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We describe the clinical findings in two previously unreported, unrelated cases with aplasia cutis congenita and epibulbar dermoids, similar to the cases reported by Toriello et al. [1993]. In addition, one patient had bladder exstrophy with epispadias. These cases provide further evidence for the identification of the oculo-ectodermal syndrome as a distinct entity.
PURPOSE: The role of environmental injury in carcinogenesis is widely recognized. Malignancy in exstrophic bladders has been reported most frequently in untreated adults and those undergoing surgical treatments which involve the mixing of fecal and urinary streams. The question of whether the closed exstrophic bladder has a similar potential for malignancy has not been resolved. The polypoid appearance of the exstrophic bladder template raises the concern of premalignant lesions. We characterized the histology of these lesions and analyzed their microscopic features with particular reference to predisposition for dysplasia. In doing so, we attempt to address the aforementioned question and set the stage for definitive quantification of the risk of malignancy in these patients with careful, long-term followup. MATERIALS AND METHODS: Under institutional board review, the slides of 38 patients with classic bladder exstrophy who had polyps excised at the time of closure were reviewed by a single genitourinary pathologist (JIE). The most common findings were reported for polyps resected at primary and secondary closure, respectively, and a comparative analysis was performed. RESULTS: Of the 38 cases 24 were primary closures and 14 were secondary closures. Six of the primary closures were delayed by 6 weeks or greater. The 2 basic types of polyps observed were fibrotic and edematous. Both types were associated with overlying reactive squamous metaplasia in approximately 50% of cases. Varying degrees of fixed on file Brunn's nests, cystitis cystica and cystitis glandularis were noted. Cystitis glandularis was observed in a significantly greater percentage of secondary closures (p = 0.0014). CONCLUSIONS: Although no dysplasia was noted, cystitis glandularis is associated with the development of adenocarcinoma of the bladder. The finding of cystitis glandularis suggests a more severe epithelial injury and it follows that the significant majority of these cases (10 of 14, 71.4%) were observed with polyps resected during secondary closure. These patients warrant future surveillance with urine cytology and cystoscopy as they enter adult life.
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Split notochord syndrome with dorsal enteric fistula is an extremely rare congenital anomaly that may be associated with meningomyelocele or meningocele, and genitourinary anomalies. This case presented with an additional finding of bladder exstrophy, raising the possibility of a relationship between this syndrome and the OEIS complex.
In a simplified view, the "normal" bladder, through a multifaceted neuromuscular event, allows the basic functions of urinary storage and emptying. More specifically, the urinary bladder accommodates increasing urinary volume with little to no increase in vesicular pressure while maintaining continence. The normal act of emptying integrates the relaxation of the urinary sphincters (external and internal) with the subsequent bladder contraction to void to completion when full. There are a multitude of conditions, both congenital and acquired, that can affect the bladder's ability to perform these functions in a smooth and coordinated fashion. The most common causes of pediatric bladder dysfunction necessitating surgical intervention are those associated with spina bifida/myelodysplasia, posterior urethral valves, and bladder exstrophy. Over the last 2 decades, the evolution of complex reconstruction for lower urinary tract dysfunction has resulted in an improved quality of life for children afflicted with upper urinary tract changes or incontinence despite maximum utilization of nonoperative therapies. Because each patient represents a unique therapeutic entity, an individualized approach to each child is recommended.
During urological reconstructive surgery small or large bowel is used to increase bladder capacity or to create a urinary reservoir. In most patients clean intermittent catheterization is necessary for urinary drainage. We report on 4 patients with a sigmoid cystoplasty who perform clean intermittent catheterization and who have experienced a serious long-term complication, urinary reservoir perforation, which was fatal in 1. Two were adolescent girls with myelodysplasia and 2 were prepubertal boys with bladder exstrophy. One patient experienced 2 separate reservoir perforations. Rupture occurred 15 to 48 months (mean 30.2 months) after reconstruction. Diagnosis was made by a static cystogram, which demonstrated extravasation in 2 of 4 patients, and ultrasound. In 2 patients the cystogram was normal. Management included intravenous antibiotics and open abscess drainage in all patients. In patients who have undergone augmentation cystoplasty or continent diversion and in whom abdominal pain and distension develop reservoir perforation should be considered in the differential diagnosis.
