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Urolithiasis (presence of stones in the urinary tract) occurs as frequently as 1 in 1,000 American children. The incidence of stones is higher among Southeast Asian children, probably due to diets high in carbohydrates and low in purines, while the mechanisms underlying stones in American children are unclear. However, urinary stones are associated with urinary tract abnormalities, infection, metabolic disease, and immobilization (lack of movement by the child). To better understand urolithiasis in children, the cases of 54 patients (22 female), ranging from newborn to 17 years of age, were studied. Symptoms varied from abdominal pain to obviously bloody urine or nausea; symptoms were absent in some cases. Diet or medication may have contributed to stone formation in nine children. High calcium levels in blood or urine occurred in eight children, two of whom had tumors in the parathyroid gland (the gland producing a calcium-regulating hormone), three of whom had been chronically immobilized, one of whom had altered protein metabolism, and three of whom had defective calcium handling. Calcium oxalate stones were most common, occurring in 17 children, while 10 children (9 of whom had previously had urinary tract infections) had stones containing magnesium ammonium phosphate. Urinary tract deformities were present in 19 patients. A total of six children with urolithiasis had been chronically immobilized to allow bone healing following auto accidents or correction of bone deformities. Almost half (25) of the patients had urinary tract infections, of whom all but one had an associated condition such as prior urinary tract surgery. Twenty-one children spontaneously passed the stones, 24 had stones removed by eight different types of surgery, and four had their stones fragmented by extracorporeal shock-wave lithotripsy (crushing of stones by shock waves applied outside the body). Of 50 patients who had follow-up examinations, 11 experienced recurrence of stones, most of whom had urinary tract deformities or prior surgery. The study suggests that existing metabolic or anatomic abnormalities should be identified in children with urolithiasis in order to limit long-term problems. (Consumer Summary produced by Reliance Medical Information, Inc.)

Background: Continence in the bladder exstrophy population requires the coordinated and staged surgical management involving the successful closure of the exstrophy bladder, early epispadias repair, and bladder neck reconstruction (BNR) after an appropriate bladder capacity. In some select cases, initial bladder closure and epispadias may be closed concomitantly. To further delineate factors that may predict eventual continence after bladder neck plasty, we have retrospectively reviewed our patient records. Methods: The records of 88 patients who underwent total bladder exstrophy reconstruction at our institution between 1975 and 1997 were reviewed. 57 patients with BNR were available for analysis after excluding 23 patients awaiting BNR, 3 patients who achieved continence without BNR, 4 patients with recent BNR, but less than one year follow-up, and 1 patients lost to follow-up. Their medical records were reviewed and data analyzed. Results: The median and mean age for primary closure were 10 days and five months, respectively. The average age of BNR was 4.4 years with a mean capacity of 88 cc. Of these 57 patients, 42 (74%) are continent and voiding urethrally without need for augmentation or CIC. 9 (16%) patients have social continence, dry for more than three hours during the day, 2 patients required diversion for continence after failed BNR. 3 patients are wet. Analysis of bladder capacity measurements prior to BNR revealed patients with bladder capacity greater than 75 cc at the time of BNR or with an average increase in capacity of more than 21 cc/year resulted in better outcome. Whereas 2 patients were wet and 2 required urinary diversion in the group with the smaller bladder capacity and poor bladder growth, only one patient was completely wet in the optimal group. No correlation between age of BNR and continence was found. Conclusion: Determinates of continence in the bladder exstrophy population are multifactorial. In our experience 74% are all completely dry, day and night, and 89% can obtain "social continence," being dry for at least 3 hours. However, with careful evaluation of bladder capacity and bladder growth, urinary continence may be improved in this population with better patient selection., David Y. Chan, MD, Robert D. Jeffs, MD, FAAP, and John P. Gearhart, MD, FAAP. James Buchanan Brady Urological Institute, Johns Hopkins Medical Institutions, Baltimore, MD. (Presented by Dr. [...]

