Duplication of the abdominal vena cava associated with cloacal exstrophy.
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Abnormalities, Multiple↗
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Experience with the artificial urinary sphincter model AS800 in 148 patients.
The latest version of the artificial urinary sphincter, AS800, was used in 148 patients with urinary incontinence of different etiologies. Followup ranged from 3 to 37 months, with an average of 20.8 months. There were 112 (76 per cent) male and 36 (24 per cent) female patients. The cuff was implanted around the bladder neck in 78 patients (53 per cent) and around the bulbar urethra in 70 (47 per cent). Socially acceptable urinary control was achieved in 90 per cent of the 139 patients with active devices in place. It was necessary to remove the sphincter in 11 patients (7.4 per cent). The reasons for removal were infection and erosion in 8 patients (5.4 per cent), infection without erosion in 2 (1.3 per cent), and erosion due to excess pressure and poor tissues in 1 (0.7 per cent). Comparison of success and failure rates associated with incontinence of different etiologies revealed that patients with incontinence after failure of a conventional antistress incontinence operation and those with incontinence after transurethral resection or radical prostactectomy had the highest success rate, and that patients with incontinence secondary to pelvic fracture or exstrophy and epispadias had the highest failure rates. The deactivation feature (the lock) of the new artificial sphincter model was beneficial for primary deactivation, urethral catheterization or cystoscopy, or for elective nocturnal decompression of the bladder neck or urethral tissues.
Equipment Design↗
Long-term outcome of terminal myelocystocele patients.
INTRODUCTION: A terminal myelocystocele, a closed form of a neural tube defect (NTD), can present as a large, fully epithelialized, cystic lumbosacral mass containing fat, cerebrospinal fluid (CSF) and neural tissue. The spinal cord terminates at a neural placode wherein the central canal opens into a CSF-filled cavity that is distinct from fluid in the subarachnoid space surrounding the spinal cord. This form of NTD, in our experience, was only associated with major caudal cell mass abnormalities, as these patients often have maldevelopment of the lower spine, pelvis, genitalia, bowel, bladder, kidney and the abdominal wall. This study will describe the clinical manifestations, surgical management and long-term outcome of our terminal myelocystocele patients. METHODS: To characterize this rare entity, a 13-year retrospective review was undertaken at our institution. RESULTS: Nine patients with terminal myelocystoceles were identified. In all cases, there were multiple congenital defects including cloacal exstrophy, imperforate anus, omphalocele, pelvic deformity, equinovarus or renal abnormality. Only 1 out of 9 patients has required a shunt for hydrocephalus. The main goal of neurosurgical intervention was to reduce the size of the mass, which can slowly enlarge over time. The spinal cord was also untethered, although these patients have no chance of bowel or bladder control. With a mean follow-up of 63 months, all patients remained neurologically stable. Impairment of lower extremity function is usually severe. However, some patients were ambulatory with the aid of a walker or orthotic device. All patients required a prolonged hospital stay as well as multiple operations prior to initial discharge. CONCLUSION: Experienced, multispecialty care is needed to optimize the long-term outcome of these complex patients.
Child↗
[Urodynamic studies on the untethering of spinal lipoma in the children].
We performed conus untethering on 44 children with spinal lipoma. In this retrospective study, we studied urological examinations obtained in 36 consecutive patients. Urological examinations were ultrasonography of bladder and kidney, voiding cystourethrography (VCG), and urodynamic cystometry. In those of them, 6 patients had already had urinary dysfunction at preoperative stage, but 4 patients' spinal lipoma was one complication of cloaca exstrophy and 2 patients' spinal lipoma after repairment of their myelomeningocele. In the remaining 30 cases, 18 patients (60.0%) as the group 1 did not have urinary dysfunction and abnormality in any urological examinations. Another 7 patients (23.3%) as group 2 also did not have urinary dysfunction, but abnormal vesicoureteral reflux (VUR) was found out in their VCG examination. At postoperative stage, abnormal VUR resolved or improved in all these 7 cases, but one of them showed transient urinary retention. Two patients of 18 in the group 1 had to require clean intermittent self-catheterization at postoperative stage. These observations confirmed that children with spinal lipoma were more likely to present with urological findings, and so it was imperative that a diagnosis be made and treatment be instituted as early as possible.