Bladder exstrophy is a rare congenital condition that occurs in 1 to 30,000 live births. Primary bladder closure is usually performed in the first days of life in conjunction with an iliac osteotomy in order to achieve a more secure bladder closure. We report a case of a large bladder stone with secondary right-sided hydronephrosis in a 3-year-old child who underwent exstrophy repair at the age of 7 months. During the exstrophy repair a no. 1 braided, polyester, non-absorbable suture was used to close the pubic bones and served as a nidus for intravesical stone formation. This case substantiates the lithogenic nature of non-absorbable sutures in contact with urine as well as the need for close post-operative follow-up in these patients.
We report a new therapeutic approach for bladder exstrophy and epispadias in one case of failed epispadias repair. The width of the pelvis was measured by what we defined as the anteroposterior diameter (APD) on combined transverse computed tomography (CT) scan cuts of the pelvis. The APD was half the normal value in an incontinent patient with failed epispadias repair. He underwent a supraacetabular osteotomy of the pelvis with progressive anterior distraction of the anterior segment of the pelvic ring. Four months later, hardware was removed, and the APD was near normal value. Within 9 months of follow-up, the patient was dry day and night. We believe that in patients with failed exstrophy and epispadias repair, APD seems to be a predictive criterion for continence, and results of the reconstructive surgery with osteotomy should be improved by distraction of the anterior segment of the pelvic ring.
Experience with transcolonic Ureterosigmoidostomy (US) in 127 patients and colonic conduit (CC) in 152 patients is presented in detail. US proved to be highly satisfactory in children, in particular in bladder exstrophy (13% post-operative and 27% late complications, deterioration in 8% of all renal units), but gave poor results in adults. This was mainly due to the patient selection, as in this group preoperative damage to the upper urinary tract by pyelonephritis, obstruction, irradiation or previous surgery was frequent. Obviously these factors prohibit US. For the same reason US excludes adjunctive radiotherapy in cancer patients. Although employed in a higher renal risk group (signs of pyelonephritis in 34% and ureteral dilatation in 54% of the renal units in the preoperative IVP) CC with non-refluxing uretero-intestinal anastomoses reliably stabilized renal morphology (improvement of ureteral dilatation in 79%, over all deterioration 10% of all kidneys, mainly by progression of pyelonephritis established already before diversion). The low late complication rates of 21% in children and 31% in adults (with benign disease) compare favourably with the results reported in comparable series of ileal conduits.
PURPOSE: The hematuria-dysuria syndrome is the most common reported complication of gastrocystoplasty. We reviewed our cases of gastrocystoplasty to determine the long-term incidence and significance of the syndrome. MATERIALS AND METHODS: We performed a retrospective study of 78 patients who underwent gastrocystoplasty at our institution between July 1989 and October 1994. A total of 72 of the 78 cases were evaluated within the last year to elicit symptoms of the hematuria-dysuria syndrome. RESULTS: Spina bifida and bladder exstrophy were the most common diagnoses of patients undergoing gastrocystoplasty. There were 3 (4%) patients who required medications on a continuous basis to control symptoms of the hematuria-dysuria syndrome. However, using broad criteria 17 (24%) patients would be categorized as having the syndrome. Of these patients 9 did not require any medications and 4 occasionally took medications to control symptoms. A significant increase in the incidence of the hematuria-dysuria syndrome was detected in those cases with a sensate compared to those with an insensate urethra. CONCLUSIONS: The hematuria-dysuria syndrome is a clinically significant problem at long-term followup in a small percentage of patients treated with gastrocystoplasty. The lowest incidence is in those children with an insensate urethra who are continent. When symptoms occur they are easily treated in the majority of cases. We believe that gastrocystoplasty remains a viable option in the armamentarium of bladder augmentation.
PURPOSE: The exstrophy-epispadias complex in combination with a duplicated lower urinary tract is an extremely rare congenital malformation. We describe 2 cases of bladder exstrophy in association with a duplicated lower urinary tract. MATERIALS AND METHODS: We analyzed the case histories of 2 patients. The first case had anteroposterior duplication of the bladder communicating through an isthmus and a duplicate urethra. The anterior system was exstrophic and epispadiac. The second case had a duplicate bladder without epispadias. There was a communication between the anterior exstrophic and posterior normal bladders. No other malformations were present. RESULTS: Both patients underwent excision of the duplicate exstrophic bladder in conjunction with primary closure of the abdominal wall. Postoperative continence was normal. Histological examination confirmed the diagnosed bladder duplication. CONCLUSIONS: Although duplication of the lower urinary tract in combination with the exstrophy-epispadias complex is extremely rare, all children presenting with an exstrophic bladder and a normal urethra alone or in association with an epispadiac anterior urethra should be evaluated for a bladder duplication with possible isthmic communication.
