Mark W. Burns

The syndrome of dysuria and hematuria in pediatric urinary reconstruction with stomach.

AbstractBetween July 1989 and March 1992 at a single institution 27 male and 30 female patients underwent lower urinary reconstruction with stomach. Mean patient age was 9.9 years (range 1.5 to 28 years). The diagnoses were epispadias/exstrophy complex (19 patients), myelodysplasia (11), cloacal exstrophy (6), posterior urethral valves (6), Hinman syndrome (4), sacral agenesis (3) and other (8). Indications for surgery were urinary incontinence, upper tract deterioration or undiversion. A total of 54 patients underwent augmentation gastrocystoplasty and 3 had total bladder replacement. Mean followup time was 23.2 months (range 12 to 39 months).The syndrome of dysuria and hematuria is defined as 1 or a combination of the following symptoms: bladder spasm or suprapubic, penile or periurethral pain, coffee brown or bright red hematuria without infections, skin irritation or excoriation and dysuria without infections. Telephone and clinic interviews identified 21 patients (36%) with symptoms of the dysuria an...

The Mitrofanoff Principle in Urinary Reconstruction

We report the use of the Mitrofanoff principle to achieve urinary continence in 47 patients. A catheterizable channel can be used as the primary continence mechanism, or as an adjunct to ensure complete bladder emptying if urethral catheterization or voiding is inadequate. Appendico-vesicostomy was performed in 25 of 47 patients (55%). Satisfactory continence was achieved in 45 patients (96%). Bladder emptying by clean intermittent catheterization was performed in all patients. Early complications included peristomal abscess (1 patient) and small bowel obstruction (1). Late complications included difficulty with catheterization (5 patients), stomal stenosis (9) and persistent incontinence (2). Our results support use of the appendix as the catheterizable segment of choice. We describe the technique and discuss the management of complications.

VOIDING DYSFUNCTION AFTER BILATERAL EXTRAVESICAL URETERAL REIMPLANTATION

PURPOSE Voiding dysfunction has been reported after bilateral extravesical ureteral reimplantation. We evaluate the incidence and duration of voiding dysfunction, and the effects of minimizing surgical dissection in a cohort of children after bilateral extravesical reimplantation. MATERIALS AND METHODS A retrospective chart review was performed on 33 consecutive children who underwent bilateral extravesical ureteral reimplantation for reflux. Preoperative and postoperative radiological studies, and postoperative post-void residuals were reviewed. Of the 33 patients 11 underwent ureteroneocystostomy using a modified Lich-Gregoir technique with ureteral advancement and 22 underwent a modified procedure in which the detrusor dissection was minimized and the obliterated umbilical artery was preserved. Both groups had similar preoperative characteristics. Postoperative surgical success and signs of voiding dysfunction were evaluated and compared between groups. RESULTS The average length of time until patients were able to void at least half of the bladder volume was 5.9 +/- 3.1 days. The average length of time until successful postoperative Foley catheter or suprapubic tube removal was 7.4 +/- 4.2 days. All children were able to void adequately eventually. Postoperative voiding cystourethrogram demonstrated that reflux was cured in 97% of the ureters. There was no postoperative vesicoureteral obstruction. There was no significant difference in length of time necessary to void, the duration of catheterization or operative success between the children who underwent standard versus limited detrusor dissection procedures. CONCLUSIONS Extravesical ureteral reimplantation is an effective method for repairing reflux without ureteral obstruction but it can also result in a high rate of transient postoperative urinary retention even when detrusor dissection is minimized.

Renal Transplantation into the Dysfunctional Bladder: The Role of Adjunctive Bladder Reconstruction

A retrospective review of pediatric renal transplantation at 3 centers identified 9 patients who had undergone major bladder reconstruction. Seven patients underwent bladder augmentation (5 gastric, 1 colonic, 1 ureteral) and 2 received gastric neobladders. Six patients required Mitrofanoff neourethras. Patient survival was 100% with initial graft survival of 56% during a mean followup of 29.4 months. Eight patients (89%) are presently dialysis-free with functioning allografts and all 9 (100%) are continent of urine. We conclude that renal transplantation and reconstruction to ensure continence are justifiable in even the most anatomically compromised children with end stage renal disease. It is essential that the pediatric urologist should have an integral role in the management of such cases.

A Gender Assessment Team: experience with 250 patients over a period of 25 years.

Purpose: To describe a Gender Assessment Team that has provided a multidisciplinary approach to the diagnosis, medical and surgical treatment, genetic counseling, and psychosocial support of patients with ambiguous genitalia, intersex disorders, and other genital anomalies, collectively termed disorders of sex development; and to determine the major diagnostic categories and approach.Methods: A retrospective review of 250 patients evaluated by the Team at Childrens Hospital and Regional Medical Center in Seattle, WA, from January 1981 through December 2005. The Team included the following specialties: medical genetics, cytogenetics, gynecology, pediatric urology, endocrinology, and psychiatry.Results: Of the subjects, 177 were infants, 46 were children or adolescents, and 27 had a multisystem genetic condition. The most common diagnoses were congenital adrenal hyperplasia (14%), androgen insensitivity syndrome (10%), mixed gonadal dysgenesis (8%), clitoral/labial anomalies (7%), hypogonadotropic hypogonadism (6%), and 46,XY small-for-gestational-age males with hypospadias (6%).Conclusion: The six most common diagnoses comprised 50% of the cohort. The expertise of a multidisciplinary team allowed for integrated care for patients with disorders of sex development and identification of novel conditions. Geneticists play an important role in a team approach through knowledge of genetic testing options and diagnosis of patients with karyotypic abnormalities and syndromes with genital anomalies.

