In 1981, Mitrofanoff described a procedure to create a continent urinary stoma for clean intermittent catheterization. Since then several procedures have been described including Yang-Monti ileovesicostomy for effective catheterization.
We report on our experience from the use of Monti’s procedure in children at our center.
Patients and Methods
Children < 18 years of age undergoing urinary diversion/reconstruction with Yang-Monti’s procedure for congenital conditions or neuropathic bladder formed the study group. All these children, post-operatively were taught clean intermittent catheterization (CIC) and put on a regime using a 14/16 Fr catheter every 3 hours. The children were followed regularly at 3, 6, 12, 18 and 24months post-operatively, with special attention paid to any problems with catheterization and incontinence.
During the period from Jan 2000 to Dec 2011, at our center, 19 children less than eighteen years of age underwent urinary diversion with Yang-Monti’s catheterizable stoma. The indications for urinary diversion was neuropathic bladder in eight, exstrophy bladder in seven , valve bladder syndrome in three and persistent urethral stricture in one. None of the children found CIC difficult during the post-operative period and there was no hindrance to the passage of the catheter.
Although the appendix remains the tissue of choice for creation of catherterizable stoma, the Yang-Monti ileovesicostomy is effective, convenient conduit for children. Long-term complications are minimal and children find this comfortable to do CIC.
Urinary Bladder; Urinary Diversion; Surgical Stomas; Ileum; Catheterization
The Mitrofanoff principle was originally described as a method to provide an alternative means to access the bladder. It creates a conduit to the bladder through which patients with a sensitive, absent, or traumatized urethra can perform clean intermittent catheterization (CIC) easily. We report our experience with complete laparoscopic Mitrofanoff appendicovesicostomy to promote a catheterizable abdominal stoma.
Materials and Methods:
A 4-port transperitoneal approach was used to create a complete laparoscopic Mitrofanoff appendicovesicostomy.
Six children with a mean age of 12.8 years (range 9-16 years) underwent laparoscopic Mitrofanoff appendicovesicostomy. Mean operative time was 139.6 min and Mean estimated blood loss was 46 cc. No cases of urinary leaks were noted. There have been no cases of either stomal stenosis or appendicovesical stenosis noted.
Pure laparoscopic Mitrofanoff appendicovesicostomy is feasible and is associated with reasonable outcome with early recovery, resumption of normal activities and excellent cosmesis.
Laparoscopy; mitrofanoff; surgical stoma; urinary diversion
Hydronephrosis, reflux and renal failure are serious complications that occur in patients with neurogenic bladder associated with myelomeningocele. When the bladder compliance is lost, it is imperative to carry out surgery aimed at reducing bladder storage pressure. An ileocystoplasty, and for patients not suitable for intermittent catheterization, using the Mitrofanoff principle to form a continent stoma and the subsequent closure of the bladder neck, can be used. We report here, for the first time to the best of our knowledge, an association between two previously described techniques (the Mitrofanoff principle and the technique of Monti), that can solve the problem of a short appendix in obese patients.
A 33-year-old male Caucasian patient with myelomeningocele and neurogenic bladder developed low bladder compliance (4.0 mL/cm H2O) while still maintaining normal renal function. A bladder augmentation (ileocystoplasty) with continent derivation principle (Mitrofanoff) was performed. During surgery, we found that the patient's appendix was too short and was insufficient to reach the skin. We decided to make an association between the Mitrofanoff conduit and the ileal technique of Monti, through which we performed an anastomosis of the distal stump of the appendix to the bladder (with an antireflux valve). Later, the proximal stump of the appendix was anastomosed to an ileal segment of 2.0 cm that was open longitudinally and reconfigured transversally (Monti technique), modeled by a 12-Fr urethral catheter, and finally, the distal stump was sutured at the patient's navel.
