Surgical Challenge in Patients Who Underwent

Original Paper

Urologia

Received: May 10, 2010 Accepted: May 21, 2010 Published online: September 18, 2010

Urol Int 2010;85:427–435 DOI: 10.1159/000319856

Internationalis

Surgical Challenge in Patients Who Underwent Failed Hypospadias Repair: Is It Time to Change? S. Perovic a, † G. Barbagli b R. Djinovic a S. Sansalone c S. Vallasciani b M. Lazzeri d

a

Department of Urology, Clinical Centre Zvezdara, University of Belgrade, Belgrade, Serbia; b Center for Reconstructive Urethral Surgery, Arezzo, c Department of Urology, University Tor Vergata, Rome, and d Department of Urology, Santa Chiara-Firenze, Florence, Italy

Key Words Hypospadias ⴢ Oral mucosa ⴢ Urethroplasty ⴢ Failed repair ⴢ Penile curvature ⴢ Corporoplasty

Abstract Introduction: Our purpose was to evaluate patients who underwent failed hypospadias repair. Patients and Methods: We evaluated 4 different groups of patients who underwent failed hypospadias repair. Group 1: patients who underwent only urethral surgery; group 2: patients who underwent only corpora cavernosa surgery; group 3: patients who underwent urethral and corpora cavernosa surgery; group 4: patients who underwent complex reconstructive surgery. Success was defined as a functional urethra without fistula, with glandular meatus and acceptable esthetic appearance of the genitalia. Results: Out of 1,176 patients, group 1 included 301 patients (25.5%), group two 60 patients (5.2%), group three 166 patients (14.1%) and group four 649 patients (55.2%). The mean follow-up was 60.4 months. Out of 1,176 cases, 1,036 (88.1%) were considered successful and 140 (11.9%) failures. Conclusion: In the majority of patients (55.2%) with failed hypospadias repair, urethral reconstruction is associated with complex surgical procedures to fully resurface glands, penile shaft and genitalia. Copyright © 2010 S. Karger AG, Basel

© 2010 S. Karger AG, Basel 0042–1138/10/0854–0427$26.00/0 Fax +41 61 306 12 34 E-Mail [email protected] www.karger.com

Accessible online at: www.karger.com/uin

In Memoriam Prof. Sava Perovic was a shining example of a scientist and surgeon. He was a master of life who always urged his pupils to do better and to reach excellence, within the parameters he set as a very humane and compassionate person. As a surgeon he was innovative indeed, he was one of the pioneers of reconstructive urological surgery in both adults and children. He developed multiple surgical procedures that are used today all over the world. I had the pleasure and privilege to work with him in Perugia and Belgrade and what I remember most about him was the enormous enthusiasm and passion he put into his work every day and the great energy he emanated that involved every person that was cooperating with him. With his demise we have lost a great surgeon, an innovator, a teacher and a sincere friend. Prof. Massimo Porena

Introduction

Hypospadias is a common congenital anomaly occurring in approximately 1/250 live male births in the US populations and 3/1,000 in European countries [1, 2]. The prevalence of hypospadias has increased since the 1970s with no obvious explanation [1–3]. The current standard of care is to repair hypospadias at the age of 6–12 months to improve penile appearance, allow voiding while standing and improve the chances of fertility M. Lazzeri, MD Department of Urology, Santa Chiara Hospital P.za Indipendenza 11 IT–50129 Florence (Italy) Tel. +39 055 50381, Fax +39 055 480 676, E-Mail lazzeri.m @ tiscali.it

[1–5]. The repair of primary hypospadias may result in postoperative complications involving the urethra, such as meatal or penile stricture, fistula, diverticulum, retrusive meatus and/or the corpora cavernosa, such as residual penile curvature or torsion, associated with loss of the preputial hood, significant scarring and ventral skin deficiency [6]. The main causes of failures are poorly executed procedures or complications occurring during postoperative care, such as infection, wound dehiscence, urine extravasation, hematoma, ischemia or necrosis of transplanted tissues [6–8]. However, hypospadias repair may also fail many years after achieving successful functional and cosmetic results by primary repair and a urethral stricture may develop decades after the initial hypospadias surgery [8]. For many years, failed hypospadias repair was considered a complex chapter in reconstructive urethral surgery, and any book on the urethra includes ‘hypospadias cripple’ or reoperative hypospadias surgery as a special topic [7, 9]. Moreover, pediatric reports on failed hypospadias repair are mainly related to the role of the urethral plate in urethral reconstruction and rarely focus on problems involving the corpora cavernosa or the esthetically poor appearance of the genitalia [10, 11]. Recently, we reported our experience with the largest series (1,176 cases) of patients with failed hypospadias repair published in the literature to date [12]. This widespread survey allowed to us to focus on some concepts and concerns, developing a new view of this old problem. Failed hypospadias repair may consist in defects of single compartments of the male genitalia (urethra, corpora cavernosa, glans, penile and scrotal skin) or in a combination of them. It is a reasonable hypothesis that the outcome of repairing failed hypospadias depends on the number of compartments involved. We investigated failed hypospadias repair, stratifying patients in different groups to evaluate the involvement of the single versus multiple anatomical compartments of male genitalia in the defect and the outcome according to the complexity of reconstruction.

