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Contents Of This Website
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Physical and Psychological Effects Of Hypospadias
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Chapter 1: General information about hypospadias |
Chapter 2: Hypospadias and surgery
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Chapter 3: Psychological, social and sexual perspectives |
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1. Introduction |
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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.
2. Consequences of hypospadias
Functional problemsMedical 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.
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).3. Objectives and principles of surgery
ves of surgery
-
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 appearanceThe 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.
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.
4. 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).
5. The preferred age for surgery
Changes in the timingInitially, 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.
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).
6. Some technical aspects in 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).
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).
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
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
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.
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).
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). 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).
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).
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.
[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).
[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).