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PubMed1986

The Boyce-Vest operation for exstrophy of the bladder. 35 years later.

Twenty-four patients with exstrophy of the urinary bladder (23 patients) or epispadias (1 patient) who were treated by the Boyce-Vest operation have been followed for a total of 482 patient-years (range 3 to 34 years, mean 22 years). In all patients upper urinary morphology and renal function have remained normal, and those with preoperative compromise have stabilized or improved. Postoperatively, no patient has developed an electrolyte imbalance or metabolic acidosis requiring treatment, urinary calculi, or malignant change in the vesicorectal reservoir. Although the procedure and the exstrophy-epispadias complex present many unresolved of management. The infant with exstrophy of the urinary bladder appears best served by early neonatal closure of the exstrophic bladder as described by Jeffs, 6,8 with attempts at establishing urinary continence later in childhood. 6,8,11 When other methods to establish urinary continence are unsuccessful and satisfactory anal continence has been demonstrated, the Boyce-Vest procedure offers an acceptable long-term alternative to establish sphincteric urinary continence. Properly managed, the newborn with uncomplicated exstrophy should have a life expectancy equal to that of any other neonate. More importantly, it is within our ability to provide such patients with adult social acceptability and relative freedom from urinary disease.

Adolescent↗
PubMed1975

One-stage reconstruction for exstrophy of the bladder in girls.

In an African female infant with exstrophy who was a candidate for complete reconstruction of the urinary tract, pelvis, and abdominal wall, the treatment program was simplified by performing iliac osteotomies at the same time as the other repairs, using a single transverse lumbosacral incision for access. A series of 3 wire loops held the symphysis in apposition. A plaster spica, followed by a plaster girdle, minimized the tension on the pubic wires. We believe that we obtained, by this method, superior strength and a better appearance of the abdominal wall and perineum.

Abdominal Muscles↗
PubMed1998

Gastrocystoplasty: long-term followup.

PURPOSE: Gastrocystoplasty is no longer favored at many institutions due to complications, including the hematuria-dysuria syndrome and metabolic alkalosis. We reviewed our experience to determine the advantages and disadvantages of bladder augmentation using stomach body wall. MATERIALS AND METHODS: We retrospectively reviewed the medical records, urodynamic studies, x-rays and laboratory evaluations of 47 children who underwent gastrocystoplasty at our institution between 1986 and June 1997. Parents and patients were contacted by telephone for detailed interviews to validate the medical record and determine whether there had been any changes since the last visit. Followup ranged from 9 months to 11 years (mean 4.4 years). Bladder dysfunction was secondary to spinal dysraphism in 38 children. Other diagnoses included cloacal and bladder exstrophy, posterior urethral valves, a persistent urogenital sinus and bilateral ectopic ureters. RESULTS: Preoperative and postoperative ultrasound studies available for 79 renal units demonstrated a stable or improved upper tract in 75 (95%). Preoperative and postoperative serum sodium, potassium and creatinine levels showed no significant changes. Mean serum chloride decreased 2.7 mEq./l. and bicarbonate increased 3.3 mEq./l. In the 3 patients with renal insufficiency serum bicarbonate increased 8 mEq./l. Mean pressure specific bladder volume at less than 20 cm. water increased 177 cc and mean maximum bladder pressure decreased from 35 to 13 cm. water. Two-thirds of the patients had bacilluria and a fourth reported a symptomatic bladder infection. No patients had bothersome mucus or required routine bladder irrigation. Symptoms consistent with the hematuria-dysuria syndrome were present in 27% of the patients. No patients had symptoms more than once weekly. Symptoms occurred in 75% of the patients without a neurogenic bladder, such as exstrophy or posterior urethral valves, and in 14% of those with neurogenic bladder dysfunction. In 1 case a bladder stone developed 8 years after surgery. Five patients required reoperation for complications related to gastrocystoplasty. No patient had perforation. CONCLUSIONS: Our data show that even after a mean of 4.4 years gastrocystoplasty has significant advantages over intestinal augmentation, including decreased chloride reabsorption, mucous production and urinary infection, and an extremely low incidence of stones and perforation. The gastric patch is associated with metabolic alkalosis and the hematuria-dysuria syndrome, which may be avoided and medically treated with proper patient selection and close followup. The procedure should be avoided in sensate patients with sufficient bowel.

