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PubMed2000

Combined bladder neck, urethral and penile reconstruction in boys with the exstrophy-epispadias complex.

OBJECTIVE: To describe a one-stage combined bladder neck, urethral and penile reconstruction for achieving urinary continence and creating a penis with good cosmesis and function in boys with the exstrophy-epispadias complex. PATIENTS AND METHODS: Seventy-three boys underwent the combined procedure, including 36 after classic bladder exstrophy closure and 37 with epispadias. All were completely incontinent at the time of combined reconstruction. The bladder capacity just before surgery was 70-180 mL and was greater in those with epispadias. The boys were 2.5-11 years old, with those in the exstrophy group being slightly older. RESULTS: Thirty-three boys (89%) with epispadias were completely continent during the day but 15 had episodes of nocturnal enuresis. Of boys with classic exstrophy, 27 (75%) were continent during the day but nine had occasional nocturnal enuresis. Eleven boys required intermittent catheterization, which they found easy to perform. In seven boys (10%) a urethrocutaneous fistula or urethral stricture developed. CONCLUSIONS: Combined bladder neck, urethral and penile reconstruction can be carried out as a one-stage procedure in selected patients with adequate bladder capacity. Reconstruction of the whole length of the urethra facilitates intermittent catheterization.

Bladder Exstrophy↗
PubMed1996

The sigma rectum pouch (Mainz pouch II).

A low-pressure reservoir for urine is created by antimesenteric splitting and side-to-side anastomosis of the rectosigmoid, the expectation being to obtain better continence rates and better protection of the upper tract than are achievable by ureterosigmoidostomy. Between 1990 and August 1993 the procedure was performed in 73 patients (59 adults and 14 children) whose mean age was 43.5 years. The indications were malignancy (n = 55), bladder exstrophy/epispadias (n = 14), trauma (n = 3), and sinus urogenitalis (n = 1). Of the 73 patients, 69 were followed for a mean period of 127 (range, 1-34) months. In all, 5 early complications were encountered (6.8%). In addition, 8 late complications occurred (10.9%), stenosis at the ureteral implantation site being the most common one. Daytime continence was 94.5% and night-time continence, 98.6%. The sigma rectum pouch achieves excellent continence rates. Despite implantation of the ureters into a low-pressure reservoir, stenosis at the site of ureteral implantation occurred in 6.8% of the patients, demonstrating the profund vulnerability of ureterointestinal anastomosis.

Adolescent↗
PubMed1976

Ileal conduit urinary diversion. Ten-year review.

The experience with ileal conduit urinary diversion at Emory University and Affiliated Hospitals over a ten-year period is presented. Included in this review are patients with benign and malignant disease. The indications, technique, results, and complications of this type of urinary diversion are considered. Some differences between ileal conduit diversion of children and adults are discussed.

Adolescent↗
PubMed2003

Metabolic findings after colocystoplasty in children.

OBJECTIVES: To investigate whether colocystoplasty has resulted in metabolic changes in the growing child during long-term follow-up according to whether cecum with ascending or sigmoid colon was used. METHODS: Twenty-eight patients (mean age at surgery 11 years) were included in the study and divided into two groups: group 1, cystoplasty with cecum and ascending colon (12 patients) and group 2, sigmoid cystoplasty (16 patients). Patients' linear growth, body mass index, and the following parameters were estimated before surgery and at 3, 6, and 12 months, and then yearly after surgery: blood and urine electrolytes (sodium, potassium, chloride, calcium, phosphorus, magnesium), creatinine, urea, blood gases, blood pH, urine pH, and blood alkaline phosphatase (ALP). All the data were statistically analyzed. RESULTS: In group 1, the blood ALP increased significantly (P = 0.026) during follow-up. Severe metabolic acidosis with or without hyperchloremia was found in 7 patients. In group 2, the serum sodium and serum calcium levels decreased significantly (P = 0.014 and P = 0.003, respectively); however, the blood ALP, urine sodium, and urine phosphorus levels increased significantly (P = 0.033, P = 0.027, and P = 0.026, respectively) during follow-up. A statistically significant decrease in blood pH (P = 0.022) was found after surgery. Severe metabolic acidosis with or without hyperchloremia was detected in 5 patients. The average linear growth decreased significantly (P = 0.001 and P = 0.016, respectively) 1 and 2 years postoperatively. CONCLUSIONS: The statistically significant increase in blood ALP and decrease in serum calcium indicate bone demineralization after colocystoplasty. Our investigations in children suggest that bone demineralization is more frequent after sigmoid cystoplasty than after the use of cecum and ascending colon.

