Journal of Pediatric Urology (2010) 6, 6e10

Long-term follow-up of male patients after

reconstruction of the bladdereexstrophye
epispadias complex: Psychosocial status,
continence, renal and genital function
Anne K. Ebert a,*, Günter Schott b, Monika Bals-Pratsch c, Bernd Seifert c,
Wolfgang H. Rösch a

a
Department of Paediatric Urology, University Medical Centre Regensburg, Steinmetzstr. 1-3, 93049 Regensburg, Germany
b
Department of Urology and Paediatric Urology, University of Erlangen, Germany
c
Centre for Gynaecological Endocrinology, Reproductive Medicine and Human Genetics, Regensburg, Germany

Received 5 May 2009; accepted 5 June 2009

Available online 10 July 2009

KEYWORDS
Long-term follow-up;
BladdereExstrophye
Epispadias complex;
Functional reconstruc-
tion;
Andrological outcome;
Genital function;
Psychosocial outcome
Abstract Objective: There is a paucity of knowledge about long-term outcome issues in the
bladdereexstrophyeepispadias complex (BEEC). Adult male BEEC patients were investigated
in respect of bladder and renal function, fertility, genital function and psychosocial facts.
Patients and methods: In a cross-sectional study, 17 adult male BEEC patients (mean age 23.4
years) from a single centre were evaluated with a questionnaire, renal and bladder ultrasound,
blood tests, hormonal profile and semen analysis.
Results: Phenotypically one patient had complete epispadias and 16 had classical bladder
exstrophy. Five patients underwent a one-stage functional reconstruction as a primary and
12 as a redo procedure. After a mean follow-up of 19.4 years, 15 bladders were preserved with
12 voiding per urethram and 3 performing intermittent catheterization; 2 were secondarily di-
verted. Significant residual urine was present in 10; kidneys were normal in 14 patients.
Sixteen patients proved ejaculations, 3 had normospermia, 7 oligoasthenospermia and 6 azos-
permia. In patients with only one single bladder neck procedure normospermia was statisti-
cally significant.
Conclusion: After functional BEEC reconstruction, long-term bladder function is preserved
with mostly normal renal function. The number of bladder neck attempts has a significant
influence on andrologic outcome. Detailed analysis may detect multifactorial pathogenesis
from the impaired sperm quality in the BEEC.
ª 2009 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.

* Correspondence to: Anne K. Ebert, Department of Paediatric Urology, University Medical Centre Regensburg, Steinmetzstr. 1-3, 93049
Regensburg, Germany. Tel.: þ49 941 3695451; fax: þ49 941 3695455.
E-mail address: anne-karoline.ebert@barmherzige-regensburg.de (A.K. Ebert).

