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PubMed1986

The symphysis pubis. Anatomic and pathologic considerations.

The symphysis pubis is a nonsynovial amphiarthrodial joint that is situated at the confluence of the two pubic bones. A thick intrapubic fibrocartilaginous disc is sandwiched between thin layers of hyaline cartilage. The inferior pubic ligament provides most of the joint's stability. Anatomic sections demonstrate a symphysis by the end of the second month of gestation. Thick cartilaginous end-plates are present at birth but become thin by the time of skeletal maturity. Congenital diseases resulting in failure of symphysis formation include exstrophy of the bladder and cleidocranial dysostosis. Both pyogenic and tuberculous infectious diseases involve the symphysis. Metabolic disease, such as renal osteodystrophy, produces widening, while ochronosis results in calcific deposits in the symphysis. Inflammatory disease, such as ankylosing spondylitis, results in bony fusion of the symphysis. Osteitis pubis, the most common inflammatory disease, is treated with anti-inflammatory medication and rest. Degenerative joint disease of the symphysis, which can cause groin pain, results from instability or from abnormal pelvic mechanics. As is the case with most joints, the symphysis serves as a barrier to tumor invasion. The patterns of trauma include diastasis, straddle fracture, intraarticular fracture and overlapping dislocation, and combinations of injuries.

Adolescent↗
PubMed1979

Colorectal neoplasm: a rare complication of ureterosigmoidostomy.

A 24-year-old woman had a rectal neoplasm which occurred 20 years after she had undergone ureterosigmoidostomy for exstrophy of the bladder. Only 41 cases of bening or malignant tumours of the large bowel developing after ureterosigmoidostomy have previously been reported in the literature. Adenocarcinoma of the colon is most frequently found and can develop even after urine has been diverted away from the colon. The presenting symptoms are either those of ureteral obstruction or of colorectal neoplasm. This rare complication remains intriguing and the possible pathogenetic mechanisms are discussed.

Adult↗
PubMed1977

[Human celosomias].

Definition and nomenclature of celosomia are based on a historical review which provides a system of classification. Morphology and organogenesis of celosomia were studied on 64 embryonic and foetal bodies. The anatomical study of 6 cases of human celosomia provided a definition of the characteristics of major celosomia, by showing the constant elements of the syndrome (particularly the parietal malformation) and the anomalies frequently found in each of the different types:--anterior (or superior) celosomia;--middle celosomia, including laparoschisis;--posterior (or inferior) celosomia, among which exstrophy of the bladder may be either one of the constant elements of the syndrome, or a limited form of the inferior celosomia;--total celosomia, of which one case was studied for this report, and of which the major forms, the schistosomia, chelinosomia and strophosomia types, seem to constitute forms of transition with other types of monstruosity. With improved knowledge of anatomy and organogenesis the possibilities of treatment have improved over the past few years, but still remain limited in the more monstrous forms of celosomia.

Abdomen↗
PubMed1980

Epispadias.

Surgical procedures in 14 patients with epispadias not associated with exstrophy of the bladder are reviewed. In the continent patient, repair of epispadias can be accomplished in one stage; however, a multistaged technique is advocated in patients with incontinence or with persistent chordee.

Epispadias↗
PubMed1997

The role of ureter in the creation of Mitrofanoff channels in children.

PURPOSE: Since 1980 numerous variations of the Mitrofanoff principle have been described. We report on 22 children in whom a ureteral Mitrofanoff channel was created. MATERIALS AND METHODS: Between 1986 and 1995 a ureteral Mitrofanoff channel was constructed as a catheterizable conduit in 22 children 2 to 15 years old (average age 6.5) with various abnormalities of the lower urinary tract, mainly exstrophy and neurogenic bladder. Indications included unavailability or unsuitability of the appendix, preference for appendix as a catheterizable colonic stoma for antegrade washouts or concomitant removal of a nonfunctioning kidney, leaving the ureter available for use. Surgical technique was based on the principles of appendicovesicostomy and in 9 cases the ureteral Mitrofanoff channel was reimplanted. RESULTS: Followup ranged from 1 to 72 months (average 30.5). Complications included stenosis of the conduit that caused difficult catheterization in 3 patients, necessitating dilation or minor revision in 2 and complete replacement by appendix in 1. Urinary leakage from the Mitrofanoff channel in 5 patients was treated with polydimethylsiloxane injection or oxybutinin. In 1 patient the channel was reimplanted, since the catheter struck the bladder neck during catheterization and caused severe pain. CONCLUSIONS: Results of the ureteral Mitrofanoff channel seem somewhat less satisfactory than those of appendicovesicostomy but they remain acceptable and even comparable, strongly supporting its use in certain circumstances.