PURPOSE: To review long-term functional results, complications, and patient satisfaction in patients with a continent catheterizable urinary diversion. MATERIALS AND METHODS: A retrospective chart review and telephone satisfaction interviews were conducted. Bladder dysfunction was attributed to neurogenic bladder (n = 48: 23 spinal cord injury, 18 myelomeningocele, 4 sacral agenesis, 3 cerebral palsy), bladder exstrophy (n = 2), posterior urethral valves (n = 1), and other (n = 3). Patients underwent continent urinary diversion with either the Mitrofanoff principle (appendicovesicostomy, n = 47) or a Monti tube (ileovesicostomy, n = 8). Outcomes were assessed by chart review. Patient satisfaction was assessed by telephone interview and scored from 1 to 10 on a Likert-type scale. RESULTS: Between 1992 and 2003, 54 continent urinary diversions were performed on 17 boys and 37 girls. Mean age was 15.3 years (range, 7-21 years). An umbilical stoma was created in all patients. Seventy-three percent (40/54) and 47% (26/54) underwent concomitant bladder augmentation and urethral sling procedure, respectively. Mean follow-up was 2.5 years (range, 3 months to 10 years). Ninety-five percent (51/54) of patients were continent, and 5% (3/54) were incontinent from the umbilical stoma after one operation. All were compliant with intermittent catheterization. Complications included bladder calculi (15%; 8/54), stomal stenosis (9%; 5/54), stomal bleeding (5%; 3/54), small bowel obstruction (2%; 1/54), and superficial wound dehiscence (2%; 1/54). Seventy-three percent (40/54) of patients were available for telephone interview. Of these, 90% (36/40) reported satisfaction, and 10% (4/40) reported dissatisfaction; 93% (37/40) reported that they would recommend the procedure to others, whereas 7% (3/40) would not. CONCLUSION: In our series, continent urinary diversion with the Mitrofanoff principle or Monti tube is associated with high continence, compliance, and satisfaction rates and a low complication rate. An umbilical stoma was achievable in all patients. Our 10-year experience is consistent with other reported series and underscores the successful long-term outcome and durability of continent urinary diversions.
Urinary incontinence owing to epispadias or bladder exstrophy is a difficult therapeutic problem. To improve the results a new method, including free autogenous muscle transplantation, was developed. Before transplantation a skeletal muscle (extensor brevis of the foot) is denervated and, 2 weeks later, it is transplanted and placed as a U-sling around the urethra in close contact with normally innervated muscles, which act as reinnervation sources. Two patients showed improvement of continence and increasing bladder capacity from 6 weeks after transplantation. Postoperative cine-studies of micturition demonstrated clearly the functional properties of the transplanted muscle.
PURPOSE: We investigated the long-term metabolic effects of gastrointestinal composite urinary reservoirs in patients with myelomeningocele or exstrophy. MATERIALS AND METHODS: Seven patients with myelomeningocele or exstrophy who required complex urinary reconstruction in the setting of metabolic acidosis or the short bowel syndrome underwent construction of a gastrointestinal composite reservoir, including a staged and a single procedure in 3 and 4, respectively. Preoperatively and postoperatively serum electrolytes were measured, and urinalysis and urine cultures were performed in all patients. In 5 patients serum pH was compared preoperatively and postoperatively, and in all serum gastrin was measured postoperatively. RESULTS: At an average followup of 62 months (range 52 to 87) serum chloride and bicarbonate significantly normalized (p <0.05) in all 7 patients with bladder exstrophy or myelomeningocele. Serum pH also significantly normalized (p <0.05) in 5 patients at long-term followup. Serum gastrin and creatinine were normal and urinary pH fluctuated insignificantly throughout followup. None of the patients had urolithiasis or symptoms of the hematuria-dysuria syndrome. Periodic symptomatic urinary tract infections developed but none required chronic antibiotic therapy. CONCLUSIONS: Gastrointestinal composite urinary reservoirs appear to be beneficial for patients with myelomeningocele or exstrophy who have preexisting metabolic acidosis or the short bowel syndrome. Serum electrolyte neutrality is achieved during long-term followup. No patient had the hematuria-dysuria syndrome or urolithiasis.