After the procedure, a suprapubic cystostomy (22 Fr) and a Foley catheter (10 Fr) through the continent conduit were left in place. The patient had recovered well and was discharged on the tenth day after surgery. He remained with the Foley catheter (through the conduit) for 21 days and cystostomy for 30 days. Six months after surgery he was continent with good bladder compliance without reflux and fully adapted to catheterization through the navel.
The unpublished association between the Mitrofanoff and Monti techniques is feasible and a very useful alternative in urologic cases of derivation continent in which the ileocecal appendix is too short to reach the skin (i.e., in obese patients).
Since the original description of the trans-appendicular continent cystostomy by Mitrofanoff in 1980, a variety of techniques have been described for creating a continent catheterisable channel leading to the bladder, which avoids the native urethra. The Mitrofanoff principle involves the creation of a conduit going into a low pressure reservoir, which can emptied through clean intermittent catheterization through an easily accessible stoma. A variety of tissue segments have been used for creating the conduit, but the two popular options in current urological practice remain the appendix and Yang-Monti transverse ileal tube. The Mitrofanoff procedure has an early reoperation rate for bleeding, bowel obstruction, anastomotic leak or conduit breakdown of up to 8% and the most common long-term complication noted is stomal stenosis resulting in difficulty catheterizing the conduit. However, in both pediatric and adult setting, reports imply that the procedure is durable although it is associated with an overall re-operation rate of up to 32% in contemporary series. Initial reports of laparoscopic and robotic-assisted Mitrofanoff procedures are encouraging, but long-term outcomes are still awaited.
Appendix; Mitrofanoff; reconstruction
It is a great challenge to select and perform continent mechanism in a stoma for urinary reservoir. A new technique by combining MONTI ileal conduit with the serosal lined trough in order to achieve continent catheterizable stoma to the umbilicus as a part of augmentation ileocystoplasty. We applied serosal-lined trough as a continent mechanism with MONTI ileal tube in 12 years smart girl underwent ileocystoplasty for neuropathic bladder due to meylomeningocele in whom continence failed to be achieved by using Mitrofanoff with submucosal tunnel of the bladder as continent mechanism before, also the previous operation included left to right transuretero-ureterostomy, ureterocystoplasty and reimplantation of the right ureter. The patient became completely continent; she was able to do self-catheterization easily through the umbilical stoma using 16-French catheter and was able to wash the mucous easily. The capacity of the augmented bladder was 300ccs. She became independent from her mother and stopped using diapers, anticholinergic and antibiotics. Combining MONTI conduit with serosal-lined extramural valve trough (The Ghoneim technique) is an effective continent technique and gives wider channel for catheterization and washing out the mucous.
Continent stoma; ileocystoplasty; Mitrofanoff; monti0; serosal lined trough; ureterocystoplasty
Surgical management for neurogenic bladder may require abandonment of the native urethra due to intractable urinary incontinence, irreparable urethral erosion, severe scarring from previous transurethral procedures, or urethrocutaneous fistula. In these patients, bladder neck closure (BNC) excludes the native urethra and provides continence while preserving the antireflux mechanism of the native ureters. This procedure is commonly combined with ileovesicostomy or continent catheterizable stoma, with or without augmentation enterocystoplasty. Alternatively, BNC can be paired with suprapubic catheter diversion. This strategy does not require a bowel segment, resulting in shorter operative times and less opportunity for bowel-related morbidity. The study purpose is to examine preoperative characteristics, indications, complications, and long-term maintenance of renal function of BNC patients.
A retrospective review of medical records of 35 patients who underwent BNC with suprapubic catheter placement from 1998 to 2007 by a single surgeon (LKL) was completed.
Neurogenic bladder was attributable to spinal cord injury in 71%, 23% had multiple sclerosis, and 9% had cerebrovascular accident. Indications for BNC included severe urethral erosion in 80%, decubitus ulcer exacerbated by urinary incontinence in 34%, urethrocutaneous fistula in 11%, and other indications in 9%. The overall complication rate was 17%. All but two patients were continent at follow-up. Forty-nine per cent of patients had imaging available for review, none of which showed deterioration of the upper tracts.
