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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_5514_Библиотеки_им_академика_М_И_Перельмана.pdf
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- •Foreword
- •Foreword
- •Preface
- •Contents
- •Contributors
- •Introduction
- •Epidemiology
- •Etymology
- •Bladder Exstrophy Pathophysiology
- •Conclusion
- •References
- •Normal Development
- •Introduction
- •Prenatal Imaging
- •Prenatal Counseling
- •Epispadias
- •Classic Bladder Exstrophy
- •Cloacal Exstrophy
- •BEEC Variants
- •Prenatal Management
- •Genetic Counseling
- •Conclusion
- •References
- •3: Bladder Exstrophy Genetics: Our Current Understanding
- •Bladder Exstrophy Genetics
- •Copy Number Variant (CNV) Studies
- •Gene Expression Studies
- •Genome-wide Association Study (GWAS)
- •Future Directions
- •References
- •4: Prenatal and Postnatal Imaging of the Bladder Epispadias-Exstrophy Complex
- •Introduction
- •Prenatal Imaging Findings
- •Bladder Exstrophy
- •Cloacal Exstrophy
- •Isolated Epispadias
- •Exstrophy Variants
- •Postnatal Imaging Findings
- •Urinary System
- •Musculoskeletal System
- •Spine
- •Conclusions
- •References
- •Introduction
- •Bladder Growth
- •Urinary Continence
- •Conclusions
- •References
- •6: Complete Primary Repair of Bladder Exstrophy and Epispadias
- •Bladder Neck Reconstruction, Bladder/Urethral Closure
- •Pubic Bone Closure
- •Umbilicoplasty
- •Immobilization
- •Urethral Plate Dissection
- •“Grady Monsplasty”
- •Complications
- •Conclusion
- •References
- •Introduction
- •Prenatal Diagnosis
- •Anatomic Anomalies
- •Immediate vs Delayed Closure
- •Surgical Reconstruction
- •Immobilization Techniques
- •Epispadias Repair
- •Achieving Urinary Continence
- •Proposed Follow-Up
- •Future Directions
- •Conclusion
- •References
- •8: The Kelly Procedure
- •Introduction
- •Tension-Free Bladder Neck Construction
- •Postoperative Management
- •References
- •Introduction
- •Anesthesia
- •Incision
- •Bladder Plate Mobilization
- •Radical Corporal Detachment
- •Osteotomy
- •Ischiopubic Osteotomy
- •Transverse Innominate Osteotomy
- •Corporal-Urethral Separation
- •Reconstruction
- •Postoperative Management
- •Follow-Up
- •Results
- •Conclusion
- •References
- •Introduction
- •Surgical Procedures
- •References
- •Bilateral Ureteral Advancement Reimplantation
- •Pelvic Osteotomy
- •Preoperative Testosterone Administration
- •Epispadias Repair
- •Penile Skin Reconstruction
- •Continence Enhancement
- •Conclusion
- •Introduction
- •Background
- •Modified Perineal Approach Surgical Technique
- •Discussion
- •References
- •Introduction
- •Posterior Iliac Osteotomies
- •Anterior/Double Iliac Osteotomies [3, 14]
- •Anterior Oblique Iliac Osteotomies [5, 11]
- •Anterior Bilateral Superior Pubic Rami Osteotomies [4]
- •Postoperative Immobilization
- •Complications/Long-Term Outcomes
- •References
- •Ureteral Reimplantation
- •Inguinal Hernia
- •Monsplasty
- •Umbilicoplasty
- •References
- •Introduction
- •Ureterosigmoidostomy
- •The Sigma-Rectum Pouch (Mainz Pouch II)
- •The Cologne Pouch
- •Conclusion
- •References
- •15: Cloacal Exstrophy
- •Introduction
- •Epidemiology
- •Embryologic Etiology
- •Prenatal Findings
- •Urinary
- •Gastrointestinal
- •Neurologic
- •Musculoskeletal
- •Genital
- •Management
- •Neonatal
- •Surgical Reconstruction
- •Secondary Procedures
- •Outcomes
- •Urinary Continence
- •Renal
- •Fecal Continence
- •Gender Rearing
- •Nutrition
- •Mobility
- •Psychosocial Outcomes
- •Conclusion
- •References
- •16: Male Epispadias
- •Embryology
- •Anatomic Features
- •Epispadias Repair
- •Pelvic Osteotomy
- •Modified Cantwell-Ransley Repair
- •Urethral Reconstruction
- •Bladder Neck Reconstruction
- •The Mitchell Repair
- •Initial Dissection
- •Penile Disassembly
- •Proximal Dissection
- •Bladder Neck Reconstruction
- •Primary Closure
- •Skin Closure
- •Outcomes
- •Fistula Formation
- •Urethral Stricture
- •Residual Curvature
- •Urinary Continence
- •Sexual Function
- •Renal Function
- •Female Epispadias
- •Outcomes
- •Conclusion
- •References
- •Introduction
- •Pre-operative Factors
- •Technical Aspects
- •Management
- •Failed Genital Reconstruction
- •Ureterosigmoidostomy
- •Augmentation Cystoplasty
- •References
- •Background
- •Preoperative
- •Monitoring
- •Intraoperative Management
- •Postoperative Management
- •Conclusion
- •References
- •Mental Health Concerns
- •Local Priority
- •Resources
- •Clinical Care
- •Capacity Building
- •Research
- •General Principles
- •References
- •Introduction
- •Defining Continence
- •Continence versus Dryness
- •Dry Interval: How Long Is Long Enough?
- •Dry Intervals: What Is Meaningful
- •Diversion Versus Continence
- •Timing
- •Challenging Dogma
- •References
- •Introduction
- •Preoperative Counseling
- •Bladder Neck Bulking Agent Injection
- •Artificial Urinary Sphincter
- •Bladder Neck Reconstruction
- •Bladder Neck Closure
- •Continent Catheterizable Channel: Mitrofanoff Principle
- •Augmentation Cystoplasty
- •Continent Urinary Diversion
- •References
- •22: Urinary Reconstruction for Bladder Exstrophy in the Developing World: Special Consideration and Technique
- •Introduction
- •Operative Technique
- •The Final Reconstruction
- •Young-Dees-Leadbetter Bladder Neck Plasty
- •Bladder Neck Closure
- •Operative details
- •Discussion
- •Conclusion
- •References
- •Introduction
- •Nephrology Evaluation
- •Measuring Kidney Function
- •Evaluating Blood Pressure
- •Imaging Studies
- •Transplant
- •References
- •Introduction
- •Post-operative Nursing Care
- •Pain Control
- •Immobilization
- •Orthopedic Care
- •Parental Teaching
- •Conclusion
- •Bibliography
- •Introduction
- •Pelvic Floor Musculature
- •Physical Therapy Evaluation
- •Participation
- •Activity
- •Impairment
- •Physical Therapy Intervention
- •Pre-toilet Training
- •Toilet Training
- •Post-toilet Training
- •Day Versus Night
- •Constipation
- •References
- •Pediatric Psychology
- •Infancy
- •Childhood
- •Adolescence
- •Adulthood
- •Future Directions
- •References
- •Females
- •Males
- •Erectile Function
- •Ejaculatory Function
- •Recommendations
- •Literature
- •Gynecologic Anatomy
- •Puberty
- •Pelvic Organ Prolapse
- •Fertility
- •Obstetric Considerations
- •Conclusions
- •References
- •Introduction
- •Patient Advocacy
- •Peer Support
- •Local Support Groups
- •Medical Advisory Council
- •Annual Conferences
- •Global Health Inequities
- •Global Health Initiatives
- •Advocacy Considerations
- •Patient-Directed Research
- •Patient Advisory Councils
- •Conclusion
- •References
- •Index

