Spinal cord injury nurses in action: partners in practice.

The creation and implementation of a Partners in Practice care delivery model on a spinal cord injury unit is described in this article. As a result of changes in the number and mix of staff, new strategies were necessary to assure the provision of patient focused, team oriented care. The goal was to increase the efficiency, effectiveness, and continuity of care while ensuring staff accountability and appropriate utilization of resources. Manthey's (1980) Partners in Practice model was adapted to meet the needs of the unit, and was implemented using Lewin's (Scott, 1994) change theory. Outcomes of the model included the establishment of a culture of partnership for providing team oriented care to spinal cord injury patients.

Surgery for Crohn's disease in infants and children.

The course of Crohn's disease is quite variable in children. To assess the frequency and indications for surgery with current medical therapy, the authors reviewed the cases of 204 children (ages, 0.2 to 18.8 years at diagnosis, median, 12.8 years) who had Crohn's disease treated at a single institution from December 1968 to January 1994, with a median of 3.8 years of follow-up (range, 0.0 to 22.2 years). Ninety-four children (46%) required surgical resection for the following indications: (1) failure of medical therapy with persistent symptoms or growth retardation (n = 44, 47%), (2) intraabdominal abscess or perforation (n = 15, 16%), (3) fistula formation (n = 13, 14%), (4) obstruction (n = 15, 16%), (5) hemorrhage (n = 4, 4%), and (6) appendectomy at exploration for diagnosis (n = 3, 3%). The probability for surgery 3 years after diagnosis is 28.8% and by 5 years is 47.2%. Resections included ileocolectomy (71 children), colectomy (n = 16), small bowel resection (n = 4), and appendectomy (n = 3). Fourteen fistulas in 13 children required surgical intervention (7 enteroenteral, 3 enterovesical, 2 enterovaginal, and 2 enterocutaneous). The median duration from diagnosis to surgery for the fistulas was 2.6 years (range 0.1 to 9.8 years). Forty patients experienced recurring disease after resection during follow-up with a median of 1.8 years (range 0.4 to 18.1 years). The authors found that the course of the disease was unpredictable, with some children requiring early surgical intervention and others continuing with medical therapy for years.

Continent urinary diversion: the Children's Hospital experience.

PURPOSE: Continent urinary diversion has become increasingly important for treating childhood urinary tract pathology that cannot be managed by direct reconstructive techniques. We review our 9-year experience with continent diversion. MATERIALS AND METHODS: Since 1986 continent diversions were created in 74 patients 3 to 38 years old (mean age 13.7). The underlying pathological condition was the exstrophy/epispadias complex in 34 patients, neurological disorders in 23, malignancy in 13 and other congenital anomalies in 4. Followup averaged 5.2 years after the last procedure. Nonbladder reservoirs in 39 patients (53%) were fashioned from ileocolic (17), colic (7), gastrocolic (6), sigmoid (3), gastrosigmoid (2), ileosigmoid (2), ileal (1) and gastroileac (1) segments. When possible, the native bladder was incorporated into the reconstructive strategy. A total of 26 patients underwent bladder augmentation with intestine or stomach, including ileal (11), gastric (8), sigmoid (3), gastroileac (2) and ileocolic (2) segments. Nine other patients did not require bladder augmentation. Continence mechanisms were a flap valve (Mitrofanoff principle) in 50 patients, nipple valve in 15 and ileal plication (Indiana pouch) in 9. When the Mitrofanoff principle was used with a native bladder reservoir in 30 cases, outlet resistance was altered by bladder neck division (15), fascial sling placement (6) or Young-Dees-Leadbetter bladder neck reconstruction (2). In the remaining 7 patients the bladder neck remained intact. RESULTS: Excellent continence was obtained. The Mitrofanoff principle initially provided continence in 41 patients (82%). Six of the 9 incontinent patients were dry after a single revision. A total of 13 patients (87%) with nipple valves and 7 (78%) with Indiana pouches were dry, and the remaining 5 were cured after a single revision. Ultimately continence was achieved in 71 of the 74 patients (96%) after a maximum of 2 operations. Of the 48 complications in 29 patients the most common were difficulty in catheterizing (11), stones (11), infection (8) and upper tract deterioration (4). CONCLUSIONS: Many options exist for reconstructing complex anomalies. Choices must be individualized based on patient anatomy. The dry state may be achieved in most cases without resorting to a bag on the abdomen.

Reconstructive options in genitourinary rhabdomyosarcoma.

PURPOSE: We attempted to develop a rational and consistent scheme for surgical reconstruction in patients with genitourinary rhabdomyosarcoma. MATERIALS AND METHODS: We reviewed the records of 35 patients with resectable genitourinary rhabdomyosarcoma treated from 1970 to 1993. RESULTS: Primary sites included bladder in 11 cases, prostate in 13, vagina/uterus in 9 and pelvic tumors of uncertain origin in 2. A total of 33 patients underwent surgery, including partial and radical cystectomy in 17 (bowel conduit diversion in 10, continent urinary diversion in 6 and ureterosigmoidostomy in 1). Overall 30 of the 33 surgical patients are free of disease 4 months to 24 years after diagnosis. CONCLUSIONS: A nonrefluxing colon conduit is appropriate at cystectomy. Continent diversion fashioned from the original conduit may be planned as the patient achieves a durable disease-free status.

Ten-year experience with the artificial urinary sphincter in children.

