Original Articles
A Combined Vertical and Horizontal Pelvic Osteotomy Approach for
Repair of Bladder Exstrophy: The Dana Experience
Eitan Segev MD1, Elias Ezra MD1, Yosef Binyamini MD2, Shlomo Wientroub MD1 and Jacob Ben-Chaim MD2
1
Department of Pediatric Orthopedics and 2Pediatric Urology Unit, Dana Children's Hospital, Tel Aviv Sourasky Medical Center,
Tel Aviv, Israel
Affiliated to Sackler Faculty of Medicine, Tel Aviv University, Ramat Aviv, Israel
Key words: bladder exstrophy, pelvic osteotomy
Abstract
Background: Bladder exstrophy is a severe congenital defect that
A
B
requires a multidisciplinary treatment approach. Soft tissue repair may
be successful during the first few days after birth, but a combination of
pelvic osteotomies and bladder reconstruction is necessary later in life.
The combination of externally fixed anterior and posterior osteotomies
has biomechanical advantages over previous techniques for achieving
primary bladder closure.
Objectives: To describe our experience with a combined vertical
and horizontal pelvic osteotomy approach for the repair of bladder
exstrophy.
Methods: Four children underwent bladder exstrophy closure; the
mean age at surgery was 19 months (range 9±33 months)... We
stabilized the osteotomies with a small Synthes AO external fixator, 4.0
mm rod diameter.
Results: All four patients had successful bladder repair with no
dehiscence; two of them achieved partial continence, and bladder neck
reconstruction is planned for the other two. Three of the four patients
sustained neurologic injury; two completely recovered, and the third
continues to suffer from right drop foot. The average follow-up was 39
months (range 10±60 months).
Conclusions: Vertical and horizontal pelvic osteotomies stabilized
by external fixator and bladder repair is an effective treatment for
bladder exstrophy.
IMAJ 2004;6:749±752
Bladder exstrophy is one of the most severe congenital defects
facing the treating physician. It occurs in 1/30,000 births and
involves multiple systems. After delivery, the bladder is seen to be
everted and completely extruded via the lower abdominal muscles.
In male patients there is complete epispadias, while in females the
clitoris is bifurcated and the vagina is short and wide. The anus is
anteriorly located in both sexes. There is a high rate of inguinal
hernia (15% of females and 82% of males). Prenatal diagnosis of this
condition is possible by ultrasound screening: it is suspected if the
bladder is not seen in the lower abdomen, if there is bulging of the
lower abdomen, and if the penis is very small [1,2]. The bony pelvis
has an external rotation deformation of the iliac wings and of the
anterior ischiopubic segments, and the pubic bones are short with
the pubis widely open [3]. The pelvic floor, composed of the levator
ani, the obturator internus and the externus muscles, is deformed
and does not support the bladder and urethra [Figure 1 A,B] [4].
The complexity of the defect necessitates the coordination of
several medical disciplines, such as pediatric urology, orthopedic
surgery, general surgery, rehabilitation and psychology in order to
IMAJ
.
Vol 6
.
December 2004
Figure 1. [A] Clinical photograph of patient #4 (age 2 years) before surgery:
exstrophy of the bladder via the abdominal wall and complete epispadias with
anterior location of the anus. [B] A-P radiograph of the pelvis: wide separation of
the pubic rami at the symphysis; the iliac bones are externally rotated with
retroversion of the acetabulae. [C] Intraoperative clinical photograph: a Gigli
saw is passed via the sciatic notch in preparation for section of the supraacetabular region. The horizontal part of the osteotomy is similar to Salter's
classical technique. [D] Intraoperative clinical photograph: the vertical part of
the osteotomy is located parallel to the sacroiliac joint and creates a
``greenstick'' fracture of the ilium ala.
