in the control group) had difficulties in initiating contact with the opposite sex; 24% reported hiding their genital organs in public situations (e.g., in showers after sport); and 26% (against 2%) evaluated negatively the appearance of their genital organs.

[47] Evaluated through the ‘Junior Dutch Personality Questionnaire’ (DPQ-J; Luteijn, Van Dijk & Van der Ploeg, 1989) with children and adolescents, and by the ‘Dutch Personality Questionnaire’ (DPQ; Luteijn, Starren & Van Dijk, 1975) with adults.

[48] Evaluated by the ‘ ocial Anxiety Scale for Children’ (SAS-C ; Dekking, 1993) for children, and by the ‘Contact with Others Inventory’ (COI ; Van Dam-Baggen & Kraaimaat, 1987) for adults.

[49] Evaluated by the ‘Child Behavior Checklist’ (CBL ; Achenbach, 1991) and the ‘Young Adult Self Report’ (YSR, Achenbach, 1991) with children and by the ‘Young Adult Self Report’ (YASR ; Achenbach, 1990) with adults.

[50] In an article tackling the issue of sexuality after an operation for hypospadias, the surgeon A. Bracka (1999) confirmed that patients having a penis less than 9 cm long when erect reported being satisfied with their sexual relations and that a penis of reduced length causes few functional difficulties once the patients succeeded in overcoming their initial inhibitions and have sexual relationships.

[51] The medical characteristics of the participants, at birth, was varied. They ranged from the presence of peno-scrotal hypospadias (posterior form), to balano-preputial hypospadias (anterior form). Except for one participant, all other participants in the study (including the researcher) had received one (or more) surgical repair operation. The operations were carried out between the ages of 3 and 9.

[52] These feelings did not become fixed until the participants started to explore sexuality and encountered cultural bias concerning ‘genital anomalies’ and ‘male power’ as represented in society (Walker, 1998, p. 98).

[53] One of the reasons for this lack comes from the medical point of view that if hypospadias is surgically repaired, the psychological problems are resolved (Walker, 1998, p. 216).

[54] For Berg & Berg (1983b, p. 162), it was quite possible that the continued perception of a deficient body could lead to disturbance in the gender identity of the person (the feeling of belonging to one sex and not the other, in this case the male sex).

Hypospadias support, Hypospadias surgery

Chapter 3: Psychological Aspects of Hypospadias (part 2)

 

  1. Synthesis of the two studies

Paucity of literature  

According to the authors, in contrast to the abundance of literature on the medical side, very little scientific research has been carried out on the possible long-term psychological impact of hypospadias and surgery on the lives of the individuals concerned.

4.2. Offering professional psychological support

The studies carried out by the Swedish research team at the beginning of the 1980’s referred to several psychological, psychosocial and psychosexual  difficulties that can arise after hypospadias surgery. The authors highlighted the fact that the later development of boys operated on for hypospadias could be influenced by factors in the environment such as the reaction of parents and peers.

This team stressed the importance of psychological support which could be offered at an early stage to the parents of children with hypospadias and to the children themselves, in order to avoid adding a ‘psychological handicap’ (Berg et al., 1982, p. 411) to their ‘physical handicap’.

The series of studies produced by the Dutch research team also included a series of observations and conclusions concerning the psychological impact of hypospadias and its repercussions on psychosocial and psychosexual development. The authors remind us that, despite constant progress in this field, hypospadias surgery does not give a perfectly normal appearance to the penis (Mureau et al., 1997, 1995c). The majority of patients continued to perceive differences between the appearance of their own penis and that of others: they were more dissatisfied with the appearance of their penis, and had more often received comments about it; they were more self-conscious and embarrassed by the appearance of their genitals, which led to inhibitions in seeking out sexual relationships. They were also more inhibited in undressing in front of other people (e.g. in showers or communal changing rooms).

Mureau et al. (1995c) also included some comments on the importance of being able to offer psychological and/or professional sexual therapeutic support to both parents and the patients.

In conclusion, both series of studies show that although surgical treatment is effective in re-establishing varying degrees of functional and aesthetic normality to the penis, it is still always possible that emotional difficulties will persist.

4.3. Another risk factor: the secret of hypospadias

Other factors contribute to a patient’s capacity to face up to his hypospadias: as mentioned by the Dutch researchers, more often than not there is a lack of information and explanation from the medical community, for both parents and the patients themselves.

But the emotional consequences of a condition such as hypospadias are equally affected by a lack of discussion within the family. Thirty years ago, Robertson & Walker (1975) reported that that hypospadias was regarded, by the parents and the child, as ‘secret information’. The secret, in the context of hypospadias, referred to the existence of a penis which was not completely perfect; this situation was shared neither with friends, nor close relations, nor even in the bosom of the immediate family.

  1. A qualitative research project (United States, 1998)

The two series of studies already presented were quantitative in nature. As far as I know, there is no qualitative research published in journals. Nevertheless, I have located a doctoral thesis on the psychology of hypospadias (unpublished). I present here, broadly, why and how this research was carried out, and its results and implications.

5.1. The subjects investigated

Walker’s (1998) doctoral thesis aimed to thoroughly examine the psychological experience of living with hypospadias as an adult. Being affected personally by hypospadias, Walker knew that this condition and the related surgery had profoundly affected his existence during both childhood and adulthood; this experience of hypospadias was a powerful motivation to explore and so better understand the experience of others in the same situation. The objective of his research was to view the lives of men with hypospadias in a holistic manner (physical, psychological, creative and spiritual), so he could better understand the role which hypospadias had played in their lives. His approach acknowledges the whole person, as well as the deficiency.

5.2. Research method used

Walker (1998) used a qualitative research method. More precisely, this researcher adopted a method known as heuristic, an approach used in social science which requires the participation and involvement of the researcher at the center of the research. The direct experience of the researcher regarding the topic being investigated is one of the principal requirements of the heuristic method (Moustakas, 1990, cited in Walker, 1998). This researcher chose this approach since it allowed him to study the phenomenon of hypospadias while being personally affected by it.

5.3. Participants in the research

The participants in Walker’s (1998) research came from a support group for hypospadias which existed on the internet at the time. The researcher had himself been active in the support group and it was in this way that he gradually chose 5 participants affected by hypospadias [51]. The participants were aged between 23 and 47 years and came from different regions of the United States.

The medical characteristics of the participants at birth was varied. They ranged from penoscrotal hypospadias (posterior form), to balano-preputial hypospadias (anterior form). All except one participant, including the author, had experienced surgical repair (some more than once). The operations took place between the ages of 3 and 9.

5.4. Some results

According to Walker (1998), the manner in which each participant reacted to surgery (from infancy to childhood) as well as to post-operative care from the family and the medical community, was different. However, this researcher was able to observe some common experiences among all the participants. Some of these experiences are presented here (see Walker, 1998, pp.97-100).

5.4.1. Lack of emotional support

One of the experiences common to all the participants was that they had not been emotionally supported by their families, nor had they benefited from any support from the medical profession. As children, they had been given little or no information, and no opportunities to talk about hypospadias.

5.4.2. Feeling different and keeping hypospadias a secret

All the participants said that they felt they were in some way ‘different’, partly as a result of the lack of information and discussion by the family and medical community. They also felt that they could not talk about this feeling of difference. This led them to hide their thoughts and feelings about this perceived difference, which engendered a kind of ‘secret life’ about the difference in their penis. This decision to ‘hide’ their difference intensified as they became adolescent, that is to say when their sexual awareness began to emerge.

5.4.3. Feelings of embarrassment

A common theme which stands out in the stories of the participants was feeling uncomfortable talking about sexuality. Equally it emerged that they were conscious of an uncertainty and embarrassment regarding their genital difference and their capacity to have normal sexual relations. The participants reported feeling sexually inadequate and anxious during their adolescence. Some of them had been stigmatized by comments from their peers about the appearance of their penis.

5.4.4. Shame and poor self-esteem

The participants reported having become timid and socially introverted during adolescence. It was also during this period that the first signs of ‘shame’ and a lowering of self-esteem became apparent [52].

5.4.5. Anxieties and fear of sexual intimacy

Having had different emotional and psychological experiences during adolescence due to hypospadias, each participant went through, in their own way, a ‘crisis’ on reaching adulthood. This crisis related to their sexual, personal and social identity. Their feelings of sexual inadequacy which emerged during adolescence, now evolved into a fear of sexual intimacy and difficulty in opening up emotionally and sexually towards their sexual partner. Moreover, they encountered difficulties in trusting others.

5.4.6. Solitude, isolation and mistrust   

Another experience commonly reported by the participants, as adults, was related to feeling alone and emotionally isolated. This led to their feelings of shame, difference and abnormality being further intensified.

5.4.7. Feelings of loss and incomplete masculinity

In response to their impression of being deficient with regard to their genitals, the participants felt that as men they were not totally complete. In some cases, they even developed a distorted body image. This affected their self-identity and self-worth.

5.4.8. Personal growth, acceptance of self and body

Despite difficult experiences, both emotional and psychological, in living with hypospadias, each of the participants described a process of personal recovery and psychological or even spiritual healing. These common experiences of recovery happened after experiencing an extreme level of physical and psychological suffering. This process of recuperation has been described as being a kind of ‘understanding of their experience of hypospadias’ which permitted them to live with their condition in a more effective way.

Each of the participants described, in his own manner, a way of learning to accept himself and accept his body. This kind of acceptance happened, for example, through: a helper or supporter from a spiritual program, different modes of therapy, or though a creative involvement in art and the use of a man’s imagination.

5.4.9. Support from other men with hypospadias

All the participants expressed an increased need to enter into contact with other men who had hypospadias, with the aims of finding mutual support, coming to a better self-acceptance, having an opportunity to reveal themselves (as having hypospadias), and telling their story to other men in a similar situation.

