- Email: [email protected]
Long-term outcomes in primary male epispadias
Journal Pre-proof Long term outcomes in Primary Male Epispadias Thomas JS, Shenoy M, Mushtaq I, Wood D PII:
S1477-5131(19)30370-5
DOI:
https://doi.org/10.1016/j.jpurol.2019.10.027
Reference:
JPUROL 3311
To appear in:
Journal of Pediatric Urology
Received Date: 18 April 2019 Accepted Date: 28 October 2019
Please cite this article as: JS T, M S, I M, D W, Long term outcomes in Primary Male Epispadias, Journal of Pediatric Urology, https://doi.org/10.1016/j.jpurol.2019.10.027. This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. © 2019 Published by Elsevier Ltd on behalf of Journal of Pediatric Urology Company.
Long term outcomes in Primary Male Epispadias Thomas JS1, Shenoy M2, Mushtaq I3, Wood D4 1.
Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK, University College London Hospital NHS Foundation Trust, London, UK
2. 3. 4.
Nottingham Children’s Hospital, Nottingham, UK Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK University College London Hospital NHS Foundation Trust, London, UK
1.
[email protected]
2.
[email protected]
3.
[email protected]
4.
[email protected] Corresponding Author: Miss Johanna S Thomas Present Address: Urology Department, Royal Berkshire Hospital NHS Foundation Trust, Reading, RG1 5AN, UK [email protected] Telephone: +44 7739 137312
Extended Summary Purpose We present a case series of 30 male patients who presented with primary epispadias between 1989 and 2002 and looked at their long term outcomes. Objectives 1. What procedures and operations did these patients require following their original surgery? 2. What were their outcomes as adults in terms of continence, cosmesis and sexual function? Materials and Methods Primary male epispadias patients who had gone through transition into Adolescent and Adult Urology services were identified retrospectively from electronic patient records. Results We identified 30 male patients with a median follow up of 18.5 years. 24 had penopubic epispadias, and 6 had penile epispadias. All initial surgery took place between 9- 48 months. 28 patients needed further surgery over the follow up period, 26 had surgery for continence and 12 required revision surgery. At follow up 15 patients were continent voiding per urethra, 9 patients reported stress leakage with volitional urethral voiding, 6 patients were using a Mitrofanoff to void, and 4 of these had an ileocystoplasty. 19 patients had documentation on their feelings towards cosmetic outcome; 17 expressed concern. 21 patients had documentation about sexual function; 20 had normal erections
1
with 6 reporting chordee and 9 reporting retrograde ejaculation. No patients were recorded to have fathered any children. Discussion There are only a few published studies looking at long-term outcomes of genito-urinary reconstruction in primary male epsipadias and their sexual function in adulthood. The majority of this patient cohort required surgery to improve their continence and had more than one continence procedure. There is limited data on continence outcomes in the literature with small cohorts and rates varying between 40 to 100% at 10 year follow up. Despite corrective surgery, nearly all the patients were concerned about their genital appearance. Other studies have shown similar outcomes in terms of patient satisfaction and sexual function. The limitations of this work are that the information was gathered retrospectively from the electronic patient record and validated instruments on outcomes were not used. Conclusions Our outcomes will be useful for clinicians who are counselling patients and parents regarding what to expect as adults. Type of Epispadias
Total Number
Number requiring continence surgery (%)
Number of Continent patients voiding through native urethra (%)
Penopubic
24
21 (86)
10 (40)
Midshaft
3
2 (66)
2 (67)
Distal
3
1 (33)
3 (100)
2
Keywords: Male Epispadias, primary epispadias, Cantwell-Ransley repair, follow up study,
3
Introduction Isolated Male Epispadias is a rare anomaly with a reported incidence of 1 in 117,000 of live male births (1). It is characterised by the failure of the urethral plate to tubularise on the dorsum of the penis and is included within the spectrum of bladder exstrophy epispadias complex. The severity is classified according to the position of the urethral opening - from penopubic to penile and glanular. The former two are associated with varying degrees of incontinence. All types of epispadias demonstrate dorsal chordee. The aims of surgical management of epispadias are twofold: (i) to give patients a straight, cosmetically acceptable and functional penis of adequate length enabling penetrative sexual intercourse, and (ii) normal urinary control with spontaneous urethral voiding. A number of primary surgical approaches have been described to correct the penile component of epispadias including the Modified Cantwell-Ransley (MCR) technique and the Mitchell-Bagli technique (2). The Modified Cantwell-Ransley technique evolved based on the studies of Woodhouse and Kellett (3) together with the original Cantwell repair (4). This approach involves 3 components: (i) corporeal rotation dorsally with carvernocavernostomy, (ii) urethral mobilisation and reconstruction that maintains an intact urethral plate and (iii) improved glanuloplasty and improved skin coverage (5) (6). The Mitchell- Bagli technique involves complete penile disassembly into three separate components. Subsequent surgery to improve continence in these children, if needed, includes injection of bulking agents around the sphincter, bladder neck reconstruction, or the Kelly procedure. Some children will need a Mitrofanoff with or without a bladder augmentation to eliminate all incontinence.
4
This two stage approach was used at our institution until 2000. MCR repair for the epispadias was carried out before the age of two. Further surgery was carried out for continence if required based on symptoms, cystoscopic findings and urodynamics. After the year 2000, approaches to the surgical management changed with the adoption of the Kelly procedure. All children who now present with epispadias undergo the Kelly procedure and a small number have a MCR repair at the age of 12-18 months. There is a limited literature examining the long-term outcomes of male epispadias surgery. The majority of published work focuses on cosmetic appearance and urethral function or the post-operative anatomical and functional complications (6) (7) (8) (9). This study investigated the long term outcomes for 30 male infants with primary male epispadias who underwent a two-staged approach. The data have examined outcomes for cosmetic appearance, continence rates, and sexual function.