Patients and Methods The study is a retrospective observational analysis of the patient chart of those who were treated for failed hypospadias repair in 2 centers from 1988 to 2007. Once the study was approved by the local Italian and Serbian institutional review boards, all data were entered into a computerized database. The data analysis began in October 2008 and was completed on 30 December 2009.

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The study inclusion criteria were patient age, 11 year to 76 years, urethral stricture and/or penile defects, and previous primary hypospadias repair. the exclusion criteria were precancerous or malignant penile lesions and any condition that in our judgment would interfere with the patient’s ability to provide an informed consent. The patients were stratified into different groups according to the involvement of a single or multiple anatomical compartment(s) of the male genitalia at the time of surgery. Group 1 included patients who underwent only urethral surgery, group 2 those who underwent only corpora cavernosa surgery, group 3 comprised patients who underwent urethral and corpora cavernosa surgery and group 4 patients who underwent complex reconstructive surgery including urethral, corpora cavernosa, glans and penile skin resurfacing. Preoperatively, all patients underwent urine culture, retrograde and voiding cystourethrography and urethroscopy using a pediatric instrument. Patients showing glans or penile curvature or torsion were investigated by photographs of the penis in full erection. Evaluations were scheduled for 3, 6 and 9 months postoperatively, and then annually thereafter. At follow-up, the patients underwent physical examination and uroflowmetry. When symptoms of decreased force of stream were present and uroflowmetry was !12 ml/s, meatal calibration, urethrography, urethral ultrasound and urethroscopy were repeated. Patients presenting with residual curvature were asked to again supply photographs of the penis in full erection. Success was defined as a functional urethra without fistula, stricture or residual chordee and a glandular meatus with a cosmetically acceptable genitalia. The need for meatal or urethral dilation and complications or a poor penile cosmesis requiring revision was considered a failure. Since the study was descriptive and not analytic, no comparison between the groups was made and descriptive statistics were applied.

Results

From 1988 to 2007, 1,176 patients with a mean age of 31 years (range = 1–76) were treated for complications after initial repair of hypospadias, including 953 patients in Serbia and 223 in Italy. Complications involving urethral function (301 cases) were meatal, penile or bulbar stricture, retrusive meatus, fistula and diverticulum (fig. 1a– e). Complications involving corpora cavernosa function (60 cases) were residual penile curvature, corpora cavernosa deformity, penile shortening or torsion (fig. 2a–d). Complications involving both urethra and corpora cavernosa functions (166 cases) were various urethral defects (stricture, fistula, diverticulum) associated with some degree of residual glans/penile curvature (fig. 3a– g). Complications involving the genitalia (649 cases) were glans dehiscence, partial glans necrosis, glans torsion or curvature, loss of penile or scrotal skin, midline septum, penile skin torsion, abnormal penoscrotal or penopubic junction, buried penis, trapped penis and other (fig. 4a–f). Perovic /Barbagli /Djinovic /Sansalone / Vallasciani /Lazzeri

Color version available online

a

b

c

d

e

Fig. 1. a Patient (group 1) with meatal

stricture, fistula and residual distal penile curvature. b The distal urethra is opened along its ventral surface and the penile urethra is fully mobilized from the corpora cavernosa to release the chordee and obtain straightening of the penis. c The original distal urethral plate is augmented using a 1-stage penile onlay skin flap. d Dartos fascial flap is moved to cover the urethra and the glans is closed over the new urethra. e The result 3 months later.

