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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_5514_Библиотеки_им_академика_М_И_Перельмана.pdf
Osteotomies forBladder Exstrophy
12
B.DavidHorn, SoroushBaghdadi,
PiyushSudamalalMittal, VinodGautam,
andStefanoCardin
Introduction
Bladder exstrophy-epispadias complex (BE) is a complex congenital anomaly
occurring with an incidence of approximately 1in 50,000 live births [20, 24]. BE is
considered a spectrum of disorders, ranging from the milder epispadias to the more
severe cloacal exstrophy (OIES syndrome). BE also affects multiple organ systems,
including the musculoskeletal, genitourinary, and gastrointestinal systems [24, 26].
Historically, BE resulted in signicant morbidity and mortality, although
Supplementary Information The online version contains supplementary material available at
https://doi.org/10.1007/978- 3- 031- 91238- 2_12.
B. D. Horn (*)
Department of Orthopedic Surgery, The Children’s Hospital of Philadelphia,
Philadelphia, PA, USA
e-mail: hornd@chop.edu
S. Baghdadi
Department of Orthopedic Surgery, UCLA, Los Angeles, CA, USA
e-mail: sbaghdadi@mednet.ucla.edu
P. S. Mittal
Department of Orthopaedic, Civil Hospital, B.J.Medical College & Government Spine
Institute, Ahmedabad, Gujarat, India
V. Gautam
Department of Orthopedics, Government Medical Collage, Gujarat, India
S. Cardin
Division of Pediatric Orthopaedics, Orlando Health– Arnold Palmer Hospital for Children,
Orlando, FL, USA
e-mail: stefano.cardin@orlandohealth.com
© The Author(s), under exclusive license to Springer Nature
Switzerland AG 2025
A. R. Shukla, R. S. Joshi (eds.), Bladder Exstrophy and Epispadias,
https://doi.org/10.1007/978-3-031-91238-2_12
199
200
B. D. Horn et al.
advancements in treatment have improved life expectancy as well as the quality of
life for individuals with BE.Despite these advances, however, individuals with BE
continue to face challenges related to urinary continence, fertility, and social stigma
[6, 24].
BE is a diverse condition inuenced by various genetic pathways [26]. BE’s
embryological pathogenesis is intricate, but an understanding of this helps shed
light on anatomical distinctions crucial for orthopedic interventions.
During embryonic development, the cloacal membrane forms through the fusion
of ectodermal and endodermal layers in the fourth week of gestation. The subsequent growth of the mesodermal layer, responsible for the musculoskeletal system,
forms the urorectal septum, dividing the cloacal membrane into urogenital and anal
membranes. Anomalies in this process are believed to stem from decient mesodermal growth as well as from disruption in the formation of the cloacal membrane [24,
26]. The failure of separation of the urogenital and rectal membranes leads to the
complex anomalies observed in BE.These include failure of closure of pubic diastasis (closure normally occurs by the eighth week) as well as the presence of an
exstrophied bladder [21, 23, 26].
From an orthopedic perspective, it’s also important to recognize that while the
pelvic oor muscles exist, they have an atypical orientation. These muscles function
to support pelvic organs, but in BE they function as a hammock that pushes organs
outward through the exstrophy. This altered role is important in managing BE, particularly during diastasis closure [22, 25, 27].
Earlier treatment approaches primarily focused on covering or closing the exstrophy, yielding high recurrence rates and complications such as wound and bladder
dehiscence. Only in the latter half of the twentieth century did surgeons understand
the importance of pelvic osteotomy to help decrease the pubic diastasis, prompting
the involvement of orthopedic surgeons in the multidisciplinary management of BE
[23, 24].
Patho-anatomy ofthePelvis inBladder Exstrophy
The most obvious osseous feature of BE is the presence of pubic diastasis. While
orthopedic surgeons are more accustomed to encountering traumatic causes of
pubic diastasis, such as open book pelvic fractures, it’s essential to recognize that
the anatomical distinctions in BE extend beyond this characteristic nding [20, 23].