Child, Preschool↗
The OEIS complex (omphalocele, exstrophy, imperforate anus, spinal defects).
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Anus, Imperforate↗
Exstrophy of the cloaca--report of a case.
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Abnormalities, Multiple↗
Male epispadias repair: surgical and functional results with the Cantwell-Ransley procedure in 40 patients.
PURPOSE: We present our experience using the Cantwell-Ransley epispadias technique, particularly focusing on postoperative anatomical and functional complications. MATERIALS AND METHODS: Between 1989 and 1997, 40 patients 1 to 28 years old underwent the Cantwell-Ransley technique for epispadias at our institution. The condition was isolated in 17 cases and associated with exstrophy in 23. Surgery involved a primary and secondary procedure in 29 and 11 patients, respectively. Cavernocavernostomy was performed in only 16 cases. RESULTS: At a mean followup of 3 years 18 patients (45%) had complications and needed further procedures, and 3 (7.5%) had major wound dehiscence. The complication rate was higher in the exstrophy than in the isolated epispadias group (65 versus 28%) and urethral complications were consistently associated with previous urethral plate sectioning. In 1 patient major and persistent loss of continence was probably related to prolonged transurethral bladder drainage. All 17 patients in the postpubertal group report erections, although 1 who did not undergo cavernocavernostomy still complains of dorsal curvature. In 36 patients (90%) a fully satisfactory anatomical and functional result was achieved. CONCLUSIONS: The Cantwell-Ransley technique of epispadias repair allows successful reconstruction in most patients. However, postoperative complications, some of which are serious, may develop that are more associated with previous procedures that compromise the blood supply to the urethral plate than the Cantwell-Ransley technique.
Adolescent↗
The syndrome of dysuria and hematuria in pediatric urinary reconstruction with stomach.
Between July 1989 and March 1992 at a single institution 27 male and 30 female patients underwent lower urinary reconstruction with stomach. Mean patient age was 9.9 years (range 1.5 to 28 years). The diagnoses were epispadias/exstrophy complex (19 patients), myelodysplasia (11), cloacal exstrophy (6), posterior urethral valves (6), Hinman syndrome (4), sacral agenesis (3) and other (8). Indications for surgery were urinary incontinence, upper tract deterioration or undiversion. A total of 54 patients underwent augmentation gastrocystoplasty and 3 had total bladder replacement. Mean followup time was 23.2 months (range 12 to 39 months). The syndrome of dysuria and hematuria is defined as 1 or a combination of the following symptoms: bladder spasm or suprapubic, penile or periurethral pain, coffee brown or bright red hematuria without infections, skin irritation or excoriation and dysuria without infections. Telephone and clinic interviews identified 21 patients (36%) with symptoms of the dysuria and hematuria syndrome. The most common symptoms were hematuria (71%) and bladder or suprapubic pain (76%). Of the patients 18 (86%) ranked the severity of symptoms as mild to moderate and 3 (14%) ranked them as severe. No medications were required to control the symptoms in 13 patients (62%) and 3 other patients only required medications on an as needed basis. Overall patients who required no medications had lower symptom scores than those who required medications. Patients with decreased renal function may be more at risk for the dysuria and hematuria syndrome than those with normal renal function. Patients who were wet were more prone to have the dysuria and hematuria syndrome than those who were totally dry. The pathophysiology of the dysuria and hematuria syndrome is currently unknown. Patients who require urinary reconstruction with stomach tissue need to be made aware of the potential of the dysuria and hematuria syndrome.
Adolescent↗
A case of a closed partial cloacal septation defect with a patent urachus.
A boy with a closed partial cloacal septation defect with a patent urachus is reported. He had an intact abdominal wall, a patent urachus, a colovesical fistula, intact genitalia and urethra, imperforate anus, and a lipomyelocystocoele. Patients with similar constellation of findings have been reported as cloacal exstrophy variants. What distinguishes this case from the other reported variants is the intact abdominal wall with the patent urachus, the small and normally formed phallus and urethra, and the presence of a lipomyelocystocoele. We discuss the possible embryologic mechanism responsible for this boy's findings and possible relationship with the cloacal exstrophy spectrum. We also discuss new terminology for the epispadias-exstrophy spectrum. Furthermore this case reminds us that there is considerable variability within the epispadias-exstrophy spectrum.