Acidosis↗
PubMed2005

Psychosocial and psychosexual development in childhood and adolescence within the exstrophy-epispadias complex.

PURPOSE: We report the psychosocial and psychosexual development of children and adolescents with the exstrophy-epispadias complex (EEC) after complete functional repair using the Erlangen single stage technique. MATERIALS AND METHODS: In a long-term retrospective followup of an average of 11.1 years 100 patients with EEC (76 boys and 24 girls, mean age 14.5 years) were evaluated with respect to medical history, and received a general questionnaire concerning their social and psychosocial situation. A total of 54 patients who were 15 years or older (mean age 18.5) received an additional questionnaire to assess detailed sexual history. RESULTS: Of the patients 81% returned the general questionnaire within 3 weeks. School education level and social integration were high. In about 25% of the patients impairment of daily life was significant, and in 58.7% peer relations were altered. Of the adolescent group 76% answered the special questionnaire. Genital satisfaction and genital touching were rated low, and avoidance of nudity in public areas was common. All patients expressed heterosexuality and 43.9% had engaged in sexual intercourse but 58.5% displayed anxiety about sexual activity. It is noteworthy that 93.9% expressed an interest in psychological assistance. CONCLUSIONS: Despite a high degree of social integration and adult adaptation, children and adolescents with EEC suffer from psychosocial and psychosexual dysfunction requiring special questionnaires for adequate assessment. Anxiety about genital appearance and sexual activity is a common phenomenon among adolescents with EEC, even when they present with nearly "normal" genitalia and participate with satisfaction in sexual activity. Further studies are needed to understand the exstrophy problem and supply all patients with EEC with the individual care they need.

Activities of Daily Living↗
PubMed2005

Reconstructive lower urinary tract surgery in incontinent adolescents with exstrophy/epispadias complex.

OBJECTIVES: To outline the management strategies applied to the adolescent patient population with exstrophy/epispadias and incontinence at our institution. These patients present a difficult management problem. At the same time they are dealing with difficult issues, including body image and sexual awareness, the added burden of ongoing incontinence causes major anxieties and lifestyle restrictions. In many, incontinence has persisted despite numerous operations. METHODS: A total of 25 (19 male and 6 female) patients who remained incontinent into adolescence or early adulthood were reviewed. Of the 25 patients, 19 had exstrophy, 4 had cloacal exstrophy, and 1 male and 1 female had epispadias. Six patients had undergone eight prior attempts at continent reconstruction, one had undergone cutaneous diversion, and one had problems after ureterosigmoidostomy. RESULTS: The mean age at continence surgery was 12.9 years. Of the 25 patients, 18 underwent bladder augmentation, with a continent stoma in 17 and an artificial sphincter in 1. An additional 5 patients underwent bladder neck transection, with a new continent stoma in 3. The ureterosigmoidostomy was converted to a Mainz II pouch. One patient underwent continent neobladder formation. Nine patients (36%) developed complications during follow-up. Three required stoma revision for stenosis and one for prolapse. Pouch stones occurred in 4 patients, and vesicocutaneous fistula developed in 1. All achieved full urinary continence. The mean follow-up was 72.4 months. CONCLUSIONS: Some children with exstrophy/epispadias reach adolescence and remain incontinent. For these patients, modern reconstructive techniques provide hope of continence. With careful preoperative assessment, exact surgical precision, and regular follow-up, a successful outcome can be expected in virtually all cases without the need for external urine collection devices.

Adolescent↗
PubMed2002

Phenotypic and functional characterization of in vivo tissue engineered smooth muscle from normal and pathological bladders.

