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PubMed2001

New surgical techniques in pediatric urology.

More sophisticated endoscopic instruments, combined with a better understanding of bladder and urethral pathology, have significantly improved the therapeutic approaches for both posterior urethral valves and ureteroceles. New generation lithotripters have allowed for a safe and efficient method of treating urinary calculi in children, which was once thought too injurious a process with first-generation machines. The rapidly advancing field of laparoscopy, aided by the development of more optically refined and diminutive instruments, has allowed for its application in a wide variety of surgical interventions in pediatric urology. The tubularized incised plate urethroplasty has challenged more traditional approaches to hypospadias repair and is now considered by many pediatric urologists to be the best approach for midshaft and distal hypospadias. The one-stage approach to exstrophy repair may hold the answer to improved continence without a formal bladder neck reconstruction. Finally, the field of tissue engineering leads the way to new advances in autologous biological substitutes in the surgically-challenged patient where there is a shortage of local tissues at the surgeon's disposal.

Bladder Exstrophy↗
PubMed2001

Construction of female urethra using buccal mucosa graft.

PURPOSE: We reviewed our experience with buccal mucosa grafts for reconstructing difficult female urethral problems. METHODS AND METHODS: Since 1994, we have used a buccal mucosa graft to reconstruct the urethra in 7 girls 3 to 13 years old. The underlying pathological condition was a fibrotic urethra after previous operations for cloacal exstrophy, cloacal malformation, iatrogenic urethral stricture, and multiple false passages in a previously reconstructed urethra of vaginal mucosa that made clean intermittent catheterization difficult. A full-thickness buccal mucosa graft was tubularized in situ as the neourethra to the base of the clitoris. In patients with cloacal exstrophy and cloacal malformation the bladder neck and urethra were widely exposed transabdominally by splitting the pubic symphysis. The fibrotic mucosa was excised and the tubularized buccal mucosa graft was wrapped with periurethral tissues. Other patients underwent transvaginal surgery in the prone position and the graft was covered with a buttock flap. RESULTS: Patients were followed for 12 to 58 months (mean 34.7). Those with cloacal exstrophy and cloacal malformation had been completely incontinent before urethral reconstruction but all attained complete continence postoperatively. They and the girl who underwent urethral reconstruction for difficult catheterization performed clean intermittent catheterization easily. The patient with urethral stricture voided via the urethra without difficulty. CONCLUSIONS: In select female patients with difficult urethral reconstructive problems a tubularized free graft obtained from the buccal mucosa may be effectively used when local tissue is fibrotic and unsuitable for creating a supple new urethra.

Adolescent↗
PubMed1997

Pseudoexstrophy of the bladder: case report and literature review.

The authors present a case of a boy newborn who had bladder pseudoexstrophy and multiple congenital anomalies. In addition to this unusual variant of the exstrophy-epispadias complex, the patient was found to have a posterior cleft palate, an omphalocele, and an imperforate anus.

Abnormalities, Multiple↗
PubMed1999

Orthotopic urethral substitution in female patients using the mitrofanoff principle.

PURPOSE: We present our experience with orthotopic urethral substitution in female patients using the Mitrofanoff principle. MATERIALS AND METHODS: We performed orthotopic urethral substitution using the Mitrofanoff principle in 18 female patients 1 to 29 years old (mean age 10). The conduit was constructed with appendix in 13 cases, tapered ileum in 4 and fallopian tube in 1. The etiology of incontinence included exstrophy in 8 patients, neurogenic bladder in 3, urogenital sinus in 3, and bilateral ectopic ureter, ectopic ureterocele, the prune-belly syndrome and post-cystectomy undiversion in 1 each. RESULTS: Mean followup was 29 months (range 9 to 72). A total of 16 patients achieved continence following a program of clean intermittent catheterization. There were 2 unsuccessful operations. In 1 case the appendix become ischemic and in 1 a vesicoperineal fistula developed. One patient catheterizes every 2 hours to avoid leakage. Catheterization was temporarily difficult in 5 patients, of whom 2 had an appendiceal and 3 had a tapered ileal conduit. Two patients with an ileal conduit have had chronic difficult catheterization. CONCLUSIONS: Orthotopic replacement of the urethra using the Mitrofanoff principle is suitable in highly select female patients who need continent diversion or a catheterizable conduit but who will not accept an abdominal stoma. It is particularly suited to patients in whom exstrophy reconstruction has failed.