Child↗
PubMed1993

Hypertension following primary bladder closure for vesical exstrophy.

Since the initiation of staged reconstruction for bladder exstrophy, hypertension has been a known complication of the procedure. The etiology of the elevation in blood pressure was presumably secondary to ureteral dysfunction caused by edema at the ureterovesical junction and secondary renal obstruction. This report details the clinical course of three patients who developed significant hypertension following primary bladder closure with posterior iliac osteotomies and bony immobilization by skin traction. None of the patients had urinary obstruction and all had their hypertension alleviated by removal of the traction. The presumed etiology of the elevated blood pressure within these patients is similar to that experimentally produce by inappropriately applied traction resulting in tension on the sciatic nerve and reflex induced vasoconstriction. The need to maintain appropriately applied traction and the necessity of monitoring the postoperative blood pressure is stressed.

Antihypertensive Agents↗
PubMed1980

Exstrophy of the bladder: long-term results of bilateral posterior iliac osteotomies and two-stage anatomic repair.

One hundred cases of exstrophy of the bladder treated with bilateral posterior iliac osteotomies to facilitate anterior closure of the pelvis and soft tissues were reviewed. Sixty-nine cases followed 5 to 18 years were evaluated in terms of: success of anterior closure (95%); degree of urinary continence (45% with near normal continence); incidence of chronic urinary tract infection, dilation of upper urinary tract and need for ileal diversion (30%); the effect of bony reconstruction on hip and pelvic development. Modifications of the technique of pelvic stabilization anteriorly and a previously unreported complication of asymmetrical pelvic closure are presented. Closure of the bony pelvis appears to increase the success of repair of both in terms of soft-tissue closure and attainment of urinary continence. Follow-up examinations of the patients throughtout the growing years with special attention to possible development of pelvic obliquity or scoliosis are important.

Bladder Exstrophy↗
PubMed2002

The endoscopic treatment of incontinence in children.

The achievement of urinary continence in children may be very difficult with some congenital malformations of the lower urinary tract, such as bladder exstrophy, or in some neurogenic dysfunctions, such as neuropathic bladder. Endoscopic injection of bulging substances into the bladder neck or posterior urethra may be regarded as a minimally invasive procedure that may provide a better dry interval in these patients. Endoscopic treatment can be performed as either a primary or adjunctive procedure after open bladder neck surgery. It is also of use to increase the functional bladder capacity in some children. Although longer follow-up is wise, results so far are encouraging. The search for a safe and reliable substance to use is still ongoing, and the new available biodegradable implants seem adequate.

Child↗
PubMed2001

Evolution of male epispadias repair: 16-year experience.

PURPOSE: We review our experience during the last 16 years, adopting different surgical approaches for epispadias repair, and compare the results of complete penile disassembly technique with perineal muscular complex reassembly since 1995 with previous repairs. MATERIALS AND METHODS: From 1984 to 1999, 58 epispadias repairs were performed in 53 male patients 3 days to 13 years old, including 18 with primary penopubic epispadias, 35 with bladder exstrophy and 5 treated with secondary genito-urethroplasty after previous repairs of the exstrophic complex. Cases were divided into 2 periods of the surgical procedure. Different techniques succeeded in 41 patients in the first decade (group 1) while the complete penile disassembly with perineal muscular complex reassembly technique was used in 17 patients during the last 5-year period (group 2). Results of both groups were compared, and Fisher's exact test was used for statistical analysis. RESULTS: Of the 41 group 1 cases complications (mostly fistula and/or urethral stenosis) in 21 (51%) required 1 or multiple operations. Continence was never achieved with urethroplasty alone. Cosmetic appearance of the phallus was not satisfactory in 23 cases (29%) and urethral catheterization was difficult in 8 (19%). Of the 17 group 2 cases only 2 complications (11%) occurred (1 fistula and 1 distal urethral stenosis). Dry intervals or voluntary continence was achieved in 6 of the 10 patients with exstrophy and in all but 1 with epispadias without bladder neck surgery. The penis had a satisfactory cosmetic appearance and no dorsal chordee with an easily catheterizable urethra was noted. The complication rate was significantly different in the 2 groups (Fisher's exact test p = 0.0042). CONCLUSIONS: The complete penile disassembly with perineal muscular complex reassembly technique, with deeper positioning of the urethra in the perineal musculature, seems to guarantee a significant step forward in functional epispadias repair.