Background: The surgical approach to the small newborn exstrophied bladder remains undetermined. Various approaches to long term management of these children have been implemented. The authors investigated their experience in late primary closure of the small exstrophied bladder template. Methods: The charts of 605 patients treated and followed for the exstrophy-epispadias complex were reviewed. Fifty of these underwent primary closure (either at our institution or else. where) after the age of six months. Twenty of these patients had a bladder template which was judged too small to close in the newborn period. The other 30 patients had delayed closure for a variety of financial, geographic, other circumstantial or unknown reasons. Results: Of the 20 children who had delayed closure due to a small bladder template there were 18 males and 2 females. Follow-up time ranged from 1-32 years (mean 21 years). Primary closure was Performed at a mean age of 1.2 years (range 6mo-2yrs). Osteotomies were performed in 17. Nine of 20 patients achieved continence after gaining sufficient bladder capacity for bladder neck reconstruction. Five of 20 patients required enterocystoplasty to augment bladder volume, and Perform clean intermittent catheterization (2 per stoma, and 3 per urethra). One patient required a colon conduit for very small bladder and one a cystectomy and ureterosigmoidostomy due to rhabdomyosarcoma. Four patients are incontinent, 2 of these are awaiting bladder neck reconstruction, and 2 had a failed bladder neck reconstruction. Conclusion: Delayed primary closure of a small exstrophied bladder template permits the native bladder tissue time to grow to a size that is feasible for successful closure in most patients. An epispadias repair can usually be performed at the same time especially with testosterone stimulation. Bladder neck reconstructive techniques have achieved continence without the need for augmentation or bladder replacement in 45% of patients. Even in patients who do not achieve adequate capacity for bladder neck reconstruction, preservation of the native bladder template allows for easier future augmentation and ureteral reimplantation., Jennifer Dodson, MD, Ilhami Surer, MD, Linda Baker, MD and John P. Gearhart, MD, FAAP. Division of Pediatric Urology, Brady Urological Institute, The Johns Hopkins Hospital, Baltimore, [...]

Background: In the exstrophy-epispadias population, male adolescents consider the oddly appearing genitalia, with a short widened penis, to be a greater psychosocial problem than incontinence and therefore every effort should be made to restore the penis to a normal status. Therefore the authors evaluated their ten year experience with the modified Cantwell-Ransley epispadias repair technique to determine the complications and long term results. Methods: A retrospective chart review was performed on 605 exstrophy-epispadias data-base patients. 93 males (79 with classic bladder exstrophy and 14 with complete epispadias) underwent a modified Cantwell-Ransley epispadias repair over the last ten years. Primary repair was performed in 77 boys (65 with classic bladder exstrophy and 12 with epispadias), and secondary repair was performed after prior failed reconstruction in 16 boys (14 classic exstrophy and 2 with complete epispadias). Epispadias repair was combined with reclosure of bladder exstrophy in 10 patients. Results: At a mean follow up of 68 months (4-120mo), 87 patients had a horizontal or downward angled penis while standing and 88 patients pass urine per urethra. Fistulas developed in 22 patients (23%). In 4 patients the fistulas closed spontaneously within the first 3 months after surgery and 18 patients (19%) underwent a separate procedure to close the urethrocutaneous fistula. Seven patients developed a urethral stricture at the proximal anastomotic area and 5 patients had minor skin separations (4 exstrophy, 1 epispadias) of the dorsal penile skin closure. Catheterization or cystoscopy in 77 children revealed an easily negotiable neourethral channel. Conclusion: The authors conclude from their ten year follow-up that the single stage modified Cantwell-Ransley epispadias repair technique creates a cosmetically satisfactory penis and produces a straight, catheterizable neourethral channel with an acceptable complication rate. However, definitive assessment of genital reconstruction must be deferred until all of these boys are sexually mature and active. While many methods of epispadias repair exist, meticulous follow-up of the urethra, patient selection and surgical experience remain the milestones for success., Ilhami Surer, MD, Linda A. Baker, MD, Robert D. Jeffs, MD, FAAP and John P. Gearhart, MD, FAAP. Division of Pediatric Urology, Brady Urological Institute, The Johns Hopkins Hospital and [...]