Adolescent↗
PubMed2003

Continent urinary diversions in the exstrophy complex: why do they fail?

PURPOSE: Urinary continence is one of the primary goals in the surgical treatment of the patient with exstrophy. Children with inadequate bladder capacity, noncompliant or neuropathic bladder, or failed bladder neck reconstruction may require creation of a continent urinary reservoir to achieve continence. While initial success rates are excellent, some patients suffer persistent urinary incontinence, severely affecting their quality of life. The reasons for such failure and subsequent management were evaluated. MATERIALS AND METHODS: A retrospective database review was performed in 748 patients with the exstrophy complex. Patients with a history of failed continent urinary diversion with incontinence from the stoma or urethra were identified. Initial diagnosis, number of previous operations, reason for and time interval after surgery when failure occurred, and management strategies were evaluated. RESULTS: Of 92 patients undergoing continent diversion procedures 19 (21%) had failure initially (15 with classic and 4 with cloacal exstrophy). Four of the 19 patients underwent primary surgery at our institution and 15 were referred. The procedure initially performed was an intussuscepted nipple valve in 8 patients, an intussuscepted ileocecal valve in 1 and an ileal or sigmoid reservoir with appendiceal flap valve (Mitrofanoff) in 10. Main reasons for failure included de-intussusception and bladder neck incompetence. Mean followup after continence was achieved was 2.9 years (range 3 months to 10 years). These patients underwent up to 8 operations to achieve continence (mean 5). Most patients (79%) were successfully treated with either 1 (47%) or 2 revisions (32%), while 1 required 3 reoperations. Overall, 89 of 92 patients (97%) are currently continent, including those requiring medications and further surgical procedures, while 3 are still wet. CONCLUSIONS: Most patients with exstrophy with failure of continent urinary diversion have a long complicated surgical history. In this series incontinence from an intussuscepted nipple stoma was mostly due to de-intussusception. In patients with an initial Mitrofanoff stoma all incontinence occurred within the first few months postoperatively. Continence was achieved by either recreation of a Mitrofanoff stoma or bladder neck transection. Patients with a failed continent urinary diversion benefit from careful preoperative evaluation, meticulous surgical execution and persistent attempts to achieve continence.

Adolescent↗
PubMed1993

Pregnancy after orthotopic continent urinary diversion.

Continent urinary diversion has become a common form of bladder management for the female exstrophy patient in whom primary reconstruction has failed. Reported are the results of successful pregnancies in four young adult females, who had previously undergone a flap vaginoplasty as part of earlier management and more recently a continent right colonic urinary reservoir with a perineal stoma (Indiana pouch). Pregnancy in each of these patients was characterized by several urinary tract infections, cervical prolapse and mild to severe maternal hydronephrosis. All of the patients had some degree of difficulty with clean intermittent catheterization. One patient required an indwelling catheter with prolonged bed rest. Maternal hydronephrosis resolved after delivery in all instances. All four patients delivered their infants by way of cesarean section, either emergently for maternal or fetal distress or electively. Cervical prolapse did not resolve in three patients and will require surgical repair. After delivery, all patients returned to their previous pattern of clean intermittent catheterization without loss of continence. All the infants delivered were healthy with appropriate weights and high Apgar scores (more than 8). Orthotopic (perineal stoma) continent urinary diversion is not a contraindication to pregnancy. However, our experience mandates delivery by cesarean section with close monitoring for maternal or fetal distress during gestation.