12 Osteotomies forBladder Exstrophy
• Pre-op Imaging:
– Hip ultrasound.
– AP pelvis X-ray.
– Pelvis CT or MRI as indicated.
• Timing:
– For younger patients (<12months) or with a diastasis <4cm, the procedure
may be performed in one stage at the time of the urological procedure.
– For older patients (>12months) or diastasis >4cm: Staged Closure.
• In older children or those with a more severe pubic diastasis, it may not be
possible to achieve immediate closure of the pubic symphyseal diastasis.
• In such cases, closure may need to be done in stages. This involves using
an external xator and gradually adjusting the bar to bring the pubic bones
closer together over 7–14days.
• Positioning:
– Patient is positioned supine on an operating room table that allows for uoro-
scopic visualization of the pelvis.
– A small (3–4 diameter) gel roll or towel bump is placed midline under
the sacrum.
– The bladder is isolated using a sterile drape.
– Both legs are placed in sterile stockinette to help keep the patient warm
(Fig.12.9).
– The pelvis is prepped and draped in a usual sterile fashion.
• Approach:
– A 5cm bikini-style incision is performed just below each ASIS along the
inguinal crease and beneath the iliac crest.
• The incision curves proximally to access the iliac crest apophysis.
– Electrocautery is used to dissect through subcutaneous tissue until external
obliques and deep fascia are clearly visualized.
209
Fig. 12.9 Both legs are
wrapped in a sterile
stockinette