Three hundred and one patients (25%, group 1) underwent surgery to repair urethral defects, 162 patients (53.8%) underwent 1-stage repair and 139 (46.2%) underwent staged repair. Sixty patients (5.2%) underwent surgery to repair corpora cavernosa defects using the Nesbit

technique in 38 cases (63.2%) (fig. 2c, 3c), plication of the corpora in 14 (23.4%) and the grafting procedure in 8 (13.4%) (fig. 4b). One hundred and sixty-six patients (14.1%) underwent combined surgery to repair both urethral and corpora cavernosa defects. In this group, some

Failed Hypospadias Repair

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a

b

c

d

Fig. 2. a Patient (group 2) with residual

distal penile curvature with a normal meatus and urethra. b The glans is fully dissected from the corpora cavernosa to better expose the apex of the corpora. c The curvature is corrected using the Nesbit procedure. d At the end of the procedure, the penis is straight with a normal urethra and meatus.

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c

b

a

e

g

d

Fig. 3. a Patient (group 3) with a urethral fistula. b Intraoperative erection shows residual distal penile curvature. c The curvature is corrected using the Nesbit procedure. d The penis is straight. e The neourethra is tubularized up to the glans. f Dartos fascial flap is moved to cover the urethra. g The result 1 year later.

f


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a

b

c

Fig. 4. a Patient (group 4) with urethral stricture, residual penile curvature and deformity of the genitalia. b The curvature is corrected using a substitute graft material as in complex Peyronie’s disease. c Corpora ca-

vernosa are covered by penile and scrotal skin flaps and the urethral meatus is at the base of the penis.

patients also required minor reconstructive steps to resurface the meatus, glans or penile/scrotal skin, but the surgery mainly involved the urethra and corpora cavernosa. Six hundred and forty-nine patients (55.2%) underwent combined procedures to repair urethral, corpora cavernosa and complex genitalia defects. Out of 1,176 cases, 1,036 (88.1%) were classified as successful and 140 (11.9%) were considered failures. The success rate was 89.7% (group 1), 96.7% (group 2), 88.5% (group 3) and 86.4% (group 4), respectively. The follow-up was 12–237 months (mean = 60.4). All data are summarized in table 1.

Discussion

The reliable incidence of failed hypospadias is still unknown, and it is probable that the data underestimate the true incidence of this problem in the adult population [13, 14]. Treatment of patients with failed hypospadias repair still represents a complex problem because this difficult population of patients has been left with deformities ful432

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ly involving the genitalia that are significantly worse than the simple primary urethral congenital anomaly [6–9]. No other congenital abnormality of the body requires a median of 5 surgical operations to be cured [12]. Hypospadias repair unfortunately deteriorates over time, and our survey clearly shows that reoperative surgery involves, in the majority of patients (55.2%), the urethra, corpora cavernosa, glans, penile shaft and skin, requiring complete resurfacing of the genitalia. In the last decades, the attitudes to sexuality and the psychological and emotional value of the sexual organs have changed. Normal anatomy of the penis has been shown to contribute positively to the patient’s self-esteem, body image, confidence and sexuality. Therefore, it is imperative that we are able to handle the wishes and feelings of the male patients who want to achieve optimal penile function and esthetic appearance of the genitalia after failed hypospadias repair. Surgeons should be aware that a reconstructive approach to failed hypospadias might represent the ideal anatomical pathway to follow in the near future. Penile reconstruction spread over Perovic /Barbagli /Djinovic /Sansalone / Vallasciani /Lazzeri


e

d

f

Fig. 4. d Six months later, the patient underwent the first stage of oral graft urethroplasty. e Six months later, the patient underwent the second stage of urethroplasty. f The result 1 year later.

61 compartments (urethra, corpora cavernosa, glans, penile and scrotal skin) should allow penis anatomy and function to remain as close as possible to the physiological situation, with normal penile appearance, erectile axis and penile length, and reduce the personal and social costs of other surgeries. One of the main goals is to provide all men who underwent failed hypospadias repair with the opportunity to be satisfied with their selfimage of body integrity, even if they should always be advised about the risk of further surgeries and the need for a long-term follow-up. It is now time to change the approach to this problem. As previously reported, our experience showed 2 different populations with failed hypospadias repair [8, 12]. Some patients had satisfactory results from primary hy-

pospadias repair, obtaining a cosmetically acceptable appearance of the genitalia, and the prevalent complication was either meatal or urethral stricture [8, 12]. When these patients required urological evaluation for urethral obstruction, they had no complaints regarding genitalia deformities. The reconstructed urethra had provided a normal urinary stream for many years, but increasing urinary problems and difficulty many years after surgery had developed. Our present study shows that, in these patients, who had undergone urethroplasty, the success rate of reconstructive urethral surgery was 89.7%. On the other hand, some patients showed multiple penile deformities that were significantly worse than the primary congenital anomaly, such as fistula, residual hypo-


