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The lessons of 40 bladder extrophies in 20 years.

A personal series of 42 consecutive unselected bladder extrophies over a 20-year period is critically analyzed. The incidence is calculated as 1 in 25,000 live births. Four died unoperated of multiple anomalies and two died following surgery. Of the remaining 36, cosmetic closure was possible in 34. Most closures were done at about 1 year, though 1 month of age is now preferred. Iliac osteotomy has been abandoned. Further staged surgery using Young-Dees bladderneck reconstruction and ureteric reimplant high in the bladder has been used in 11 selected cases. It has been effective in five but there have been three bilateral ureteric obstructions in small bladders. Twelve patients are under 5 years of age and considered too young for definitive evaluation. Of 22 patients available for close analysis, three and possibly four will be acceptably dry. Twelve have had colon or ileal loop diversion and a further two are awaiting diversion. Three are wet. On our experience we hope to successfully reconstruct about one in five unselected extrophies. For the remainder, ureteric transplant into an isolated colon loop offers the most options for the future. The selection of cases and criteria of continence in young children are important considerations in assessing results.

Bladder Exstrophy↗

Imperforate anus in females: frequency of genital tract involvement, incidence of associated anomalies, and functional outcome.

Of 162 female patients with imperforate anus, 21% had a noncommunicating and 79% a communicating anomaly of the rectum or anus. Associated anatomical abnormalities were found in the lower urinary tract (15%), upper urinary tract (25%), lower genital tract (27%), upper genital tract (35%), and additional organ systems (51%). Death occurred in 26 patients and in 19, this was attributed to the associated abnormalities. Functional outcome was assessed in those patients 13 years of age or older. Bowel function was normal or near normal in 85%, as was urinary and renal function. In 44% of patients evaluated, there was persistent vaginal abnormality or scarring and in 25%, this was severe enough to require future surgery.

Abnormalities, Multiple↗

The relationship between Helicobacter pylori infection and acid-hematuria syndrome in pediatric patients with gastric augmentation-II.

BACKGROUND/PURPOSE: The acid-hematuria syndrome, which presents with dysuria, hematuria, and perineal pain still remains a problem in those patients who have undergone bladder augmentation using the gastrocystoplasty technique. Additionally, there is still a question regarding the explanation for postoperative metabolic problems such as metabolic alkalosis, hypocholoremia, hypergastrinemia, and various complications related to gastritis and peptic ulcer disease. The aim of this study was to investigate the relation of Helicobacter pylori (HP) infection in this patient group and the relationship between HP infection and the above-mentioned clinical problems and complications. METHODS: In this study, 10 children with a history of previous gastrocystoplasty (five girls, five boys; mean age, 6.75+/-2.53 years; range, 2.5 to 12 years) were evaluated. Blood samples for HP detection were analyzed by serological testing (ELISA technique). Histopathologic studies were performed for gastric tissue specimens, obtained by endoscopic procedures from the stomach and augmented bladder. Urine pH and serum gastrin levels were measured in all patients. RESULTS: Regarding the serological studies, four of ten patients had a positive ELISA test result (40%). The four patients with HP-positive serological test results, were the patients who had acid-hematuria syndrome. These patients also had low urine pH levels (mean, 4.5) when compared with those of HP-negative patients. HP-positive patients also had high serum gastrin levels in comparison with those of HP-negative patients. CONCLUSIONS: The relation between HP infection and problems such as hypergastrinemia, acid output, and ulcer disease is well known. Our study demonstrates a correlation between the HP-positive gastrocystoplasty patients and the above-mentioned symptoms and complications. Because of the potential risk of HP infection, we suggest that HP infection be investigated in patients with gastrocystoplasty or in candidates for a gastrocystoplasty operation. HP-positive patients should be treated, to reduce the risks of postoperative complications.

Bladder Exstrophy↗

Ureteral graft in urological reconstruction: clinical experience and review of the literature.

We report 7 cases in which grafts of ureter were used as tube and patch segments in urethral reconstruction and formation of a continent catheterizable stoma. The ureteral grafts survived without long-term stricture in all cases. Meticulous preparation of the graft and recipient bed is required for success, as previously demonstrated experimentally. When available, ureter is an excellent source of tissue for urological reconstruction.

Adolescent↗

The gastroileoileal pouch: an alternative continent urinary reservoir for patients with short bowel, acidosis and/or extensive pelvic radiation.