One of the topics most often raised by all the participants was the need to make parents – and the medical community involved with hypospadias – more conscious of the importance of offering psychological and emotional support at different developmental stages. This should include psychological support for parents, as well as for the young patients, but equally for adolescents and adults during their emotional and sexual development.

5.4.10. Hypospadias as a part of an overall life experience   

One last theme, but no less important, shared by all participants was coming to understand that hypospadias is only a part of their overall  experience. All had learned, in different ways, to extend their consciousness of being emotionally isolated with their experience of hypospadias (in which they were negatively focused on their ‘problem’) so that they finally understood they are part of a wider group of men who have hypospadias. To varying degrees this has enabled them to better accept their condition as being important but still only one part of their overall life experience.

5.5. Implications of the research

Walker’s research has brought out a number of implications. These are aimed both at professionals in the fields of mental health and medicine and at the family members of people with hypospadias (Walker, 1998, pp. 215-223)

5.5.1. Implications for professionals

Many participants expressed their appreciation at receiving, at certain moments in their life, professional support which helped them learn to live with hypospadias as a medical condition. However, it was not easy for them to find such help, partly because of the lack of mental health services specializing in this condition. Even in health centers and hospitals, no service existed to support the psychological problems associated with hypospadias [53]. The author recommended that new studies be carried out, and that both medical and mental health professionals be trained so that hospital services specializing in the surgical correction of hypospadias have the possibility of offering an educational and psychological support service, as well as guidance to patients and their parents, as a central part of the overall treatment for this condition (Walker, 1998, pp. 215-216).

5.5.2. Implications for family members  

The participants constantly described feelings of not having been supported by their family members, and this had an impact on their experience of hypospadias. According to the author, it was vital to be able to create a climate of openness, support and communication in the heart of the family (Walker, 1998, p. 121). In this way, children could feel more free to ask questions and express their anxieties. Parents should be able to educate, guide and listen actively to their child. One of the ways to achieve, according to the author, would be to create a specialized guidance service for parents so that they themselves may explore their own anxieties, hopes and fears. Finally, as well professional support, it would be extremely beneficial for parents to be able to access support groups for men and boys with hypospadias (p.122).

Hypospadias support

Chapter 3: Psychological Aspects Of Hypospadias (part 1)

Chapter 3: Psychological, psychosocial and psychosexual perspectives (part 1)

  1. Introduction

This chapter presents the principal studies which have been carried out on the psychological, psychosocial and psychosexual impact of hypospadias. Given the paucity of existing literature on the impact of hypospadias and its surgical repair, I have concentrated on three studies:

First, two series of studies using quantitative methods of investigation carried out by Swedish researchers in the early 1980’s and by Dutch researchers in the middle of the 1990’s. Second, a rather more qualitative study carried out by a north American researcher towards the end of the 1990’s.

  1. First series of studies (Sweden, 1980’s)

2.1. Aspects studied 

Svensson and his colleagues (Svensson, R. Berg and G. Berg, 1981) studied the psychological, psychosocial and psychosexual impact of hypospadias surgery on patients. Various aspects of these studies have been reported in different publications, but all used the same sample of subjects. The method of investigation consisted of semi-structured interviews and psychological testing, as described below. The results of the interviews and various tests were statistically analyzed.

2.2. Sample studied

Svensson et al. (1981) had access to a sample of 34 adult men aged between 21 and 34 years drawn from a population of patients treated for hypospadias (urethroplasties) during their childhood in the 1950’s and 1960’s [42], in a pediatric surgery service in the Stockholm region (Sweden).

In order to establish comparisons, a control group was composed of 36 men aged between 20 and 34 who had not had this type of surgery, but who had been operated on for appendicitis at around the same age.

2.3. Principal results

2.3.1. Delay in psychosexual development

One of the first aspects studied was related to psychosexual development (R. Berg, Svensson and Åström, 1981). When compared to the control group, the results of the interviews with men who had been operated on for hypospadias showed that their first experiences of a sexual nature (e.g., first flirtations, first sexual relations) happened at a later age and that generally they had a significantly reduced number of sexual partners.  However, in spite of these differences, the majority of the men interviewed reported having a relatively satisfying sex life (Berg et al., 1981).

2.3.2. Psychological and interpersonal disturbances

The second aspect studied concerned the psychological implications of hypospadias repair and its repercussions on mental health (G. Berg & R. Berg, 1983a ; R. Berg, G. Berg and Svensson, 1982). Using two psychiatric interviews and a personality test [43], the researchers established that the men who had had hypospadias operations considered themselves to have been more timid and isolated during childhood.

During adulthood, they reported more symptoms of depression and anxiety, and encountered more difficulties in establishing interpersonal relationships.  Finally, a tendency towards low self-esteem was observed in these subjects.

2.3.3. Doubts relating to masculinity

A third area which the research focused on was the concept of gender identity and sexual orientation (R. Berg & G. Berg, 1983b). According to the authors, who used psychological testing to measure these dimensions of psychosexual development [44], the adults who had been operated on during childhood seemed more uncertain regarding their masculinity.

This study, which also aimed to produce data on how the group behaved with regard to typical masculine and feminine roles, found evidence that the group had a higher propensity to adopt more feminine gender role behavior. However, according to Berg & Berg (1983), little difference was observed between the sexual orientation adopted by the target group and the control group, with most reporting a heterosexual choice of partner.

2.4. Implications of the data

The results of these studies clearly indicate that some differences exist between the psychological, psychosexual and psychosocial development of men operated on for hypospadias and men who have not received this type of surgery. These discoveries led the authors to propose several possible explanations.

Their first hypothesis was that there could be a link between the level of hormones in the hypospadias patients and the psychological traits they displayed. However, another study (R. Berg, G. Berg, Edman & Svensson, 1983c), demonstrated no significant relationship between the hormonal profiles and psychological characteristics such as personality traits. These results undermined the endocrinal hypothesis proposed by the researchers.

A second explanation, of a more psychoanalytic flavor, supposed that genital surgery, practiced at a psychologically vulnerable age (during the oedipal phase) could lead to an exaggerated castration anxiety and disturbances of a neurotic type (Berg & Berg, 1983a).

A third explanation, more psychosocial in nature, is based on the importance of the penis-phallus, at least in western culture: ‘symbolically, the penis represents activity, self-affirmation, social success, strength, masculinity, etc’ (Berg et al., 1982, p. 411).

Given the symbolic significance of the penis, it could be that a sense of deficiency surrounding this organ has led to fears of incapacity or incompetence, in the patients and their parents, which extend beyond the purely physical function (e.g. urination or sexual function) and into the domain of psychological function, social relations and sexual behavior.

Moreover, the development of the observed psychological characteristics, low self-esteem in particular, could have been induced by the reaction of their peers regarding their deficiency (e.g., mockery, jokes) (Berg & Berg, 1983a, 1983b).

The authors referred to the importance of some kind of professional survey or follow-up for both children having surgery and their parents in order to understand the nature of their responses to both hypospadias and the experience of genital surgery.

Such a survey could help in the development of psychotherapeutic assistance specially adapted to the needs of this group of people (Berg & Berg, 1983a, 1983b ; Berg et al., 1982).

  1. Second series of studies(Netherlands, 1990s)

3.1. Aspects studied  

The second series of studies published, this time carried out by a team of Dutch researchers, dates back to the middle of the 1990’s. These studies aimed to collect information on the genital perception  (Mureau, Slijper, Slob & Verhulst, 1995c), psychosexual development (Mureau, Slijper, Nijman et al., 1995a ; Mureau, Slijper, van der Meulen et al., 1995b) and psychosocial development (Mureau, Slijper, Slob & Verhulst, 1997) of patients operated on for hypospadias at different ages. Again, this study was designed to compare data from subjects operated on for hypospadias with data from a non-hypospadic control population.  

3.2. Samples studied

The samples used by this research team came from a population of patients treated for hypospadias during childhood, in two medico-surgical departments attached to hospitals located in the Rotterdam region. One department specialized in plastic and reconstructive surgery; the other in pediatric urology.

The patients treated in plastic/reconstructive surgery (between1960 and 1990) and those treated in pediatric urology (between 1980 and 1992) received surgical repairs (urethroplasty) giving different aesthetic results after the operations: technically speaking, the repairs were either ‘ventral’ (bringing the urethral meatus to the level of the coronal ridge) or ‘terminal’ (excavating a canal within the glans, bringing the urethral meatus to the tip).

Two samples were organized, according to age. The first sample comprised 73 adults aged 18 to 38 years who had all received ventral repairs. The second comprised 116 children and adolescents aged between 9 and 18 who, depending on the department to which they were admitted, had ventral or terminal repairs.

The researchers formed two other control groups, composed of children and adolescents (88 in number) and adults (50 in number), who had not experienced surgery on the external genital organs, but who had been hospitalized during childhood, in the same hospital, for an inguinal hernia.

3.3. Principal results

3.3.1. Different and more negative genital perception

The first study reviewed here was carried out on ‘genital perception’ (Mureau et al., 1995c). In this study, researchers wanted to know to what extent, following surgical treatment, the subjects with hypospadias continued to perceive differences between the appearance of their penis and that of others.

According to the authors, several factors play a role in the development of a different genital perception (Mureau et al., 1995c, pp. 290-291). The first factor is that the perception of feeling and touch in the penis is perceptibly changed after the operation.

Before surgery, many boys with hypospadias are not easily able to direct their stream of urine, often forcing them to sit down to urinate.  After surgery, they are able to urinate standing up and are better able to direct the stream. Similarly, the sensation produced by a curved, erect, pre-operative penis may differ from that after the operation, when the erection is straight.

A second factor is the level of a child’s awareness of his penile abnormality. Very young children are not usually conscious of having a congenital variation of the penis and urethra. It is only as they grow up and their cognitive functions develop that they become conscious that their penis looks different to other people’s (e.g. in comparison with their father, their brother(s) or their peers).

