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Background: We analyzed shape and stress on the hip joint in exstrophy and reviewed the status of hips in adults with uncorrected exstrophy. Methods: Fourteen patients aged 16 to 52 years born with classic bladder exstrophy were studied. Patients were matched for age and gender with 14 controls. AP pelvis radiographs were used for biomechanical analysis based on the single-stance phase of gait. Joint force and joint stress (force/area) were calculated relative to partial body weight by the method of Legal (1987). Clinical assessment was by Iowa hip score and radiographic grading. Statistical analysis was done using t tests. Results: For the exstrophy patients, the mean relative joint force was significantly higher than control (4.2 [+ or -] 0.91; 3.0 [+ or -] 0.3) (p [is less than] 0.01). The mean relative joint stress for exstrophy patients was significantly higher as well (p [is less than] 0.05). The increase in force and stress appeared to be due to three factors: (1) the mean distance from the body center to the center of the femoral head was significantly increased (p [is less than] 0.001) in bladder exstrophy (12.35 [+ or -] 1.05 cm) versus controls (10.31 [+ or -] 0.70 cm) (approximately 30% increased). (2) The mean distance from the greater trochanter to the femoral head center (f) was significantly less (p [is less than] 0.02) for exstrophy patients (5.2 [+ or -] 1.2 cm) compared to controls (6.5 [+ or -] 0.8 cm). (3) The center-edge angle (CO angle) was significantly decreased in exstrophy patients (25.0 [+ or -] 9 deg.) Versus control (33 [+ or -] deg) (p [is less than] 0.05). Two of the adults with exstrophy had decreased Iowa hip scores and radiographic evidence of arthrosis. Conclusions: The force and stress on the hip joint are increased in adult bladder exstrophy patients. Increased joint load and stress can lead to an earlier onset of DJD. These results need corroboration by larger series. It remains to be seen whether closing the pelvic ring at exstrophy closure may help reduce these factors and affect the outcome of the hip., M.M. Jani, BA, Paul D. Sponseller, M.D., FAAP, John P. Gearhart, M.D., FAAP. Johns Hopkins Hospital, Baltimore [...]

Background: To assess results, applications, and complications of pelvic osteotomy producing continence in patients with the exstrophy/epispadias complex. Methods: Eighty-five patients who underwent pelvic osteotomy and external fixation were reviewed at a minimum of two year follow up (mean 4.8 years). Seventy-two patients had classic bladder exstrophy and 13 patients had cloacal exstrophy. Indications for osteotomy were to achieve a tension-free closure of the bladder and lower abdominal wall and/or to approximate pelvic floor muscles at the time of later bladder neck reconstruction. The patients were stratified into five different groups of age at surgery for analysis. Of these patients, 37 had anterior innominate osteotomy, 40 combined anterior innominate and posterior iliac osteotomy, 6 posterior lilac osteotomy, and 2 suprapublic ramotomy osteotomies were performed. Results: The mean age at surgery was 2.9 [+ or -] 3.7 years (range 3 days to 13 years old). Osteotomy was performed at the time of initial bladder closure in 21, reclosure in 34, and bladder neck reconstruction in 30. In classic exstrophy patients, diastasis was corrected to an initial mean of 2.8 cm and a final mean of 3.8 cm. Cloacal exstrophy patients had significantly greater initial and residual diastasis. Maintenance of diastasis correction increased continuously with age at surgery. Wound dehiscence or bladder prolapse occurred in 4% of patients after primary closures and 0% after reclosure. Daytime continence was achieved in 75% of patients. The degree of continence was not correlated with percent correction of diastasis. Complications include transient femoral nerve palsy in 7 cases (8%) which all resolved spontaneously after three months and were probably due to tension on the inguinal ligament; delayed union in 3; late pin-track osteomyelitis in one. Conclusion: Pelvic osteotomy is useful in helping achieve two goals of exstrophy treatment: successful bladder and abdominal wall closure along with urinary continence. The authors prefer an anterior approach because of the single stage positioning and accuracy of fixator application. Long-term maintenance of diastasis is least in the younger patients, probably because of continued undergrowth of the anterior pelvic segment. The authors prefer closure without osteotomy in young infants if this can be achieved early on without tension. However, in other cases, pelvic osteotomies are affective in achieving treatment aims with an acceptably low complication rate., Paul D. Sponseller, M.D., FAAP, M.M. Jani, BA, and John P. Gearhart, M.D., FAAP. Johns Hopkins Hospital, Baltimore, [...]