Adult↗
PubMed1989

Terminal loop cutaneous ureterostomy in cadaveric kidney transplantation.

The presence of a nonfunctional bladder is a common finding in some patients with end-stage renal disease in whom transplantation is a proposed option. Over the last 20 years, we have performed six terminal loop cutaneous ureterostomies for urinary drainage during kidney transplantation. Neurogenic bladder in five patients and exstrophy in one patient prohibited use of the bladder. Four long-term graft survivors (19 months and 4, 10, and 20 years) experienced no short-term problems with the procedure, but two episodes of pyelonephritis have occurred. In our patients no graft dysfunction or loss could be attributed to infection, and no stomal stenosis or revision has occurred. We conclude that terminal loop cutaneous ureterostomy is feasible using normal-caliber ureters, and is a safe, effective means of urinary drainage during kidney transplantation when the bladder is not available. It also eliminates the necessity of preparatory bowel surgery that has its attendant risk of contamination in an immunocompromised patient.

Adult↗
PubMed1998

Anatomical correction of the exstrophy-epispadias complex: analysis of 34 patients.

OBJECTIVE: To evaluate the surgical procedures required for anatomical reconstruction of the bladder and penis in the exstrophy-epispadias complex. PATIENTS AND METHODS: All primary exstrophy-epispadias repairs carried out by one surgeon between 1987 and 1997 were reviewed. Bladder closure consisted of full extraperitoneal mobilization, transpositional omphaloplasty, drainage with ureteric and urethral catheters and immobilization with a 'frog-leg' plaster-cast or 'mermaid' dressings. Osteotomies were always performed when bladder closure was attempted after 37 h of age. Before 1990 the osteotomies were posterior vertical iliac (one patient) and subsequently anterior oblique iliac (10 patients). Pre-peritoneal herniotomies, in the absence of a clinical hernia, were included in the primary procedure after 1992. A modified Cantwell technique was used for epispadias repair and this was undertaken at a median of 16 months after bladder closure (range 6-30). RESULTS: Thirty-four patients (27 male) were reviewed; one patient had a chromosomal abnormality, a deletion in the short arm of chromosome 4. The male infants required a median of four procedures (range 2-5) for bladder closure, epispadias reconstruction and herniotomies, while the females needed a median of two (range 2-5). Complete bladder dehiscence, requiring re-closure with osteotomies, occurred in three cases (9%, two male). There were no dehiscences in the primary osteotomy group. Fistulae after epispadias repair occurred in four patients (17%). The bladder capacity increased to > 60 mL in 10 of 15 males by 36 months after epispadias repair. Only two of seven female infants attained a capacity of > 60 mL. Of the 15 infants who did not undergo herniotomy at primary closure, 13 subsequently developed inguinal hernias (one uni- and 11 bilateral) with incarceration occurring in two. Twelve infants underwent herniotomy at primary closure and six developed subsequent hernias (two uni- and four bilateral; P = 0.05) with documented incarceration in two. CONCLUSIONS: Anatomical correction of the exstrophy-epispadias complex remains challenging, but can be achieved with complication rates of < 20% for each stage. Bladder volumes large enough to permit adequate bladder neck reconstruction can be anticipated after epispadias repair in a large proportion of male infants, but remains small in female infants with low outlet resistance. Inguinal herniotomy at the time of bladder closure significantly reduces the incidence of subsequent herniation, which nevertheless remains high.

Bladder Exstrophy↗
PubMed1999

Inferior vesical fistula.

Exstrophy variants are uncommon developmental anomalies of the bladder; the variants involving only the bladder neck are extremely rare. There are only two case reports of inferior vesical fissure to date, and inferior vesical fistula (IVF) as an exstrophy variant has not been described previously. We report a case of IVF in a 20-month-old female. The urethra was normal in calibre and position and the bladder capacity was adequate. Simple closure of the fistula was followed by a normal micturition pattern.