Abnormalities, Multiple↗
Male epispadias: experience with 45 cases.
PURPOSE: We evaluate the long-term results of surgery for isolated (without exstrophy) male epispadias. MATERIALS AND METHODS: A total of 45 male patients with isolated epispadias were treated and followed at Debrousse Hospital between 1971 and 1993, 8 in whom repairs performed elsewhere had failed and 14 had total urinary incontinence. Incontinence was treated with a modified Young-Dees reconstruction of the bladder neck and the ureters were reimplanted in all cases. Long-term followup was available in 18 cases of a Cantwell-Young-Gross, 4 Duplay, 7 full thickness skin graft, 1 bladder graft and 10 Cantwell-Ransley urethroplasties. Penile reconstruction was performed using a 2-stage procedure in 8 patients (freeing of the corpora followed by urethroplasty), and an isolated urethroplasty in 13. All other patients underwent a single stage repair combining dissection of the penis and urethroplasty. RESULTS: All patients were reassessed clinically. Of 13 incontinent patients followed for 1 to 20 years 11 acquired continence, 1 remains incontinent and 1 underwent a repeat procedure recently. Urethroplasty was immediately satisfactory in 66% of the cases, minor complications developed in 25% and serious complications (especially free graft urethroplasty) developed in 10%. Assessment of genital cosmesis is subjective but it was considered acceptable in most cases. All 29 patients with long-term followup had erections, 24 performed regular sexual intercourse, 17 had normal ejaculations and 4 fathered children. CONCLUSIONS: The principles described by Cantwell-Young and Gross, and modified by Ransley, have considerably improved penile reconstruction. Free graft and Duplay urethroplasties provided unacceptable results in our series. Our results of surgery for incontinence associated with isolated male epispadias (84%) were better than for male exstrophy (63%).
Adolescent↗
Limb-body wall complex: 4 new cases illustrating the importance of examining placenta and umbilical cord.
Limb-body wall complex (LBWC) is a rare, sporadic, congenital defect defined as a combination of at least two of three characteristics: 1. limb defects, 2. anterior body wall defects, and 3. exencephaly or encephalocoele with/without facial clefts. Three pathogenic mechanisms have been proposed: early amnion rupture, vascular disruption and embryonic dysgenesis. In this study we carried out the pathological evaluation of four fetuses with LBWC and their placentas. None of the cases had craniofacial defects. Three fetuses showed an abdominal wall defect with eventration of abdominal organs, cloacal exstrophy, absent external genitalia, abnormal internal genitalia, scoliosis and lower limb defects. One fetus showed failure of closure of both thoracic and abdominal walls with ectopia cordis, evisceration of left lung and abdominal organs, severe reduction defect of left arm, but normal colon, anus, bladder, genitalia and lower limbs. All cases had a short, malformed umbilical cord, incompletely covered by amnion. The umbilical vessels were embedded in an amniotic sheet which connected the skin margin of the anterior body wall defect to the placenta. These anomalies suggest an abnormal body stalk development as a pathogenic mechanism for LBWC. Prenatally, the abnormal fetoplacental attachment can be detected ultrasonographically by the end of the first gestational trimester. Postnatally, the examination of placenta, umbilical cord and membranes is crucial in confirming the diagnosis of LBWC.
Abdominal Muscles↗
Novel use of acellular dermal matrix in the formation of a bioprosthetic silo for giant omphalocele coverage.
A case report is presented on the utilization of an acellular dermal matrix in the formation of a biologic silo conducive to early epithelial grafting in the treatment of a giant omphalocele associated with cloacal exstrophy.
Bioprosthesis↗
Cloacal exstrophy: a 25-year experience with 50 cases.
