SALVAGE
RECONSTRUCTIVE SURGERY IN AN ADULT PATIENT WITH FAILED PREVIOUS REPAIR
OF AN EXTROPHY-EPISPADIAS COMPLEX. AN OPERATION WITH A FUNCTIONAL AND
AESTHETIC PURPOSE
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STEVEN JONIAU, ANNE-MARIE STOEL, HEIN VAN-POPPEL, ROBERT HIERNER
Department of Urology and Department of Plastic, Reconstructive and Aesthetic Surgery, Center for Interdisciplinary Reconstructive Surgery, Microsurgery, Hand Surgery, Burns, University Hospital Leuven, Leuven, Belgium
ABSTRACT
Salvage
surgical procedures after failed reconstruction for an extrophy-epispadias
complex are extremely challenging. The goals are to restore continence
and improve aesthetic appearance in order to provide quality of life and
an improved body image to the patient.
We describe the surgical steps in an adult
patient who presented anal urinary incontinence and a poor body image
due to the absence of an umbilicus and the presence of hypertrophic scars.
He underwent a modified Mainz II reconstruction of the lower urinary tract
at childhood for an extrophy-epispadias complex. Restoration of continence
was achieved by the construction of a modified Mainz I pouch with a continent
stoma in a neo-umbilicus. Body image improved dramatically by the construction
of a neo-umbilicus, a surgical revision of the hypertrophic abdominal
scars and an abdominoplasty.
It is mandatory that such demanding surgery
should only be attempted as a combined multidisciplinary effort with urologists
and plastic/reconstructive surgeons.
Key
words: bladder exstrophy; epispadias; reconstructive surgical
procedures
Int Braz J Urol. 2007; 33: 810-4
INTRODUCTION
Children
with the extrophy-epispadias complex pose a great challenge to the multidisciplinary
teams caring for them. The main goals are to achieve urinary continence
and fashioning the external genitalia in order to be capable of adequate
sexual intercourse. At present, most teams will opt for a staged reconstruction
with closure of the bladder plate, and a bilateral iliac osteotomy at
a first stage. Later treatment is directed to render the patient continent,
which often implies in the construction of urinary reservoirs and conduits
for intermittent catheterization. An alternative is the construction of
a rectal pouch (Mainz II procedure), with the anal sphincter providing
continence. In addition, in this later stage, the external genitalia are
reconstructed (1).
Although continence now can be achieved
in up to 80% of children, only about 40% of adults are dry in the best
hands. Apart from this, hypertrophic scars, the absence of an umbilicus
and malformation of the external genitalia often compromise the aesthetic
aspects later in life. In these cases surgical procedures, which are complex
and demanding, should be performed by multidisciplinary teams (1-5).
We describe the surgical steps undertaken
in an adult patient who experienced secondary incontinence and a poor
body image after failed previous surgery for an extrophy-epispadias complex.
The main challenge was to restore continence and to create a neo-umbilicus,
which served both a functional (access to the urinary reservoir) and aesthetic
purpose.
CASE REPORT
A
35-year-old man presented at the urology outpatient clinic. As a neonate,
he had been treated for extrophy-epispadias complex by the construction
of a modified Mainz II pouch. The rectum served as a urinary reservoir,
while the sigmoid colon was pulled down forming the posterior compartment.
A corporoplasty, combined with a urethroplasty was performed at the same
time. At the age of 14, a reimplantation of the left ureter was performed
for an anastomotic stricture. A Nesbitt-plication and a scar revision
were performed at the age of 29. Furthermore, he underwent an inguinal
hernia repair, an appendectomy.
Initially, continence was as expected in
a rectal neo-bladder: normal during the day but at night there was some
loss of urine. At the age of 32, the patient sustained a persistent transphincteric
recto-perineal fistula. He was treated conservatively with a tress-thread
intending not to affect the function of the anal sphincter. In spite of
good clinical practice, there was a partial loss of continence. A bulking
agent was unsuccessfully implanted inter sphincter.
The main problems at presentation were a
poor body image caused by the absence of the umbilicus and the broad,
hypertrophic abdominal scars. The urinary incontinence was another main
concern, as he had always been very active in different sports and had
to stop these activities because of embarrassment with his situation.
At physical examination, wide and hypertrophic
abdominal scars were evident, as was the absence of an umbilicus. The
symphysis was widened because a pelvic osteotomy was not performed at
childhood. The penis was short and broad.
Upper tract function was normal, with balanced
split renal function on renal scintigraphy. There was no evidence of malignant
malformation on rectoscopy. A retrograde urethrography showed a widened,
saccular urethral remnant.
Considering the reconstructed urethra could
not be catheterized and the fact that the umbilicus of the patient was
missing as a result of the first operation, a neo-umbilicus had to be
constructed to provide an aesthetic and functional access to the pouch.