210
B. D. Horn et al.
– The external obliques are gently dissected away from the iliac crest apophysis.
• Care is taken not to cauterize the cartilage.
– A partial anterior (Smith-Peterson) approach to the hip is used.
• The interval is between the sartorius and tensor fascia lata (TFL) muscles.
• The lateral femoral cutaneous nerve (LFCN) is located within this interval.
• The authors identify the TFL and carefully incise the overlying fascia to
protect the LFCN.Blunt dissection is then used to develop the interval
between the TFL and Sartorius muscles.
– A 15-blade is then used to split the iliac apophysis midline.
– A subperiosteal elevator is used to expose the inner and outer tables of the
pelvis as far as the sciatic notch. Medial dissection on the inner table should
stop just short of sacroiliac joint (SIJ) if a double osteotomy is to be performed.
• Osteotomy
– A 1.1mm diameter smooth Kirschner wire is placed from the ASIS to the
dome of the sciatic notch to serve as a guide for the osteotomy. Fluoroscopy
is used to conrm the proper positioning of this wire. Typically the bony cut
will be just caudal to this wire (Fig.12.10).
– Anterior (Salter-cut) Osteotomy (single):
• Retractors are carefully placed into the sciatic notch to protect its contents.
• The osteotomy is then performed from just below the ASIS to the
Sciatica notch.
• An oscillating saw may be used to perform most of the osteotomy (about
4/5 of its length). Osteotomes are then used to complete the last portion of
the cut under direct visualization.
• In older children, a Gigli saw may be used to perform the cut.
Fig. 12.10 Fluoroscopic
image showing the position
of guide wire prior to the
osteotomy

12 Osteotomies forBladder Exstrophy
Fig. 12.11 Postoperative
pelvis radiograph of a
3-month-old after bilateral
anterior osteotomies
• This osteotomy frees up the distal (acetabular) segment of the pelvis and
allows it to internally rotate (Fig.12.11).
– Incomplete posterior Iliac Osteotomy (for double osteotomy—performed in
conjunction with the anterior (Salter) osteotomy)
• An incomplete vertical osteotomy is then performed about 1cm laterally
to the SIJ by leaving the posterior cortex intact.
• This can be made with a burr, rongeur, or oscillating saw.
• An osteotome can be used distally to free the distal cortical portion of
the bone.
• This creates a “greenstick fracture,” which will be used to hinge the iliac
wing anteriorly while the hemipelvis continues to be internally rotated
(Fig.12.12).
• Application of external xator pins
– Fluoroscopic guidance recommended.
– Two pins are inserted lateral to medial through the internally rotated distal
fragment and parallel to the anterior cut. Bicortical xation should be obtained
with each pin.
– A third pin is then inserted between the inner and outer tables of the ilium in
the iliac wing to stabilize the fragment located between the double osteotomies (Fig.12.13).
• Pubic Symphysis Diastasis Reduction:
– Closure of both incisions is then performed in layers.
• Care is taken to close the apophysis over the ilium in a separate layer.
• The overlying external oblique fascia is then closed, followed by subcutaneous tissue and skin closure.
– For younger patients (<12months) or with a diastasis <4cm. The reduction is
performed in one stage.
211

212
Fig. 12.12 Drawing
illustrating anterior iliac
wing incomplete
osteotomy and correction
of deformity. (From de
Mattos etal. [3])
Fig. 12.13 Fluoroscopic
image showing external
xator pin position
B. D. Horn et al.
• The genitourinary repair is then performed by the urology team.
• After the urological procedure, the pubis symphyseal diastasis is reduced.
– The xator is adjusted and tightened with the pelvis reduced.
– A large, monolament, absorbable suture may also be used to maintain
the pubis symphysis reduction.

