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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: 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 [...]