Also, children may perceive differences in their genital appearance because, despite technical progress, hypospadias surgery never gives the penis a perfectly normal appearance (e.g., the penis appears to be circumcised).

A third factor relates to the attitudes of the people around the child. As shown in previous studies (Robertson & Walker, 1975), the parents of children with hypospadias may be anxious about the future masculinity of their children (e.g., his fertility or sexual potency).

There is a risk of these parental anxieties being transmitted to the child and this may affect the child’s capacity to accept his bodily difference.  The responses of peers can also, in certain cases, increase a child’s awareness of being different (e.g. if he is unable to urinate standing up or project his stream a certain distance), which may lead to him devaluing his penis and avoiding situations where his difference may be discovered (e.g., urinating in groups or publicly).

A fourth factor concerns unrealistic expectations regarding the aesthetic outcome of the operation, from the parent’s point of view as well as the child’s. Boys who have been told that their penis will be ‘normal’ after the surgery expect to have a penis which looks similar to that of other boys. But if their expectations are not fully realized they may feel disappointment and become fixated on the appearance of their penis.

Specific questionnaires measuring ‘genital perception’  [45] were completed by the experimental subjects in two different versions: one for adults and one for children and adolescents.  Statistical analysis of the replies to these questionnaires (Mureau et al. 1995c pp. 293-295) produced some significant information.

The first observation concerns  the perception of difference in the appearance of the penis, in comparison with others.  78% of children and adolescents, and 84% of adults, operated on for hypospadias (as against 13% and 40% respectively in the control groups) were conscious of having a penis which differed in appearance from that of other people.  One of the most commonly reported issues was the post-operative circumcised appearance of the penis (the authors noted that circumcision is an uncommon practice in the Netherlands).

A second observation related to the degree of satisfaction with the appearance of the post-operative penis.  Almost 25% of the subjects operated on for hypospadias (against 5% of the children/adolescents and 12% of the adults in the control group) were dissatisfied with the appearance of their penis. Their lack of satisfaction was related to the size and shape of their penis and the position of the urethral meatus. The authors concluded that these subjects had a ‘more negative genital perception’ (ibid, p. 295). In addition, a desire for both functional and aesthetic improvement was expressed by almost 40% of children/adolescents and 37% of adults who underwent urethroplasties.

A third observation concerned comments from other people.  Almost 41% of children/adolescents and 33% of adults operated on for hypospadias reported having received comments on the appearance of their penis, in public, in places where it was necessary to undress in front of others (e.g., in the locker room while changing for sports, or in public toilets).

3.3.2. Psychosexual inhibitions

The investigation of psychosexual development in patients who had undergone operations for hypospadias consisted of semi-structured interviews based on prepared questionnaires.  Some of these questions related to the physical and psychological aspects of sexuality; others to the functional and aesthetic results of surgery. Some of the principal results from these two studies (see Mureau et al., 1995a, pp. 1352-1354, and 1995b, pp. 1903-1905) are presented below.

One common observation was that post-operative differences in genital appearance could result in people operated on for hypospadias experiencing ‘inhibitions’ in certain circumstances.

Almost 40% of children/adolescents and 33% of adults in the target groups (against 2% and 3% in the control groups) reported expecting to feel inhibited or having been inhibited in the search for intimate contact, both non-genital (e.g. in flirtations) and genital (sexual relationships).  One of the reasons often given for these inhibitions was a fear of appearing ridiculous in front of their partner, if he or she ever discovered the difference in their genital appearance.

Embarrassment, combined with a more negative genital perception, can extend to other situations and cause men to try and conceal their genitals. For example, the researchers discovered that people operated on for hypospadias hid their genitals in situations where it is conventional to be exposed (e.g., in public showers or public toilets).

According to these authors, even though the hypospadias-operated men showed certain differences when compared to the control group, such as a more negative genital perception, difficulties in establishing sexually-related contacts, and more frequent concealment of their penis in front of others [46], their sexual adjustment (e.g., the age of first sexual feelings, contacts or first sexual relations) and their sexual behavior (e.g., number of partners or frequency of sexual activity or masturbation) were considered ‘similar’ (Mureau et al., 1995a, p. 1354, and 1995b, p. 1905).

Only a few differences were observed in sexual function, including problems caused by chordee, a too-short penis, and pain during erection/orgasms.  In summary, therefore, the psychosexual development of subjects operated on for hypospadias has been evaluated as relatively ‘normal’ (Mureau et al., 1995a, 1995b, 1995c).

3.3.3. Lack of guidance and communication

At the end of the research Mureau et al. (1995a) allowed the adult subjects who had been operated on for hypospadias to express their thoughts and to ask questions. This qualitative data throws some light on the difficulties faced by this group of men.

The first difficulty was their lack of information. Many adult men asked basic questions about  hypospadias, mostly about its frequency, how it occurs and its impact on fertility.

They wanted to be informed about the precise nature of their condition. A major complaint was related to the lack of guidance and explanations received during treatment; some patients did not even understand exactly why they were being operated upon.

The second problem was the lack of communication surrounding hypospadias and the experience of surgery. According to Mureau et al. (1995a), for some men it was taking part in this research that had, for the first time, allowed them to confront and explore their hypospadias, their surgery and how it had affected their sex life.

A similar level of secrecy was clear among the children and adolescents (Mureau et al., 1995b). More than one third of them (33%) had never told anyone they had had an operation on their penis, for fear of being ridiculed.

3.3.4. Psychosocial development

The third aspect studied by the Dutch team was related to ‘psychosocial development’. According to the authors (Mureau et al., 1997, p. 372), surgical repair for hypospadias was accompanied by a series of stressful events (e.g., repeated hospitalization, parental anxieties, the experience of surgery on the genitals, consciousness of having genitals different from the norm). Thus patients operated upon would be ‘at risk’ of developing subsequent psychosocial problems.

With the aim of verifying whether people operated upon for hypospadias encountered further problems in their psychosocial development, the researchers used several standardized questionnaires which were also used with the control groups. One questionnaire related to the genital perception of the subjects (already recounted); the other questionnaires were based on certain principal variables such as:

–   social inadequacy and self-confidence [47];

–   social anxiety and social competence [48];

–   the presence of emotional and behavioral problems [49].

The results showed no significant difference in the variables investigated. Only a few significant correlations were found between the genital perception of the subjects and their psychosocial development.

These seemed to indicate that  ‘the genital perception of the subjects operated on for hypospadias was negative, their psychosocial functioning was better’ (p. 384). While generally the results of this study indicated that patients operated upon for hypospadias did not have a poorer psychosocial development than the control subjects, some results showed that patients who were more dissatisfied with the appearance of their penis ran a greater risk of developing psychosocial problems later on.

3.3.5. Implications of the data

In their discussions (Mureau et al., 1995a, 1995b), the researchers emphasized the importance of being able to follow-up patients treated for hypospadias during childhood into adulthood as standard medical practice. Patients seem little inclined on their own initiative to seek out medical advice, even if they encounter considerable physical or psychological difficulty.

Moreover, Mureau et al. (1995c, pp. 295-297) stressed the usefulness of offering professional psychological assistance and/or sexual therapy to patients who had difficulty, after the operation, in accepting the appearance of their penis, especially its size and circumcised appearance.

According to the authors, it is important to clearly inform parents and patients that: (i) after surgery the penis will have a circumcised appearance due to the absence of a foreskin, and the glans being permanently exposed;  and (ii) that a penis ‘circumcised’ through hypospadias surgery looks very similar to a penis circumcised for religious or cultural reasons.

It is also important to tell patients who are worried about the small size of their penis that surgical operations for hypospadias do not aim to increase the size of the penis, and it is important to reassure them that it is possible to have a satisfactory sex life with a small penis [50].

Some psychological support would equally be necessary for more vulnerable patients, for whom the appearance of their penis could be a risk factor in developing psychosocial problems later on (Mureau et al., 1997).

Hypospadias surgery

Chapter 2: Hypospadias and Surgery (part 3)

Chapter 2 Continued (part 3)

  1. Conclusion

The understanding of the etiology and the surgical treatment of hypospadias (or hypospadiology), remain two very active fields in medical research and literature.

Hundreds of surgical procedures developed over a century and half bear witness to the interest of surgeons, and more recently pediatric urologists, in this condition.  One of the principle objectives of surgery for hypospadias has always been to improve the functional aspects of the penis: to permit urination standing up and satisfactory sexual relationships.

For several years now, specialists in this surgery have also tried to give the penis an appearance which comes as close as possible to the aesthetic norm.  This is realized in those techniques which allow the creation of a urethral meatus in a terminal position and also result in a penis of a circumcised appearance.

Another important change concerns the required age for surgical treatment. Current practice is to advise that the operation should take place as soon as possible in order to avoid emotional consequences of awareness of a congenital deficiency and the experience of surgery.

In the same context, it’s now seen as important that the parents accompany the child during the pre- and post-operative processes. Another principle objective is to prepare the parents for the child’s surgery, giving them information on surgical procedures, the duration of hospitalization, the possible complications and the required post-operative care.

A review of the medical literature shows that hypospadias is much studied from a surgical position, but research on the psychological, psychosocial and psychosexual effects of hypospadias and its surgery is very much less represented in the literature. This is the subject of the next chapter.

Footnotes to text of Chapter 2

[21] It is notable that only a small number of publications have dealt with the physical consequences of unoperated hypospadias, regarding sexuality and reproduction as an adult (see, for example, Moudouni, Tazi, Nouri et al., 2001; Viville, 1993).

[22] In simple cases of hypospadias, an aesthetic correction should only be planned after first discussing the psychological aspects linked to hypospadias as well as clarification of any functional difficulties.