Materials and Methods A retrospective review was conducted examining the medical records for 30 male patients with primary epispadias who were referred to a single institution between 1989 and 2002. All patients were extracted from a database kept at the institution. Inclusion criteria were male gender patients with distal, midshaft or penopubic epispadias who underwent an epispadias repair. Exclusion criteria included male patients with glanular epispadias and bladder exstrophy patients. All of these patients had already transitioned to the adolescent and adult Urology services at 16 to 18 years of age. Data including age at presentation, type of epispadias, age at primary surgery, further surgery (revision or continence procedures), number of procedures, continence 5
outcomes (with means of bladder emptying) and sexual function were gathered. Continence was defined as not needing to wear any protection (eg. incontinence pad for urine leakage) with patient reporting to be dry between voids or catheterisations. Mild stress incontinence was classified as using 1 pad for protection in a 24 hour period. Major stress incontinence was defined as the need to use more than this. Reports given by the patients themselves or by their caregivers were recorded in the medical records, as recorded by medical doctors and nurses. No validated instruments were used for the assessment of these patients. Documentation was solely based on what the patient reported to the doctor or nurse at the time of the consultation.
Results All 30 patients had documentation on primary epispadias reconstruction and associated continence surgery. Information about sexual function was documented in 21 patients (70%). All patients were born between 1998 and 2001, and follow up ranged from 15-27 years (median 18.5 years). 21 patients had sub-symphyseal epispadias, and 3 had proximal penile epispadias- these were defined as penopubic epispadias (80%). Of the remaining patients 3 (10%) patients had midshaft epispadias and 3 (10%) had distal epispadias. 22 (73%) of the patients had a MCR repair, 3 had a Tumble-Type repair, 1 had a reversed Duckett repair and 4 had primary surgery at another centre and details were not available. Primary surgery took place between the ages of 9 months and 48 months. All patients who had their initial surgery at our institution were administered 3 months preoperative testosterone (Sustanon250).
6
28 (93%) patients required additional surgery following their primary epispadias repair. The remaining 2 patients required investigation by means of cystoscopy: 1 had a penopubic penile defect and 1 a distal defect. Both patients had undergone a MCR repair before 48 months. Neourethroplasty and Final appearance 12 patients (40%) required some form of revision surgery within the follow up period. Reasons for surgery included skin revision (17%), fistulae (20%), abscess formation (3%) and an excision of urethral diverticulum (3%). Of those patients who had a MCR repair only 6% developed a fistula. In 19 patients we identified documentation in relation to the patients’ opinion on the cosmetic outcome of their epispadias surgery. 17/19 (89%) expressed concern about the physical appearance of their genitalia. One patient had undergone a radial forearm flap phalloplasty at the age of 23 and 1 further patient was documented to be considering radial forearm flap phalloplasty surgery. 1 patient had corporeal plication for persistent dorsal chordee as an adult and 1 patient underwent division of the penile suspensory ligament to improve the cosmetic appearance of the penis.
Bladder dynamics and Continence Outcomes Patients underwent urodynamic and cystoscopic evaluation of bladder capacity and function. Complete data of urodynamic assessments were not available so these were not included in the results. 26 (86%) patients had surgery to improve continence within the follow up period. Continence surgery included injection of bulking agents (Macroplastique™) at the
7
posterior urethra, bladder neck reconstruction (Young-Dees-Leadbetter), bladder neck sling, Kelly procedure and a combination of above. The mean number of procedures per patient (excluding cystoscopy) during childhood and adolescence was 3.4 (range 0-8) – see Table 1. 11 (37%) patients had Macroplastique™ only. 4 (36%) of these complained of some stress leakage persisting after puberty. 3 (27%) complained of minor stress leakage. 1 (9%) had major stress leakage. Further continence surgery in the form of bladder augmentation and Mitrofanoff was documented as being discussed with him before he was lost to follow up. 15 (50%) patients were reported to be dry and voiding though their native urethra. 1(3%) had had a bladder neck repair and needed to double void. 9 (30%) patients were voiding through their urethra but reported some form of stress leakage. Volitional transurethral voiding was recorded in all patients who were voiding through their urethra (80%). 6 (20%) patients were using clean intermittent catheterisation through a Mitrofanoff channel of which four patients also had undergone a bladder augmentation surgery. Age at augmentation ranged from 7-15 years (median 11.5). See Table 2 and Diagram 1. Other reported complications included bladder stones in 2 patients following bladder neck reconstruction and a further bladder stone in a patient with a closed bladder neck and bladder augmentation. We were not able to obtain information on upper tract status or urinary tract infections. Sexual Function 21 patients had documentation about sexual function; 20 (95%) were documented as able to achieve full erections. The one patient with poor erections had a forearm flap
8
phalloplasty. 6 (29%) patients had reported persistent dorsal chordee as an adult and 9 (43%) reported retrograde ejaculation. Of the 4 patients who had a Kelly procedure, 3 reported normal erections. 1 had poor erections and was the patient who had a radial forearm flap phalloplasty as an adult; it was not clearly documented whether the erectile dysfunction was present before or after the Kelly procedure. No patients were recorded to have fathered any children; however two had sought fertility advice.