PURPOSE: The engineering of bladder tissue involves obtaining a biopsy from a host, expanding the cells, seeding them onto a matrix and implanting the cell-matrix composite back into the host. Clinically, cells used for these techniques may be harvested from abnormal bladders. It is not known whether abnormal bladder cells may be engineered into functionally normal tissue. We investigated the phenotypic and functional characteristics of tissue engineered bladder smooth muscle derived from patients with functionally normal bladders and functionally abnormal exstrophic and neuropathic bladders. MATERIALS AND METHODS: Human smooth muscle cells derived from functionally normal bladders, exstrophic bladders and neurogenic bladders were grown, expanded and seeded onto polymer scaffolds. Sixteen cell seeded scaffolds were analyzed in vitro and 40 cell seeded scaffolds were implanted in athymic mice. The tissue engineered constructs were retrieved and analyzed at 2 weeks and 2 months. The scaffolds were evaluated immunocytochemically, histologically, with organ bath studies and with Western blot analyses. RESULTS: Human bladder cells showed similar expression of smooth muscle marker proteins (alpha-actin and myosin) in vitro and after 2 months in vivo, regardless of their origin. All scaffolds showed similar muscle formation in vivo. The cell seeded scaffolds demonstrated the typical "contraction-relaxation" response to supramaximal electrical field and carbachol stimulation. There were no statistical differences among the experimental groups (normal, exstrophic, neurogenic). CONCLUSIONS: Tissue engineered muscle from normal and diseased bladders retain their phenotype in vitro and after implantation in vivo. The cells exhibited the same degree of contractility to electrical and chemical stimulation regardless of their origin. These results suggest that there are no phenotypic or functional differences between muscle cells obtained from urodynamically normal or pathological bladders, and that bladder muscle cells, regardless of their origin, may have the potential to be engineered into normal bladder tissues.

Actins↗
PubMed1976

Follow-up of 13 children after ureterosigmoidostomy.

Follow-up of 13 children who had had a ureterosigmoid anastomosis 3 1/2 to 10 years previously and whose initial urogram had been satisfactory, showed that growth was normal and that there was no serious metabolic disorder. In particular whole-body potassium did not differ significantly from normal values (as given by Langham, 1961). Asymptomatic urinary infection is the chief hazard in these cases but is difficult to diagnose and may lead to progressive dilatation of the ureters.

Adolescent↗
PubMed1972

Pediatric urology.

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Bladder Exstrophy↗
PubMed1976

Cloacal exstrophy: 18-year survival of untreated case.

A patient is described who was 18 years old and attending high school when initially treated for cloacal exstrophy. The history, embryology and treatment of cloacal exstrophy are discussed, emphasizing that this congenital anomaly is compatible with a useful and happy life.

Adolescent↗
PubMed1987

Complications of artificial urinary sphincter around intestinal segments in reconstructed exstrophy patients.

Nine patients 15 to 28 years old underwent placement of the artificial urinary sphincter around an internal intestinal urinary reservoir. A total of 15 complications was experienced in 8 patients and 13 secondary procedures were necessary. Eight patients are completely continent and 1 has nighttime enuresis. The most common complication encountered was reduction in bowel luminal circumference underlying the sphincter cuff.

Adolescent↗
PubMed1986

Use of bowel in undiversion.

Urinary undiversion is becoming an uncommon procedure because fewer patients are being diverted. The lessons learned from undiversion, however, have made possible early primary reconstructions in patients who would previously have been candidates for diversion. Intermittent clean catheterization and the use of bowel to augment or form a compliant storage vessel for urine have permitted reconstruction in patients with abnormal bladder and urethral function. Thus patients with neurogenic bladder or with severe lower urinary tract abnormalities can look forward to healthy kidneys and urinary continence without the use of appliances. Certainly there are yet many lessons to be learned, but the potential is just starting to be appreciated.

Adolescent↗
PubMed1978

[Colonic segments as visceral substitutes in children].

The many ways in which segments of the colon can be used as visceral substitutes are described in the light of a personal series of neonates with multiple deformities, including atresia of the oesophagus and rectum, and atresia of the rectum coupled with agenesis of the vagina. At birth, colostomy at the right and extremity of the transverse colon is recommended (e.g. in cases of anorectal atresia, since this does not interrupt the vascular arcades required to give a good blood supply to the visceral reconstruction (as described) at a later date.

Bladder Exstrophy↗