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Orthopaedic Management of Bladder Exstrophy
Jessica J. M. Telleria, MD Resident PYG-1 Department of Orthopaedics and Sports Medicine University of Washington, Seattle, WA, USA Pediatric Surgery Weekly Conference 07/07/2011
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Disclosures No disclosures
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Outline Anatomic anomalies in bladder exstrophy
Indications & goals for surgery Operative approaches Complications Conclusions
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Urology Perspective
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Ortho Perspective
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What do we do with this?
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What is Normal in Exstrophy
Sacral width Iliac segment (posterior Pelvis) length Microscopic histology normal Boney and cartilaginous differentiation & development Endochondral ossification (cartilage model)
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Defects in Boney Anatomy
Pubic diastasis incomplete pelvic ring Mean ~4 cm (birth) 8 cm (10 yrs) Normal 0.6 cm (all ages) Ischiopubic segment (anterior pelvis) is 30% shorter Reduced symphyseal tension/mechanical stress Anterior segment externally rotated extra 18º Posterior segment externally rotated extra 12º
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Defects in Boney Anatomy
Wider hips 31% greater distance between triradiate cartilage Acetabular retroversion 13° retroversion, normal = 0°
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Defects in Boney Anatomy
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Defects in Muscular Anatomy
Obturator internus externally rotated extra 15º Obturator externus externally rotated extra 17º “Frame” for pelvic diaphragm Levator ani 15° greater anterosuperior rotation 68% of puborectus sling is posterior to rectum (normal = 52%) Further from bladder neck less support incontinence Hiatus is 2x wider & 1.3x longer Wider/flatter Greater pelvic organ prolapse
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Why Correct Boney Deformity?
Prior to boney correction well executed soft-tissue repairs subject to complications: Dehiscence/Poor wound healing Fistula formation Wound infection Incontinence Recurrence of exstrophic defect Many related to excess soft tissue tension on bladder/urethra/abdominal wall Pubic diastasis & innominate external rotation
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Goals of Surgery Restore stability to pelvic ring
Close anterior ring Reconstitute “scaffold” for pelvic diaphragm Provide tension-free closure for bladder/soft tissues wound healing
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Before After
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Approaches Posterior iliac osteotomy
Anterior osteotomy of superior pubic rami Anterior diagonal iliac osteotomy Anterior transverse iliac osteotomy Combine posterior vertical and anterior transverse iliac osteotomy
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Combined Vertical/Transverse
Corrects both anterior & posterior defects Transverse osteotomy: ~10mm proximal to AIIS most proximal (superior) sciatic notch Posterior vertical osteotomy: 2-3 cm lateral to SI joint sacral notch. Symphysis secured with wire through obturator foramen External table left intact Vertical closing wedge (hinged greenstick)
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Combined Vertical/Transverse
Ex-fix to close pubic symphysis, x 4 wks Applied under direct visualization Adjustable if incomplete reduction Better symphyseal approximation and lower recurrence ( p < 0.05 compared to posterior alone)
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Combined Vertical/Transverse
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Anterior Transverse Osteotomy
Preoperative
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Anterior Transverse Osteotomy
Immediate Postoperative
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Anterior Transverse Osteotomy
Preoperative 3 Years Postoperative
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Posterior Iliac Osteotomy
Landmark procedure (1958) 1st stage: Vertical osteotomy 2-3 cm lateral to iliosacral joints. Iliac crest sacral notch. 2nd stage: Pubic rami closed/secured with wire through obturator foramina Sturdy anterior ring prevents prolapse, infection, dehiscence of bladder Soft tissue reconstruction proceeds Improved urinary continence 5% 43-69%
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Posterior Iliac Osteotomy
Preoperative
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Posterior Iliac Osteotomy
Immediately Postoperative
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Posterior Iliac Osteotomy
Preoperative 14 Years Postoperative
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Post-Operative Management
Options: Bucks traction (4-6 wks) External fixator + modified buck traction (4-6 wks) Close follow-up, monitor for complications Pin-site infection, bladder outlet obstruction, etc
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Complications Rate of orthopaedic complications 4 - 6% Boney
Delayed union, non-union, SI joint pain, leg length inequality/asymmetry Neurological Femoral, sciatic, peroneal, superior gluteal nerves Most recover, some with permanent palsy Soft tissue Pressure sores, compartment syndrome due to overly tight bandages/traction Deep infection, osteo
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Complications Overall complication rates higher (up to 25%)
Include urologic complications: bladder prolapse, dehiscence, bladder outlet obstruction, ischemic injury to penis, etc.
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Symptomatic non-union (limp) 10 yrs following vertical osteotomy
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Vertical migration of ilium following vertical osteotomy, deficit increased with growth
3 cm leg length inequality
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Take Home Points Care of these patients requires a multidisciplinary approach Benefits of osteotomy outweigh risks Major technical surgery, requires: Careful planning Creativity Experienced hands Know your limits Success dependent on tension free construct Evolution of management, continued research and reporting
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References Sponseller PD, Bisson LJ, Gearhart JP, et al. The anatomy of the pelvis in the exstrophy complex. J Bone Joint Surg Am 1995;77-A: Stec AA, Wakim A, Barbet P, et al. Fetal bony pelvis in the bladder exstrophy complex: Normal potential for growth. J Pediatr Urol 2003; 62: Delaere O, Dhem A. Prenatal development of the human pelvis and acetabulum. Acta Orthop Belg 1999;65: Stec AA, Pannu HK, Tadros YE, et al. Pelvic floor anatomy in classic bladder exstrophy using 3-dimensional computerized tomography: Initial insights. J Urol 2001;166:
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Thanks!
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Ant. Osteotomy Sup. Pubic Ramus
Goal: simplify process for completion by pediatric urologist, not ortho (1980s) Concurent boney & soft tissue repair Faster, no repositioning, fewer incisions Tension free closure of abdominal wall Bilateral superior pubic ramus osteotomies between pectineus & adductor insertions Medial segments tilted toward midline, suture secured through cartilagenous symphysis Problem: almost always have complete recurrence of diastasis
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Anterior Diagonal Iliac Osteotomy
Originated from computer modeling (1990s) Diagonal osteotomy, greater sciatic notch 1-2 cm posterior to ASIS Optional bone graft in defect Pelvis compressed rami approximated symphysis secured with suture Best of both worlds: Tension free closure, faster, no repositioning Lowest wound infection and dehiscence rates Failure rate similar to posterior iliac osteotomy
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