PURPOSE: The aim of this study was to evaluate the outcomes of reconstruction of all cloacal exstrophy cases seen by the authors from 1974 to 1999. METHODS: A retrospective chart review and personal follow-up was conducted on all 50 patients, who ranged in age from newborn to 35 years. Thirty-eight were secondary cases, 12 were primary (no previous surgery). Twenty-eight (56%) were genetic boys; 6 were raised as boys and 22 as girls. Forty patients underwent extensive reconstructive surgery; 6 await reconstruction, and 4 were seen only in consultation. For urinary continence, 21 had bladder neck narrowing, 7 received a bowel nipple, and 12 had a Mitrofanoff catheterizable conduit on the abdominal wall, using tapered intestine in 8, ureter in 3 and the appendix in 1. Four patients had a urostomy. Bladder augmentation was performed in 35 patients (18 with stomach, 11 small bowel, and 6 with both). Twenty-five patients had pull-through of their colon to the perineum. Thirty-two have had a vagina constructed. Forty-seven of the 50 patients had spinal cord tethering, and most underwent neurosurgical release. RESULTS: Overall survival rate was 98%; 1 patient died preoperatively at another institution. Acceptable bowel continence was achieved with enema washouts in 19 of 25 pullthroughs; 4 failed and were reversed. One case is too recent to judge. Of 40 reconstructed cases, 31 were dry, 3 had slight leakage, and 1 is too early to evaluate. Five had enough leakage to require further surgery. CONCLUSIONS: The once hopeless anomaly of cloacal exstrophy is treatable with extensive reconstructive surgery. Continence of urine (mainly by catheterization) and stool (mainly by enema washouts) is achievable in most. The hindgut, including the cecum and the usually rudimentary distal colon, should be saved and used as colon, not for urinary or vaginal reconstruction. In genetic boys, the authors continue to believe that gender assignment should depend on the likelihood for reconstructing an adequate phallus.
Adolescent↗
The ultimate challenge of cloacal exstrophy.
PURPOSE: Our review addresses the various system abnormalities associated with cloacal exstrophy and revisits the question of gender assignment. Gender assignment decisions and psychological aspects of gender issues have become the "ultimate challenge." Exploration of gender identity, gender role behavior and sexual orientation has just begun. MATERIALS AND METHODS: A comprehensive literature review was performed with all world literature regarding the current management of cloacal exstrophy. Research focused on management principles, outcomes and documentation of concurrent anomalies. We also describe original research evaluating gender identity in our own series of patients raised as girls to illustrate the challenge of treatment and augment the available literature, which is scant. RESULTS: Abnormalities of the vertebral column ranged from hemivertebra to myelomeningocele. With magnetic resonance imaging, the incidence of spinal dysraphism approached 100% and cord tethering was also more frequently recognized. For children with the short bowel syndrome, advances in antibiotic usage, and parenteral and enteral nutrition have increased the survival rate and reduced morbidity. A neurological component has been recognized for bladder function, bladder neck continence, lower extremity function and erectile capacity. Mitrofanoff-type reconstruction with bladder neck closure and continent catheterizable stoma dramatically increased continence. Diminutive or absent penis has been documented in 30% of males, and no documentation of paternity exists. The majority of females have bicornuate uterus. However, ovaries and tubes were generally normal. No reports of proven fertility exist. CONCLUSIONS: The strategy and timing of surgery relating to gender assignment remain controversial. A desperate need exists for research focusing on gender development and quality of life. Until that time, a cautious watchful approach may be most appropriate as our patients with cloacal exstrophy mature into adulthood.
Abnormalities, Multiple↗
Initial experience with the transurethral self-detachable balloon system for urinary incontinence in pediatric patients.
PURPOSE: A new endoscopic technique to treat urinary incontinence in children using a self-detachable balloon device was studied. MATERIALS AND METHODS: The study includes 11 patients with a mean age of 14.6 years and all of whom had intrinsic sphincter deficiency due to myelomeningocele in 9, spinal artery bleed in 1 and cloacal exstrophy in 1. All patients were on clean intermittent catheterization preoperatively and postoperatively. Endoscopic balloon treatment was performed on an outpatient basis. A mean of 5 balloons (range 2 to 8) were placed per patient. All patients underwent formal urodynamic study preoperatively and at 6 weeks and 6 months following balloon placement. RESULTS: Of the 9 patients without prior bladder neck surgery 7 had improvement in urodynamic parameters, including urethral pressure profile in all 7 and functional bladder capacity in 6, 4 were markedly improved clinically and 2 were dry. Two patients with prior bladder neck surgery were clinically unchanged following balloon placement, although 1 had urodynamic improvement. CONCLUSIONS: Our initial experience with the transurethral self-detachable balloon system as a minimally invasive outpatient procedure to treat urinary incontinence in children has been encouraging. To date this procedure appears most applicable to the patient who has not undergone surgery and has a neurogenic etiology for urinary incontinence.