1477-5131/$36 ª 2009 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
doi:10.1016/j.jpurol.2009.06.002
Long-term follow-up of bladdereexstrophyeepispadias complex
Introduction
Over the last decades enormous efforts have been made to
further improve the functional and cosmetic outcome of
the bladdereexstrophyeepispadias complex (BEEC) [1].
However, outcome issues of reconstruction concepts are
still a matter of debate [2,3]. At the time when the adult
BEEC individuals of this series were born, reconstruction
strategies in Germany widely differed from the contempo-
rary approach. Smaller and less promising bladder
templates were excised and a urinary diversion was set in
place. Larger bladders were closed at 3e18 months of age,
and bladder neck reconstruction, usually a Young-Dees-
Leadbetter procedure, was performed when the patients
were 4e7 years old [4]. Epispadias was corrected as late as
the bladder neck plasty or even later according to Young-
Cantwell. In 1983, Schrott introduced his single-stage
functional reconstruction, and this technique has continued
to be used in a standardized fashion in two high-volume
BEEC centres in Germany to date [5]. The possible benefit
of this and all other one-stage reconstruction techniques is
the unique combination of all major reconstruction steps,
allowing unrestricted access to the bladder neck, and by
this means resulting in fewer scars, and therefore ideally in
an efficient developing bladder capacity by rhythmic filling
and emptying against adequate bladder outlet resistance.
Hence, since there are sparse long-term follow-up data
about bladder function, fertility, genital function and
psychosocial outcome in BEEC patients after reconstructive
surgery, we conducted a cross-sectional study in adult male
BEEC patients. The aim was to update long-term informa-
tion about fertility and continence within the reconstruc-
tive concept, and determine potential prognostic factors
for fertility and continence in BEEC.
Methods
Patient recruitment
A study call via a postal letter was send to all male patients
older than 16 years after functional reconstruction in
a single high-volume BEEC centre in Germany. We offered
a non-invasive check up with sonography, blood tests and
semen analysis. No other exclusion criteria were
determined.
Study design
This study was based on a cross-sectional design and
enrolled 17 male BEEC patients who followed our call
during the period from 08/2004 to 12/2007. The local
Human Research Ethics Committee approved this study,
which was a part of a research project about long-term
outcome within the BEEC. All BEEC patients signed
a written consent.
Assessments
All patients presented in our outpatient clinic for a semi-
structured interview about medical and andrologic history,
7
continence status, psychosocial status, and genital and
sexual function. All had a clinical examination with testic-
ular, bladder and renal ultrasound. Complete continence
was defined according to the literature as dry intervals of
more than 3 h and dry nights, partial as between 1 and 3 h
with dry nights, and incomplete as nocturnal and daytime
incontinence [1]. Blood samples for hormone analysis were
taken before 10 am. Semen analysis was retrieved through
masturbation and was analysed according to WHO standard
2002.
Statistical analyses
Statistical analyses mainly made use of descriptive
methods, including counts, means and ranges. Prognostic
factors for fertility and continence were calculated using
2  2 cross tabulations; P  0.05 was considered as statis-
tically significant. All statistical analyses were performed
using SPSS 15.0.
Results
Patient characteristics
Operative characteristics, functional voiding outcome and
continence are listed in Table 1. Mean age of all male BEEC
patients was 23.4 years (range 18.3e33.9) when they pre-
sented for this study. Regarding the phenotype, 16 had
classical exstrophy and 1 complete epispadias.
Initial operative treatment
All patients underwent the following standardized func-
tional single-stage reconstruction at a high-volume BEEC
centre in Germany described by Schrott [5]. Usually,
complete reconstruction is timed during the first 8e10
weeks of life. In contrast to the Young-Dees-Leadbetter
procedure, a superficial oblique incision is performed on
each side up to the lateral margin between the upper and
lower parts of undamaged trigone, until blunt preparation
splits the bladder wall area and posterior urethra, in order
to preserve the neurovascular bundles. Urethral prolonga-
tion is achieved by tubularization of the trigonal muscle and
the anterior bladder wall is reinforced by a second
muscular invagination, to ensure the anatomically correct
positioning of the colliculus in the posterior urethra. At this
time epispadias correction was achieved by the Young-
Cantwell repair. As the newborn and infant pelvis is soft
enough, the symphysis is approximated in a stepwise
fashion with the help of absorbable traction sutures. After
immobilization for 10 days there usually is formation of
a substantial connective tissue bridge.
Reconstruction results and voiding outcome
No dehiscence regarding bladder, symphysis or abdominal
wall was noted after 5 primary and 12 secondary proce-
dures at the mean age of 31.3 months (range 3e144
months). In the long-term follow-up of mean 18.7 years
(range 12.3e29.1), 15 of 17 functional reconstructed
8 A.K. Ebert et al.