Bladder Exstrophy↗
PubMed1996

Treatment of urinary incontinence in children by endoscopically directed bladder neck injection of collagen.

PURPOSE: We assessed optimal selection criteria for and expected outcome of bladder neck injection of collagen in children with intrinsic sphincteric deficiency. MATERIALS AND METHODS: We retrospectively reviewed the charts of children with intrinsic sphincteric deficiency treated with bladder neck injection of collagen at 3 pediatric urology centers. RESULTS: We reviewed the records of 12 boys and 6 girls 6 to 18 years old (median age 10.5). The underlying etiology of incontinence was neurogenic bladder in 10 patients, the epispadias-exstrophy complex in 6, ureterocele in 1 and trauma in 1. Previous surgery included bladder augmentation in 4 cases, bladder neck plasty in 7 and epispadias repair in 4, and concurrent medical management comprised anticholinergics in 8, alpha-agonists in 1 and intermittent catheterization in 9. Skin tests in all patients were negative for collagen. Seven patients had 1 injection, 9 had 2 and 2 had 3. The volume of collagen injected ranged from 2.4 to 13 cc (median 5) per treatment and 3 to 28 cc (median 7) per patient. Followup ranged from 5 to 21 months (median 15). Of the 14 patients evaluated 5 are dry, 4 are improved, 1 is unchanged and 4 underwent further continence surgery. No treatment related morbidity was seen. CONCLUSIONS: Endoscopically directed injection of collagen around the bladder neck is successful in 64% of children with intrinsic sphincteric deficiency. It is an outpatient treatment with no significant morbidity. Further continence surgery is not precluded when necessary. Patients with adequate capacity and compliant neurogenic bladders, and those with the exstrophy-epispadias complex and failed bladder neck plasty seem to be optimal candidates for collagen injection.

Administration, Intravesical↗
PubMed2002

The effect of endoscopic injections of dextranomer based implants on continence and bladder capacity: a prospective study of 31 patients.

PURPOSE: A prospective study was conducted to assess the efficacy of dextranomer based implants as a new bulking agent for endoscopic treatment of pediatric structural incontinence. MATERIALS AND METHODS: A total of 33 children and adolescents 5 to 18 years old with severe incontinence due to sphincteric incompetence (exstrophy-epispadias in 13, neuropathic bladder in 16, bilateral ectopic ureters in 4) were enrolled in the study. All but 1 patient wore diapers. Preoperative evaluation consisted of medical history, pad test, urine culture, urinary tract ultrasound and videourodynamics. This evaluation was repeated 6 months and 1 year after treatment and then on a yearly basis. Of the patients 14 had 2 and 1 had 3 treatment sessions to achieve a definitive result. At each evaluation the patient was considered cured-dryness interval of 4 hours confirmed by pad test, significantly improved-minimal incontinence requiring no more than 1 pad a day with less than 10 gm. leakage during pad test; and no further treatment required, and treatment failure-no significant improvement. Videourodynamics were mainly useful to study the evolution of the bladder capacity, activity and compliance. Followup after the last injection ranged from 6 to 36 months (mean 18). RESULTS: The mean injected volume was 3.9 ml. (range 1.6 to 12) and the procedure lasted a mean of 30 minutes (10 to 60). In the postoperative period 2 patients had temporary dysuria and 10 had a nonfebrile urinary tract infection. At 1 month 24 of the 33 patients (73%) were dry or improved. Two patients were subsequently excluded from study for noncompliance with followup. At 6 months 17 of 31 patients (55%), at 1 year 13 of 28 (46%), at 2 years 10 of 23 (43%) and at 3 years 10 of 20 (50%) were dry or improved. Similar success occurred in cases of neuropathic bladder (57%) and exstrophy-epispadias complex (46%). Success rate of re-treated patients was 35%. Of 13 patients with a normal initial bladder capacity 3 had a bladder decompensation requiring augmentation after 6 months. Also an increase of at least 50% in capacity was observed in 12 of 18 patients with an initial small bladder. No side effect related to the substance was observed. CONCLUSIONS: Endoscopic treatment of pediatric structural urinary incontinence with dextranomer implant, a nontoxic, nonimmunogenic, nonmigrant synthétic substance, was effective after 3 years in half of our patients. It may also be beneficial for patients with small bladder functional capacity. As the success rate decreased during the first year of followup, the result observed at 1 year seems to remain stable subsequently.