Discussion There are only a few published studies looking at long-term outcomes of genito-urinary reconstruction in primary male epsipadias and their sexual function in adulthood (7) (8) (9) (10)
. Complications of these reconstructive techniques can take decades to develop and
pubertal growth can alter the final functional and cosmetic aspects of the corrected genitalia (11). It is therefore vital that long-term outcomes are recorded. These data in this historical series show both favourable and unfavourable long-term outcomes for male patients with primary epispadias comparable to previously published data which is limited. Primary male epispadias is a rare condition which is best managed in specialist paediatric urology centres. To date there is no uniform agreement on the best surgical management and thus each centre performs surgery in line with their expertise and experience. Currently two major reconstruction principles are used in specialist paediatric urology centres, the MCR repair and the Mitchell-Bagli complete disassembly technique (2) (12) (13). In 1989 Ransley and co-workers described a modification to the to the Cantwell repair that resulted in fewer complications and better cosmesis (14). Reports on this technique
9
tend to focus on the cosmetic appearance of the penis and urethral function or the postoperative anatomical and functional complications (6) (15) . Lottman and colleagues reported complications that required further surgical reconstruction in 45% of patients who underwent a MCR (n=40). Just under half of these patients had primary epispadias. Follow up was for a mean of 3 years. 50% of patients reported a good cosmetic outcome (7) . Mollard et al published long term follow up data in 29 patients. Those patients who had a MCR repair were considered to have a more cosmetically acceptable penis (8). Fistula rates have been reported between 0% to 28% in a number of studies with follow up varying between 5 to 8 years (9) (15) (14). Only 2 patients (6%) in our series who had a MCR repair developed a fistula. The remaining patients who developed a fistula had either had an alternative surgical procedure or the information on the primary surgery was not available. The overall fistula rate was therefore 20%. In those patients considered to have had successful corrective surgery, nearly all of them were concerned about their genital appearance as an adolescent and adult with 26% concerned about persistent dorsal curvature. Other studies have shown similar outcomes in terms of patient satisfaction once they reach adulthood (10) (11). Psychosexual counselling is an integral part of the management of epispadias and this was available to this cohort of patients. This shows that despite the best efforts and intentions of surgeons, surgical techniques alone will not enable the patients to cope with structural and aesthetic challenges of epispadias. 4 (13%) patients went on to have corrective surgery for cosmetic or functional reasons as adult. One patient who went on to have the radial forearm flap phalloplasty had undergone multiple surgeries with a number of post-operative complications
10
Continence In this series 80% had penopubic epispadias and the remainder had a penile defect. It has been thought in the past that occurrence of incontinence is related to the degree of epispadias. However Kramer and Kelalis published a paper of their experience with 82 male patients with epispadias. 49 were penopubic (sub-symsyphyseal), 21 were penile and 12 were glanular. 46/49 penopubic epispadic patients and 15/21 penile epispadic patients were also incontinent (16). In fact in our cohort of patients all but 4 patients required some form of surgery for incontinence. Out of these 4 patients, 3 had penopubic epispadias, and 1 had distal epispadias. It has been shown that the most frequently identified urodynamic pattern before surgical correction of the bladder neck in patients with isolated epispadias is a low capacity, highly compliant bladder with minimal detrusor dysfunction (17) (18). Mouriquand et al looked at 17 male patients with primary epispadias who had undergone Young-Dees-Leadbetter bladder neck reconstruction. Continence was satisfactory in 41% at mean follow up of 10 years (19). From the long-term follow up published by Mollard et al 14 out of 40 patients were classified as having incontinent epispadias. These patients had a bladder neck reconstruction before penile reconstruction. 84% of patients were continent at follow up (8). Canon et al looked at 6 patients with primary male epispadias with a distal defect all with urinary incontinence. 5 patients had a bladder neck repair and all were continent at mean follow up of 9.8 years (20) . A recent study by Cendron et al looked at the anatomical findings associated with epispadias. This showed a significant number of both penile and penopubic epispadias patients had an abnormal bladder neck and continence rates were similar between both groups
(21)
.
11
Duffy and Ransley looked at early outcomes of endoscopic treatment of urinary incontinence in children with primary epispadias (22). In our cohort 19 (63%) of patients had Macroplastique™ as an initial procedure. The majority of these required repeated injections. 8 patients had a further continence procedure following Marcroplastique™ injection. Median number of total Marcroplastique™ injections for all patients was 3 per patient (range 1-5).Of those patients who had Macroplastique™ injection only, 36% had persistent mild stress urinary incontinence at follow up.
Sexual Function Where documentation was available on sexual function, 95% of patients were reported to achieve a full erection. 26% of these patients described persistent dorsal chordee and 43% had retrograde ejaculation. Reddy et al showed 67% of their patient cohort described some problem with sexual function despite overall satisfaction and half reported abnormal ejaculation (10). Of the 6 epispadias patients in a Finnish study, the majority of these were satisfied with their sexual function (23). Mollard et al reported sexual outcomes in 29 patients (mean 27 years follow up). 80% reported regular sexual intercourse, 55% had normal ejaculation and four patients had fathered children (8). We were able to identify that two patients had sought fertility advice but could not find any record of paternity. Previous studies have shown fertility rates to be between 1766% (10) (11). Limitations of this study are that it is a retrospective study of patient records. Data are therefore reliant on the detail of the information obtained and recorded by the clinician, and no validated instruments were used in the assessment of LUTS, sexual function and patients’ opinion on cosmetic outcomes. 9 patients had no reported data on sexual
12
outcomes; however they were kept in the study in order for those conducting to study to understand their continence outcomes.
Conclusions Published long-term continence and sexual function outcomes for men with epispadias are sparse. This study shows the majority of patients with epispadias have a good chance of achieving continence with volitional urethral voiding. More than 80% will need additional procedures to achieve socially acceptable continence. This study also shows high rates of erectile and ejaculatory function; however, dissatisfaction with genital appearance is significant. Our findings are consistent with previous reports and can be used to counsel patients and their parents on what they can expect as they progress through adolescence into adulthood.