Our results suggest that BNC in conjunction with suprapubic catheter diversion provides an excellent chance at urethral continence with a reasonable complication rate.
Bladder; Urethra; Surgical treatment
There are only 7 cases reported in the literature of squamous cell cancer of the bladder in patients performing intermittent self-catheterization (ISC). We report on an eighth case, and the first case described in a patient with a Mitrofanoff continent appendicovesicostomy. A description of the case and review of the literature are presented. Risk factors for squamous cell cancer include recurrent urinary tract infections, keratinising squamous metaplasia (leukoplakia) and local mucosal trauma from intermittent self-catheterization. There is no recognized or validated monitoring program for patients performing ISC who may also have these risk factors. Reasonable protocols may include regular urinary cytology and cystoscopy with random or targeted bladder biopsies. Squamous cell cancer may present late in this cohort of patients and is associated with a dismal prognosis.
We report on the evaluation and management of a 47-year-old white male found to have primary carcinoid tumor of the ileal segment of his diverting ileovesicostomy thirty-five months after initial creation. Subsequent to presentation with intermittent gross hematuria, CT urogram highlights an 8 mm enhancing lesion near the enterovesical junction of urinary diversion. Office cystoscopy confirms presence of a lesion that was later endoscopically resected and found to be a well-differentiated carcinoid tumor. Evaluation with serum markers, direct visualization utilizing endoscopy, and imaging was without finding of alternate primary or metastatic lesions. The patient ultimately had the proximal ileal portion of his ileovesicostomy excised and the distal portion converted into an ileal conduit. After briefly discussing the carcinoid tumor and the carcinoid syndrome it may cause, we review the literature on the incidence of carcinoid tumors in a population requiring the use of intestine in the urinary tract.
Objectives. To review the literature regarding ileovesicostomy and evaluate our patient population for clinical characteristics. Methods. Various surgical reconstructive techniques allow management of difficult clinical scenarios involving patients with neurogenic bladder, irretraceable lower urinary tract symptoms, lower urinary tract disaster, and urethrocutaneous fistulae. One such reconstructive technique employed is the ileovesicostomy. This procedure provides patients with a low-pressure urinary conduit utilizing the ileum and native bladder that empties without catheterization. We describe our patient population who underwent ileovesicostomy for 5 consecutive years ending 2007 at Detroit Receiving Hospital. Results. Most common diagnosis was neurogenic bladder secondary to spinal cord injury. Our population and clinical outcomes are similar to those previously reported in the literature. Conclusions. Based on our experience, we suggest that patients with severe lower urinary tract symptoms and who are unable to perform clean intermittent catheterization and/or refractory to medical therapy ileovesicostomy should be the procedure of choice.
Distal ureteric stricture is a common complication of urinary schistosomiasis which is a disease more prevalent in the tropics and subtropics. The surgical management of this complication is more challenging when it affects more than half of both ureters. We report the case of a 17-year-old Nigerian with a long standing recurrent painless terminal hematuria associated with bilateral colicky loin pains. Ultrasound scan showed bilateral hydro ureters and hydro nephrosis with deranged biochemical renal function. The patient had bilateral tube nephrostomy and antibiotic therapy. Definitive bilateral ureteric substitution was done using Mitrofanoff technique for the right ureter and Yang-Montie technique for the left ureter. The patient′s renal function became normal and he was discharged home without complication. The related literatures were reviewed. Surgical nonurothelial ureteral substitution is necessary for long, extensive, severe bilateral ureteric strictures so as to prevent progressive renal damage and end stage renal failure.
Appendix; intestine; schistosomiasis; stricture; ureter; ureteric substitution
To evaluate the outcome of innervation preserving sphincteroplasty along with anatomical bladder neck reconstruction (IPS-ABNR) compared to classic Young-Dees-Leadbetter (YDL) bladder neck reconstruction in exstrophy with insufficient bladder capacity requiring detubularized-ileocystoplasty.