Adolescent↗
PubMed1998

Initial experience using a catheterizable ileovesicostomy (Monti procedure) in children.

OBJECTIVES: To present a review of our experience using the recently described Monti ileovesicostomy as an alternative to appendicovesicostomy as a continent catheterizable stoma in children. METHODS: Between January and December 1997, a Monti ileovesicostomy was performed as part of a reconstructive procedure in 13 children. Mean patient age was 1 1 years, and diagnosis included neurogenic bladder in 11 patients, cloacal exstrophy in 1, and cloacal anomaly in 1 patient. Simultaneous bladder augmentation was performed in 10 children, bladder neck reconstruction or closure in 7, and Malone antegrade continent enema (MACE) in 4. RESULTS: All 13 patients have continent stomas (100%) and catheterize the Monti ileovesicostomy without difficulty. Mean follow-up was 7 months (range 1 to 14), and there have been no stomal problems or postoperative complications attributed to the ileal channel. CONCLUSIONS: The Monti ileovesicostomy is a new technique for creation of a continent catheterizable stoma and has allowed preservation of the appendix for the MACE procedure, providing an additional option in patients with an absent or inadequate appendix. Early results of this technique for pediatric reconstruction have been excellent.

Adolescent↗
PubMed2006

Long-term effects of dextranomer endoscopic injections for the treatment of urinary incontinence: an update of a prospective study of 61 patients.

PURPOSE: To treat sphincteric deficiency in children endoscopic bladder neck injections may avoid or salvage more complex procedures. A prospective study to assess the efficacy of bladder neck injections of dextranomer based implants (Deflux(R)) was done in a 7-year period in 61 patients. MATERIALS AND METHODS: From September 1997 to September 2004 we enrolled in the study 41 males and 20 females 5 to 18 years old with severe sphincteric incompetence, including exstrophy-epispadias in 26, neuropathic bladder in 27, bilateral ectopic ureters in 5, and miscellaneous in 3. Preoperative evaluation consisted of medical history, urine culture, urinary tract ultrasound and videourodynamics. This evaluation was repeated 6 months and 1 year after treatment, and yearly thereafter. Of the patients 17 underwent 2 and 4 underwent 3 treatment sessions to achieve a definitive result. At each evaluation the case was considered cured-a dryness interval of 4 hours between voids or CIC, significantly improved-minimal incontinence requiring no more than 1 pad daily and no further treatment required, and treatment failure-no significant, long lasting improvement. Videourodynamics were mainly useful to study the evolution of bladder capacity, activity and compliance. Followup after the last injection was 6 to 84 months (mean 28). RESULTS: Mean injected volume per session was 3.9 cc (range 1.6 to 12). Postoperative complications were temporary dysuria in 2 patients nonfebrile urinary tract infection in 10, orchid-epididymitis in 1 and urinary retention with pyelonephritis in 1. The incidence of dryness or improvement during followup was 79% (48 of 61 patients) at 1 month, 56% (31 of 55) at 6 months, 52% (24 of 46) at 1 year, 51% (18 of 35) at 2 years, 52% (16 of 31) at 3 years, 48% (12 of 25) at 4 years, 43% (9 of 21) at 5 years, 36% (4 of 11) at 6 years and 40% (2 of 5) at 7 years. The success rate according to pathological condition was similar in cases of neuropathic bladder and the exstrophy-epispadias complex (48% and 53%, respectively). The success rate in re-treated cases was 38%. After treatment a contracted bladder developed in 6 patients. Also, of the 35 patients with at least 2 years of followup an increase in capacity of at least 50% was observed in 12 of 18 with an initially small bladder. No side effects related to the substance were observed. CONCLUSIONS: Endoscopic treatment for pediatric severe sphincteric deficiency with dextranomer implant, a nontoxic, nonimmunogenic, nonmigratory synthetic substance, was effective up to 2 years in half of the patients. Subsequently at up to 7 years of followup a slow decrease in efficacy was observed and treatment remained beneficial in 40% of the patients.