PURPOSE: Appendicovesicostomy was introduced in the United States in 1982 at our hospital. It has become the most popular alternate continence channel for catheterization. We reviewed the experience of 1 surgeon with appendicovesicostomy during a 15-year period. MATERIALS AND METHODS: We retrospectively reviewed the operative reports and clinical records of 50 consecutive patients in whom appendicovesicostomy was performed by 1 surgeon between 1982 and 1998. The underlying diagnosis was myelomeningocele in 31 cases, bladder exstrophy in 6, the prune-belly syndrome in 2, posterior urethral valves in 2 and other disorders in 10. Mean patient age at surgery was 13.1 years (range 4 months to 25 years) and mean followup was 4.3 years (range 3 months to 16.3 years). RESULTS: Of the 50 patients 96% continue to catheterize the appendicovesicostomy. Stomal stenosis developed in 5 cases (10%) and other complications included stricture and appendiceal perforation in 2 each. Eight patients (16%) required appendicovesicostomy revision at a median of 7.3 months (range 1 month to 5.8 years) after the initial procedure. Median time to revision for stomal stenosis was 13 months (range 1 month to 5.8 years). Appendicovesicostomy continence was achieved in 49 patients (98%). CONCLUSIONS: Our series demonstrates the successful long-term outcome and durability of appendicovesicostomy in children. Careful adherence to technique at initial surgery helps ensure a high long-term success rate.
The authors report a case of atypical osteodystrophy of visceral origin (uretero-sigmoid by-pass for bladder exstrophy) which was characterized by the association of osteocondensation and looser-Milkman lines. They emphasize the rareness of osteodystrophy after uro-digestive by-pass and the exceptional character of the osteocondensation.
Sixty-four children underwent colon conduit urinary diversion because of a neurogenic bladder owing to myelomeningocele and 39 children required a ureterosigmoidostomy because of bladder exstrophy. The average length of followup was 4.6 years after colon conduit and 5.6 years after ureterosigmoidostomy. Colon conduit diversion was secondary in 3 children and ureterosigmoidostomy was secondary in 5. Of the children with a colon conduit 9.4% and of those with ureterosigmoidostomy 12.8% had postoperative surgical complications. Late surgical complications were encountered after colon conduit in 14.5% and after ureterosigmoidostomy in 20%. Of the children with normal renal function preoperatively 8.3% showed late deterioration after colon conduit and 8.7% after ureterosigmoidostomy. Conversely, only 16.7% with impaired renal function preoperatively did not improve at long-term followup after colon conduit. Of the renoureteral units 91% were normal after colon conduit and 95.2% were normal after ureterosigmoidostomy. The colon conduit halted the preoperative pyelonephritis in 74% of 31 affected kidneys.
BACKGROUND: The purpose of this study was to report experience with the revived surgical concept of ureterosigmoidostomy in its low pressure modification and to discuss its value within the current spectrum of urinary diversion. METHODS: Between February 1992 and September 1997 modified ureterosigmoidostomy (rectosigmoid pouch; Mainz pouch II) was performed in 34 patients aged 1.9-76.9 (mean 55.8) years as a primary urinary diversion after radical cystectomy for bladder cancer (n = 30) and benign conditions (bladder exstrophy, three patients; intractable urinary incontinence, one). All patients were followed prospectively according to a standard protocol including assessment of continence, renal function and acid-base balance. RESULTS: There were no perioperative deaths. In one patient dislocation of a ureteral stent in the early postoperative course required insertion of a percutaneous nephrostomy. All patients were continent during the day. One patient experienced night-time incontinence but rejected a conversion procedure. In one case ureterosigmoidostomy was replaced by an ileal conduit after several episodes of septicaemia. One nephrectomy was performed for ureterointestinal obstruction. Mild hyperchloraemic acidosis was seen in two patients. CONCLUSION: Bowel frequency and urge incontinence, the major weaknesses of classical ureterosigmoidostomy, can be overcome by detubularization of the rectum. As the modified procedure is quick, safe and easy to perform with highly satisfactory results, the rectosigmoid pouch has potential in reconstructive urology.