[23] The earliest forms of treatment date back to the !st and 2nd centuries BC, when surgeons amputated the penis at the level of the meatus and cauterized the wound with a hot iron! (De Sy & Hoebeke, 1996; Smith, 1997; Zaontz & Packer, 1997).

[24] I cite in this regard the use of scrotal skin (Cecil, 1932, cited by Horton & Devine, 1972), vesicle mucosa (Marshall & Spellman, 1955, cited by Coleman, 1981) or even skin from the penis (Denis Browne, 1949, cited by Gearhart & Witherington, 1979) to carry out urethroplasty.

[25] In fact, according to Paparel et al. (2001), replacement of the missing urethra using different skin grafts often produced unsatisfactory results and too high a number of repetitive operations (repetitive failures). This period was known as the ‘dark age of hypospadias surgery‘, since so many ‘disasters’ occurred!

[26] The reason for waiting was mainly ‘strategic’: surgeons had to have a reserve of skin sufficient to carry out reconstruction of the urethra. This did not happen until the age of around 3 years, or even later (AAP, 1975).

[27] When reconstructions in multiple stages were the rule (AAP, 1975), these were usually separated at intervals of 6 to 12 months, and children stayed in hospital a number of days (between 5 to 14 days) after each operation; very often rooms for parents to stay in were not provided. According to the AAP (1996), the need for several days of post-operative hospitalization is tending to be replaced by outpatient procedures, permitting patients to go home the same day. If a stay is required, many pediatric centers take care to minimize the separation time and offer rooms adapted to allow for this.

[28] MAGPI: Meatal Advancement and Glanuloplasty Incorporated. A surgical procedure consisting of opening the glans by a longitudinal incision, then vertically suturing the two sides.  This maneuver creates a flattening of the glans and repositioning of the urethral meatus to the level of the glans apex (Sheldon & Duckett, 1987).

[29] In fact, many authors have confirmed a regression or secondary recoil of the meatus after using MAGPI (Paparel et al., 2001).

[30] GAP : Glans Approximation Procedure. This technique pulls the sides of the glans together.  Its indication is limited to hypospadias where the meatus is wide and deep (Gites et al., 1998).

[31] The principle of this intervention is to use the elasticity of the urethra to bring it forward into a good position on the apex of the glans (Atala, 2002).

[32] Tubularization: creation of a new urethral canal (or neo-urethra) by rolling the tissue around a urethral catheter and suturing it in the form of a tube (Wilcox & Ransley, 2000).

[33] Embryologically, the urethral plate creates the penile urethra. In the case of a hypospadic penis, the urethral plate represents an important anatomic entity: it is a smooth urethral mucosa of variable dimensions which extends from the hypospadic meatus as far as the glans (Perovic et al., 1999). Anatomical studies have demonstrated that its use works well in the fabrication of a neo-urethra: this structure is free from hair, is richly vascularised and nerve-free, and it possesses good muscular and connective tissue components (Erol, Baskin, Li & Liu, 2001).

[34] TIP: Tubularized Incised Plate Urethroplasty.

[35] Mathieu’s technique (1932) was for a good part of the 20th century considered a model technique in repairing anterior hypospadias (the limit of its use is median hypospadias). Its method is as follows: a piece of skin, taken in advance from the ventral side of the penis, is freed, moved forward and stitched between the flanks of the urethral canal to create a new conduit (Ravasse, Petit & Delmas, 2000).

[36] The technique, the preputial pedicle flap is more often known as the ‘Onlay-Island Flap’. Its general principle is as follows: a flap of preputial skin is dissected and moved onto an isolated pedicle in the subcutaneous tissue of the dorsal side of the penis.  It is then turned over onto the ventral side and sutured as a patch on the urethral canal to constitute a new canal as far as the glans apex (De Sy, 1996b). See also: Braz J Urol, 26: 621-629, 2000 online at http://www.brazjurol.com.br/novembro/Baskin_621_629.htm

[37] This technique, better known as ‘Tubularized Transverse Preputial Island Flap’, is employed when the urethral plate has had to be dissected to straighten the penis. It utilizes the inner portion of the foreskin, retaining its own blood supply. The remaining outer portion of foreskin resurfaces the front of the penis. (See also: Hayashi et al., 2003).

[38] Buccal mucosa: mucosa taken in advance from either the inside of the cheek or from the inside upper lip.

[39] I note here that certain new techniques such as GRAP (Glanular Reconstruction and Preputioplasty) (Gray & Boston, 2003), allow a combined reconstruction of the glans and the prepuce (preputioplasty) in the case of anterior hypospadias repair. Reconstruction of the prepuce may be carried out for many reasons: aesthetic, personal convenience or even cultural (Bruézière, 1996 ; Klijn, Dik & de Jong, 2001).

[40] In English medical literature this term is known as ‘hypospadias cripples’ (Stecker, Horton, Devine & McCraw, 1981).

[41] These numerous operations are the result of an accumulation of technical faults, traumatic dissections, use of poorly vascularised skin, bad sutures or even post-operative infections (Paparel et al., 2001).

Hypospadias surgery

Chapter 2: Hypospadias and Surgery (part 2)

Chapter 2 Continued (Part 2)

6 Some technical aspects of hypospadiology

 

Progressively and over the course of time, surgeons and pediatric urologists have developed an impressive number of techniques for repairing the various forms of hypospadias. When considered globally, it is estimated that more than 300 techniques (including variations) for the correction of hypospadias have been described in literature (Arap & Mitre, 2000).

6.1. Correction of chordee 

The choice of of a urethroplasty technique is made after correction of chordee (De Sy, 1996a). Different techniques for normalizing the penis have been described (see Baskin, Duckett & Lue, 1996 ; Hayashi, Kojima, Mizuno et al., 2002). The presence of chordee is generally more easily visible when the penis is erect rather than flaccid, and in young infants this is tested by an artificial erection induced by an injection of physiological serum (Wese et al., 1994). The artificial erection test allows a judgment to be made about the extent of straightening and the length of the operation (Baskin et al., 1996 ; Hayashi et al., 2002).

6.2. Urethroplasty

According to De Sy (1996a), the choice of a particular urethroplasty technique is dependent on physical factors present (e.g., chordee, location and appearance of the urethral meatus, the shape and size of the glans, the quality of ventral skin covering the urethra, length of the urethral plate, quantity of preputial skin available). For Wilcox and Ransley (2000), the available techniques can be synthesized into four large groups.

The techniques for advancing the urethra refer to processes of adjusting the distal extremity of the penis, used in repairing anterior forms of hypospadias, without associated chordee.  One of the most popular techniques of urethral advancement was the ‘meatal advancement and glanuloplasty incorporated’  or MAGPI [28], which appeared in the 1980s (Sheldon & Duckett, 1987).

However, the aesthetic results of surgical repair of hypospadias by MAGPI are mixed [29], and pediatric urologists are tending to abandon this technique (Paparel et al., 2001). Other techniques have been proposed for repair of anterior forms of hypospadias, such as the ‘glans approximation procedure’ or GAP [30] (Zaontz, 1988, cited by Gittes, Snyder & Murphy, 1998). There is also a technique known as ‘mobilization of the urethra with advancement’ [31]
(Koff, 1981, cited in Atala, 2002).

The techniques known as tubularization [32] of the urethral plate [33] were described by the pioneers of hypospadias surgery almost a century and a half ago (Duplay, 1874). Somewhat forgotten, they were only rediscovered in the 1980s and then widely popularized during the 1990s (Wilcox & Ransley, 2000).

The general principle of these techniques is, as their name indicates, to use the urethral plate, which, once preserved, can be tubularized upon itself as far as the tip of the glans (Bouhafs, Mege, Dubois et al., 2002 ; Mege, Pelizzo, Dubois et al., 1999).

As the urethral plate is too narrow for tubularisation, a method introduced by Snodgrass (1994) overcomes this limitation: a longitudinal incision along its whole length causes the urethral plate to broaden and it can then be tubularized.

The Snodgrass technique, first used for the repair of anterior hypospadias, was later extended to posterior forms (Cendron & Ellsworth, 1999 ; Snodgrass, 1999 ; Snodgrass & Lorenzo, 2002a ; Sugarman, Trevett & Malone, 1999). The urethroplasty technique of ‘tubularized incised plate urethroplasty’ or TIP [34] is nowadays one of the most popular in surgical repair of hypospadias (Cheng, Vemulapalli, Kropp et al., 2002 ; Snodgrass & Nguyen, 2002b).

The techniques known as ‘vascularised flaps’ consist of the creation of a new urethral tube using various flaps of skin taken directly from the penis. I should mention here the Mathieu technique [35] (modified) for repair of anterior hypospadias (Hoebeke, Boemers & De Jong, 1996 ; Ravasse, Petit & Delmas, 2000).

Other techniques have been proposed when chordee is present, for example ‘transversal preputial pedicle flap’ [36] for the repair of both anterior and median hypospadias (Duckett, 1981, cited in De Sy, 1996b), and also the technique known as ‘tubular transversal preputial pedicle flap’ [37], a technique for complete replacement of the urethra, used for repair of posterior hypospadias (Duckett, 1980, cited in Hayashi, Kojima, Nakane et al., 2003).

The techniques using free grafts from elsewhere on the body, popular throughout a large part of the twentieth century, are no longer recommended except in a minority of cases: extreme posterior forms (e.g., perineal) (Ferro et al, 2002 ; Meyer et al., 2002), and multi-operated hypospadias where the skin from the penis (e.g., preputial skin) is no longer useable (van der Werff & van der Meulen, 2000).

However, I would add that certain current techniques make increasing use of the buccal mucosa [38] for making grafts (Caldamone, Edstrom, Koyle et al., 1998; Hensle, Kearney & Bingham, 2002).