Modified Osteotomy for Bladder Exstrophy
749
Original Articles
B
C
Figure 2. [A] Clinical photograph of the patient in Figure 1 just after surgery: the pelvic osteotomies are stabilized by an external fixator (small AO external fixator
set). The bladder has been repaired and the defect in the abdominal wall is closed. The epispadias has been repaired, and there are tubes in the bladder and ureters
for temporary drainage. [B] Radiograph of the pelvis in the A-P position after surgery: the pubic rami are approximated by the external fixator and the iliac bones are
internally rotated via the osteotomies. [C] Follow-up radiograph 7 months after surgery: the pelvic bone has remained internally rotated with reduction of the
symphysis pubis.
achieve successful repair and outcome. We describe our experience
with four children who were operated by our combined pediatric
orthopedics and pediatric urologist team.
Treatment modalities
Initial management
Upon the discovery of bladder exstrophy in the newborn, the
extruded bladder is immediately washed with saline and dressed
with plastic film to protect the bladder mucosa. The umbilical
clamp should be replaced by a thick silk ligature. Maintenance of
meticulous hygiene is crucial while waiting for surgical correction.
Closure of the bladder
In neonates a few days old, it is possible to close the bladder and
abdominal wall without pelvic osteotomies [5]. In older infants,
however, pelvic osteotomies are necessary to allow the release of
soft tissue tension and to approximate the symphysis pubis in the
midline [6±8]. The bony repair helps to maintain the bladder neck
and posterior urethra in the pelvis and improves continence by
approximating the pelvic floor muscles [4,9±11]. The inguinal canal
is explored and the frequently occurring hernia is repaired [12].
Repair of epispadias
This includes urethroplasty and repair of the dorsal curvature of the
penis. The procedure is usually done at age 12±18 months or
simultaneously with the initial bladder repair. In complicated or
redo reconstructions it is possible to use free grafts from the buccal
mucosa to lengthen the urethra and penis [13,14].
Bladder neck reconstruction
Reconstruction of the bladder neck is usually undertaken at age 4±5
years when the volume of the bladder is above 60 ml. At this age the
child is able to exercise bladder control to achieve continence.
Muscle flaps from the bladder are sutured in the bladder neck area
(the Young-Deeds-Leadbetter technique) and the ureters are
reimplanted proximally to correct the reflux (Cohen technique)
[15±17].
750
E. Segev et al.
Pelvic osteotomy
This is essential to allow bladder exstrophy and abdominal wall
closure, unless the baby is less than 48 hours of age and the pelvis
is flexible enough to allow approximation and closure of the pelvic
rami without an osteotomy.
We use the technique described by Sponseller et al. [18,19],
performing horizontal and vertical osteotomies in each hemi pelvis.
The pelvis is stabilized with a small external fixator, 4.0 mm rod
diameter, and the child remains in traction for 6 weeks [Figure 2A].
The patient is positioned supine on a transparent table. An
iliofemoral incision is performed to expose the iliac bone, and the
apophysis is split and reflected down medially and laterally to the
sciatic notch. A Salter [20] osteotomy is done using a Gigli saw
between the anterior-superior iliac spine and the anterior-inferior
iliac spine, and two 2 mm K-wires are inserted into the supraacetabular bone. A vertical osteotomy of the anterior cortex only is
then carried out in the iliac wing parallel to the sacroiliac joint, and
two 2 mm K-wires are inserted into the iliac wing [Figure 1 C,D].
The bladder is repaired leaving the urethral catheter, cystostomy
and ureteral stents for full urinary drainage. The pubic bones are
approximated in the midline with a thick non-absorbable mattress
suture, and an external fixator frame is applied to the pelvis to keep
the symphysis pubis together [Figure 2 B,C].
The child is transferred to the intensive care unit for a few days
and remains in modified buck traction of both legs for 6 weeks (hips
in slight flexion and knees straight), after which the frame is
removed and rehabilitation is initiated. The patient resumes
gradual weight-bearing with range of motion exercises for the hips;
hydrotherapy is encouraged. Full unrestricted ambulation is
allowed 3 months after surgery.