Adolescent↗
Preventing reservoir calculi after augmentation cystoplasty and continent urinary diversion: the influence of an irrigation protocol.
OBJECTIVE: To evaluate the influence of an irrigation protocol in preventing reservoir calculi forming after augmentation cystoplasty and continent urinary diversion. PATIENTS AND METHODS: Between 1985 and 1995, 91 patients had an augmentation cystoplasty and/or continent urinary diversion (group 1; 54 females and 37 males, mean age 11.1 years, range 1-31); these patients were not routinely instructed to use irrigation after surgery. The segments used included ileum (44), colon (36), stomach (eight) and ureter (three). Between 1995 and 2000, 42 patients (group 2) underwent urinary reconstruction (22 females and 20 males, mean age 14.8 years, range 4-27), the segment used being ileum (30), colon (five), ureter (five) and stomach (two) but in contrast to group 1 they then were placed on a standard prophylactic irrigation protocol. The occurrence of stones in the reservoir was then assessed. RESULTS: Thirty-nine of the 91 patients (42.8%) in group 1 presented with reservoir calculi after reconstruction and 22 had several episodes. The mean time to presentation was 30 months. The incidence of stone formation by underlying diagnosis included: myelomeningocele, 32/48 (66%), exstrophy five/25 (25%), posterior urethral valves two/20 (10%) and rhabdomyosarcoma, none of three. Fifty of the 91 patients had an abdominal stoma, with stone formation in 33 (66%), while 41 used the native urethra, with stone formation in six (15%). Three (7%) of the 42 patients in group 2 developed reservoir calculi after reconstruction, two in patients with myelomeningocele and one in a trauma patient who had residual bone spicules in the bladder; the mean time to presentation was 26.5 months. CONCLUSIONS: These data suggest that the irrigation protocol used in group 2 significantly reduced the number of reservoir calculi after urinary tract reconstruction when bowel was used as part of the reconstruction (43% vs. 7%). The most calculi in both groups were in immobile patients with sensory impairment. Also, patients with an abdominal stoma had a greater risk of reservoir calculi (66%) than those using the native urethra (15%).
Adolescent↗
Cloacal exstrophy: radiologic findings in 13 patients.
Cloacal exstrophy is a complex, multisystem malformation that has not received much attention in the radiologic literature. The clinical histories and imaging studies of 13 cases of cloacal exstrophy proved surgically or at autopsy were reviewed. Radiologic observations were based on skeletal plain films (13), contrast-enhanced examinations of the genitourinary system (12), abdominopelvic sonograms (nine), gastrointestinal studies (seven), CT scans of the pelvis and CNS (seven), MR images of the pelvis and CNS (six), renal nuclear medicine scans (three), myelography (three), and three-dimensional scans of the pelvis (two). Genitourinary and gastrointestinal abnormalities identified in our 13 patients included bladder malformations (13), hindgut anomalies (13), undescended testes (five), malrotation (five), duplicated müllerian structures (four), and renal ectopia (four). Skeletal abnormalities included symphysis pubis diastasis (13), posterior element dysraphism (12), vertebral segmentation anomalies (12), clubfeet (six), and hip dislocations (five). CNS anomalies included meningocele (nine) and Chiari malformation (one). Immediate surgical closure of the defect with maximal bowel preservation and diverting colostomy offers optimal chances for survival. Appropriate preoperative gastrointestinal and genitourinary evaluation is essential.
Abnormalities, Multiple↗
Prospects for the prenatal diagnosis and obstetric management of cloacal exstrophy. A report of two cases.
Two cases of cloacal exstrophy, a rare congenital anomaly featuring exstrophy of the urinary, genital and intestinal systems, occurred. Possibilities for the prenatal diagnosis of such structural anomalies are alpha-fetoprotein screening, ultrasonography and fetal heart rate and activity patterns. Careful preoperative evaluation of affected neonates and staging of the surgical correction are recommended.
Abnormalities, Multiple↗