6.3. Reconstruction of the ventral side of the penis

The third stage of surgery refers to reconstruction of the ventral side of the penis (Paparel et al., 2001). This final stage is usually incorporated within current urethroplasty techniques [39]. Reconstruction is carried out by means of three surgical techniques.  I mention them here, without going into details:

–       remodeling of the urethral meatus (meatoplasty) and the ventral side of the glans (glanuloplasty)

–       reconstruction of the mucous collar around the glans.  This is a technique which gives the penis an appearance very close to that of a normal circumcised penis (Kolligian & Firlit, 2000)

–       a correctly vascularised cutaneous covering

  1. Possible complications

Although the objectives of hypospadias repair aim to obtain a penis with normal functions of urination, erection and appearance, it is well known that there are a number of possible complications related to this type of surgery (Paparel et al., 2001; Wilcox & Ransley, 2000). Here is an outline of some of them.

7.1. Urethral fistulas 

One of the more commonly encountered complications after hypospadias surgery is the urethral fistula.  Characterized by an outflowing of urine at the site of the repair on the ventral side of the penis, a urethral fistula is at least an annoyance during urination (Dubois, Pellizo, Nasser et al., 1998). This complication can occur during the first six months following the operation or even several years later.  Sometimes, fistulas close themselves spontaneously. However, if this complication persists, it is possible to operate again, according to the severity of the situation (e.g. size of the fistula, multiple fistulas) (Elbakry, 2001; Shankar, Losty, Hopper et al., 2002). Some fistulas which are more complex to close than others require specific surgical procedures (Richter, Pinto, Stock & Hanna, 2003).

7.2. Stenosis of the urethral meatus

A second complication is stenosis of the urethral meatus. Rarer than fistulas, stenoses consist of a shrinking of the urethral meatus, which lessens the stream of urine during urination (Ellsworth et al., 1999). This complication can lead to serious problems due to the difficulty of completely emptying the bladder (e.g. damage to the upper urinary tract, urinary infections).  A stenosis can be treated manually by dilatation with the help of a catheter or surgically (a meatotomy) (Wilcox & Ransley, 2000).

7.3. Persistent chordee

A third complication refers to the persistence of chordee. In general, this complication is due to its inadequate correction at the time of the first operation. This complication is becoming less frequent thanks to the development of new surgical techniques in orthoplasty and the potential to check the straightening of the penis throughout the surgery (Wilcox & Ransley, 2000).

7.4. Unsatisfactory aesthetic results 

A fourth complication relates to disappointing aesthetic results. The aesthetic quality of the repair can, for example, be compromised by the presence of irregular suture points or an excess of skin on the ventral side of the glans (Paparel et al., 2001). The aesthetic objective (to give the patient a penis with as near normal an appearance as possible) can be also be subject to other complications.

Even after surgery, the urethral meatus may be situated below the apex of the glans or may have a circular form instead of a vertical orientation (slit) (Holland, Smith, Ross & Cass, 2001). With the aim of evaluating the aesthetic results of repair operations, some pediatric urologists have, for example, put forward a ‘system of objective analysis’, using photographic negatives taken at different moments of the intervention (just before, just after and between three months and one year afterwards) (Baskin, 2001).

7.5. Multiple failures

A fifth complication refers to multiple failures in operations carried out on what some authors have called hypospadias ‘cripples’ [40] or ‘disasters’ (Paparel et al., 2001). This terminology refers to boys or men who, even if they have been operated on several times [41], still suffer major functional or aesthetic problems (e.g., badly scarred tissue, persistent chordee or fistulas, stenosis of the meatus) (van der Werff & van der Meulen, 2000).

  1. Pre-admission and post-operative care

Given that the candidate population for hypospadias surgery is composed mostly of young children, the parents should benefit from a variety of information before giving their consent to the operation to be performed on their child (Ellsworth et al., 1999).

The information given out before admission is notably related to the surgical procedures and their objectives, risks and complications associated with such procedures and the duration of hospitalization. Pre-admission information also concerns post-surgical care (Sanders, 2002).

Parents should be informed that a catheter will probably be left in place for several days following the operation, to drain the urine from the bladder (Mondet, Johanet, Larroquet et al., 1999). They should also know that after the operation the penis will be covered in a specialized dressing, the application of which is recommended for several reasons: to keep the penis stabilized, to allow for the best possible healing of tissues, to ensure sterility of the wound, to reduce the risk of bleeding by keeping a moderated pressure, and to maintain the catheter in the correct position (Searles & Mackinnon, 2001).

Continued here.

Hypospadias information, Hypospadias surgery

Chapter 2: Hypospadias and Surgery (part 1)

Chapter 2: Hypospadias and Surgery (part 1)

  1. Introduction

This second chapter is dedicated to exploring medical treatment. We shall look at the physical issues which are taken into account in the decision to operate and I shall describe briefly the necessary objectives and principles of surgery.

After a review of the history of surgery for hypospadias, I shall move on to the question of the appropriate age for surgery, where I will try to show that changes have taken place in this field in recent years.  I will present a few surgical techniques in use today, and cite some complications inherent in this type of surgery.  Finally, I will touch briefly on several elements concerning pre-admission and post-operative care.

  1. Consequences of hypospadias

2.1. Functional problems

Medical practitioners believe that hypospadias runs the risk of a number of adverse physical consequences if it is not treated surgically (Bukowski & Zeman, 2001 ; De Sy & Hoebeke, 1996). Surgical intervention is generally recommended for posterior and median forms of hypospadias as well as distal forms presenting an associated pathology (e.g., chordee).

The existence of functional problems related to urination, sexuality and reproduction, has often been reported in the  literature (Arap & Mitre ; Baskin, 2000 ; Zaontz & Packer, 1997). For example, misplacement of the urethral meatus can alter the direction of the stream of urine: depending on the anatomical condition, the stream of urine tends to deviate backwards, making it more difficult to urinate standing up.  In posterior forms, deviation may be such that the individual has to sit down to urinate.

On attaining adolescence or adulthood [21], the presence of chordee may hinder sexual activity (e.g., through pain during intercourse). In adulthood, fathering a child may potentially be made more difficult if, due to the location of his urethral meatus, a man’s ejaculation takes place lower down the vagina, reducing the chances of semen reaching far enough into the vagina for insemination. 

2.2. Aesthetic repercussions 

To the three difficulties mentioned above (urination, sexual activity, fatherhood) must be added a fourth, which refers to problems of an aesthetic nature. The appearance of a hypospadic penis may be rather different to that of a ‘normal’ penis (Baskin, 2000; Sheldon & Duckett, 1987).

The external aspect of a penis with hypospadias must be taken into account during the clinical examination, even if there are no physical difficulties with the stream of urine or chordee [22] (Zaontz & Packer, 1997).

  1. Objectives and principles of hypospadias surgery

3.1. Objectives of surgery

Surgical treatment aims to re-establish the functions (urination, erection) and anatomy of the genito-urinary organ, rendering it as similar as possible to a ‘normal’ penis (Wese et al., 1994). The objectives are to obtain, at the end of the operation, various functional and aesthetic characteristics  (Zaontz & Packer, 1997):

–       a straight penis (in erection)

–       a urethral meatus situated, if possible, at the end of the glans

–       urination with a straight, well-aimed stream

–       a penis of aesthetically normal appearance

The straightening of the penis is carried out to permit the individual to have satisfactory sexual relationships later on.  The creation of a urethral meatus as close as possible to the glans, or even at the tip, using new procedures, is designed to make it possible to urinate standing up, without misdirection of the stream of urine. The position of the newly created urinary meatus also permits insemination for reproduction. Finally, the objective is also to render the penis as close as possible to ‘normal’ in appearance.

3.2. Principles of surgery

According to Paparel et al. (2001), the current approach to hypospadias is governed by three fundamental principles which should, if possible, be dealt with at the same stage (Paparel et al., 2001); they are:

–       correction of chordee

–       reconstruction of the missing piece of urethra (urethroplasty)

–       reconstruction of the ventral side of the penis

Before going into any further detail on the techniques currently utilized by pediatric urologists specializing in the surgical approach to hypospadias – known as ‘hypospadiology’ (Sheldon & Duckett, 1987) – I will briefly review the history of surgery for hypospadias as well as providing an overview of the main changes which have taken place regarding opinions about the best age for the operation.

  1.  Surgery for hypospadias: an historical view

Surgical treatment for hypospadias has long represented  ‘a great challenge’ (Perovic, Scepanovic, Vukadinovic et al., 2000; Macedo & Srougi, 1998), for generation of surgeons and pediatric urologists, and it is very likely to remain so in the future.

While different types of surgical treatment have been mentioned over the centuries [23], the ‘modern principles’ of surgery for hypospadias were only described for the first time during the 19th century and beginning of the 20th century, in Europe, by German and French surgeons (e.g., Dieffenbach, 1936 et Tiersh, 1869 ; Duplay, 1874 ; Novè Josserand, 1897 ; Ombrédanne, 1923 ; Mathieu, 1932) (Smith, 1997). Moreover, the techniques in use today mainly stem from modifications based on discoveries made by the pioneers of this surgery (Santanelli, 2002).

The surgical procedures in existence during the 20th century have been divided into two main types: multi–stage reconstructive techniques and single stage reconstructive techniques.

In multi-stage procedures, a first operation was necessary to correct the chordee.  Urethroplasty only took place afterwards, necessitating one or more operations (Horton & Devine, 1972). These procedures, used during the major part of the last century, and which used multiple skin grafts [24] as substitute material for the urethroplasty thus lengthening the time taken for the operation, are not in use today [25].

In the field of surgery for hypospadias, as stressed by Babut (1996, p. 64), ‘the ideas are evolving and certain principles have been abandoned’. If some surgeons still make use of multi-stage techniques, these are no longer likely except in very severe cases (Ferro, Zaccara, Spagnoli & al., 2002 ; Gershbaum, Stock & Hanna, 2002).