Patients and Results
We describe the surgical approach and report the outcome in four
children whose mean age at surgery was 19 months (range 9±33).
The average follow-up is 39 months (range 10±60). All four patients
had successful bladder repair with no dehiscence, and two of them
achieved partial continence. Bladder neck reconstruction is planned
IMAJ
.
Vol 6
.
December 2004
Original Articles
Table 1. Patients' data, procedures and outcome
Patient
(gender)
1 (M)
Date of birth
1 January 1998
2 (F)
11 May 1999
3 (M)
16 March 1998
4 (M)
9 January 2001
Diagnosis
Bladder
exstrophy,
epispadias
Bladder
exstrophy
Bladder exstrophy
(failure of 2 previous
procedures)
Bladder exstrophy
Mean
Age and Surgery
12 mo. Double
osteotomy, exstrophy,
epispadias repair
9 mo. Double
osteotomy,
exstrophy repair
33 mo. Double
osteotomy,
exstrophy repair
22 mo. Double
osteotomy,
exstrophy repair
19 mo
for the other two patients. All four patients are independent
walkers, although they have a wide-based gait. The pelvic X-rays
show some degree of widening of the space between the pubic
bones with no effect on the bladder repair [Figure 2 C].
With regard to complications of surgery, three of the four
patients suffered unilateral neurologic injury, and two completely
recovered. The third has a combination of quadriceps weakness and
drop foot, and electromyographic studies revealed root nerve injury
at the level of the spine from L2 to L5.
All the parents expressed satisfaction with the results of surgery
and with the improvement in the child's quality of life [Table 1].
Discussion
The success of the first attempt at bladder closure has a significant
affect on the chances of achieving continence by later surgery
[21,22]. One failure of closure will reduce the chance for continence
to 40% and a second failure will further reduce this chance to 20%
[23].
Bladder neck reconstruction will result in continence for 75±85%
of patients after a period of training (normally one year); the
operation is considered to have failed if this is not achieved after 2
years [10,24,25]. The majority of patients in whom the bladder neck
reconstruction had failed will need urinary diversion (Mitrofanoff
technique), bladder augmentation and bladder neck closure. After
successful reconstruction surgery, the patient will be continent with
intermittent self-catheterization every 4±6 hours for his/her entire
lifetime.
Most epispadias repairs and penis reconstructions (CantwellRansley technique) are successful, and additional skin flaps can be
used to improve the length and appearance of the penis. According
to reports in the literature, most patients will achieve continence
and normal kidney function, and the external genitalia can be
reconstructed with good cosmetic appearance and, in most cases,
capability of sexual activity.
The combination of anterior and posterior innominate
osteotomies has several advantages over previous techniques.
The posterior osteotomies were performed with the patient
initially in a prone position so that he/she had to be turned on
IMAJ
.
Vol 6
.
December 2004
Follow-up
60 mo
48 mo
38 mo
10 mo
39 mo
Outcome
Good bladder
volume;
dry for 1.5 hours
Good bladder
volume
Small bladder
volume,
dry for 1 hr
Good bladder
volume
Complications
Peroneal palsy
resolved at
6 weeks
Peroneal palsy
resolved at
6 weeks
Peroneal palsy,
femoral paresis
not resolved
the back to complete the surgery. Mobilization of the pelvic
bones is better with anterior osteotomies compared to posterior
osteotomies, and the combination of anterior and vertical
posterior osteotomies allows even better correction of the
distorted bony anatomy [19]. The anterior supra-acetabular
osteotomies as described by Salter [20] are well known and
commonly practiced by pediatric orthopedic surgeons, and the
addition of posterior osteotomies through the same surgical
exposure simplifies the procedure.