Conflict of Interest None to declare Funding None References 1. PDE, Mouriquand. Congenital disorders of the bladder and urethra In: Textbook of Genitourinary Surgery 2nd Edition. 1998. 2. Management of Epispadias. Grady RW, Mitchell ME. s.l. : Urology Clinics of North America, 2002, Vol. 29. 349-360. 3. Anatomy of the penis and its deformities in exstrophy and epispadias. Woodhouse CRJ, Kellett MJ. s.l. : J Urol, 1984, Vol. 132. 1112. 13
4. Operative treatment of epispadias by transplantation of the urethra. FV, Cantwell. s.l. : Ann Surg, 1895, Vol. 22. 5. Male Epispadias. Diamond DA, Ransley PG. s.l. : J Urol, 1995, Vol. 154. 6. The evolution of Penile reconstruction in Epispadias repair: A report of 180 cases. Kajbafzadeh AM, Duffy PG, Ransley PG. s.l. : J Urol, 1995, Vol. 154. 858-861. 7. Male Epispadias repair: Surgical adn fucntional results with the Cantwell-Ransley proceudre in 40 patients. Lottman HB, Yaqouti M, Melin Y. s.l. : J Urol, 1999, Vol. 162. 1176-1180. 8. Male Epispadias: Experience with 45 Cases. Mollard P, Basset T, Mure PY. s.l. : J Urol, 1998, Vol. 160. 55-59. 9. The Modified Cantwell-Ransley repair for Exstrophy and Epispadias: A 10 year experience. Surer I, Baker LA, Jeffs RD, Gearhart JP. 1040-1043, s.l. : J Urol, 2000, Vol. 164. 10. Sexual Health Outcomes in Adults with Complete Male Epispadias. Reddy SS, Inouye BM, Anele UA, Adelwahab M, Le B, Gearhart JP, Rao PK. s.l. : J Urol, 2015, Vol. 194. 1091-1095. 11. Long-term outcome of male genital reconstruction in childhood. Tourchi A, Hoebeke P. s.l. : J Paed Urol, 2012, Vol. 9. 980-989. 12. Diagnosis and management of epispadias. D, Frimberger. s.l. : Seminars in Paediatric Surgery, 2011, Vol. 20. 85-90. 13. The Surgical Management of Male Epispadias in the New Millenium. Cho P, Cendron M. s.l. : Curr Urol Rep, 2014, Vol. 15. 472. 14. Applications of the modified Cantwell-Ransley epispadias repair in the exstrophy-epispaidias complex. Baird AD, Gearhart JP, Mathews RI. s.l. : J Paed Urol, 2005, Vol. 1. 331-336. 15. Results of Epispadias Repair Using the Modified Cantwell-Ransley technique. Bar-Yosef Y, Sofer M, Ekstein MP, Binyamini Y, Ben-Chaim J. s.l. : Urology, 2016, Vol. 99. 221-224. 16. Assessment of urinary continence in epispadias. Review of 94 patients. Kraemer SA, Kelalis PP. s.l. : J Urol, 1982, J Urol, Vol. 128, pp. 2636-42. 290-293. 17. Urodynamic findings in children with isolated epispadias. Kaefer M, Andler R, Bauer SB, Hendren WH, Diamond D, Retik AB. s.l. : J Urol, 1999, Vol. 162. 1172-1175. 18. Bladder function and dysfunction in exstrophy and epispadias. Hollowell JG, Hill PD, Duffy PG, Ransley PG. s.l. : Lancet, 1991, Vol. 338. 926. 19. Long-term results of bladder neck reconstruction for incontinence in children with classical bladder exstophy or incontinent epispadias. Mouriquand PDE, Bubanj T, Feyaerts A, Jandric M, Timsit M, Mollard P, Mure PY, Basset T. 92, s.l. : BJUI, 2003. 997-1002. 20. Pathophysiology and management of urinary incontinence in case of distal penile epispadias. Canon S, Robert R, Koff SA. 180, s.l. : J Urol, Vol. 2008. 2636-42.
14
21. Anatomic findings associated with epispadias in boys: Implications for surgical management and urinary continence. Cendron M, Cho PS, Pennison M, Rosoklija I, Diamond DA, Borer JG. 2018, Vol. 14(1). 42-46. 22. Endoscopic treatment of urinary incontinence in children with primary epispadias. Duffy PG, Ransley PG. s.l. : British Journal of Urology, 1998, Vol. 1998. 309-311. 23. Sexual Function in Patients Operated on for Bladder Exstrophy and Epispadias. Suominen JS, Santilla P, Taskinen S. s.l. : J Urol, 2015, Vol. 194. 195-199.
15
Table 2: Continence Outcomes for Patients according to Type of epispadias Type of Epispadias
Total Number
Number requiring continence surgery (%)
Number of Continent patients voiding through native urethra (%)
Number of patients with persistent Stress Incontinence (%)
Number of patients voiding via Mitrofanoff (%)
Penopubic
24
21 (86)
10 (40)
9 (38)
5 (21)
Midshaft
3
2 (66)
2 (67)
0
1 (33)
Distal
3
1 (33)
3 (100)
0
0
Table 1: Continence Procedures and Continence outcomes Continence Procedure
Total number
Number of Continent patients voiding through native urethra
Number of patients with persistent mild Incontinence
Number of patients voiding via Mitrofanoff
Macroplastique™ only
11
7
4
0
Bladder neck reconstruction (BNR) only
3
2
0
1
Bladder neck sling only
1
0
1
0
1. Macroplastique™ BNR 1. BNR 2. Macroplastique™ 1. Macroplastique™ 2. Kelly 1. BNR 2. Kelly procedure Kelly procedure
6
1
2
3
1
0
1
0
2
2
0
0
1
0
0
1
1
0
0
1
No Continence procedure
4
3
1
0
Diagram 1 : Flow diagram on continence outcomes CIC = Clean intermittent Catheterisation
30 Patients 24 Penopubic (PP) 3 Midshaft (M) 3 Distal (D)
15 (50%) Dry and voiding through native urethra 10 PP 2M 2D
8 (27%) Minor stress leakage – urethral voiding
1 (3%) Major Stress leakage – urethral voiding
8 PP
1 PP
6 (20%) CIC through Mitrofanoff 5 PP 1 MS
4 Ileocystoplasty
S1477-5131(19)30370-5
DOI:
https://doi.org/10.1016/j.jpurol.2019.10.027
Reference:
JPUROL 3311
To appear in:
Journal of Pediatric Urology
Received Date: 18 April 2019 Accepted Date: 28 October 2019
Please cite this article as: JS T, M S, I M, D W, Long term outcomes in Primary Male Epispadias, Journal of Pediatric Urology, https://doi.org/10.1016/j.jpurol.2019.10.027. This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. © 2019 Published by Elsevier Ltd on behalf of Journal of Pediatric Urology Company.