In BE, the pelvis exhibits anterior deciency and external rotation across all of
its segments. Detailed analysis of pelvic CT scans in patients with BE revealed an
average 30-degree external rotation of each hemipelvis, with the posterior segment
(the iliac wing) contributing approximately 12° and the anterior segment around 18°
to this rotation. In more severe cases, particularly in cases of OEIS syndrome, the
posterior segment showed an average of 25° and the anterior segment 24° of external rotation, respectively, compared to healthy controls. Additionally, the pubic
rami in BE are found to be, on average, 30% shorter than in patients of the same age
without BE.There is a mean of 4.2cm of diastasis at birth in BE and 7.2cm in
12 Osteotomies forBladder Exstrophy
Fig. 12.1 Bony changes
seen in exstrophy,
including (A) increased
inter-triradiate distance,
(B) shortened pubic rami,
and (C) external rotation of
anterior and posterior
pelvis
201
OEIS, which progressively increases with age, reaching a mean of 14.2cm in adulthood (Fig.12.1) [20, 22, 26].
Another characteristic feature observed in BE is acetabular retroversion, typically measuring an average of 13° compared to healthy controls, with potential
increases into adulthood. This acetabular retroversion, coupled with decreased
internal rotation of the hip, contributes to a foot progression angle that may exceed
20° of external rotation [20, 28]. Additional deformities noted in BE patients include
increased inter-triradiate distance, rotation and deformity of the sacroiliac joints,
and sacral dysmorphism. In summary, the average BE pelvis is characterized by a
posterior segment of normal size, a shortened anterior segment, and external rotation across all segments [14, 24].
Summary of key pelvic anomalies in CBE (Fig.12.1):
• Posterior segment externally malrotated by 12°
• Anterior segment externally malrotated by 18°
• Retroverted acetabula by 15°
• Pubis foreshortened by 30%
• Increased intra-pelvic volume by 30–40%
• Pubis diastasis of 4.2cm at birth and 14.2cm in adulthood
Osteotomies inBEReconstruction
As previously discussed, the complex patho-anatomy of BE coupled with its rarity
and variability in severity make soft tissue correction alone challenging. It wasn’t
until the 1960s that the benets of osteotomies and reapproximating the pubic
diastasis became apparent. Over time, closure of the pelvic ring at the time of urological repair has been shown to have multiple benets. These include decreased
rates of bladder dehiscence, decreased tension on the wound, increased continence
rates since the urethra and bladder neck can be placed within the bony pelvis,
improved genital reconstruction because of decreased tension on the hemi corpi,
and decreased rates of late uterine prolapse. Other theoretical advantages include
improved gait, decreased rates of sacroiliac joint arthritis, and decreased rates of
202
B. D. Horn et al.
hip arthrosis [14, 23, 24, 26]. Shultz, in 1958, reported on the rst use of pelvic
osteotomies in BE reconstruction [19]. The initial reports described posterior osteotomies, including Lloyd Roberts in 1959 and O’Phelan in 1963 [10, 16]. The
anterior approach was rst described in 1989 with superior pubic rami osteotomies
[4, 17]. This technique gained traction in the 1990s in Europe and was typically
performed concomitantly with urologic reconstruction by urologists. This procedure involves cutting the pubis medial to the iliopectineal eminence, within the
obturator foramen, and closure with sutures. However, a drawback of this technique is its failure to address external rotation of the pelvis. Overall, the low complication rates and improved outcomes associated with the use of osteotomies have
made pelvic osteotomies integral to the care of patients with BE [24, 26].
In general, pelvic osteotomies should be strongly considered in all infants and
children more than 72hours old and in newborn infants with a large pubic diastasis
[14, 24]. Osteotomies can be performed either anteriorly or posteriorly. Currently,
all of the osteotomies, whether posterior or anterior, have the disadvantage of
decreasing intra-pelvic volume [9]. This can make placement of the reconstructed
bladder and bladder neck into the pelvis challenging, potentially leading to ischemia of the reconstructed bladder and genitalia.
Posterior osteotomies involve bilateral vertical bony cuts just lateral to the sacroiliac joints. This approach requires prone positioning, so the patient must be repositioned after the osteotomies are performed to allow for the urological reconstruction.
Advantages include potential improvement in the acetabular retroversion, although
there are concerns that correction may be limited by the soft tissue surrounding the
osteotomized segments.