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Table 1. Success rate according to type of repair

Group

Type of repair

1 2 3 4

urethroplasty corporoplasty urethroplasty, corporoplasty genitalia resurfacing

Total

Patients 301 (25.5) 60 (5.2) 166 (14.1) 649 (55.2) 1,176

Mean follow-up months

Success rate

Failure rate

58.6 [12–186] 63.2 [12–237] 60 [12–210] 59.8 [12–192]

270 (89.7) 58 (96) 147 (88.5) 561 (86.4)

31 (10.3) 2 (3.3) 19 (11.5) 88 (13.6)

60.4 [12–237]

1,036 (88.1)

140 (11.9)

Figures in parentheses are percentages and values in brackets represent ranges.

spadias, penile curvature and a cosmetically unacceptable appearance of the genitalia [8, 12]. These patients had undergone numerous surgeries to repair urethral and penile defects without a satisfactory outcome and preferred to wait many years before further surgical attempts [8]. Eventually, they would again seek correction of these deformities, believing that improvements in surgery would offer better results than in previous years [8]. Are urologists now able to offer better results than many years ago with regards to the esthetic reconstruction of the corpora cavernosa and genitalia? In the patients who underwent urethral and genitalia resurfacing, the success rate of reconstructive surgery, in our specialized centers, was 79.5%, the lowest success rate in the entire series, showing the complexity of this type of repair. Our present study also shows that failed hypospadias repair is not a problem for the pediatric urologists, as the mean age of our patients was 31 years, or for the urethral surgeon, as the surgery was restricted to the urethra in only 25.5% of the cases in our series. Pediatric urologists underestimate the complexity of reconstruction in patients with failed hypospadias repair, suggesting that this population underwent ‘old-fashioned’ repair compared to the more up-to-date surgical techniques currently used, which provide preservation of the urethral plate and either tubulization or augmentation [10]. The role of the urethral plate is also emphasized in redo-repair, suggesting an algorithm for the approach to failed hypospadias repair only based on presence or absence of a supple urethral plate, without any reference to the necessity of genitalia reconstruction [11]. It is probable that pediatric and adult urologists care for different populations of patients with failed hypospadias repair. Clearly, so as to improve the anatomical and functional outcome, it is essential to found centers specialized in treatment of these patients. Only full collaboration between the urethral surgeon and the surgeon widely skilled 434

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in reconstructive surgery of the corpora cavernosa (penile prosthesis implantation, surgery for Peyronie’s disease, surgery for male to female transition) can ensure the best cosmetic and functional outcome. Patients presenting with failed hypospadias repair should avail themselves of centers where surgeons have matured great experience in plastic and reconstructive surgery of the male genitalia. In our series, 13.4% patients required complex corpora cavernosa grafting procedures to repair residual penile curvature, such as in patients with severe deformity due to Peyronie’s disease. Should patients with complex failed hypospadias repair not be referred to a center of expertise? Medically and ethically speaking, it is the right thing to do [15]. Our study also confirms that 2-stage hypospadias repair is a misnomer because this technique rarely requires only 2 stages, with the majority of cases requiring at least 1 additional procedure to obtain satisfactory results [8, 12]. This means high personal and social costs for the treatment of this population of patients. Our study presents several weaknesses. The patient’s perception of the disease and its treatment is unknown and we failed to gain information on patient quality of life. In the future, it is mandatory to develop a specific patient-reported outcome instrument for those who have undergone failed hypospadias repair surgery, on the basis of a clearly defined conceptual framework which indicates the importance of the patient’s perspective and/or expectations [12].

Conclusion

The surgical repair of complications in patients who underwent primary hypospadias repair still represents a nonnegligible, difficult issue. For many years, surgical rePerovic /Barbagli /Djinovic /Sansalone / Vallasciani /Lazzeri

pair of failed hypospadias was akin to surrendering into the hands of urethral surgeons. Urethral complications in patients with failed hypospadias repair are less frequent than complications involving the corpora cavernosa and the esthetic appearance of the genitalia. To re-

pair these defects, collaboration between the urethral surgeon and the surgeon who has developed vast experience in plastic and reconstructive surgery of the male genitalia is indeed mandatory.

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