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: While some case series have suggested delayed linear growth after enterocystoplasty, none of these were case-controlled and the series did not necessarily include patients whose bladders were augmented for identical reasons. Therefore, the present study was performed to examine the hypothesis that intestinal bladder augmentation is associated with reduced linear growth. Methods: Fifty patients who had undergone bladder augmentation for incontinence resulting from bladder exstrophy were randomly selected from our institution's patient data-base and matched for gender, age and type of exstrophy with 50 non-augmented bladder exstrophy patients. Patients were then contacted and asked to permit their pediatricians to release their growth charts. Once consent was obtained, the charts were requested from the pediatricians. Evaluable data (defined as at least one height pre and post augmentation) were obtained for 20 (40%) augmented and 14 (28%) non augmented patients. Results: The mean age at operation was 7.7 years. Delayed growth defined by a postoperative drop in percentile height occurred in 18 (90%) of augmented patients, with a mean loss of 17 percentile points. In the control group, delayed growth after 7.7 years of age occurred in 4 (29%) patients, with the control group as a whole gaining average of 8 percentile points. A Shapiro-Wilk test found the heights to be normally distributed (p=0.19 and 0.93 respectively) and a t-test showed the mean height change to be significantly different between the two groups (p=0.006). The average follow up periods of 6 and 7.4 years in the augmented and non-augmented groups, respectively, were not significantly different (p=0.19). Conclusion: Intestinal bladder augmentation is associated with a nearly universal decrease in percentile height. Analysis of subtle metabolic alterations may provide information to help minimize or prevent growth impediment in the future. Alternatives to intestinal augmentation should continue to be pursued., Jennifer L. Dodson, MD, David-Alexandre C. Gros, MD, Uri A. Lopatin, BA, John P. Gearhart, MD, FAAP, Richard I. Silver MD, Steven G. Docimo, MD, FAAP. Division of Pediatric Urology, [...]

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, [...]

Objectives. To identify and study adults (21 years or older) who have a 46,XY karyotype and presented as infants or children with genital ambiguity, including a small phallus and perineoscrotal hypospadias, reared male or female. Methods. Participants were classified according to the cause underlying their intersex condition based on review of medical and surgical records. Long-term medical and surgical outcome was assessed with a written questionnaire and physical examination. Long-term psychosexual development was assessed with a written questionnaire and semistructured interview. Results. Thirty-nine (72%) of 54 eligible patients participated. The cause underlying genital ambiguity of participants included partial androgen insensitivity syndrome (n = 14; 5 men and 9 women), partial gonadal dysgenesis (n = 11; 7 men and 4 women), and other intersex conditions. Men had significantly more genital surgeries (mean: 5.8) than women (mean: 2.1), and physician-rated cosmetic appearance of the genitalia was significantly worse for men than for women. The majority of participants were satisfied with their body image, and men and women did not differ on this measure. Most men (90%) and women (83%) had sexual experience with a partner. Men and women did not differ in their satisfaction with their sexual function. The majority of participants were exclusively heterosexual, and men considered themselves to be masculine and women considered themselves to be feminine. Finally, 23% of participants (5 men and 4 women) were dissatisfied with their sex of rearing determined by their parents and physicians. Conclusions. Either male or female sex of rearing can lead to successful long-term outcome for the majority of cases of severe genital ambiguity in 46,XY individuals. We discuss factors that should be considered by parents and physicians when deciding on a sex of rearing for such infants. Pediatrics 2002;110(3). URL: http://www. pediatrics.org/cgi/content/full/110/3/e31; intersex, sex assignment, gender, androgen insensitivity, gonadal dysgenesis, psychosexual, genital reconstruction, hormone replacement.

Describes two pediatric cases of lower genitourinary tract trauma associated with break dancing. Characteristics of the male patients; Types of injuries associated with break dancing; Diagnosis and treatment of urethral injuries.