Materials and Methods:
Sixteen male patients of exstrophy bladder who required ileocystoplasty from 2004 to 2010 were randomized into group A (n = 7) and group B (n = 9). After detubularized-ileocystoplasty with Mitrofanoff stoma and ureteric reimplantation in all, group A received YDL bladder neck repair while group B received IPS-ABNR repair through a midline scrotoperineal approach. Outcome measurement included operative and postoperative problems, continence, and upper tract status.
In group A, two had incompetent bladder neck with gross incontinence, while four had a dry interval of more than 3 h without the ability of voiding per urethra. In group B, seven patients had dry interval of more than 3 h with an ability of urethral voiding and midstream holding in five.
Exstrophy patients requiring augmentation cystoplasty and repaired with IPS-ABNR can achieve dynamic bladder outlet resistance with adequate leak point pressure and ability to void voluntarily with midstream holding capability. The children had the satisfaction of voiding per urethra with ability to stop in midstream similar to that in normal children.
Anatomical bladder neck reconstruction; augmentation cystoplasty; exstrophy bladder; ileocystoplasty; innervation preserving sphincteroplasty; Young-Dees- Leadbetter bladder neck reconstruction
Malignant melanoma originating in the urethra is considered extremely rare and has a very poor prognosis. Consequently, therapeutic reviews are retrospective describing assorted treatments. We report how to perform a radical urethrectomy with bladder preservation and a continent catheterizable stoma (Yang-Monti technique) Radical urethrectomy with bladder preservation and a continent catheterizable stoma may be appropriate in selected patients with tumours that do not invade the bladder neck.
Female genital; primary urethral melanoma; radical urethrectomy; Yang-Monti technique
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.
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.
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.
Urinary diversion; adenocarcinoma colon; exstrophy
The surgical management of urinary bladder exstrophy is challenging. This paper describes the personal experience in a tertiary care hospital over a period exceeding a quarter of a century.
During the period 1984-2010, 248 patients of the epispadias-exstrophy complex have been treated. The cases of classical bladder exstrophy (n = 210) form the basis of this paper. The stages/procedures used in the surgical reconstruction of bladder exstrophy included bladder closure with anterior abdominal wall reconstruction, bladder neck repair, ureteric reimplantation, epispadias repair and augmentation colocystoplasty in various combinations. Some of these patients had their initial operations done prior to 1984 or in other hospitals. Evaluation methods included, amongst others, clinical evaluation and urodynamic assessment. Eight patients opted out of treatment; 15 patients underwent permanent urinary diversion by either ureterosigmoidostomy or colon conduit. The remaining 187 patients were treated with bladder reconstruction, and of these, 132 patients have had at least one attempt at bladder neck reconstruction with 56 of these patients having undergone an augmentation colocystoplasty.
A total of 105 patients had socially acceptable continence: 57 from the bladder neck reconstruction group and 48 from the bladder augmentation group. Further attempts at continence surgery have been offered to the inadequately continent patients.
Surgical management of bladder exstrophy demands patience and perseverance. It is possible to provide all patients with socially acceptable continence with bladder neck division and catheterizable continent stoma as the last resort. Urodynamic assessment has emerged as an essential tool in the follow-up evaluation of these patients. Anticholinergic medication with imipramine or oxybutinin is a useful adjunct in the overall management.
Anticholinergic medication; bladder augmentation; bladder closure; bladder neck repair; epispadias repair; ureteric reimplantation; urinary bladder exstrophy; urinary continence; urodynamics
To gain a better understanding of the outcomes of the Mitrofanoff procedure for urinary diversion in children with spinal cord injury (SCI).
Individuals 6 to 27 years of age with SCI with at least 1 year follow-up after the Mitrofanoff procedure. Objective data collected via retrospective chart review include general demographics and medical/surgical history. Data collected via structured telephone interview include history of adverse urological events, bladder management, bladder management independence scores, patient satisfaction, and quality of life.