The single stage procedures are surgical procedures which permit correction of chordee and urethroplasty in one and the same operation. These procedures, mostly using penile skin (skin from the penis or prepuce), were introduced during the 1960s, then popularized around the 1980s (Lottman, 1998). They have never ceased to be remodeled and subtly improved (Belman, 1997). In the last few years, hypospadias repair has seen unprecedented evolution. Reconstruction work, originally purely functional, has today combined with aesthetic repair work of increasing finesse  (see for example, Hoebeke, De Kuyper et Van Laeke, 2002).

  1.  The preferred age for surgery

5.1. Changes in the timing

Initially, surgical reconstructions for hypospadias were practiced during later childhood, or even postponed to early adolescence (Ellsworth et al., 1999). It was only around the second half of the 19th century that certain north American surgeons proposed that the operations be carried out before children entered primary school, to permit them to urinate standing up in the boys’ toilets (Culp, 1951, cited in Mills, McGovern, Coleman et al., 1981).

The idea of operating on boys born with hypospadias before they started school spread throughout the USA where it became accepted practice at the end of the 1970s (Hodgson, 1981). At that time, according to the American Academy of Pediatrics (AAP), technical considerations remained of prime importance (American Academy of Pediatrics 1975) [26].

At the beginning of the 1980s, two publications appeared concerning the technical possibilities of starting to operate on very young children (Belman & Kass, 1982 ; Manley & Epstein, 1981). As technical changes reduced the possible age of surgery, an awareness began to develop about the psychological implications of surgery at such a young age. For example, in the mid-1970s, Robertson and Walker (1975) were able to observe that the child candidates for hypospadias surgery, and their parents, could be anxious regarding the surgical procedures used or regarding the condition of hypospadias itself (Robertson & Walker, 1975).

And at the end of the decade, Lepore and Kesler (1979) described a specific pattern of negative behavior in a group of children who had just been operated upon. The authors alerted the medical community to the danger to the children’s psychological health caused by genital surgery around the age of three years.

Around the same time, the publication of an article by Schultz, Klykylo and Wacksman (1983) contributed further to revision of the timing of the operation. These authors primarily emphasized the fact that the psychological impact of hypospadias, or a repair operation, varied according to age. According to a review of the literature at that time, Shultz et al. (1983) suggested – with regard to emotional and cognitive development and the emergence of self-image and sexual identity – that surgical interventions should be practiced during the first year of the child’s life.

They also stated that it should be arranged so that parent-child separation during hospitalization was minimized and that parental accompaniment should be encouraged, with the aim of reassuring parents and allowing them to express their anxieties, worries or feelings of guilt, emotions which are often present.

The issues of a psychological order tackled thirty years ago by Shultz et al. (1983), as well as those concerning technological advances in the domain of ‘hypospadiology’, were reprised in a more recent article from the American Academy of Pediatrics (AAP) (American Academy of Pediatrics, 1996).

According to the AAP (1996), it has become technically possible (e.g., using optical magnifiers, micro-instruments, delicate materials and sutures, and with progress in anesthesia) to operate on almost all forms of hypospadias in very young children while minimizing not only the time spent in hospital and the amount of separation from parents [27], but also the number of operations required (e.g., by using single stage procedures).

According to the AAP (1996), because emotional and cognitive development and body image can be profoundly affected both by the genital condition and the experience of surgery, the ‘best’ time to operate is between 6 and 12 months of age.

5.2. Current practice

Current practice, in north America, is to carry out this type of surgical intervention between the ages of 6 and 18 months (AAP, 2000). However, there is still no true consensus on this question internationally.  In France, for example, some surgeons prefer to wait until the child is one or two years old (Paparel et al., 2001). Overall, however, the tendency is to operate increasingly early, with as few operations as possible and in as limited a time period as possible, with the aim of diminishing the psychological impact of both the hypospadias and the experience of surgery  (Arap & Mitre, 2000; Baskin, 2000; Paparel et al., 2001).

Continued here.

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Physical and Psychological Effects Of Hypospadias (Chapter 1 part 5)

Chapter 1 continued

7. Epidemiology
7.1. Frequency of Hypospadias

In the specialist field of pediatric urology, it is reported that hypospadias is a common congenital malformation. The estimations of frequency are placed between 1 in 300 (0.3 %) and 1 in 250 (0.4%) of male births (Baskin, 2000 ; Sheldon & Duckett, 1987 ; Paparel et al., 2001).

7.2. Incidence of hypospadias

However, certain scientific studies published towards the end of the 1990s reported an increasing incidence of hypospadias in several western countries. Based upon the analysis of American and European surveys of congenital malformations and earlier epidemiological reports, Paulozzi, Erickson et Jackson (1997) observed that the rate of hypospadias almost doubled [18] between 1970 and 1990 in the United States. Paulozzi (1999) also stated that similar increases in hypospadias had been reported in several European countries [19].

A series of recent publications, based upon hospital birth registers, have reported an increase in hypospadias. One study carried out by Pierik, Burdof, Nijman et al. (2002) produced evidence that the frequency of hypospadias rose to 0.7 percent in child health centers situated in the south west region of the Netherlands; this represented a rate 6 times higher than that generally reported in the region by national registers.

Another study by Hussain, Chagtai, Herndon et al. (2001) showed an incidence 10 times higher (4.0% vs 0.4 %) of hypospadias in 2000 compared to 13 years previously (1987) in neo-natal intensive care units in the USA (Connecticut) [20]..

Other studies using different registers have discovered a higher frequency of hypospadias than is generally recognized. After an analysis of American national military registers, Gallentine, Morey and Tompson (2001) estimated a frequency of hypospadias rising to 0.7% (709 cases of hypospadias counted in 99210 males). By contrast, this frequency (0.7%) is almost double that calculated by other researchers in Italy, in a male population over 18 years of age who were examined during enrolment for military service, which reached 0.36% (42 cases per 11649 males) (Mondaini, Ponchietti, Bonafè et al., 2001).

What explanations does the scientific community give regarding these rises?

The concept of endocrinal disruption, as discussed above, has been increasingly accepted and proposed as a way of accounting for the increased incidence of hypospadias observed in more industrialized nations (Dolk, 1998 ; Harrison, Holmes & Humfrey, 1997 ; Wakefield, 2001).

However, a number of authors agree that new epidemiological research of a multi-disciplinary nature must be carried out, in order to clarify the part played by chemical substances disrupting endocrine function in the reported increased frequency of hypospadias (Baskin et al., 2001; Rittler & Castilla, 2002; Sultan et al., 2001; Toppari et al. 2002; Weber et al., 2002).

Taken together, the published studies draw attention to the fact that nowadays hypospadias is a congenital condition far more common than is normally supposed (Sharpe, 2003).

8. Conclusion

Hypospadias benefits from a vast literature concerning the investigation of its physical aspects. Currently, different medical disciplines are working together to understand better the etiology of this condition. The data currently available on this subject stem from endocrinology, molecular biology, epidemiology and even eco-toxology. Although some considerable progress has been made in these fields, supplementary research is required to better understand the factors combining to produce this condition.

This review of the relevant literature shows that hypospadias is a much more common condition than is generally supposed, that it is becoming increasingly common, and indeed that it is more common now than ever before.

Footnotes to Chapter 1

[1] The mechanisms of prenatal sexual differentiation have been to a large part elucidated by the many experiments carried out, some 50 years ago, on different animal species (batrachians, mammals and birds) (for a review see Aron, 1973, pp. 109-121).
[2] The gonadic outline is called undifferentiated when it contains both ovarian and testicular tissue.
[3] SRY : Sex determining Region Y gene. Only a tiny region (known as the ‘short arm’ of the Y chromosome contains the gene(s) of sexual determination (see Haqq & Donahoe, 1998, pp. 2-5).
[4] In the absence of SRY, the undifferentiated gonad develops as an ovary.
[5] In the absence of AMH (anti-Müllerian hormone) and testosterone, sexual differentiation follows a fixed pattern, characterized by the regression of the Wolfian ducts and the development of Müllerian ducts (the upper third of the vagina and uterus).
[6] This severity of hypospadias is also classified as vulviform; the external genital organs of the newborn male (46XY) can appear as the phenotypic feminine.
[7] The prepuce is known as a dorsal hooded foreskin.
[8] In fact, certain varieties of hypospadias with a complete prepuce have been reported in the literature (see, for example, Cold & Taylor, 1999).
[9] A penis of under-developed size, sometimes observable in hypospadias, is not to be confused with what has been called a micropenis or microphallus: here the penis is morphologically normal and the urethra’s outlet is well positioned at the end of the glans, but the size is very much reduced in comparison to the available norms (Bourgeois, 2003).
[10] The non-descent of testicles (without the association of hypospadias) is a condition frequently encountered in newborns of the masculine sex, its incidence being estimated at around 3 percent of full-term newborns (Leissner, Filipas, Wolf & Fish, 1999).
[11] Impacting of the penis in the scrotum is defined by the presence of scrotal skin, either low or high on the body of the penis. Total impacting is rare, but if this is the case only the dorsal face is visible, the ventral side remains stuck in the scrotum.
[12] The notion of sexual ambiguity relates to an undifferentiated or badly differentiated appearance of the external genital organs, or to a state of discordance between the internal and external genital organs (Encha-Razavi & Escudier, 2000). The notion of intersexuality refers to a variation of normal development whereby the appearance of the external genitals makes it difficult to assign a sex to the child at birth (Hugues, 2002).
[13] The balano-preputial ridge is the anatomical transition between the glans and the shaft of the penis.
[14] The explanation of such a classification is relatively easy to explain: the position of the urethral meatus is considered an untrustworthy criterion for judging the severity of hypospadias as, in general, the meatus moves backwards after surgical repair of the chordee (orthoplasty), worsening to some extent the degree of hypospadias confirmed initially (De Sy & Hoebeke, 1996).
[15] The concept of endocrinal disturbance refers to the molecules capable of imitating sexual hormones naturally produced by the body (xenoestrogens) or of blocking certain hormones (anti-androgens) at the stage crucial for sexual development in utero (Toppari, 2002).
[16] I cite as an example the research of Facemire, Gross and Guillette (1995) and that of Guillette (2000) (in Florida, USA). In a first study, the researchers clearly gave evidence that the abnormally high presence of cryptorchidism, observed in a population of panthers, could be explained by the fact that these felines lived very close to an agricultural area where pesticides were widely distributed. (Facemire et al., 1995). In a second series of studies, the researchers noticed a notable diminishing of the penis size of alligators born in a polluted lake (Lake Apopka) following a leakage of toxic substances (Guillette, 2000; see also Semenza, Tolbert, Rubin et al., 1997).
[17] Diethylstilbestrol (DES) is a synthetic estrogen which was widely prescribed to pregnant women between 1938 and 1975 to prevent miscarriage. The effects of DES as evidenced are avowed to be transgenerational. In the article by Klip et al. (2002), the hypothesis is that DES would be associated with a disturbed function of the placenta, resulting in a diminishing of placental and fetal hormones which could disrupt fetal development; this would predispose a likelihood of hypospadias.
[18] The rate has gone from 20/10000 in 1970 to 40/10000 in 1993 in USA (Paulozzi et al., 1997).
[19] Norway, Sweden, United Kingdom, Denmark, Italy and France (for details see Paulozzi, 1999).
[20] Other American researchers have reported an incidence, also in neonatal intensive care units (in the Atlanta region), ten times higher than normally estimated in that particular population (11% vs 1%) (Gatti, Kirsch, Troyer et al., 2002).