Long term outcomes in Primary Male Epispadias Thomas JS1, Shenoy M2, Mushtaq I3, Wood D4 1.
Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK, University College London Hospital NHS Foundation Trust, London, UK
2. 3. 4.
Nottingham Children’s Hospital, Nottingham, UK Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK University College London Hospital NHS Foundation Trust, London, UK
1.
[email protected]
2.
[email protected]
3.
[email protected]
4.
[email protected] Corresponding Author: Miss Johanna S Thomas Present Address: Urology Department, Royal Berkshire Hospital NHS Foundation Trust, Reading, RG1 5AN, UK [email protected] Telephone: +44 7739 137312
Extended Summary Purpose We present a case series of 30 male patients who presented with primary epispadias between 1989 and 2002 and looked at their long term outcomes. Objectives 1. What procedures and operations did these patients require following their original surgery? 2. What were their outcomes as adults in terms of continence, cosmesis and sexual function? Materials and Methods Primary male epispadias patients who had gone through transition into Adolescent and Adult Urology services were identified retrospectively from electronic patient records. Results We identified 30 male patients with a median follow up of 18.5 years. 24 had penopubic epispadias, and 6 had penile epispadias. All initial surgery took place between 9- 48 months. 28 patients needed further surgery over the follow up period, 26 had surgery for continence and 12 required revision surgery. At follow up 15 patients were continent voiding per urethra, 9 patients reported stress leakage with volitional urethral voiding, 6 patients were using a Mitrofanoff to void, and 4 of these had an ileocystoplasty. 19 patients had documentation on their feelings towards cosmetic outcome; 17 expressed concern. 21 patients had documentation about sexual function; 20 had normal erections
1
with 6 reporting chordee and 9 reporting retrograde ejaculation. No patients were recorded to have fathered any children. Discussion There are only a few published studies looking at long-term outcomes of genito-urinary reconstruction in primary male epsipadias and their sexual function in adulthood. The majority of this patient cohort required surgery to improve their continence and had more than one continence procedure. There is limited data on continence outcomes in the literature with small cohorts and rates varying between 40 to 100% at 10 year follow up. Despite corrective surgery, nearly all the patients were concerned about their genital appearance. Other studies have shown similar outcomes in terms of patient satisfaction and sexual function. The limitations of this work are that the information was gathered retrospectively from the electronic patient record and validated instruments on outcomes were not used. Conclusions Our outcomes will be useful for clinicians who are counselling patients and parents regarding what to expect as adults. Type of Epispadias
Total Number
Number requiring continence surgery (%)
Number of Continent patients voiding through native urethra (%)
Penopubic
24
21 (86)
10 (40)
Midshaft
3
2 (66)
2 (67)
Distal
3
1 (33)
3 (100)
2
Keywords: Male Epispadias, primary epispadias, Cantwell-Ransley repair, follow up study,
3
Introduction Isolated Male Epispadias is a rare anomaly with a reported incidence of 1 in 117,000 of live male births (1). It is characterised by the failure of the urethral plate to tubularise on the dorsum of the penis and is included within the spectrum of bladder exstrophy epispadias complex. The severity is classified according to the position of the urethral opening - from penopubic to penile and glanular. The former two are associated with varying degrees of incontinence. All types of epispadias demonstrate dorsal chordee. The aims of surgical management of epispadias are twofold: (i) to give patients a straight, cosmetically acceptable and functional penis of adequate length enabling penetrative sexual intercourse, and (ii) normal urinary control with spontaneous urethral voiding. A number of primary surgical approaches have been described to correct the penile component of epispadias including the Modified Cantwell-Ransley (MCR) technique and the Mitchell-Bagli technique (2). The Modified Cantwell-Ransley technique evolved based on the studies of Woodhouse and Kellett (3) together with the original Cantwell repair (4). This approach involves 3 components: (i) corporeal rotation dorsally with carvernocavernostomy, (ii) urethral mobilisation and reconstruction that maintains an intact urethral plate and (iii) improved glanuloplasty and improved skin coverage (5) (6). The Mitchell- Bagli technique involves complete penile disassembly into three separate components. Subsequent surgery to improve continence in these children, if needed, includes injection of bulking agents around the sphincter, bladder neck reconstruction, or the Kelly procedure. Some children will need a Mitrofanoff with or without a bladder augmentation to eliminate all incontinence.
4
This two stage approach was used at our institution until 2000. MCR repair for the epispadias was carried out before the age of two. Further surgery was carried out for continence if required based on symptoms, cystoscopic findings and urodynamics. After the year 2000, approaches to the surgical management changed with the adoption of the Kelly procedure. All children who now present with epispadias undergo the Kelly procedure and a small number have a MCR repair at the age of 12-18 months. There is a limited literature examining the long-term outcomes of male epispadias surgery. The majority of published work focuses on cosmetic appearance and urethral function or the post-operative anatomical and functional complications (6) (7) (8) (9). This study investigated the long term outcomes for 30 male infants with primary male epispadias who underwent a two-staged approach. The data have examined outcomes for cosmetic appearance, continence rates, and sexual function.