Adolescent↗
PubMed2004

Quo vadis? Ureteric reimplantation or ignoring reflux during augmentation cystoplasty.

OBJECTIVE: To decide whether antireflux surgery should be used in the presence of vesico-ureteric reflux (VUR) in children, in whom an augmentation procedure is needed, because secondary VUR in children with a neurogenic bladder, infravesical obstruction and primary VUR in the exstrophy-epispadias complex is expected to resolve after augmentation, which decreases the intravesical pressure and increases capacity. PATIENTS AND METHODS: Between 1987 and 2001, the bladder was augmented in 38 children, using no antireflux surgery in group 1 (15 patients) and antireflux surgery in group 2 (23 patients). RESULTS: VUR was detected in all patients on cysto-urethrography before surgery; reflux resolved after augmentation cystoplasty in 97% and 93% of refluxing units in groups 1 and 2, respectively. The increase in the expected bladder capacity was from 35% to 86% in group 1 and from 38% to 90% in group 2. No patient had any deterioration in renal function. CONCLUSIONS: We recommend using only augmentation in patients with low- or high-grade VUR and a neurogenic bladder, infravesical obstruction and exstrophy-epispadias. Combining antireflux surgery with cystoplasty has no significant effect on either the resolution of VUR or renal function.

Child↗
PubMed2001

The Yang-Monti ileovesicostomy: a problematic channel?

OBJECTIVE: To compare the differences in the quality of Mitrofanoff channels created using appendix and re-tubularized small bowel (the Yang-Monti ileovesicostomy). Patients and methods The case-notes were reviewed retrospectively for all patients who underwent a Mitrofanoff procedure using either appendix or small bowel, over a 5-year period from June 1994 to July 1999. RESULTS: In all, 92 patients underwent 94 Mitrofanoff procedures; the appendix was used in 69 and small bowel in 25. The underlying diagnoses were exstrophy-epispadias complex (38), neuropathic bladder (21), anorectal malformations and cloacal anomalies (15), posterior urethral valves (nine) and miscellaneous (nine). The mean (range) age at operation was 9.2 (1.1-18.3) years. The mean (range) follow-up for the appendix group was 37 (6.7-65) months and for the Monti group 25 (6-66) months. Catheterization problems occurred in 18 (27%) patients from the appendix group; two needed an adjustment of technique, six dilatation and 10 revision. Stomal stenosis occurred in 10 (15%) patients, bladder level stenosis in four (6%) and conduit necrosis in two. Catheterization problems were reported in 15 (60%) patients from the Monti group; five needed revision, three dilatation and seven are being managed conservatively. The incidences of stomal stenosis (four, 16%) and bladder level stenosis (two, 8%) were comparable with the appendix group. In addition, two patients had distal channel (sub-stomal) stenosis and two had mid-channel stenosis. The problem unique to the Yang-Monti channel was a pouch-like dilatation in seven patients (28%), all of whom presented with catheterization problems; five are being managed conservatively and two have needed pouch resection. Stomal prolapse occurred in five (7%) patients in the appendix group, but in none of the Monti group. CONCLUSIONS: The appendix is the conduit of choice for a Mitrofanoff procedure. Re-tubularized small bowel conduits have a considerably higher incidence of catheterization problems. Anatomical factors may contribute to the unique incidence of pouch formation.

Adolescent↗
PubMed2004

Carcinoma of sigmoid colon following urinary diversion: a case report and review of literature.