Sixteen subjects (13 female, 3 male) with a mean age of 19 years (range 6–27 y) who underwent the Mitrofanoff procedure were interviewed. Length of postoperative follow-up ranged from 1 to 8 years (mean 4.25 y). Complications included stomal stenosis 25% (n = 4) with a mean of 19 months to first occurrence of stenosis; urethral incontinence 75% (n = 12); renal/bladder calculi 19% (n = 3); and stomal leakage 44% (n = 7). Independence scores for bladder management after the Mitrofanoff procedure improved in 84% of subjects with tetraplegia and 25% of subjects with paraplegia. Eighty-eight percent (n = 14) were satisfied with the procedure, while 12% (n = 2) were somewhat satisfied. A thematic analysis of quality of life revealed that freedom (35%) and independence (35%) were most commonly cited.
While some subjects experienced complications, satisfaction was relatively high and level of independence in bladder management was greatly improved. This study demonstrates that the Mitrofanoff procedure is a beneficial option to improve independence and ease of bladder management in children with SCI.
Spinal cord injuries; Spina bifida; Neurogenic bladder; Mitrofanoff procedure (appendico vescicostomy); Urinary diversion; Vesicosphincteric dyssynergy; Tetraplegia; Paraplegia
Despite advances in the management of exstrophy epispadias complex (EEC), the quality of life of these patients is far from good. The post-operative period is complicated by numerous and variable events - infection, dehiscence, upper tract dilatation with deterioration, fistulas, stone formation and incontinence to name a few of the major complications. Redo surgery for bladder closure, bladder neck reconstruction, epispadias repair and closure of fistulas are frequently required. The current focus is on limiting the frequency and morbidity of the reconstructive procedures. A successful initial closure and early satisfactory cosmetic and functional results are gratifying for the family and the health care team, but this is only the beginning of the lifelong care necessary for bladder exstrophy (BE) patients. In this article, the long-term outcome of various treatment options and the continent procedures in BE has been reviewed, tracing the journey of these patients into adolescence and adulthood.
Exstrophy; epispadias; incontinence; bladder neck; ureterosigmoidostomy
Aims and Objective:
To postulate a hypothesis to explain the embryogenesis of exstrophy bladder based on our clinical observations.
Materials and Methods:
In 27 cases of exstrophy, we measured the distance between the lowermost inguinal skin crease to the root of the penis (clitoris) (B) and the distance between the penis (clitoris) and the scrotum (labia majora) (C). These were compared with age, height and XP distance (distance between xiphisternum and symphysis pubis) matched control group of normal children. The distance between the lowermost inguinal skin crease and the penis (clitoris) (A) was measured in control group.
The observation was A = B + C. This implies that in exstrophy bladder, the position of the penis (clitoris) has moved cephalad from the lower border of A to the junction of B and C.
Based on the observations, we postulate that abnormal origin of genital tubercle may be the cause of exstrophy bladder. The abnormal origin of primordia of the genital tubercle in more cephalad direction than normal causes wedge effect, which will interfere with the medial migration of the mesoderm as well as the midline approximation of mesodermal structures in the lower abdominal wall, thereby resulting in the exstrophy of bladder.
Bladder exstrophy; embryology of cloaca; genital tubercle
We report our experience with the treatment of classic exstrophy of the bladder in a small series of seven adult males. There are very few documented cases of adults presenting with exstrophy of bladder in literature.
Materials and Methods
Adult males presenting with classic exstrophy of the bladder and complete epispadias underwent detailed evaluation including psychological assessment and counseling. All were explained regarding the surgical procedure and informed about the need for self-catheterization. Prior to 2002 all patients underwent cystectomy of the existing bladder plate, with creation of catheterizable modified Mainz pouch. Since 2002 patients were assessed for bladder reconstruction with augmentation ileocystoplasty, bladder neck reconstruction, and abdominal wall closure.