Adolescent↗
PubMed2000

Surgical complications of bladder augmentation: comparison between various enterocystoplasties in 133 patients.

OBJECTIVES: Ileal and sigmoid augmentation are equally effective at increasing bladder capacity and compliance. Therefore, knowledge of the incidence of major complications, including perforation, small bowel obstruction (SBO), anastomotic complications, calculus formation, and indications for revision may be useful in choosing the ideal segment. We compared the complications of ileocystoplasty and two types of sigmoidocystoplasty that required reoperative surgery. METHODS: Between 1981 and 1997, 158 patients with a mean age of 11 years (range 2 to 25) underwent augmentation cystoplasty. Ileum or sigmoid colon was used in 133 patients, who were the subjects of this study. The mean follow-up was 64 months (range 6 to 185). Indications included neurogenic bladder (n = 100), bladder exstrophy (n = 12), cloacal exstrophy (n = 6), posterior urethral valves (n = 3), and miscellaneous (n = 12). Ileum was used in 65 patients and sigmoid colon in 68. Of these, 48 underwent conventional colocystoplasty and 20 seromuscular colocystoplasty lined with urothelium (SCLU). Seventy-nine percent required additional procedures to achieve continence or facilitate catheterization, which included bladder neck procedures in 56% or continent stomas alone in 23%. RESULTS: There were no deaths or complications of bowel anastomosis. Overall, continence was achieved in 95%. Spontaneous bladder perforation was highest in patients with neurogenic bladder. Calculi developed more frequently in patients with continent stomas (P = 0.04) and in patients with bladder/cloacal exstrophy (32%) than in patients with neurogenic bladder (P = 0.01). Additional procedures and route of catheterization did not increase the risk of perforation. One patient with SCLU with known hypercalciuria developed bladder calculi. CONCLUSIONS: Sigmoid colon showed a trend of a lower rate of SBO with no difference in perforation or stone formation compared with ileum. Primary diagnoses of bladder or cloacal exstrophy and continent stomas are risk factors for the development of calculi. SCLU has a low rate of surgical complications and no incidence of perforation or SBO thus far; therefore, we advocate the use of SCLU when feasible, and sigmoid as the preferred bowel segment for augmentation cystoplasty.

Adolescent↗
PubMed2006

MTHFR 677 TT genotype in a mother and her child with Down syndrome, atrioventricular canal and exstrophy of the bladder: implications of a mutual genetic risk factor?

Apart from Husmann and Vandersteen [in: Gearhart JP, Matthews R (eds) The Epispadias-Exstrophy Complex. Kluwer, New York, pp 199-206, 1999], we report only the second case of Down syndrome (DS) associated with exstrophy of the bladder (EB). Besides the appearance of DS, the newborn exhibited a complete atrioventricular canal (CAVC) and classical EB, including diastases of the symphysis, an epispadic penis and an open bladder plate. Despite current recommendations, the mother had not supplemented her intake of folic acid during the periconceptional period. In a comparable case, Al-Gazali et al. (Am J Med Genet 103:128-132, 2001) found the homozygous 677T allele of the methylenetetrahydrofolate (MTHFR) gene 677C-->T polymorphism in a mother and her child with DS and cervical meningomyelocele. They found that the mother, who also had not supplemented her folic acid intake, had a secondarily altered folate status with an increased homocysteine level, suggesting that the homozygous TT mutation in the MTHFR gene in both mother and her child had contributed to the presentation of DS and a neural tube defect. The combined clinical findings of the present case and the observations of Al-Gazali et al. led us to investigate the 677C-->T polymorphism in our mother-child pair. Likewise we found that mother and child were homozygous for the mutant 677T allele. Our findings support the suggestion of Al-Gazali et al. that the MTHFR 677TT could be a mutual genetic risk factor for the co-occurrence of trisomy 21 and midline defects, the risk of which may be reduced by periconceptional folic acid supplementation.

Bladder Exstrophy↗
PubMed1979

Improved life expectancy for children with exstrophy of the bladder.

Life-table techniques demonstrate a 98% ten-year survival, a 96% 20-year survival, and 91% 35-year survival of children with exstrophy of the bladder. These figures represent a vast improvement over the earlier figures, due in large part to technical advances that have been made in areas of surgery, anesthesia, and antibacterial and metabolic therapy.

Adolescent↗