The use of a small external fixator as opposed to a cast for
stabilization of the pelvic bones provides room for the anticipated
postoperative swelling. There is free access to the abdomen and
genitalia, and the patient can be turned in bed. The fixator is easily
removed in the ward with the use of light sedation after union of the
osteotomies has been established. This technique has been
modified and is used in older patients (age range 11±16 years);
gradual closure of the pelvis with an external fixator has enabled
successful cloacal extrophy repair after previous failure in adolescent patients.
As we encountered during the follow-up of our cases and as
reported in the literature, there may be some recurrence of the
symphysis pubis diastasis. A dense fibrous tissue develops to
bridge the gap between the symphysis rami, however, and this has
no affect on the soft tissue repair, which has stabilized itself [19].
We believe that the neurologic complications in three of our
patients were the result of the traction injury to the sciatic and
femoral nerves that occurs when the pelvic bones are approximated
together.
Treatment by a team of pediatric orthopedic surgeons and
urologists is a key factor for successful vital initial reconstruction of
this difficult defect.
References
1. Gearhart JP, Ben-Chaim J, Jeffs RD, Sander RC. Criteria for prenatal
diagnosis of bladder extrophy. Obstet Gynecol 1995;85:961±4.
2. Jaffe R, Schoenfeld A, Ovadia J. Sonographic finding in the prenatal
diagnosis of bladder extrophy. Am J Obstet Gynecol 1990;162:675±8.
3. Stec AA, Pannu HK, Tadros YE, et al. Evaluation of the bony pelvis in
Modified Osteotomy for Bladder Exstrophy
751
Original Articles
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
15.
16.
classic bladder exstrophy by using 3D-CT: further insights. J Urol
2001;58:1030±5.
Stec AA, Pannu HK, Tadros YE, Sponseller PD, Fishman EK, Gearhart JP.
Pelvic floor anatomy in classic bladder exstrophy using 3-dimensional
computerized tomography: initial insights. J Urol 2001;166:1444±9.
Ansell JS. Surgical treatment of extrophy of the bladder with emphasis
on neonatal primary closure: personal experience with 28 consecutive
cases treated at the University of Washington hospital from 1962 to
1977: techniques and results. J Urol 1979;121:650±3.
Gearhart JP, Forschner DC, Sponseller PD, Jeffs RD, Ben-Chaim J.
Combined vertical and horizontal pelvic osteotomy approach for the
initial and secondary repair of bladder exstrophy. J Urol 1996;155:689±93.
Gokcora IH, Yazar T. Bilateral transverse iliac osteotomy in the correction
of neonatal bladder extrophies. Int Surg 1989;74:123±5.
McKenna PH, Khoury AE, McLorie GA, Churchil BM, Babyn PB, Wedge
JH. Iliac osteotomy; a model to compare the options in bladder and
cloacal extrophy reconstruction. J Urol 1994;151:182±6.
Osterling JE, Jeffs RD. The importance of a successful initial bladder
closure in the surgical management of classic bladder exstrophy:
analysis of 144 patients treated at The Johns Hopkins Hospital between
1975 and 1985. J Urol 1987:137:258±62.
Gearhart JP, Jeffs RD. State of the art reconstructive surgery for bladder
exstrophy at the Johns Hopkins Hospital. Am J Dis Child 1989;143:1475±
8.
Jeffs RD, Guice SL, Oesch I. The factors in successful extsophy closure.
J Urol 1982;127:974±6.
Connolly JA, Peppas DS, Jeffs RD,Gearhart JP. Prevalence and repair of
inguinal hernia in children with bladder exstrophy. J Urol 1995;154:1900±
1.
Hendren WH. Penile lengthening after previous repair of epispadias.
J Urol 1979;121:527±34.
Thomalla JV, Mitchell ME. Ventral preputial island flap technique for the
repair of epispadias with or without extrophy. J Urol 1984;132:985±7.
Peppas DS, Blanch-Tgien M, Jeffs RD, et al. Collagen smooth muscle
rations in the bladder exstrophy patient J Urol 1989;162:2119±22.