BACKGROUND: The association of ureterosigmoidostomy with colonic cancer is well established. A 100-fold increased risk of malignancy has been proposed in association with ureterosigmoidostomy. Characteristically there is a latent period of around 20-30 years before the occurrence of cancer. CASE PRESENTATION: An unusual case of adenocarcinoma of the colon in a 36-year-old patient is presented. The patient underwent three operations in his infancy for exstrophy but after failure to close bladder, ureterosigmoidostomy was attempted at the age of 5 years and was converted to an ileal conduit after 8 months. At the age of 36 years, 30 years following ileal conduit urinary diversion for exstrophy, he presented in emergency with large bowel obstruction due to adenocarcinoma of the sigmoid colon. CONCLUSION: Patients who undergo urinary diversion for exstrophy may be kept on a regular follow-up surveillance colonoscopy as most of these young adults may later present with vague abdominal symptoms which may not be taken seriously until they increase to an extent as to present with intestinal obstruction as in the present case.

Journal Article↗
PubMed2004

Using color Doppler sonography to identify the perivesical umbilical arteries: a useful method in the prenatal diagnosis of omphalocele-exstrophy-imperforate anus-spinal defects complex.

OBJECTIVE: To describe the different prenatal sonographic findings in 3 cases of omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex, or cloacal exstrophy. METHODS: Three patients with OEIS complex were examined by sonography. In 2 (cases 2 and 3) of the 3 cases, color Doppler sonography was applied to the area of cord insertion and the abdominal mass to determine the origin of the abdominal mass. RESULTS: Three cases of OEIS complex with different sonographic appearances are included in this series. An absent bladder without an abdominal mass but with bowel floating in the amniotic cavity was revealed in case 1; an absent bladder with a lower anterior abdominal mass was found in the second trimester in case 2; and a large cystlike mass located in the anterior abdominal wall was found in case 3. Color Doppler imaging showed that the abdominal mass originated from the urinary bladder in cases 2 and 3; therefore, OEIS complex was presumptively diagnosed antenatally in these cases. In all cases, OEIS complex was confirmed postnatally. CONCLUSIONS: Omphalocele-exstrophy-imperforate anus-spinal defects complex should be considered in patients with an absent bladder combined with either an anterior abdominal wall mass or defects. Special attention should be given to search for other combined anomalies. We suggest that color Doppler sonography for identifying the perivesical umbilical arteries is a very useful method in establishing of the diagnosis of OEIS complex.

Adult↗
PubMed2001

Pseudoexstrophy in a female child.

A six-year-old female patient presenting with a swelling in the infraumbilical part of the abdomen, bulging out on straining, was diagnosed to have pseudoexstrophy bladder. The urinary tract was normal. The patient had bifid clitoris. There was no other associated malformation. Surgical repair of abdominal wall defect was done successfully. A new classification of exstrophy variants is proposed.

Abdominal Muscles↗
PubMed1998

Cloacal exstrophy--improving the quality of life: the Johns Hopkins experience.

PURPOSE: Exstrophy of the cloaca is a multisystem anomaly involving the gastrointestinal, nervous, musculoskeletal and genitourinary tracts which should be managed with a multidisciplinary approach. Improvement in management has led to survival for the majority of infants, and the focus has shifted to improvement in quality of life. The experience with management of cloacal exstrophy at a large center is evaluated. MATERIALS AND METHODS: Demographic data as well as functional results of management of the multiple anomalies in 37 patients with cloacal exstrophy were evaluated. Surgical reconstruction was aimed at providing the best functional and cosmetic results. RESULTS: Average patient age at review was 13.6 years. Most patients (32 of 37) had undergone an initial attempt at bladder closure from birth to greater than 24 months of age. Colostomy was performed when possible and if initial ileostomy was performed, the bowel was augmented later with the hindgut segment. When the hindgut segment was not used for bowel reconstruction, it was preserved for bladder augmentation or genital reconstruction. While urinary continence was achievable in many children, it was usually after augmentation and/or continent diversion. CONCLUSIONS: Improvements in perinatal management have increased survival in cloacal exstrophy. Therefore, the focus of reconstruction has shifted to reduction in the numbers of incontinent stomas, assistance with ambulation and improved cosmesis. All of these goals are achievable using a multidisciplinary approach to the management of this complex anomaly.

Abnormalities, Multiple↗
PubMed1991

Vesical neck reconstruction in patients with epispadias-exstrophy complex.