12 Osteotomies forBladder Exstrophy
– For older patients (>12months) or diastasis >4cm: Staged Closure
• In older children or those with a more severe pubic diastasis, it may not be
possible to achieve immediate closure of the pubic symphyseal diastasis.
In such cases, closure may need to be done in stages. This involves using
an external xator and gradually adjusting the bar to bring the pubic bones
closer together over 7–14days.
• At the time of the urological procedure, anterior xation may be performed.
– Consider anterior xation in children >2years and in cloacal exstrophy.
– An appropriately sized screw (such as a small pedicle screw) may be
placed in the canal of the superior pubic ramus.
– A small plate or rod can then be used to connect these screws to provide
anterior stabilization (Figs.12.14 and 12.15).
• Postoperative immobilization
– Light skin traction is used (Fig.12.16).
– Assess healing after 4–6weeks.
Fig. 12.14 Anterior
xation with pedicle screw
within canal of superior
pubic ramus in a 4-yearold with cloacal exstrophy
before xator removal
213
Fig. 12.15 6months after
xator removal

214
Fig. 12.16 External
xation and skin traction
after double anterior
osteotomies
B. D. Horn et al.
Anterior Oblique Iliac Osteotomies [5, 11]
• Indications
– Patients with classic BE <1year of age.
– Patients >1year of age with cloacal exstrophy or epispadius with diasta-
sis <4cm.
• Performed at the time of the urological procedure.
• Positioning:
– Same as for anterior osteotomy.
• Approach:
– Same as for anterior osteotomy.
• Osteotomy (Fig.12.17)
– The osteotomy procedure is similar to anterior (Salter-cut) osteotomies except
that it extends from just posterior to the ASIS to the sciatic notch.
• For neonates, a bone cutter and osteotome may be used to create the
osteotomy.
• A Gigli saw or oscillating saw may be used in older children.
• Pubic Symphysis Diastasis Reduction is performed as for anterior osteotomies.
• Postoperative xation/immobilization
– A spica brace/cast may be used.
– External xation along with light skin traction may be used (typically >1year
of age).
– 4–6weeks of immobilization is typically required.

12 Osteotomies forBladder Exstrophy
Fig. 12.17 AP
uoroscopic image
showing trajectory of an
oblique osteotomy
Anterior Bilateral Superior Pubic Rami Osteotomies [4]
• Positioning:
– The patient is prepared and positioned supine on a attop table.
– A rolled towel is placed beneath the patient’s buttock to aid in positioning.
• Approach:
– After urogenital reconstruction is completed, the abdominal skin and its sub-
cutaneous tissues are mobilized through the same incision.
– The insertion of the rectus femoris on the pubis is identied.
– The rectus sheath is incised at the lateral border of its insertion where the
pubis bone is readily encountered.
– The origin of the gracilis muscle and adductor longus, magnus, and brevis is
identied just medial to the origin of the pectineus muscles.
• These originate in the same order medially to laterally starting in the pubis
symphysis.
– An intermuscular approach to the superior pubic ramus is made between
adductor brevis and pectineus muscle.
• This will also be just medial to the insertion of the inguinal ligament.
– The superior pubic ramus periosteum is incised longitudinally along the axis
of the bone and circumferentially carefully elevated.
• Osteotomy:
– Hohmann retractors are placed surrounding the superior pubic ramus deep to
the elevated periosteum.
• This will protect the obturator foramen neurovascular contents.
– A full-thickness osteotomy is performed with the use of osteotomes or a
Gigli saw.
215