Various anterior iliac osteotomies have been described, including bilateral Saltertype osteotomies extending from just above the anterior inferior iliac spine (AIIS)
to the sciatic notch [14, 21], as well as a more oblique osteotomy propagating diagonally from just posterior to the anterior superior iliac spine (ASIS) to the sciatic
notch [5, 8, 11]. These are all shown to be successful. In addition, double pelvic
osteotomies have been described [14] (Fig. 12.2). These osteotomies will be
described in greater detail later in the chapter.
Regardless of the exact technique performed, there are several common preoperative considerations that need to be considered before performing pelvic osteotomies for BE reconstruction. There is approximately a 5% rate of hip dysplasia
associated with BE.Patients should be screened for this with a hip ultrasound 4–6
weeks post gestational age [13]. In addition, plain X-rays consisting of an AP pelvis
should be obtained to aid in surgical planning. In more complex cases, such as cloacal exstrophy (OEIS) or revision surgery, axial imaging like CT or MRI scans may
be helpful to further evaluate pelvic anatomy and search for associated pathologies
such as SI joint dysplasia. Close coordination with the urological team is also vital
to optimize surgical ow and minimize operative time. Finally, patient factors
should be optimized prior to surgery. Patients with cloacal exstrophy, for example,
may have nutritional issues that should be addressed prior to undertaking a complex
reconstruction.
12 Osteotomies forBladder Exstrophy
Fig. 12.2 Location of
commonly performed
pelvic osteotomies.
Red—posterior, Blue—
oblique, Yellow—anterior
and Ahmedabad, Red +
Yellow—double anterior
203
Intraoperative pubic reduction xation and postoperative immobilization should
also be considered in the treatment plan. Many different strategies have been
described and used with success. Intraoperative xation to maintain pubic reduction
may involve the use of large absorbable sutures, internal xation, and/or external
xation. Postoperative immobilization may include skin traction, including modied Bryant’s traction, spica casting, and bracing [24].
The role of osteotomies in the treatment of BE is to facilitate the urological
reconstruction. The primary goals, therefore, are urological in nature: enhanced
healing of the bladder and abdominal wall, urinary continence, renal health, and
improved genital function and appearance. Many studies demonstrate improved
outcomes when pelvic osteotomies are performed in conjunction with repair of BE
[1, 6, 24]. From an orthopedic perspective, the pubic diastasis invariably recurs.
This is typically accompanied by external rotation of the pelvis and acetabular retroversion. To date, these differences seem to be well tolerated and have not been
associated with decreased ambulatory function, activity level, pain, or hip arthrosis
[14, 24, 26]. It is important to bear in mind, however, that the longest follow-up is
only in individuals in the fourth decade of life, so true long-term data are not available for this population.
Complications may occur regardless of the specic osteotomy performed. There
seems to be an overall 4–5% complication rate associated with osteotomies themselves. These include wound and pin tract infections, skin complications from traction or casting, and nerve palsy (particularly femoral nerve palsy, which was
transient) [15, 18].
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B. D. Horn et al.
Posterior Iliac Osteotomies
• Indications:
– Patients with classic BE <1year of age.
• Pre-op Imaging:
– Hip ultrasound.
– AP pelvis X-ray (Fig.12.3).
– Pelvis CT or MRI as indicated.
• Timing
– The osteotomies are usually performed prior to the urologic repair.
– The osteotomies can be performed 3–7days prior to the urological procedure
or at the same time as the urological surgery (the latter requires repositioning
the patient supine for the exstrophy repair).
• Positioning:
– The patient is placed prone on gel rolls with all bony prominences appropri-
ately padded (Fig.12.4).
• Approach:
– Two parallel longitudinal skin incisions are made. Each should be 4–5cm in
length and about 1cm lateral to the PSIS (Fig.12.5).
– Dissection is performed through subcutaneous fat until deep fascia is reached.
– Electrocautery is then used to dissect the muscle bers of the paralumbar
muscles off the posterior iliac crest to expose the iliac apophysis.
– A 15-blade is then used to split the apophysis (the pelvis orientation is verti-
cal). To split the pelvic apophysis, the knife handle will be almost parallel to
the body.