The achievement of satisfactory continence in the management of patients with the epispadias-exstrophy complex remains a challenge. Between 1971 and 1989, 22 children underwent vesical neck reconstruction at our hospital. Satisfactory continence was achieved in 17 of the 22 patients (77%), including 8 (36%) who are continent after a single vesical neck reconstruction and 9 of 13 who achieved continence after additional and/or adjunctive procedures. Additions and/or adjunctive procedures included revision of the vesical neck reconstruction, bladder augmentation and/or placement of an artificial urinary sphincter. We conclude that vesical neck reconstruction with or without additional and/or adjunctive procedures can result in an acceptable continence rate in patients with the epispadias-exstrophy complex.

Adolescent↗
PubMed1997

Urolithiasis in the exstrophy-epispadias complex.

PURPOSE: We sought to determine the incidence of urolithiasis in patients with the exstrophy-epispadias complex, associated risk factors and guidelines for the proper clinical management of this problem. MATERIALS AND METHODS: We retrospectively reviewed the chart and x-rays of 530 patients with the exstrophy-epispadias complex treated at our institution. RESULTS: Stones formed in 77 of the 530 patients (15%), including 16% of those with classic exstrophy, 25% of those with cloacal exstrophy and 3% of those with epispadias. White and male individuals were most commonly affected. Most calculi formed in the bladder, whether native or augmented by enterocystoplasty. Standard techniques were successful for stone removal. Most calculi consisted of calcium apatite, calcium oxalate monohydrate or magnesium ammonium phosphate, usually in combination with at least 1 other element. The risk of stone formation was associated with augmentation cystoplasty (p < 0.001) and a bladder neck procedure to increase outlet resistance (p < 0.001). Other risk factors included urinary tract infection, foreign bodies, vesicoureteral reflux and urinary stasis but not acidosis or immobilization. Stones recurred in 30 patients (39%), including equal numbers of those treated with open and closed techniques, and recurrence was associated with urinary tract infection or struvite composition (p < 0.05). Serum calcium abnormalities were not present in any patient, including those with metabolic acidosis or prolonged immobilization. Urinary chemistry studies were incomplete or unavailable. CONCLUSIONS: These data suggest that urolithiasis in the exstrophy-epispadias complex is related to risk factors associated with surgical reconstruction of this condition. The role of metabolic abnormalities that may predispose to urolithiasis is unknown but under investigation. Standard treatment is effective but stone recurrence remains a significant problem. Urine chemistry data may provide information to help minimize stone development in this patient population.

Abnormalities, Multiple↗
PubMed2006

Modified bladder neck reconstruction in patients with incontinence after staged exstrophy/epispadias closures.

PURPOSE: Reconstruction of the urethra without adequate circumferential muscular support is a significant problem in bladder neck surgery for urinary incontinence. Fascial, muscular and artificial slings have been used for support of the bladder neck after reconstruction. We used a demucosalized detrusor muscle pedicle to wrap around the bladder neck base along with other continence techniques in children who experienced incontinence after staged closure of exstrophy and epispadias. We describe our experience using the pedicle wraparound along with the Mitchell modification of Young-Dees-Leadbetter bladder neck reconstruction. MATERIALS AND METHODS: We reviewed our continence rates using a detrusor wraparound in 8 eligible patients with failed staged exstrophy-epispadias repair. We defined incontinence as any degree of leakage through the bladder neck day or night. RESULTS: Of the 8 patients studied 2 were female and 6 were male. Mean patient age at surgery was 7.6 years (range 4 to 11). Mean followup was 3.2 years (range 0.5 to 5). All patients with staged exstrophy-epispadias repair failure are currently continent. Five patients underwent simultaneous bladder augmentation. All but 2 patients catheterize via a Mitrofanoff channel. Three patients void volitionally and 5 use clean intermittent catheterization per Mitrofanoff. Two patients required dextranomer/hyaluronic acid injections at the bladder neck postoperatively to achieve complete dryness. CONCLUSIONS: The detrusor bladder neck wraparound, while successful, may require concomitant surgery, including augmentation, clean intermittent catheterization and endoscopic injection therapy, to achieve continence following failure of staged exstrophy-epispadias repair. The detrusor bladder neck wrap appears to be a safe and effective adjunctive procedure in this patient population. We believe it has an important role in the achievement of urinary dryness.