Materials and Methods A retrospective review was conducted examining the medical records for 30 male patients with primary epispadias who were referred to a single institution between 1989 and 2002. All patients were extracted from a database kept at the institution. Inclusion criteria were male gender patients with distal, midshaft or penopubic epispadias who underwent an epispadias repair. Exclusion criteria included male patients with glanular epispadias and bladder exstrophy patients. All of these patients had already transitioned to the adolescent and adult Urology services at 16 to 18 years of age. Data including age at presentation, type of epispadias, age at primary surgery, further surgery (revision or continence procedures), number of procedures, continence 5
outcomes (with means of bladder emptying) and sexual function were gathered. Continence was defined as not needing to wear any protection (eg. incontinence pad for urine leakage) with patient reporting to be dry between voids or catheterisations. Mild stress incontinence was classified as using 1 pad for protection in a 24 hour period. Major stress incontinence was defined as the need to use more than this. Reports given by the patients themselves or by their caregivers were recorded in the medical records, as recorded by medical doctors and nurses. No validated instruments were used for the assessment of these patients. Documentation was solely based on what the patient reported to the doctor or nurse at the time of the consultation.
Results All 30 patients had documentation on primary epispadias reconstruction and associated continence surgery. Information about sexual function was documented in 21 patients (70%). All patients were born between 1998 and 2001, and follow up ranged from 15-27 years (median 18.5 years). 21 patients had sub-symphyseal epispadias, and 3 had proximal penile epispadias- these were defined as penopubic epispadias (80%). Of the remaining patients 3 (10%) patients had midshaft epispadias and 3 (10%) had distal epispadias. 22 (73%) of the patients had a MCR repair, 3 had a Tumble-Type repair, 1 had a reversed Duckett repair and 4 had primary surgery at another centre and details were not available. Primary surgery took place between the ages of 9 months and 48 months. All patients who had their initial surgery at our institution were administered 3 months preoperative testosterone (Sustanon250).
6
28 (93%) patients required additional surgery following their primary epispadias repair. The remaining 2 patients required investigation by means of cystoscopy: 1 had a penopubic penile defect and 1 a distal defect. Both patients had undergone a MCR repair before 48 months. Neourethroplasty and Final appearance 12 patients (40%) required some form of revision surgery within the follow up period. Reasons for surgery included skin revision (17%), fistulae (20%), abscess formation (3%) and an excision of urethral diverticulum (3%). Of those patients who had a MCR repair only 6% developed a fistula. In 19 patients we identified documentation in relation to the patients’ opinion on the cosmetic outcome of their epispadias surgery. 17/19 (89%) expressed concern about the physical appearance of their genitalia. One patient had undergone a radial forearm flap phalloplasty at the age of 23 and 1 further patient was documented to be considering radial forearm flap phalloplasty surgery. 1 patient had corporeal plication for persistent dorsal chordee as an adult and 1 patient underwent division of the penile suspensory ligament to improve the cosmetic appearance of the penis.
Bladder dynamics and Continence Outcomes Patients underwent urodynamic and cystoscopic evaluation of bladder capacity and function. Complete data of urodynamic assessments were not available so these were not included in the results. 26 (86%) patients had surgery to improve continence within the follow up period. Continence surgery included injection of bulking agents (Macroplastique™) at the
7
posterior urethra, bladder neck reconstruction (Young-Dees-Leadbetter), bladder neck sling, Kelly procedure and a combination of above. The mean number of procedures per patient (excluding cystoscopy) during childhood and adolescence was 3.4 (range 0-8) – see Table 1. 11 (37%) patients had Macroplastique™ only. 4 (36%) of these complained of some stress leakage persisting after puberty. 3 (27%) complained of minor stress leakage. 1 (9%) had major stress leakage. Further continence surgery in the form of bladder augmentation and Mitrofanoff was documented as being discussed with him before he was lost to follow up. 15 (50%) patients were reported to be dry and voiding though their native urethra. 1(3%) had had a bladder neck repair and needed to double void. 9 (30%) patients were voiding through their urethra but reported some form of stress leakage. Volitional transurethral voiding was recorded in all patients who were voiding through their urethra (80%). 6 (20%) patients were using clean intermittent catheterisation through a Mitrofanoff channel of which four patients also had undergone a bladder augmentation surgery. Age at augmentation ranged from 7-15 years (median 11.5). See Table 2 and Diagram 1. Other reported complications included bladder stones in 2 patients following bladder neck reconstruction and a further bladder stone in a patient with a closed bladder neck and bladder augmentation. We were not able to obtain information on upper tract status or urinary tract infections. Sexual Function 21 patients had documentation about sexual function; 20 (95%) were documented as able to achieve full erections. The one patient with poor erections had a forearm flap
8
phalloplasty. 6 (29%) patients had reported persistent dorsal chordee as an adult and 9 (43%) reported retrograde ejaculation. Of the 4 patients who had a Kelly procedure, 3 reported normal erections. 1 had poor erections and was the patient who had a radial forearm flap phalloplasty as an adult; it was not clearly documented whether the erectile dysfunction was present before or after the Kelly procedure. No patients were recorded to have fathered any children; however two had sought fertility advice.