Seven adult males with classic exstrophy of the bladder and complete epispadias who had not received any previous treatment presented to us during the period 1991-2006. Five of these underwent cystectomy with continent pouch and the remaining two underwent bladder reconstruction. All have been doing well with improved self-esteem and social interaction. Two of these have married and leading a satisfactory sexual relationship.
Surgical correction in adults with exstrophy of the bladder greatly improves self-esteem, confidence, and social relationship.
Adult; augmentation ileocystoplasty; bladder reconstruction; epispadias; exstrophy bladder
Few groups have examined health related quality of life for adolescents with bladder exstrophy-epispadias. We studied parent reported health related quality of life for adolescents with bladder exstrophy-epispadias using the Child Health Questionnaire-Parent Form 50.
Materials and Methods
We recruited 11 to 17-year-old participants with bladder exstrophy-epispadias and their parents. Parents served as proxy respondents for the adolescents by self-administering a validated generic health related quality of life instrument, the Child Health Questionnaire-Parent Form 50. We collected urinary incontinence, catheterization status, and medical and surgical history data. Mean questionnaire scores were compared to population based norms.
Median age of the 55 patients was 14 years, 69% were male and 84% were white. Diagnoses included bladder exstrophy in 48 cases and epispadias in 7. Of the participants 29 (53%) reported urinary incontinence. The median number of lifetime surgeries was 9. Although physical and psychosocial summary measure scores were comparable to norms, the mean general health perception score was significantly worse than that of a population based sample (65.8 points, 95% CI 61.4–70.2 vs 73, 95% CI 71.3–74.7, p = 0.004). Mean family activity and parent emotional impact scores were also significantly worse than in a population based sample (83.6 points, 95% CI 79.3–88.0 vs 89.7, 95% CI 87.9–91.5, p = 0.02 and 67.7, 95% CI 61.9–73.6 vs 80.3, 95% CI 78.4–82.2, p <0.0001, respectively). Comparison of incontinent to continent children revealed a lower mean score on the parent emotional impact scale (62.6 points, 95% CI 55.5–69.8 vs 73.4, 95% CI 63.9–82.9), which approached significance (p = 0.06).
Although overall adolescent quality of life was comparable to norms, parents reported significantly impaired adolescent general health and family activity as well as a negative parental emotional impact. Further research is needed to identify interventions that can decrease the adverse impact of bladder exstrophy-epispadias on family activity and parent emotional distress.
urinary bladder; bladder exstrophy; quality of life; adolescent; congenital; hereditary and neonatal diseases and abnormalities
Bladder exstrophy is a very rare congenital disorder, in which the first stage of reconstruction is usually performed within the first 72 hours of life. The most feared form of failure of the reconstruction is postoperative dehiscence of the bladder and abdominal wall. We present an 11-year-old girl with bladder exstrophy. She underwent three iliac bone supra-acetabular osteotomies with repair of the bladder exstrophy. Unfortunately the diastasis of the symphysis recurred widely open with dehiscence of bladder and abdominal wall.
Bilateral pubic and ischial rami osteotomies with adequate soft tissue release were carried out, which allowed the urology team to perform a tension-free repair of the bladder and the abdominal wall.
Here we report an osteotomy with the soft tissue release that succesfully allowed the closure of the pelvis, bladder and anterior abdominal wall in a recurrent case with more than three years follow up.
Bladder exstrophy; Ischail osteotomy; Pelvic osteotomy; Pubic osteotomy; Recurrent repair; SMS osteotomy; Steel osteotomy
Urinary carcinogens promote late malignant transformation of the colon after a ureterosigmoidostomy. An unusual case is presented where, despite the early removal of the latter and hence cessation of urine flow, a colonic carcinoma developed at the site of previous anastomosis. The importance of surveillance of all patients who have undergone this procedure to avoid an iatrogenic cancer is emphasised.