– A small periosteal elevator is used to subperiosteally dissect the inner and
outer iliac tables until the sciatic notch is reached on both sides.
Fig. 12.3 Preoperative
radiograph of a 3-monthold girl shows a widened
pubic diastasis
characteristic of BE
12 Osteotomies forBladder Exstrophy
Fig. 12.4 Positioning for
posterior iliac osteotomies.
The hips should be exed
to improve access to the
posterior iliac crests
Fig. 12.5 Incisions for
posterior osteotomies
205
• The surgeon should be aware that the notch tends to be located more medially than expected when dissecting the outer table.
• In contrast, the notch tends to be located more laterally than expected during the inner table dissection.
– Once the notch is identied, small Hohmann retractors or periosteal elevators
are used to protect its contents.
• Osteotomy:
– A vertical osteotomy is then performed.
• This may be created by using small rongeurs, osteotomes, or an oscillating saw.
• Small osteotomes are used to safely complete the osteotomy.
• Laminar spreaders may be used to conrm the completion of the osteotomy.
– Completion of the osteotomy is then veried by hinging the ilium anteriorly,
which will result in the osteotomy opening posteriorly.
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B. D. Horn et al.
– Closure is then performed in layers.
– Care should be taken to close the apophysis over the ilium in a separate layer.
• The overlying fascia is then closed, followed by subcutaneous tissue and
skin closure.
– Repeat the surgical technique on the contralateral side.
• Pubic Symphysis Diastasis Reduction:
– Performed at the time of the urological procedure.
– The patient is positioned supine once both osteotomies are performed, and the
posterior incisions are closed.
– At the appropriate time in the urology procedure, the pubic symphysis diasta-
sis is typically reduced with manual reduction (a pointed bone reduction
clamp may also be used).
• Pubic diastasis reduction is facilitated by pushing on the greater trochanters of each hip.
– The reduction is then held by a large, monolament, absorbable suture
between the pubic bones. This helps stabilize the posterior osteotomies.
– At the conclusion of the procedure, the patient is placed into either a hip spica
or a previously made hip spica brace (Fig.12.6).
• Postoperative immobilization
– Either a spica cast or a spica brace may be used.
– Spica brace is the author’s preference—two techniques
• Custom brace fabricated by an orthotist 1–2 weeks prior to surgery
(Fig.12.7)
Fig. 12.6 Postoperative
radiograph demonstrating
posterior osteotomies
(dotted lines) and
decreased pubic diastasis
12 Osteotomies forBladder Exstrophy
Fig. 12.7 Custom
fabricated spica brace
207
• Bivalved spica cast was placed on the day of surgery
– The procedure begins with molding for the hip spica brace prior to the
beginning of surgical repair.
– The patient is placed on a spica table, and a double hip spica cast is
applied. Care is taken to avoid excessive hip abduction and to keep the
hips as adducted as possible. Hip exion should be 30–60°.
– The cast is then bivalved and a hip spica brace is then made from the
bivalved spica (Fig.12.8).
• Postoperative Care:
– Patient remains in spica brace full time for 5days.
– The brace may then be removed three times per day for skin checks.
– The total time of immobilization in a Spica brace is 4–6weeks (clinical pic at
the end of the video).
– Spica brace allows access for wound care, drains, and hygiene.
– First postoperative radiographs performed at 4weeks (postop AP XR pic).
208
Fig. 12.8 Bivalved spica
cast brace
B. D. Horn et al.
Anterior/Double Iliac Osteotomies [3, 14]
• Indications
– Single
• Patients with classic BE <1year of age.
• Patients >1year of age with Cloacal Exstrophy or Epispadius with diastasis <4cm.
– Double
• Substantial diastasis of >4cm.
• Late or failed closure of BE in a patient who is >28days old.
• Aesthetic purposes for a patient who is >8years old.
• Females with BE and accompanying cervical prolapse.
• Cloacal exstrophy (typically performed when a child is >18months old).
• The double osteotomy is usually performed 7–14days before the urological procedure and stabilized with external xation.
• The diastasis is then gradually reduced by adjusting the xator until the
urology surgery.