Adolescent↗
PubMed1991

Techniques to create urinary continence in the cloacal exstrophy patient.

Of 15 patients with cloacal exstrophy currently under management 11 have undergone procedures for the establishment of urinary continence. There were 8 genetic female subjects, 6 genetic male subjects raised as girls and 1 genetic male subject raised as a boy. The genetic female subjects had all undergone urethral reconstruction. In 4 patients urethral reconstruction was done with local tissues and they have subsequently undergone bladder neck reconstruction: 1 is continent and voids through the urethra, 2 are dry on intermittent catheterization and 1 is dry on intermittent catheterization after augmentation with a hindgut patch. One girl underwent a Kropp procedure along with bladder augmentation, and she is dry on intermittent catheterization, in 1 urethral reconstruction was done with an ileal nipple through which she performs intermittent catheterization and 2 await a continence procedure. The urethral reconstructions in the genetic male subjects were more difficult. Of the 6 genetic male subjects raised as girls 5 have undergone continence procedures and 1 awaits establishment of urinary continence. In only 1 patient was urethral reconstruction possible from local tissues to allow intermittent catheterization. In the other 4 the perineal urethra was closed in favor of an abdominal stoma for intermittent catheterization. Ileal plication with nipple formation of the stoma was done in 3 patients and a Benchekroun stoma was used in 1. The genetic male subject raised as a boy underwent exstrophy reclosure plus epispadias repair and subsequent bladder neck reconstruction and augmentation. Augmentation was performed in 5 patients with hindgut segments in 3 and ileum in 2. All 5 patients are currently dry on intermittent catheterization, although 1 required revision of the ileal nipple to a Benchekroun stoma. Staged reconstruction can produce acceptable urinary continence in this complex anomaly. An innovative approach is required to find the most suitable solution for each patient anatomy, bladder size and function, and mental, neurological and orthopedic status.

Bladder Exstrophy↗
PubMed1998

The prenatal diagnosis of cloacal exstrophy.

PURPOSE: We assess and clarify diagnostic features for making the prenatal diagnosis of cloacal exstrophy. MATERIALS AND METHODS: We evaluated 9 patients born with cloacal exstrophy at our institutions (2 prospectively and 7 retrospectively) for diagnostic features on prenatal ultrasound studies. We also thoroughly reviewed the literature on 13 previous prenatally diagnosed cloacal exstrophy cases. Diagnostic criteria were assessed by combining the findings in our patients and those in previous reports. RESULTS: Of the 22 patients with prenatal ultrasound studies and cloacal exstrophy whom we analyzed 1 of our 9 and 2 in the literature had a cloacal membrane that persisted at 22 weeks of gestation. Major ultrasound criteria for diagnosing cloacal exstrophy prenatally are nonvisualization of the bladder, a large midline infraumbilical anterior wall defect or cystic anterior wall structure (persistent cloacal membrane), omphalocele and lumbosacral anomalies. Seven less frequent or minor criteria include lower extremity defects, renal anomalies, ascites, widened pubic arches, a narrow thorax, hydrocephalus and 1 umbilical artery. CONCLUSIONS: We propose major and minor criteria to assist in the prenatal diagnosis of cloacal exstrophy. Despite these major and minor criteria the certainty of establishing a prenatal diagnosis remains challenging. Persistence of the cloacal membrane beyond the first trimester in 1 patient was an exception to the classic concept of cloacal exstrophy embryogenesis. An accurate prenatal diagnosis requires validation of these criteria by further correlation of prenatal and postnatal observations.

Cloaca↗
PubMed2006

Morphology of the enteric nervous system in the hindgut of an infant with cloacal exstrophy.