Discussion There are only a few published studies looking at long-term outcomes of genito-urinary reconstruction in primary male epsipadias and their sexual function in adulthood (7) (8) (9) (10)
. Complications of these reconstructive techniques can take decades to develop and
pubertal growth can alter the final functional and cosmetic aspects of the corrected genitalia (11). It is therefore vital that long-term outcomes are recorded. These data in this historical series show both favourable and unfavourable long-term outcomes for male patients with primary epispadias comparable to previously published data which is limited. Primary male epispadias is a rare condition which is best managed in specialist paediatric urology centres. To date there is no uniform agreement on the best surgical management and thus each centre performs surgery in line with their expertise and experience. Currently two major reconstruction principles are used in specialist paediatric urology centres, the MCR repair and the Mitchell-Bagli complete disassembly technique (2) (12) (13). In 1989 Ransley and co-workers described a modification to the to the Cantwell repair that resulted in fewer complications and better cosmesis (14). Reports on this technique
9
tend to focus on the cosmetic appearance of the penis and urethral function or the postoperative anatomical and functional complications (6) (15) . Lottman and colleagues reported complications that required further surgical reconstruction in 45% of patients who underwent a MCR (n=40). Just under half of these patients had primary epispadias. Follow up was for a mean of 3 years. 50% of patients reported a good cosmetic outcome (7) . Mollard et al published long term follow up data in 29 patients. Those patients who had a MCR repair were considered to have a more cosmetically acceptable penis (8). Fistula rates have been reported between 0% to 28% in a number of studies with follow up varying between 5 to 8 years (9) (15) (14). Only 2 patients (6%) in our series who had a MCR repair developed a fistula. The remaining patients who developed a fistula had either had an alternative surgical procedure or the information on the primary surgery was not available. The overall fistula rate was therefore 20%. In those patients considered to have had successful corrective surgery, nearly all of them were concerned about their genital appearance as an adolescent and adult with 26% concerned about persistent dorsal curvature. Other studies have shown similar outcomes in terms of patient satisfaction once they reach adulthood (10) (11). Psychosexual counselling is an integral part of the management of epispadias and this was available to this cohort of patients. This shows that despite the best efforts and intentions of surgeons, surgical techniques alone will not enable the patients to cope with structural and aesthetic challenges of epispadias. 4 (13%) patients went on to have corrective surgery for cosmetic or functional reasons as adult. One patient who went on to have the radial forearm flap phalloplasty had undergone multiple surgeries with a number of post-operative complications
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Continence In this series 80% had penopubic epispadias and the remainder had a penile defect. It has been thought in the past that occurrence of incontinence is related to the degree of epispadias. However Kramer and Kelalis published a paper of their experience with 82 male patients with epispadias. 49 were penopubic (sub-symsyphyseal), 21 were penile and 12 were glanular. 46/49 penopubic epispadic patients and 15/21 penile epispadic patients were also incontinent (16). In fact in our cohort of patients all but 4 patients required some form of surgery for incontinence. Out of these 4 patients, 3 had penopubic epispadias, and 1 had distal epispadias. It has been shown that the most frequently identified urodynamic pattern before surgical correction of the bladder neck in patients with isolated epispadias is a low capacity, highly compliant bladder with minimal detrusor dysfunction (17) (18). Mouriquand et al looked at 17 male patients with primary epispadias who had undergone Young-Dees-Leadbetter bladder neck reconstruction. Continence was satisfactory in 41% at mean follow up of 10 years (19). From the long-term follow up published by Mollard et al 14 out of 40 patients were classified as having incontinent epispadias. These patients had a bladder neck reconstruction before penile reconstruction. 84% of patients were continent at follow up (8). Canon et al looked at 6 patients with primary male epispadias with a distal defect all with urinary incontinence. 5 patients had a bladder neck repair and all were continent at mean follow up of 9.8 years (20) . A recent study by Cendron et al looked at the anatomical findings associated with epispadias. This showed a significant number of both penile and penopubic epispadias patients had an abnormal bladder neck and continence rates were similar between both groups
(21)
.
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Duffy and Ransley looked at early outcomes of endoscopic treatment of urinary incontinence in children with primary epispadias (22). In our cohort 19 (63%) of patients had Macroplastique™ as an initial procedure. The majority of these required repeated injections. 8 patients had a further continence procedure following Marcroplastique™ injection. Median number of total Marcroplastique™ injections for all patients was 3 per patient (range 1-5).Of those patients who had Macroplastique™ injection only, 36% had persistent mild stress urinary incontinence at follow up.
Sexual Function Where documentation was available on sexual function, 95% of patients were reported to achieve a full erection. 26% of these patients described persistent dorsal chordee and 43% had retrograde ejaculation. Reddy et al showed 67% of their patient cohort described some problem with sexual function despite overall satisfaction and half reported abnormal ejaculation (10). Of the 6 epispadias patients in a Finnish study, the majority of these were satisfied with their sexual function (23). Mollard et al reported sexual outcomes in 29 patients (mean 27 years follow up). 80% reported regular sexual intercourse, 55% had normal ejaculation and four patients had fathered children (8). We were able to identify that two patients had sought fertility advice but could not find any record of paternity. Previous studies have shown fertility rates to be between 1766% (10) (11). Limitations of this study are that it is a retrospective study of patient records. Data are therefore reliant on the detail of the information obtained and recorded by the clinician, and no validated instruments were used in the assessment of LUTS, sexual function and patients’ opinion on cosmetic outcomes. 9 patients had no reported data on sexual
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outcomes; however they were kept in the study in order for those conducting to study to understand their continence outcomes.
Conclusions Published long-term continence and sexual function outcomes for men with epispadias are sparse. This study shows the majority of patients with epispadias have a good chance of achieving continence with volitional urethral voiding. More than 80% will need additional procedures to achieve socially acceptable continence. This study also shows high rates of erectile and ejaculatory function; however, dissatisfaction with genital appearance is significant. Our findings are consistent with previous reports and can be used to counsel patients and their parents on what they can expect as they progress through adolescence into adulthood.