Lee BR, Perlman E, Partin AW, Jeffs RD, Gearhart JP. An analysis of
17.
18.
19.
20.
21.
22.
23.
24.
25.
collagen subtypes in the newborn exstrophy bladder. J Urol 1996;
156:2034±6.
Canning DA, Gearhart JP, Peppas DS, Jeffs RD. The cephalotrigonal
reimplant in bladder neck reconstruction for patients with exstrophy or
epispadias. J Urol 1993;150:156±8.
Gearhart JP, Forschner DC, Jeffs RD, Ben-Chaim J, Sponseller PD. A
combined vertical and horizontal pelvic osteotomy approach for primary
and secondary repair of bladder exstrophy. J Urol 1996;155:689±93.
Sponseller PD, Jani MM, Jeffs RD, Gearhart JP. Anterior innominate
osteotomy in repair of bladder exstrophy. J Bone Joint Surg Am 2001; 83A:184±93.
Salter RB. Role of innominate osteotomy in the treatment of congenital
dislocation and subluxation of the hip in the older child. J Bone Joint Surg
1966;48-A(7):1413±39.
Low FC, Jeffs RD. Wound dehiscence in bladder exstrophy: an
examination of the etiologies and factors for initial failure and
subsequent success. J Urol 1983;130:312±15.
Gearhart JP, Ben-Chaim J, Sciortino C, Sponseller PD, Jeffs RD. The
multiple bladder exstrophy closure: what affects the potential of the
bladder? J Urol 1996;47:240±3.
Gearhart JP, Peppas DS, Jeffs RD. The failed exstrophy closure: strategy
for management. Br J Urol 1993;71:217±20.
Lottman H, Melin Y, Beeze-Beyrie P, et al. Is it possible to achieve
continence with spontaneous voiding? A restrospective study of 57
cases. European Society of Pediatric Urology. London, March 1996,
Abstract #102.
Connors JP, Hensle TW, Lattimer JK, Bubridge KA. Long-term follow-up
of 207 patients with bladder exstrophy: an evolution in treatment. J Urol
1989;142:793±5.
Correspondence: Dr. E. Segev, Dept. of Pediatric Orthopedics, Dana
Children Hospital, 6 Weizmann Street, Tel Aviv 64239, Israel.
Phone: (972-3) 697-4261
Fax: (972-3) 697-4542
email: [email protected]
God gives, but man must open his hand
German proverb
Capsule
NF-kB and tumorigenesis
There has been a resurgence of interest in the concept that
inflammatory mechanisms can profoundly affect the pathogenesis of many common human diseases. In the case of cancer,
much research has focused on the role of NF-kB, a transcription
factor that is normally activated in response to pro-inflammatory
cytokines and that regulates the expression of more than 200
genes. Many tumor cell lines show constitutive activation of NFkB signaling, but there has been conflicting evidence as to
whether this promotes or inhibits tumorigenesis. Three groups
have studied mouse models of intestinal (Greten et al., Cell
2004;118:285), liver (Pikarsky et al., Nature 2004;10.1038/nature02924), and mammary (Huber et al., J Clin Invest 2004;114:569)
tumors; they conclude that activation of the NF-kB pathway
752
E. Segev et al.
enhances tumor development and may act primarily in late
stages of tumorigenesis. Inhibition of NF-kB signaling uniformly
suppressed tumor development but, depending on the model
studied, this salutary effect was attributed to an increase in
tumor cell apoptosis, reduced expression of tumor cell growth
factors supplied by surrounding stromal cells, or abrogation of a
tumor cell dedifferentiation program that is critical for tumor
invasion/metastasis. Although collectively these results support
the development of NF-kB inhibitors as potential anticancer
agents, they illustrate that such inhibitors could have complex
physiologic effects.
E. Israeli
IMAJ
.
Vol 6
.
December 2004