This was planned in two phases. First, an inverted T abdominoplasty was
performed to remove old scars. (Figure-1). At the same time, an umbilicus
was reconstructed by means of two lateral flaps. Wound-healing was compromised:
there was an area of dehiscence of the vertical part of the scar. During
the second stage, a redo umbilical reconstruction was performed simultaneously
with the successful construction of continent iliocaecal neobladder (Mainz
I-pouch) with a continent (Monti) stoma (post-appendectomy status), Figure-1.
An additional liposuction was performed in order to improve the epigastric
and lateral (“love handles”) contour. After the second stage,
wound healing was not compromised and the patient was taught to catheterize
via the neo-umbilicus. At 12 months postoperatively, the patient was very
pleased by both the functional and aesthetic results of the surgery. (Figure-1)
COMMENTS
The
extrophy-epispadias complex is an uncommon malformation, characterized
by disorders of the lower abdominal wall, pelvic floor, external genitalia
and pelvis. Nowadays, staged reconstructions can achieve acceptable continence
rates and aesthetic appearance. Nevertheless, a number of patients will
fail, and will need additional surgery at a later stage. There are not
many reports describing surgical steps to repair failed hypospadias surgery
in adult patients (1,2).
The presented case illustrates a number
of considerations one has to make before embarking in such complex and
challenging surgery.
Secondary urinary incontinence after a previous
rectal pouch reconstruction can only be treated by constructing a new
urinary reservoir with a continent diversion from another bowel segment.
In the presented case, a modified Mainz I pouch reconstruction was performed.
A Monti type continent outlet was used, because the appendix had already
been resected earlier in life. Reconstructive lower urinary tract surgery
in secondary incontinent patients or patients remaining incontinent after
prior surgery can achieve very high success rates, provided customized
surgical procedures are chosen on a case-by-case basis (1).
The lack of the umbilicus was an important
aesthetic burden for the patient. The absence of the only tolerated scar
can be associated with poor body image and abnormal emotional behavior.
Having not only an aesthetical function, the umbilicus can be used as
a handy access for a continent diversion. Different techniques of umbilical
reconstruction have been published, describing both the immediate reconstruction
after primary closure of the bladder plate, as the delayed reconstruction
at a later stage (3,4).
In analogy with Choudhary & Taams we
suggest 4 details for functional and aesthetic reconstruction (5):
1)
Preservation of the umbilicus: Contrary to old techniques, the umbilicus
or even remnants should be preserved during primary treatment of bladder
extrophy.
2) The position of the neo-umbilicus should
be carefully planned: it should be placed on the joining lines of both
iliac crests. The distance between the xiphisternum and the umbilicus
- distance between the umbilicus and the pubic symphysis (1.6:1). - The
distance between the umbilicus and the anterior superior iliac spine-distance
between both the anterior superior iliac spines (0.6:1). - The distance
between the umbilicus and the anterior superior iliac spine-distance between
the umbilicus and the pubic symphysis (0.9:1).
3) The appearance of the neo-umbilicus:
The new umbilicus needs to have an adequate diameter (1.5 - 2 cm). The
navel is ‘perfect’ when he is T - or vertical shaped. The
hooding should be different with the posture (superior in standing positioning).
An ‘innie’ being better than an ‘outie’. Better
results are reached when the subcutaneous fat layer is more prominent.
The natural cone depression must be pursued while a circumferentially
scar should be avoided. Furthermore, it should have a normal slant superiorly
and a normal shape.
4) The function of the neo-umbilicus: it
should be continent and one should strive for minimal irritation at the
transient zone between mucosa and normal skin. Adequate hygiene is of
utmost importance in patients with hair growth. Hair follicles must be
removed definitively by the exeresis or laser-treatment.
CONCLUSION
Body image, together with adequate urinary continence and sexual function are crucial in the surgical treatment of the extrophy-epispadias complex. When primary treatment fails at an adult age, surgery by an experienced multidisciplinary team can successfully improve patient’s body image and restore continence.
CONFLICT OF INTEREST
None declared.
REFERENCES
- Woodhouse CR, North AC, Gearhart JP: Standing the test of time: long-term outcome of reconstruction of the exstrophy bladder. World J Urol. 2006; 24: 244-9.
- Meyer KF, Freitas Filho LG, Martins DM, Vaccari M, Carnevale J: The exstrophy-epispadias complex: is aesthetic appearance important? BJU Int. 2004; 93: 1062-8.
- Barroso U Jr, Jednak R, Barthold JS, Gonzalez R: A technique for constructing an umbilicus and a concealed catheterizable stoma. BJU Int. 2001; 87: 117-20.
- Hanna MK, Ansong K: Reconstruction of umbilicus in bladder exstrophy. Urology. 1984; 24: 324-6.
- Choudhary S, Taams KO: Umbilicosculpture: a concept revisited. Br J Plast Surg. 1998; 51: 538-41. Erratum in: Br J Plast Surg 1999; 52: 78.
____________________
Accepted after revision:
September 28, 2007
_______________________
Correspondence address:
Dr. Steven Joniau
Dept. of Urology University Hospital Leuven
Herestraat 49
3000, Leuven, Belgium
E-mail: Steven.Joniau@uzleuven.be