Conflict of Interest None to declare Funding None References 1. PDE, Mouriquand. Congenital disorders of the bladder and urethra In: Textbook of Genitourinary Surgery 2nd Edition. 1998. 2. Management of Epispadias. Grady RW, Mitchell ME. s.l. : Urology Clinics of North America, 2002, Vol. 29. 349-360. 3. Anatomy of the penis and its deformities in exstrophy and epispadias. Woodhouse CRJ, Kellett MJ. s.l. : J Urol, 1984, Vol. 132. 1112. 13
4. Operative treatment of epispadias by transplantation of the urethra. FV, Cantwell. s.l. : Ann Surg, 1895, Vol. 22. 5. Male Epispadias. Diamond DA, Ransley PG. s.l. : J Urol, 1995, Vol. 154. 6. The evolution of Penile reconstruction in Epispadias repair: A report of 180 cases. Kajbafzadeh AM, Duffy PG, Ransley PG. s.l. : J Urol, 1995, Vol. 154. 858-861. 7. Male Epispadias repair: Surgical adn fucntional results with the Cantwell-Ransley proceudre in 40 patients. Lottman HB, Yaqouti M, Melin Y. s.l. : J Urol, 1999, Vol. 162. 1176-1180. 8. Male Epispadias: Experience with 45 Cases. Mollard P, Basset T, Mure PY. s.l. : J Urol, 1998, Vol. 160. 55-59. 9. The Modified Cantwell-Ransley repair for Exstrophy and Epispadias: A 10 year experience. Surer I, Baker LA, Jeffs RD, Gearhart JP. 1040-1043, s.l. : J Urol, 2000, Vol. 164. 10. Sexual Health Outcomes in Adults with Complete Male Epispadias. Reddy SS, Inouye BM, Anele UA, Adelwahab M, Le B, Gearhart JP, Rao PK. s.l. : J Urol, 2015, Vol. 194. 1091-1095. 11. Long-term outcome of male genital reconstruction in childhood. Tourchi A, Hoebeke P. s.l. : J Paed Urol, 2012, Vol. 9. 980-989. 12. Diagnosis and management of epispadias. D, Frimberger. s.l. : Seminars in Paediatric Surgery, 2011, Vol. 20. 85-90. 13. The Surgical Management of Male Epispadias in the New Millenium. Cho P, Cendron M. s.l. : Curr Urol Rep, 2014, Vol. 15. 472. 14. Applications of the modified Cantwell-Ransley epispadias repair in the exstrophy-epispaidias complex. Baird AD, Gearhart JP, Mathews RI. s.l. : J Paed Urol, 2005, Vol. 1. 331-336. 15. Results of Epispadias Repair Using the Modified Cantwell-Ransley technique. Bar-Yosef Y, Sofer M, Ekstein MP, Binyamini Y, Ben-Chaim J. s.l. : Urology, 2016, Vol. 99. 221-224. 16. Assessment of urinary continence in epispadias. Review of 94 patients. Kraemer SA, Kelalis PP. s.l. : J Urol, 1982, J Urol, Vol. 128, pp. 2636-42. 290-293. 17. Urodynamic findings in children with isolated epispadias. Kaefer M, Andler R, Bauer SB, Hendren WH, Diamond D, Retik AB. s.l. : J Urol, 1999, Vol. 162. 1172-1175. 18. Bladder function and dysfunction in exstrophy and epispadias. Hollowell JG, Hill PD, Duffy PG, Ransley PG. s.l. : Lancet, 1991, Vol. 338. 926. 19. Long-term results of bladder neck reconstruction for incontinence in children with classical bladder exstophy or incontinent epispadias. Mouriquand PDE, Bubanj T, Feyaerts A, Jandric M, Timsit M, Mollard P, Mure PY, Basset T. 92, s.l. : BJUI, 2003. 997-1002. 20. Pathophysiology and management of urinary incontinence in case of distal penile epispadias. Canon S, Robert R, Koff SA. 180, s.l. : J Urol, Vol. 2008. 2636-42.
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21. Anatomic findings associated with epispadias in boys: Implications for surgical management and urinary continence. Cendron M, Cho PS, Pennison M, Rosoklija I, Diamond DA, Borer JG. 2018, Vol. 14(1). 42-46. 22. Endoscopic treatment of urinary incontinence in children with primary epispadias. Duffy PG, Ransley PG. s.l. : British Journal of Urology, 1998, Vol. 1998. 309-311. 23. Sexual Function in Patients Operated on for Bladder Exstrophy and Epispadias. Suominen JS, Santilla P, Taskinen S. s.l. : J Urol, 2015, Vol. 194. 195-199.
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Table 2: Continence Outcomes for Patients according to Type of epispadias Type of Epispadias
Total Number
Number requiring continence surgery (%)
Number of Continent patients voiding through native urethra (%)
Number of patients with persistent Stress Incontinence (%)
Number of patients voiding via Mitrofanoff (%)
Penopubic
24
21 (86)
10 (40)
9 (38)
5 (21)
Midshaft
3
2 (66)
2 (67)
0
1 (33)
Distal
3
1 (33)
3 (100)
0
0
Table 1: Continence Procedures and Continence outcomes Continence Procedure
Total number
Number of Continent patients voiding through native urethra
Number of patients with persistent mild Incontinence
Number of patients voiding via Mitrofanoff
Macroplastique™ only
11
7
4
0
Bladder neck reconstruction (BNR) only
3
2
0
1
Bladder neck sling only
1
0
1
0
1. Macroplastique™ BNR 1. BNR 2. Macroplastique™ 1. Macroplastique™ 2. Kelly 1. BNR 2. Kelly procedure Kelly procedure
6
1
2
3
1
0
1
0
2
2
0
0
1
0
0
1
1
0
0
1
No Continence procedure
4
3
1
0
Diagram 1 : Flow diagram on continence outcomes CIC = Clean intermittent Catheterisation
30 Patients 24 Penopubic (PP) 3 Midshaft (M) 3 Distal (D)
15 (50%) Dry and voiding through native urethra 10 PP 2M 2D
8 (27%) Minor stress leakage – urethral voiding
1 (3%) Major Stress leakage – urethral voiding
8 PP
1 PP
6 (20%) CIC through Mitrofanoff 5 PP 1 MS
4 Ileocystoplasty