Intraoperative Cardiovascular Collapse Secondary to Latex Allergy

An apparent allergic reaction has been noted in children undergoing open urological surgery. This condition is characterized by precipitous hypotension, tachycardia and upper body flushing, and it often causes termination of the procedure. Latex allergy has been identified as the possible inciting event. Contact of latex rubber gloves with intra-abdominal structures (handling bowel) appears to be the most dramatic trigger mechanism for this reaction. We report on 10 patients with latex allergy, 6 of whom have myelomeningocele, who have undergone reconstructive surgery. Severe anaphylactic shock developed intraoperatively in 5 patients and during a barium enema performed with a latex catheter in 1 patient. These 6 patients had previous allergic reactions to latex material, which was not detected preoperatively. In the remaining 4 patients latex allergy was diagnosed preoperatively. A total of 6 patients agreed to a skin prick test to liquid latex. Three patients reacted with a wheal size greater than or equal to a histamine control at a dilution of 1:1,000 and 3 patients at 1:100. In contrast, none of the 5 normal controls reacted to any of the concentrations including full strength latex. A history of exposure to latex products (balloons, surgical gloves, catheters, condoms and so forth) with allergic reactions should heighten surgeon awareness of a potentially severe intraoperative reaction. Furthermore, a skin prick test may be used to screen high risk patients such as those with myelomeningocele. A protocol involving preoperative corticosteroid and antihistamine therapy is recommended.

Reexamination of the bromophenols in the red alga Rhodomela larix

Abstract A reinvestigation of the red alga Rhodomela larix gave dipotassium 2,3-dibromo-5-hydroxy-benzyl-1′,4-disulfate and 2,3-dibromo-4,5-dihydroxybenzy] methyl ether. Aqueous hydrolysis of the salt yielded 2,3-dibromo-4,5-dihydroxybenzyl alcohol. Simple phenols reported in algae are probably artifacts of the isolation procedure.

Interstitial Cystitis in Children

PURPOSE Interstitial cystitis has been rarely reported in children. We present our experience with 16 children in whom clinical symptomatology and cystoscopic findings were consistent with the diagnosis of interstitial cystitis. MATERIALS AND METHODS We retrospectively reviewed the charts of 20 children referred for chronic sensory urgency, frequency and bladder pain who underwent cystoscopy and hydrodistension. Four patients were excluded from study because of infection identified at cystoscopy or lack of cystoscopic changes with hydro-distension. RESULTS Patient age at onset of first symptoms ranged from 2 to 11 years (median 4.5) and age at diagnosis ranged from 3 to 16 years (mean 8.2). Of the 16 children 14 (88%) presented with symptoms of urinary frequency and sensory urgency, and in 13 (81%) lower abdominal pain was relieved by voiding. None of the children had motor urgency. Urodynamic evaluation in 8 cases revealed early bladder sensation with no evidence of involuntary bladder contractions. Diffuse glomerulations and terminal hematuria were demonstrated in all patients after hydro-distension. Followup was available for 14 patients. Relief of symptoms occurred after hydro-distension in all children except 1. Seven children (50%) required repeat hydro-distension. CONCLUSIONS Children with symptoms of bladder pain, urinary frequency and sensory urgency have bladder changes on cystoscopy consistent with interstitial cystitis. Although rare, this condition is recognizable and it should not be confused with dysfunctional voiding in which complaints are secondary to involuntary bladder contractions.

Treatment of bladder dysfunction in children with end-stage renal disease☆

The appropriate management of the urinary bladder in patients requiring a renal transplant is significantly different in children than in adults. The etiology of end-stage renal disease (ESRD) in 13 of 50 children (26%) transplanted since 1985 was obstructive uropathy/dysplasia. Five of these children had small-capacity, poorly compliant bladders. Our current approach is to restore bladder compliance, improve emptying, and transplant into the restored bladder rather than divert. Pretransplant gastrocystoplasty was performed in three children and the donor ureter was implanted into the augmented bladder. One child awaits transplantation following his bladder augmentation. Bladder function is followed postoperatively by voiding cystourethrography (VCUG) and urodynamics. All of the children who have received transplants into augmented bladders are infection-free, voiding per urethra, and have functioning allografts. We recommend: (1) an initial VCUG in all children; (2) complete urodynamics, if appropriate; (3) urological reconstruction to include augmentation prior to transplantation; (4) transplantation into the reconstructed bladder; and (5) comprehensive follow-up including regular urodynamic assessment.

Aggressive surgical and medical management of autosomal recessive polycystic kidney disease

The treatment of autosomal recessive polycystic kidney disease (ARPKD) is evolving. Overall prognosis is difficult to determine, but most neonates without severe pulmonary hypoplasia will survive. It is difficult to predict which neonates with ARPKD requiring immediate ventilatory support have pulmonary hypoplasia incompatible with survival. Massively enlarged kidneys restricting diaphragmatic excursion can cause respiratory distress in addition to pulmonary hypoplasia. Three infants with ARPKD underwent bilateral nephrectomies and institution of peritoneal dialysis in an attempt to improve ventilatory status and assess long-term pulmonary function. Two children are alive at twelve and thirty months postoperatively and awaiting renal transplantation. One infant died of acute cardiorespiratory complications.