1. 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

2. Disclosures
No disclosures

3. Outline Anatomic anomalies in bladder exstrophy
Indications & goals for surgery
Operative approaches
Complications
Conclusions

4. Urology Perspective

5. Ortho Perspective

6. What do we do with this?

7. What is Normal in Exstrophy
Sacral width
Iliac segment (posterior Pelvis) length
Microscopic histology normal
Boney and cartilaginous differentiation & development
Endochondral ossification (cartilage model)

8. 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

9. Defects in Boney Anatomy
Wider hips
31% greater distance between triradiate cartilage
Acetabular retroversion
13° retroversion, normal = 0°

10. Defects in Boney Anatomy

11. 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

12. 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

13. 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

14. Before
After

15. 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

16. 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)

17. 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)

18. Combined Vertical/Transverse

19. Anterior Transverse Osteotomy
Preoperative

20. Anterior Transverse Osteotomy
Immediate Postoperative

21. Anterior Transverse Osteotomy
Preoperative
3 Years Postoperative

22. 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%

23. Posterior Iliac Osteotomy
Preoperative

24. Posterior Iliac Osteotomy
Immediately Postoperative

25. Posterior Iliac Osteotomy
Preoperative
14 Years Postoperative

26. 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

27. 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

28. Complications Overall complication rates higher (up to 25%)
Include urologic complications: bladder prolapse, dehiscence, bladder outlet obstruction, ischemic injury to penis, etc.

29. Symptomatic non-union (limp) 10 yrs following vertical osteotomy

30. Vertical migration of ilium following vertical osteotomy, deficit increased with growth
3 cm leg length inequality

31. 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

32. 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:

33. Thanks!

34. 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

35. 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