nocturia, stress incontinence and urgency, as well as the use

Table 1 Patients’ characteristics, functional voiding
of diapers on various occasions, either for protection or in
outcome and continence.
preparation for nocturnal incontinence, were found
BEEC patients predominately in the redo group.
after functional
reconstruction (N Z 17) Renal and bladder function
Initial operative concept Single-stage concept
Primary attempt 5 Renal function according to a mean serum creatinine of
Mean age at primary 8.5 months (range 3e14) 0.85 mg/dl (0.8e1.0) was normal in all study patients.
reconstruction Sonographically normal bilateral kidneys were seen in 14
Secondary attempt 12 patients, whereas three patients had a contralateral
Mean age at secondary 23 months (range 5e144) smaller kidney with a stabile 99mTc-DMSA renal scan. No
reconstruction renal scars or upper tract dilatation were detected in the
study group. Recurrent non-febrile UTIs were reported in
Functional voiding outcome
four patients, one each after urinary diversion or ileum
Long-term bladder preservation 15
augmentation. As a possible risk factor significant residual
Spontaneously voiding 12
urine (>50 ml) was detected in seven of the 12 spontane-
Residual urine 7
ously voiding patients.
in spontaneously voiding
Augmentation 3
CIC through 2 Psychosocial details
Mitrofanoff stoma
Spontaneously voiding 1 Psychosocial markers and partnership characteristics are
CIC per urethram 1 listed in Table 2. Though one patient was practising
Secondary urinary diversion 2 exclusively one-night stands, most relationships were
lasting a longer period. Despite a very good education level,
Continence heavy smoking, drugs and alcohol were common. One
Subjective continent 16 patient had finished alcohol withdrawal treatment. Three
Complete continence 10 patients were in psychiatric long-term treatment and under
per definition observation mainly for anxiety or psychosexual adaptation
Stress incontinence 4 problems.
Urgency 1
Nocturia 4
Incontinence at night 7 Andrologic history, genital function and potential
(>1 night/month) fertility status
Use of diapers 7
All 17 study patients reported erections, leading to
successful sexual intercourse in 10 patients. Four patients
were too young to establish a sexual relationship and three
failed when they tried to practise intercourse due to penile
exstrophic bladders could be preserved. Four of the five length. Dorsal penile curvature was present in 13 patients,
primary reconstructed patients reported reliable whereas only six had discomfort with penile deviation.
continence, three of them voiding spontaneously and one Clinical examination showed various genital findings like
performing intermittent catheterization after ileum firm and enlarged epididymis in six, hydroceles in two, and
augmentation through a Mitrofanoff channel. Owing to a varicocele III in one patient. Fourteen patients had
persistent incontinence, one patient was diverted to an a positive semen microbiology. Spontaneously voiding and
ileocoecal pouch after puberty. In the redo group, 10 of 12 residual urine were not statistically significant for the
patients stated being completely dry, one patient reported occurrence of epididymitis. Sonographically intratesticular
to be partially incontinent, and one patient was diverted microcalcifications were detected in two patients and
during puberty after three unsuccessful bladder neck hypoechogenic lesions in one patient. Two patients had
repairs. In this redo group, nine adult patients voided a testicular biopsy. Histology proved a testicular intra-
spontaneously and one of these emptied his bladder epithelial neoplasia (TIN) in one patient, who underwent as
without residual urine after sigma augmentation. Inter- a consequence radiation therapy, and a benign stroma
mittent catheterization was performed per urethram and tumor in the other patient.
through a Mitrofanoff channel after ileum augmentation in During semen analysis 16 patients proved prograde
one patient each (Table 1). ejaculations with a mean volume of 1.8 (range
0.1e7.0 ml). Three patients had normospermia, two
Continence asthenospermia, four oligoasthenospermia and seven azo-
spermia (Table 3). Though hormonal profile was within
Irrespectively to finally reconstruction method 16 BEEC normal range for 13 patients, follicle-stimulating hormone
patients declared themselves continent. According to the (FSH) was elevated in four patients as a sign of spermato-
objective continence criteria however, complete continence genesis failure. Normospermia however was statistically
was only verified in 10 patients. Voiding symptoms such as significant after a single bladder neck attempt (P Z 0.018).
Long-term follow-up of bladdereexstrophyeepispadias complex 9