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Physical and Psychological Effects Of Hypospadias (Chapter 1 part 4)

Chapter 1 continued

6. Etiological hypotheses of hypospadias

Because of its frequency in the male population, there have been many investigations into the etiology of hypospadias (Baskin, 2000). It’s now recognized that the etiology of hypospadias comprises hereditary, genetic, endocrinal and environmental factors (Silver, 2000)

6.1. Hereditary factors

The existence of a hereditary causal factor for hypospadias has been demonstrated by certain studies which show the presence of several affected people in the same family. In one relatively old study (Bauer, Bull et Retik 1979; quoted in De Sy & Hoebeke, 1996), the authors concluded that if the father of a family has hypospadias, the probability that one of his sons will carry it is increased to around 8 percent, and the likelihood that one of his brothers will also be affected reaches 12 percent. Moreover, the risk for the next generation increases to around 26 percent if two members of the same family are carriers of hypospadias (e.g., when the father and one of the sons are affected).

According to a more recent study concerning the heredity of hypospadias (Fredell, Kockum, Hansson et al., 2002), almost 7 percent of the families interviewed – in which a child presented one or other form of hypospadias at birth – reported knowing of the existence of another affected family member.

6.2. Genetic factors

The pathogenesis of hypospadias equally comprises factors of a genetic order. Earlier molecular analyses have revealed that there might be, among certain boys presenting an isolated case of hypospadias, mutations of the gene responsible for the enzyme activity of 5-alpha-reductase, leading to disturbance of the production of dihydrotestosterone which is necessary for development of the male genito-urinary tract (Silver & Russel, 1999).

Recent genetic studies carried out on humans (Frisén, 2002), and on animals (Morgan, Nguyen, Scott & Stadler, 2003), have shown that alterations affecting certain genes – such as gene HOXA13 – are susceptible to change the response of the receptor to androgens (located at the level of the genital tubercule) and to lead to the phenotypic expression of hypospadias.

6.3. Endocrinal factors

The presence of hypospadias may also be explained through certain endocrinal factors. Nowadays, it is widely accepted that androgens play a crucial role in the development of the male external genital organs (Hugues, 2001). More precisely, recent anatomical studies support the hypothesis that androgens are essential for the formation of the ventral portion of the human urethra (Liu, Cunha, Russel et al., 2002).

This type of research supports even more strongly the possibility that hypospadias can appear following deficiencies during the biosynthesis of 5-alpha-reductase or via a defect in the androgen receptors.
It is notable also that other hormone deficiencies, such as an excessive production of the anti-Müllerian hormone (AMH) (Austin, Siow, Fallat et al., 2002), may equally play a role in the etiology of hypospadias by interfering with the biosynthesis of testosterone.

6.4. Environmental factors

In recent years a debate has begun in the scientific community about the negative impact of environmental factors on male reproduction in humans (Weber, Pierik, Dohle & Burdof, 2002).

This debate has mostly centered on the hypothesis that certain substances of exogenous origin, known as endocrine disruptors [15], could be responsible for the appearance of disorders of sexual differentation (Toppari, 2002). More precisely, disturbances in masculine sexual differentiation may be induced by two categories of products: the xenoestrogens and the anti-androgens (Sultan, Balaguer, Terouanne et al., 2001).

Endocrine disruptors will affect in particular the normal development of the urethra, but will equally influence the physiology of testicular descent and spermatogenesis in the male (Rittler & Castilla, 2002 ; Toppari, Haavisto and Alanen, 2002).

The list of products incriminated as endocrine disruptors is a long one. According to Sultan et al. (2001), this list comprises diverse synthetic products such as insecticides, pesticides, fungicides, industrial chemical products, substances used in the pharmaceutical industry, detergents and materials used in the fabrication of plastics.

Certain natural substances of vegetable origin but having similar properties to hormones naturally produced in the body (animal and human), known as phyto-estrogens, have also been classified as potential endocrine disruptors (Santti, Makela, Strauss et al., 1998). It is now almost ten years since researchers alerted the scientific community to the possible links between the presence of these substances in our environment and the incidence of demasculinization phenomena [16] in diverse animal species living in the natural environment (Hose & Guillette, 1995).

A number of experiments, some carried out on animals, have drawn attention to how substances with xenoestrogenic or anti-androgenic effects may exert their influence on the development of the male reproductive and genito-urinary system  (Gray, 1998; Gray, Wolf, Lambright et al., 1999; Hayes, Collins, Lee et al., 2002).

The injection of certain potentially anti-androgenic substances have induced hypospadias in rodents, as has finisteride (an inhibitor of 5-alpha-reductase type 2) in rabbits (Kurzrock, Jegatheesan, Cunha et al., 2000), and flutamide (an inhibitor of testosterone fixation on the special receptors) in mice (Kojima, Hayashi, Mizuno et al., 2002).

Note that these experiments using animals do not exactly mirror the situations which could be encountered by humans, but nevertheless they provide valuable information about the stages of normal genito-urinary development, as well as throwing light on the biochemical activities leading to the appearance of hypospadias.

Scientists have recently proposed the hypothesis that endocrine disruptors may be partly responsible for hypospadias in humans (Baskin, Himes, & Colborn, 2001). But only a limited number of studies on this subject exist. Klip, Verloop, van Goel et al. (2002) showed that boys born to mothers who had been exposed to diethylstilbestrol [17] (DES) in utero had a higher risk of hypospadias.

Another study (North & Golding, 2000), suggested that a potentially excessive consumption of phyto-estrogens (via a strictly vegetarian diet in the mother) could disrupt genito-urinary development in the male fetus. Some studies, however, have found no accumulated risks in certain target populations. Vrijheid, Amstrong, Dolk et al. (2003) recently reported that mothers exposed (through their professional occupation) to products classed as endocrine disruptors have no increased risk of giving birth to a child with hypospadias (Vrijheid, Armstrong, Dolk et al., 2003).

Current knowledge notwithstanding, the absence of an exact causal agent for hypospadias in humans continues to pose a problem (Baskin et al., 2001). Even today, the etiology of the majority of observed cases remains, unexplained (Boehmer, Nijman, Lammers et al., 2001 ; Sharpe, 2003).

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Physical and Psychological Effects Of Hypospadias (Chapter 1 part 3)

Chapter 1 continued

4. Anatomical Considerations Of Hypospadias

As already outlined, the term hypospadias refers to the position of the genito-urinary orifice – the urethral meatus – when it opens somewhere along the path of the urethra (Wese, Opsomer, Abi-Aad et al., 1994).

To recap, in a man the urethra is a canal in the form of a tube which runs the whole length of the penis and opens as a hole or slit positioned at the end of the glans. The primary functions of this tubular structure are to carry urine and to allow the passage of semen (Tortora & Grabowski, 1994).

4.1. General anatomical characteristics

Three anatomical characteristics have traditionally been associated with hypospadias (Paparel, Mure, Margarian et al., 2001), as follows:
– an ectopic urethral meatus
– an incomplete prepuce
– chordee

The unusual placement of the urethral meatus is a reliable anatomical characteristic of hypospadias. Hypospadic urethral openings may be located at a variable distance from the extremity of the glans. This ranges from a slightly displaced orifice under the ventral side of the glans to an orifice located in the perineum.

The incomplete prepuce refers to an excess of preputial skin on the dorsal side of the penis which is absent on the ventral side [7]. (This appears as a hooded foreskin covering the dorsal half of the glans only.)

This characteristic regarding the prepuce often accompanies hypospadias but is not always constant [8].
The term chordee refers to the existence of a ventral curve of the penis, especially noticeable during an erection. In general, the extent of chordee is relative to the degree of hypospadias.

In the last few years, some specialists (pediatric urologists) have opted for a more anatomical approach. Thus, as per Paparel et al (2001, p. 741), hypospadias can be defined as a ‘hypoplasy of the tissues forming the ventral side of the penis’. Anatomically, a penis known as hypospadic is characterized by an immaturity of the tissues (hypoplasy) situated on the inferior side of the penis: those forming the urethra, the corpus spongiosum and sometimes even the corpora cavernosae. The most distinct morphological signs of this diminished formative activity in these tissues are: a part of the urethra missing; a division in the corpus spongiosum (where it does not surround the existing portion of urethra); a ventrally incomplete prepuce; an absence of frenulum on the glans; and a chordee.