Keywords: rhabdomyosarcoma; ureterosigmoidostomy; colonic carcinoma
Bladder exstrophy is a rare congenital anomaly, caused by abnormal development of the cloacal membrane. To our knowledge, 18 familial patients with this malformation have been described. Two sets of familial cases with bladder exstrophy are reported here: two cousins and a mother and son and the published reports of the 18 familial cases among 682 index patients with bladder exstrophy are reviewed. Ultrasonography is advocated as the investigation of choice for early prenatal diagnosis.
Surgery for bladder exstrophy has been evolving over the last four to five decades. Because survival has become almost universal, the focus has changed in the exstrophy-epispadias complex to improving quality of life. The most prevalent problem in the long-term function of exstrophy patients is the sexual activity of the adolescent and adult males. The penis in exstrophy patients appears short because of marked congenital deficiency of anterior corporal tissue. Many patients approach for genital reconstruction to improve cosmesis as well as to correct chordee. We report our series of male patients seeking genital reconstruction following exstrophy repair in the past.
Materials and Methods:
Fourteen adolescent/adult male patients attended urology services during the period January 2000-December 2009 seeking genital reconstruction following exstrophy repair in the past.
Three patients underwent epispadias repair, four patients had chordee correction with cosmetic excision of skin tags and seven patients underwent chordee correction with penile lengthening. All patients reported satisfaction in the answered questionnaire. Patients undergoing penile lengthening by partial corporal dissection achieved a mean increase in length of 1.614 ± 0.279 cm dorsally and 1.543 ± 0.230 cm ventrally. The satisfactory rate assessed by the Short Form-36 (SF-36) showed that irrespective of the different genital reconstructive procedures done, the patients were satisfied with cosmetic and functional outcome.
Surgical procedures have transformed the management in these patients with bladder exstrophy. Bladders can be safely placed within the pelvis, with most patients achieving urinary continence and cosmetically acceptable external genitalia. Genital reconstruction in the form of correction of chordee, excision of ugly skin tags and lengthening of penis can be performed to give the patients a satisfactory cosmetic and functional system.
Exstrophy; function; genital reconstruction; outcome
Bladder exstrophy occurs in approximately 1 in 35,000 live births and is associated with an increased incidence of bladder cancer.
The primary mucinous adenocarcinoma of the bladder is an extremely rare urologic entity, which is found in less than 2% of all urinary bladder tumours and is often presented as metastatic. This is the first case in literature of a primary mucinous adenocarcinoma of an unreconstructed exstrophic bladder.
A 55-year old male patient was diagnosed with a primary mucinous adenocarcinoma of an unreconstructed exstrophic bladder. Examination of the entire gastrointestinal tract shown there were not other primary cites. Immunohistochemistry confirmed the nature of the tumour. The patient underwent a radical cystoprostatectomy with en block bilateral pelvic lymphadenectomy, urinary diversion with a cutaneous ureterostomy and epidpadias repair.
Adult bladder exstrophy and epispadia correction is a very rare practice in urology due the fact that this congenital disease is diagnosed and corrected in neonates. We advocate the radical surgical management, after exclusion of any primary malignant sites related to the gastrointestinal tract.
A 16-year-old female presented with dribbling of urine along with voluntary voiding since birth. Renal imaging revealed hydroureteronephrosis on the right side; the uterus and ovary were normal. A radionuclide scan showed a left nonfunctional kidney. On cystovaginoscopy, the urethra was shown to be normal and the urinary bladder was tubular with small capacity and an absent trigone. Although the vagina was capacious, no ureteric orifices were found. Computed tomography corroborated the diagnosis of bilateral, single ectopic ureters draining into a grossly dilated vagina. This case is unique because it is a bilateral single-system ureteral ectopia in a completely differentiated female genital tract that presented late in adolescence. To the best of our knowledge, this is the second such ureteral abnormality reported in the literature so far. The patient underwent ileocystoplasty with right ureteric reimplantation and nephroureterectomy for the left nonfunctional kidney, which histopathology showed to be tuberculosis. The patient is continent with cystometric capacity of more than 300 mL.