Table 2 Patients’ psychosocial and partnership Table 3 Hormone and semen analysis.
characteristics. BEEC patients Primary Secondary
BEEC patients (N Z 17) (N Z 17) functional functional
Education level reconstruction reconstruction
High school diploma 13 (N Z 5) (N Z 12)
Secondary school level 4 Semen analysis possible 5 11
FSH elevation 0 4
Occupation
Normospermia 3* 0*
Attending high school 1
Asthenospermia 0 2
Studies at university (medicine) 12 (4)
Oligoasthenospermia 0 4
Working self-employed 1
Azospermia 2 5
Employees 3
Epididymitis 2 4
Family of origin structure Semen microbiology positive 4 10
No siblings 2 * Statistically significant with P Z 0.018.
One sibling 6
Two or more siblings 9
either in voiding or storage dysfunction [2,4]. In this cross-
Partnership characteristics sectional study, long-term information was updated for
Married 1 male patients after functional reconstruction in a single
Not married 16 centre in Germany. After a follow-up of mean 18.7 years
Number of sexual partners most exstrophic bladders were preserved, allowing many
No sexual partnership ever 4 patients spontaneous voiding per urethram (Table 1).
Only one sexual partner 8 However, in three cases additional surgery was needed to
Two or three 2 optimize bladder storage and emptying function (Table 1).
sexual partners Although overall kidney function remained normal, three
>3 partners 3 study patients from the redo group had impaired unilateral
kidney split function. In addition to voiding symptoms,
Duration of sexual partnerships remarkable residual urine was detected in more than half
Longer than 1 year 8
of the spontaneously voiding BEEC patients. Neither voiding
Between 6 and 12 months 2 pattern nor residual urine was statistically significant for
Less than 6 months 3 recurrent UTIs, but at least one patient was regularly
No sexual relationship at present 4 catheterizing per urethram. Significant residual urine
Drugs and alcohol mandatorily indicates the necessity for sonographic moni-
Heavy smoking 10 toring, double voiding and urodynamic evaluation when
Marihuana or synthetic drugs 6 complications occur.
Regular alcohol 3 Some actual long-term results regarding true fertility and
in a notable amount potential reproductive function of male BEEC patients have
Alcohol withdrawal treatment 1 been reported, but there is still controversy over whether
Psychiatric treatment 3 functional reconstruction or urinary diversion is more
harmful to future fertility [1,7,8,16e19]. As expected, about
60% of the study patients practiced sexual intercourse and
had longstanding relationships with only a few partners
[7,8]. A specific wish to have own children was expressed by
None of the other potential fertility-restricting factors, like
two patients with azospermia in a stable partnership, but
epididymitis, residual urine, pathological semen analysis
none intended in vitro fertilization to father own children
and FSH elevation, were significantly different between the
yet. In contrast to the current doctrine of a usually normal
primary and secondary reconstruction techniques.
hormonal status in BEEC, FSH was elevated in 20% of the
study patients, indicating spermatogenesis failure. As
Discussion a result, attention should be paid to the hormonal status of
male BEEC patients after puberty. Due to the well-known
There is no doubt that the BEEC has tremendous impact on fact that reconstructive surgery must per definition interfere
adult life for both sexes [1e3,6e8]. Long-term follow-up with bladder neck, colliculus seminalis and penile anatomy,
should focus on renal and bladder outcome but also on complications of early reconstructive surgery seem to be
genital and potential reproductive function. In the litera- disastrous to fertility [7,8,16e19]. In addition to scarring of
ture, a reasonable evaluation of continence in adulthood the bladder neck region, postoperative damage of the bul-
and validated scores have not been introduced or tailored bospongiosus muscle interferes with an undisturbed ante-
to the particular needs of adult BEEC patients. Most avail- grade ejaculation [7,8]. Detailed semen analyses are
able outcome information about continence cover post- available in about 80 adult BEEC patients worldwide from 10
operative periods of 5e12.5 years [1e3,9e15]. Moreover, series, showing azospermia in about half of the patients,
often only necessary subsequent surgery provides indirect normospermia in only about 10%, and good sperm quality
evidence for muscular bladder deterioration resulting more likely in diverted than in reconstructed patients
10
[7,8,16e19]. As a result, paternity was twice as high in
diverted or even unreconstructed than in reconstructed
BEEC patients [8,17e19]. All 17 BEEC patients of our study
had erections and 16 proved antegrade ejaculations, irre-
spective to timing and method of urethral reconstruction.
The mean age at functional reconstruction was 8.5 months
for the primary and 23 months for the redo cases. Potential
fertility-restricting factors like epididymitis, residual urine,
pathological semen analysis and FSH elevation were not
significantly different between primary and secondary
reconstruction groups. Normospermia, however, was statis-
tically significant in patients after a single bladder neck
attempt (P Z 0.018). Not the timing but the number of
bladder neck attempts seems to be one prognostic factor for
fertility. Furthermore, in addition to the fate of the bulbo-
spongiosus muscle, only an anatomically correct positioning
of the colliculus during the bladder neck procedure will allow
antegrade ejaculation in the BEEC.
Conclusion
Long-term observation of the BEEC is necessary for appro-
priate counseling of parents and patients. Fertility and
continence are major issues for male adolescent and adult
BEEC patients. After a single-stage functional reconstruc-
tion long-term bladder function can be preserved with
normal renal function in most cases. Spontaneous voiding
was possible even after redo reconstruction. However, signs
of muscular deterioration such as residual urine or urinary
symptoms were present in a considerable number of
patients. The number of bladder neck attempts however
had a significant influence on andrologic outcome. An
astonishingly high rate of positive semen microbiological
results should focus attention on a post-infectious problem
and possible treatment strategy. Severely impaired fertility
in male BEEC patients is most probably multifactorial. True
individual fertility potential and spontaneous chance of
pregnancy for a couple with one partner affected by BEEC
are surely not predictable. This study underlines the
importance of careful long-term follow-up for all BEEC
patients.
All authors have made a significant contribution to the
findings and methods in the paper. All authors state that
there are no financial or commercial interests and no
conflicts of interest and funding.
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