Sometimes, following an arrest of growth in the tissues forming the corpora cavernosae, under-development of the penis may occur [9] (Paparel et al., 2001).

4.2. Anomalies Associated With Hypospadias

Hypospadias appears most often in an isolated manner (Hencha-Razavi & Escudier, 2000 ; Larsen, 1996). However, it is known that other genital malformations or anomalies, some more serious than others, sometimes accompany this condition.

Alongside hypospadias, cryptorchidism [10] may also be present in the newborn male (Wese et al., 1994). Cryptorchidism may be defined as ‘the uni- or bi-lateral absence of the testicles in the scrotal cavity’ (Garnier & Delamare, 2000). The association of cryptorchidism with hypospadias (observed in around 9% of cases) may be understood in relation to the endocrinal process during testicular descent (passage of the testicles through the abdomen and into the scrotum) which is similar to the embryology of hypospadias (Huston, 2002).

More rarely, the penis may be combined with the scrotum [11] ; or the position of the scrotum may change place with the root of the penis (peno-scrotal transposition) (Avolio, 2002).

Hypospadias is most severe when accompanied by these genital anomalies (Eberle, Überreiter, Radmayr et al., 1993).

5. Diversity and Classification of Hypospadias

There is a diversity of hypospadias. As remarked by Arap et Mitre (2000, p. 304), hypospadias may be observed in a variety of configurations, varying from an ‘ambiguous aspect of the external genital organs’ to ‘a completely formed penis with only a superficial deficiency’.

5.1. Hypospadias or intersexuality ?

The broader issues of sexual ambiguity and intersexuality [12] may arise when the degree of severity of hypospadias is pronounced (e.g., with perineal hypospadias) and where other genital problems (e.g., cryptorchidism; under-developed penis) are added to the picture (Hutcheson, 2002).

In some cases it is necessary to carry out a series of examinations (e.g., palpation of the gonads, magnetic resonance imagery, caryotype) in order to determine the most probable sex of the child (Kaefer, Diamond, Hendren et al., 1999 ; Lapointe, Wei, Hricak et al., 2001 ; McAlleer & Kaplan, 2001).

However, in the vast majority of cases, the presence of hypospadias does not lead to doubt as to the male sex of the child (46XY) or to his acceptance as a boy (American Academy of Pediatrics [AAP], 2000 ; Wilson & Reiner, 1999 ; Zemel & Slover, 2002).

5.2. Named variations of hypospadias

Various classifications of hypospadias have been proposed, defined mostly by the location of the urethral meatus at birth. For example, one such scheme is shown below (Wese et al., 1994):
Names for variations of hypospadias /  Possible locations of the urethral meatus:
1) Glanular  / On the underside of the glans
2) Coronal / At the level of the glanular-preputial ridge [13]
3) Anterior penile  /  On the anterior third of the shaft of the penis
4) Median penile / On the middle third of the shaft of the penis
5) Posterior penile / On the posterior third of the shaft of the penis
6) Peno-scrotal / At the junction of the penis and scrotum
7) Scrotal / At the level of the scrotum
8) Perineal / At the level of the perineum

Over time, several different classifications of hypospadias have been suggested based on the position of the urethral meatus (Sheldon & Duckett, 1987). However, many pediatric urologists have adopted a particular classification developed, some thirty years ago, by a surgeon named Barcat (Barcat, 1973, quoted in Zaontz & Packer, 1997).

This classification, based on (possible) associated chordee, considers that hypospadias can only be classified after a surgical straightening of the penis has taken place [14].

Sheldon & Duckett (1987) contributed to the popularity of such a system, believing that there are 3 principal forms of hypospadias, within which are grouped different degrees of abnormality. These are known as anterior (or distal); median; and posterior (or proximal) (see Figure 6). In terms of frequency, the anterior forms are the most frequent (70% of cases), followed by posterior (20%) and median (10%).

FIGURE 6

Source: Weiner and Hensle (2000).

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Physical and Psychological Effects Of Hypospadias (Chapter 1part 2)

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3. Embryological Origins Of Hypospadias

Hypospadias may be considered as a relatively common malformation of the male genito-urinary organs (Hencha-Razavi & Escudier, 2000).

It is caused by arrested development of the urethra during the period of embryological sexual differentiation (Larsen, 1996).  To better understand this, I shall review some principles of embryonic sexual differentiation [1], the process which leads to development of the genito-urinary tract in humans.

I will pay particular attention to this development in the male. To do so, I will refer to Hugues (2001, p. 3281), for whom the process of sexual differentiation can be defined as ‘phenotypic development of the internal and external genital structures, under the action of hormones which have been produced following the determination of the gonad’.

3.1. Normal sexual determination and differentiation: a recap

The differentiation of the gonad (which at the beginning of the process is the same in both sexes [2]) into a testicle or an ovary is a genetic process known as sexual determination (Hugues, 2001).

The factors responsible for sexual determination are partly chromosomal and partly at the level of the gene (Hencha- Razavi & Escudier, 2000). At the moment of conception the sex of the embryo is determined by the combination of sexual chromosomes: in males, the chromosomal sex is most often 46 XY, in females 46 XX.

In addition, there is a specific gene sequence responsible for testicular development. It appears that the gene SRY [3], carried by the Y chromosome, is the principal initiator of the cascade of genetic interactions which determine the development of the undifferentiated gonad into a testicle [4].

As for the term gonadic sex, this is used to describe the status of the gonads: whether they comprise testicular tissue, or ovarian tissue.

Following the gonad’s determination as a testicle, masculine sexual differentiation is dependent on the production and action of androgens – hormones of gonadic origin (Hugues, 2001).

Until the sixth week, whatever the sex of the embryo, the internal genital passages are represented by two pairs of genital canals: the Wolferian and Müllerian ducts. These ducts take one or other direction according to the hormones produced by the gonads (Hencha-Razavi & Escudier, 2000). 

In male embryos (46 XY), the internal male phenotype is achieved thanks to the secretion and action of two androgens. One is the anti-Müllerian hormone (AMH) which permits a regression of the Müllerian ducts. The other is testosterone, which contributes to the maintenance and development of the Wolferian ducts (epididymis, vas deferens, seminal vesicles and ejaculatory ducts [5].)

The constitution of the external male phenotype sex (external genital organs and genito-urinary sinus) needs the conversion of testosterone into a more powerful hormonal derivative – dihydrotestosterone or DHT – which happens with the help of a specific enzyme, type 2 5-alpha-reductase (Hugues, 2001).

To recap, the external genital structures develop in both sexes (46 XY or 46 XX), from the same original tissues. In the state known as undifferentiated (Hencha-Razavi & Escudier; Larsen, 1996), these tissues are identical and comprise a pair of labio-scrotal folds, a pair of genito-urinary folds and a genital tubercule (see Figure 1).

FIGURE 1 Source : Larsen (1996)

Normally, in an individual of male genetic sex (46 XY), between the 8th and 14th week the genital outlines change under the influence of DHT which fixes itself on special cell receptors (Larsen, 1996). On one side the pads of genital tissue knit together to form the scrotum. On the other, the genital tubercule elongates to form the shaft and glans of the penis.

As the penis grows, the genital folds are drawn forward, then knit together under the penis. During this process of knitting together the genito-urinary membrane forms the urethral canal, which, in fusing, forms a tube the whole length of the penis, comprising the penile urethra.

This process continues forward and draws nearer the glans: this is the glanular urethra. The extremity of the glans, containing the most distal part of the urethra, is formed through an invagination of epithelial cells hollowing out the centre of the glans.

Finally, the urethra opens at the extremity (or apex) of the glans in a vertically oriented slit (see Figure 2). The formation of a complete prepuce (foreskin), with its final cutaneous fold surrounding the glans on its ventro-dorsal part, signals the successful end of this process.

FIGURE 2 Source: Larsen (1996)

In the absence of androgens and DHT, the external sex organs differentiate in the female way (Larsen, 1996). The genital tubercule inclines towards the base to form the clitoris, the genito-urinary folds remain separated to form the small lips (labia minora) of the vulva, and the labio-scrotal folds do not knit together and consequently form the large lips (labia majora).

3.2. Hypospadias: embryogenesis

Different manifestations of hypospadias can appear, depending on the moment when, during the embryonic development of the phenotypic masculine sex, the different fusion processes which form the urethra are disrupted (Larsen, 1996).

According to Frisén (2002), the severity of hypospadias can be seen as a continuum. The earlier the fusion process is interrupted, the hypospadias will be classified as ‘severe’ or ‘complex’. Conversely, ‘simple’ cases of hypospadias will arise from failures during the terminal phases of urethral development.

The degree of hypospadias depends on the location and the length of the urethral orifice (Larsen, 1996). The most severe cases of hypospadias arise when the pads of genital tissue do not fuse at all. The urethra opens in the perineum and the hypospadias is called ‘perineal’ [6].

In the case where the genital pads fuse only partially, and the urethral orifice opens between the base of the penis and the root of the scrotum, the hypospadias is classified as ‘peno-scrotal’ (see Figure 3).

FIGURE 3 Source: Larsen (1996)

An incomplete fusion of the genital folds produces an opening of the urethra at a point along the ventral side of the penis, which is ‘penile’ hypospadias (see Figure 4).

FIGURE 4 Source: Larsen (1996)

Finally, when the epithelial invagination of the glans is defective, the urethral opening is located under the glans, and this is known as ‘glanular’ hypospadias (see Figure 5).

FIGURE 5  Source: Larsen (1996).

The conditions described (Larsen, 1996), represent the four major forms of hypospadias likely to be encountered. However, as we shall see, numerous anatomical varieties of this condition are possible.

Continued here.