Adolescent↗
PubMed1983

Treatment of the epispadias-exstrophy complex with the AS792 artificial urinary sphincter.

The AS792 artificial urinary sphincter was implanted in 11 incontinent patients with the epispadias-exstrophy complex. To date 10 patients (91 per cent) are totally continent of urine with normal renal function and intact urinary systems. The recent reports concerning success with bladder neck reconstruction in this syndrome are reviewed. Owing to the relatively poor success of this procedure in achieving total urinary continence it is recommended strongly that insertion of the artificial urinary sphincter be the initial anti-incontinence procedure for these patients. The success rate with the artificial sphincter currently is superior to any form of bladder neck reconstruction in patients with the epispadias-exstrophy complex.

Adolescent↗
PubMed2003

Intravesical phallus in cloacal exstrophy.

We report a case of a fused phallus located within the urinary bladder in a child with cloacal exstrophy. Surgical exploration revealed a phallus formed by fusion of the left and right corpus cavernosum and completely covered by bladder urothelium. The phallus was surgically separated from the bladder and mobilized to a more normal position and a neourethra created from tubularized bladder urothelium. Skin coverage was done using perineal skin flaps to cover the penile shaft, with the bladder urothelium covering the tip of the phallus being left intact to provide the appearance of a glans penis.

Abnormalities, Multiple↗
PubMed1997

Comparison of preoperative innervation pattern and postreconstructive urodynamics in the exstrophy-epispadias complex.

A primary neurogenic component is often being postulated to be responsible for unfavourable postoperative results of bladder growth and continence in the exstrophy-epispadias complex. On the other hand, we have seen favourable clinical situations and urodynamic follow-up after primary reconstruction employing the 'Erlangen technique' without evidence of primary dysinnervation. Since there are only few data available on this issue, we decided to apply immunocytochemistry and histochemistry for neuronal markers as a further step to elucidate this problem. Transmural biopsies were obtained during reconstructive surgery from the bladder dome and trigone of 22 children between September 1994 and June 1995. Indirect immunocytochemistry for vasoactive intestinal polypeptide (VIP), neuropeptide Y (NPY), substance P (SP) calcitonin gene-related product (CGRP) and protein gene product (PGP) 9.5, a universal marker for neuronal tissue and histochemistry for nicotinamide adenine dinucleotide phosphate diaphorase (NADPHd), was performed on 14-micron cryostat sections. During the same period of time, control biopsies from 6 healthy bladders of an age-compatible group were subjected to the same examination. In addition, 19 patients were examined urodynamically after reconstruction in order to compare postoperative bladder function with the preexisting innervation pattern. No evidence of dysinnervation was found either morphologically or urodynamically in cases of isolated epispadias and classical exstrophy. Cases of exstrophies after failed reconstruction had muscular innervation deficiencies but increased sub and intraepithelial innervation. This group, according to morphological changes, also demonstrated bladder wall instability, decreased bladder compliance and absent detrusor contractions during micturition. All cloacal exstrophies had an extremely uneven innervation pattern with noticeable calibre differences of nerve fibres and bundles with simultaneously increased innervation density. Functionally these bladders were marked by small capacity and decreased compliance and absent detrusor function. All exstrophies in conjunction with an anal atresia or with a caudal regression syndrome (so-called 'transition forms') had a nearly universal pathological innervation pattern, compatible with cloacal exstrophies and had equally unfavourable functional findings. Cloacal exstrophies and 'transition forms' seemed to have primarily a completely different pattern of innervation when compared to normal bladders. Prognosis of bladder function in these children remains unclear.

Adolescent↗