216
– The contralateral superior pubic ramus osteotomy is then performed in the
same fashion.
• Pubic Symphysis Diastasis Reduction
– Both osteotomized fragments are freed and tilted toward the midline.
– An absorbable strong suture is used for approximation of the cartilaginous
symphysis with little or no tension.
• Care must be taken to not entrap the already reconstructed posterior urethra within the approximated symphysis.
– An external xator in the form of an A-frame can also be used for further xa-
tion in older patients.
– The rectus muscles and their sheaths are then closed together in the midline
with interrupted suturing.
• The lateral windows previously made for the approach are then closed in
similar fashion.
– The wound is then meticulously irrigated and closed in multiple layers.
B. D. Horn et al.
Ahmedabad Osteotomy Technique (P.Mittal, MD
andV.Gautam MD, Personal Communication, January
22, 2023)
• Indication
– May be useful in low-resource environments—can be done without
uoroscopy.
• Positioning:
– The patient is prepared and positioned supine on a attop table.
– A rolled towel is placed beneath the patient’s ipsilateral buttock to aid in
positioning.
• Approach:
– The osteotomy is performed after mobilization and reconstruction of the blad-
der and bladder neck.
– A 2–4cm incision is performed centered over ASIS, parallel to the iliac crest.
– Electrocautery is used to dissect through subcutaneous tissue until external
obliques and deep fascia are clearly visualized.
– The external obliques are gently dissected away from the iliac crest apophysis.
• Care is taken not to cauterize the cartilage.
– To access the ASIS, a path is created between the TFL and sartorius muscles,
and the lateral cutaneous nerve of the thigh is gently retracted medially.
– Both sides of the ilium are exposed down to the sciatic notch
subperiosteally.
– Retractors are carefully placed into the sciatic notch to protect its contents.
• We do not recommend dividing the psoas or rectus muscle tendons.

12 Osteotomies forBladder Exstrophy
• Pin placement
– Place 3–3.5mm diameter half pins in by hand
• Two on each hemipelvis
– First pin is superior to ASIS in the iliac crest.
– Second pin is inferior to ASIS in the supraacetabular region.
• Pins to be placed at different angles in the axial plane to keep them between
the inner and outer tables.
• Insert by hand and under direct visualization so you can feel when they
reach the far cortex.
– We screen the hip clinically (move each hip through its range of motion while
palpating for crepitus or limitation of motion) to ensure there is no joint
penetration.
– We do not normally use uoroscopy.
• Osteotomy (variation of anterior osteotomy)
– Perform Salter-type osteotomies with osteotomes and mallet.
– Avoid levering downward with the osteotome when nishing the cut near the
sciatic notch until the osteotomy is complete to prevent propagation of
the crack.
– The wound is then meticulously irrigated and closed in multiple layers.
– The urological reconstruction is then completed by the urology team.
– The symphysis is approximated and then held by a large, monolament,
absorbable suture.
• An external xator is then applied (Figs.12.18 and 12.19)
– External xator stays in place for 4–6weeks.
– It is removed when there is satisfactory bone healing.
217
Fig. 12.18 AP Pelvis
radiograph showing
osteotomy and external
xator

218
Fig. 12.19 Postoperative
appearance of xator
B. D. Horn et al.
Postoperative Immobilization
Various immobilization techniques have been explored alongside osteotomies,
ranging from elastic bandages and traction to casts, braces, and external xation.
The primary objective of immobilization is to maintain the pelvis in the desired
position until osteotomies have healed sufciently to withstand external rotatory
forces. For younger children and smaller diastases, a spica cast or brace with the
legs in internal rotation often offers adequate immobilization. However, in more
severe cases, especially in repeat osteotomies, some form of rigid xation may be
necessary [12, 18, 24]. As mentioned, external xation presents an appealing option,
facilitating access to the surgical site for urogenital reconstruction and enabling
gradual correction [12, 14]. In older patients, internal xation can be safely
employed, including traditional symphyseal plating or pelvic inx, spanning the
symphysis [2].
Postoperative immobilization carries inherent risks of complications. Spica casts
can lead to pressure sores, and there exists a theoretical risk of compartment syndrome. Internal xation devices may encounter xation loss due to the softer bone
structure in younger children. Pin tract infections are common with external xation
devices but are typically benign and resolve with proper pin care or pin removal [15].
Complications/Long-Term Outcomes
Long-term outcomes in BE cases are typically contingent upon the severity of
anomalies and the success of urologic reconstruction. The anterior abdominal defect
closure rate reported in the literature stands at 95%, regardless of whether osteotomies are performed. Notably, the Johns Hopkins group reported the highest closure
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