The authors describe the morphology of the enteric nervous system in the hindgut of an infant with cloacal exstrophy. Cloacal exstrophy was diagnosed at 32 weeks' gestation using prenatal ultrasonography. The baby was delivered at 34 weeks' gestation and underwent a separation of the cecum from bladder halves, reapproximation of hemibladders, closure of the omphalocele and pubic symphysis, and a distal colostomy. Intestinal wall specimens were obtained at colostomy from the distal end of the rudimentary hindgut. Serial frozen sections were prepared for histochemical acetylcholinesterase staining. Histological investigations demonstrated a strikingly crowded, immature enteric ganglia and prominent bundles of wandering cholinergic nerves. These findings suggest the unique pathology of the enteric nervous system development in cloacal exstrophy, in which the rudimentary hindgut behaves as a blind alley of the migratory pathway for neural crest-derived cells during embryogenesis. Histological examinations of the hindgut enteric nervous system in cloacal exstrophy may be beneficial for evaluating the postnatal development of the distal colon which might be utilized for a pull-through procedure.

Bladder Exstrophy↗
PubMed2000

Outcome of gastrocystoplasty in tertiary pediatric urology practice.

PURPOSE: Although the use of stomach for bladder reconstruction has become popular during the last decade, it is not a panacea. We review our experience with gastrocystoplasty. MATERIALS AND METHODS: We completed a retrospective chart review of 11 females and 12 males, 1.5 to 22.5 years old (mean age 10) who underwent gastrocystoplasty at Hôpital Sainte-Justine, Montreal, Quebec and Children's Hospital, Winnipeg, Manitoba, Canada between December 1990 and 1998. Primary diagnoses included spinal dysraphism in 14 patients, posterior urethral valves in 3, cloacal exstrophy in 2, cloacal outlet anomaly in 2, multiple failed ureteral reimplantations with bladder dysfunction in 1 and neurogenic bladder of uncertain etiology in 1. Three patients presented with chronic renal failure. Concurrent reconstructive surgery included ureteral reimplantation in 10 patients, bladder neck plasty in 4 or closure in 4, and continent urinary diversion in 5. RESULTS: Acute postoperative complications included urosepsis in 2 cases, bowel obstruction in 2 and ureteral obstruction in 1. Followup ranged from 4 to 86 months (mean 45). Long-term complications consisted of intractable hematuria-dysuria syndrome in 5 cases, inability to catheterize in 3, perineal urinary fistula in 2, new onset hydronephrosis in 2, continent stomal stenosis in 1 and bladder calculus in 1. Proton pump inhibitors and/or histamine 2 antagonists were used in 16 of the 23 patients to prevent the hematuria-dysuria syndrome. In 5 cases the hematuria-dysuria syndrome was poorly controlled medically and 3 were converted to another form of urinary reconstruction. In 18 of 20 cases voiding cystourethrography revealed no vesicoureteral reflux, and in 18 of 21 ultrasound documented stable or improved upper tracts. Socially acceptable urinary continence was attained in 19 of the 21 patients. CONCLUSIONS: The use of stomach for bladder augmentation may be considered in patients with cloacal exstrophy and/or metabolic acidosis. Histamine blockers and/or proton pump inhibitors may be required to prevent the hematuria-dysuria syndrome. Symptoms of the hematuria-dysuria syndrome may be disabling and may mandate alternative forms of urinary tract reconstruction.

Acidosis↗
PubMed1989

Splitting the pubis for exposure in difficult reconstructions for incontinence.

In the last 13 years splitting the pubic symphysis has been used in 46 patients 1 to 32 years old for wide access to the bladder neck and entire urethra in female, or the bladder neck and urethra down into the bulb in male patients. Each patient had a complex problem to reconstruct and most had undergone a prior operation. Underlying pathological conditions included exstrophy in 16 patients, epispadias in 11, cloacal exstrophy in 6, posterior urethral valves in 3, severe trauma in 2, bilateral single ectopic ureters in 2, radical surgery for sarcoma in 2, myelodysplasia in 2, cloacal anomaly in 1 and iatrogenic injury of the urethra in 1. The pubis, or interpubic ligament in exstrophy cases, was divided in the midline. The pubic rami were spread with a laminectomy spreader to create a gap sufficient to operate on that segment of the urethra, which is usually not accessible with an intact pubis. No patient had a problem of nonunion or pubic osteitis after using this approach. This series includes 2 patients undergoing simultaneous iliac osteotomies for a widened symphysis pubis. In no patient was a segment of pubis resected, as has been used in transpubic repair of certain traumatic urethral strictures.

Adolescent↗