We report on 6 patients who underwent a new type of continent urinary diversion: the gastroileoileal reservoir. These are a select group of patients who presented with the short bowel syndrome, acidosis, borderline diarrhea and/or severe pelvic radiation, which precluded the use of terminal ileum and the ileocecal segment. Considering these factors, and based on the different functional properties of the stomach as well as the need for a large reservoir, a segment of stomach and proximal ileum was used to construct the reservoir. Four patients have been followed for at least 6 months, with the longest followup being 12 months. Temporary dysphagia requiring hydrogen blockers developed in 1 patient. Results indicate excellent function of the continent urinary system, lack of metabolic complications, absent diarrhea and excellent patient tolerance. This procedure could be a useful alternative in some difficult clinical situations when continent urinary diversion is desirable.

Acidosis↗

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.

Appendix↗

Nonrefluxing colonic conduits: a long-term life-table analysis.

We evaluated 25 patients undergoing urinary diversion with a nonrefluxing colonic conduit. Renal scarring developed in 10% of the kidneys during a median followup of 12.7 years. In 11 renal units (22%) deterioration of the ureteroenteric anastomosis occurred due to the development of reflux or stricture. Of these kidneys 45% had scarring; no renal unit in which the anastomosis remained intact had renal scars (p less than 0.001). Of our patients 96% had bacterial colonization of the colonic conduit but this was not associated with renal scarring if the urinary diversion was anatomically intact.

Bacteriuria↗

Mechanical gastric outlet obstruction after continent urinary diversion.

A number of complications have been associated with complex continent urinary diversions. We report a case of postoperative mechanical gastric outlet obstruction after continent urinary diversion with distal ileum and ascending colon. Following adequate urinary reservoir drainage gastric outlet obstruction resolved. Such mechanical effects from distended urinary reservoirs must be considered in any patient undergoing continent urinary diversion.

Adult↗

Orthoplasty in epispadias.

Orthoplasty (a term used in Europe to refer to chordee excision and penile straightening) in patients with epispadias has provided less than ideal results in the past. We propose a sequential stepwise approach for the assessment and correction of the corporeal deformity, incorporating dermal grafts on the dorsal aspect of the corpora cavernosa when necessary to achieve functional penile lengthening and straightening. Success, defined as significant improvement in functional penile length, a penis that dangles at 45 degrees below the horizontal and over-all improvement in cosmetic result, was achieved in 7 of 8 patients.

Adolescent↗

Ammoniagenic coma following ureterosigmoidostomy.

Ammoniagenic coma or episodic stupor is a rare complication of ureterosigmoidostomy. We report a case with the unusual features of normal liver function and normal liver biopsy. With extremely rare exception, ammoniagenic coma will not occur in the presence of normal ornithine-urea cycle synthesis in the liver.

Adolescent↗

Penile lengthening after previous repair of epispadias.

Unsatisfactory appearance and function of the penis are common after repair of complete epispadias. Reoperation can benefit the majority of these patients. The principles of repair in secondary cases as well as primary cases include wide mobilization of the corpora to gain length, resection of dorsal chordee to straighten the shaft, augmentation of urethral length with a graft, secure closure of the corpora over the urethra to avoid a fistula, wide mobilization of the mons to place hair-bearing skin in proper location and Z-plasty closure.

Adolescent↗

Exstrophic anomalies: followup of 36 cases.

Since 1950, 36 patients with exstrophic anomalies were seen at the Medical College of Virginia Hospitals. There were several modalities of treatment used, including primary reconstructive procedures in 11 patients, 6 of whom continue to have a functional bladder closure. Our results and current management are discussed.

Bladder Exstrophy↗

Comparison of techniques to determine continence zone.

The urethral pressure profiles for 36 patients were analyzed specifically for continence zone by 3 techniques: 1) triangle, 2) planimeter and 3) weight. Graphically, the best agreement was between the weight and planimetry methods but, statistically, there was a significant difference among techniques except for the subgroup that had the smallest continence area.

Bladder Exstrophy↗

Ureterosigmoidostomy.

Some historical aspects of ureterosigmoidostomy are described, and various techniques are discussed and illustrated. Our own satisfaction with the results when the procedure is done through the open sigmoid colon is expressed. Ureterosigmoidostomy, which has in some surgeons' hands fallen into disuse, will continue to be used and probably should be used more than it is at present. When ureterosigmoidostomy is done meticulous care is important in producing a long submucosal tunnel with direct anastomosis of the ureter to the bowel. Preoperative bowel preparation is mandatory. Patients who have undergone ureterosigmoidostomy should remain on a low chloride diet indefinitely with an adequate supplement of sodium potassium citrate to diminish the dangers of electrolyte imbalance and hyperchloremic acidosis. Careful postoperative management and followup care are vital to success.

Bladder Exstrophy↗