PURPOSE: To assess the efficacy of the artificial urinary sphincter survival and continence were first evaluated 5 years ago. We now evaluated the effects of the artificial urinary sphincter more than a decade after implantation. MATERIALS AND METHODS: Before 1985 an artificial urinary sphincter was implanted in 22 male and 14 female consecutive patients 4 to 30 years old (median age 12). In addition, between 1985 and 1990, 18 other children underwent artificial urinary sphincter implantation. Results of both series were compared. RESULTS: Of the 25 sphincters in the original series that were functioning after 5 years 1 was removed and 2 patients were lost to followup, resulting in 22 functioning implants (61% of the patients). Mean survival time of the prostheses was 12.1 years and average followup for functioning sphincters was 13.7 years. There was no statistically significant difference in artificial urinary sphincter survival between the original group at 5 years and the second group treated after 1985. Of the patients in both groups with sphincters in place 32 of 39 (82%) were dry. Augmentation cystoplasty was performed in 9 of 18 patients (50%) in the second series (5 preoperatively and 4 postoperatively) compared to 10 of 36 (28%) in the original series at 5 years (3 preoperatively and 7 postoperatively). Renal failure developed in 6 patients from both series. CONCLUSIONS: The artificial urinary sphincter is a durable long-term solution for children with intractable incontinence. Long-term surveillance of the urinary tract is mandatory because of the potential for renal failure in patients who have bladder hypertonicity after placement of the device.

Congenital posterior urethral perineal fistulae: a unique form of urethral duplication.

Three unique cases of urethral duplication, often referred to as congenital posterior urethral perineal fistulae, are presented. Presenting signs and symptoms, evaluation, and treatment are discussed, and the literature is reviewed. Although labeled as a fistula in the literature, this entity most likely represents a distinct type of urethral duplication. A functional dorsal urethra separates it from the other forms of urethral duplication.

Laparoscopy in pediatric urology: too much of a good thing?

Laparoscopy is still an evolving technique; its rightful place in pediatric urology has yet to be defined. Although both the first reported use of diagnostic laparoscopy in pediatric surgery and its initial use in pediatric urology were published in 1973, the field lay dormant until recently. Once operative laparoscopy was initiated with the report of laparoscopic cholecystectomy in 1987, a torrent of articles followed; many operative procedures in adult urology and subsequently in pediatric urology appeared. This article presents the advantages and the disadvantages of several of the more important and common possible indications for laparoscopy in pediatric urology.

The long-term urological response of neonates with myelodysplasia treated proactively with intermittent catheterization and anticholinergic therapy.

PURPOSE: Urinary tract management in children with myelodysplasia is controversial. Some advocate observation alone, while others believe that the prophylactic institution of intermittent catheterization and anticholinergic therapy may help to prevent deterioration. MATERIALS AND METHODS: A nonrandomized prospective study was instituted to compare the urological outcomes of a cohort of children who were at risk for urological deterioration on the basis of bladder-sphincter dyssynergia and/or high filling or voiding pressures. Those at risk were observed until deterioration occurred, or were placed on prophylactic intermittent catheterization with or without anticholinergic medication. RESULTS: Of 44 children at risk 35 followed by observation alone had urinary tract deterioration, whereas only 3 of 20 at risk treated with prophylactic intermittent catheterization had deterioration with time. CONCLUSIONS: Proactive bladder treatment significantly reduced the incidence of upper urinary tract deterioration and need for surgical intervention.

Comparative urodynamics of appendiceal and ureteral Mitrofanoff conduits in children.

We report the cystometric and conduit pressure profilometric findings of 20 children for whom the Mitrofanoff principle was applied to the construction of 21 continent catheterizable urinary conduits. Mitrofanoff conduits were fashioned from ureter in 10 cases, appendix in 8, ileum in 1, stomach in 1 and a combination appendix and ureter in 1. Conduit pressure profiles showed that functional profile length correlated strongly with the static profile maximal Mitrofanoff closure pressure (p = 0.04) and dynamic profile maximal Mitrofanoff closure pressure (p = 0.02). There was a statistically significant difference between clinical continence rates for children above and below the functional profile length threshold of 2.0 cm. (p = 0.05). The zone of continence corresponded to the region of the conduit intramural tunnel. Only 2 of 21 conduits were incontinent but both were constructed with ureters implanted into bowel reservoirs. Compared to appendiceal conduits, ureteral conduits had a lower functional profile length (p < 0.01) and static profile maximal Mitrofanoff closure pressure (p < 0.01), indicating a possible advantage to the use of the appendix.

Management of severe hypospadias with a 2-stage repair.

From 1986 to 1993 we treated 1,437 children with hypospadias of whom 58 had scrotal or perineal hypospadias with severe chordee and a small phallus. These patients underwent a 2-stage surgical repair. The first stage of the procedure included correction of the chordee and advancement of preputial flaps ventrally and distally to the superior aspect of the glans. After 6 to 12 months the second stage of the procedure was performed using the previously transferred preputial skin to reconstruct the glans and urethra. A second layer of subcutaneous tissue or tunica vaginalis was used in several instances. A penile nerve block and a transparent biomembrane dressing (Tegaderm) allowed for early postoperative mobilization. Excellent functional and cosmetic results were achieved. There is a subset of patients with severe proximal hypospadias, chordee and a small phallus who may benefit best from a 2-stage procedure. In these children a 2-stage repair allows for a better cosmetic appearance and a lower complication rate than a 1-stage repair with a free or vascularized graft.

Perforation of the augmented bladder.

In 12 of 264 children treated with enterocystoplasty 15 spontaneous perforations occurred. Of the 12 children 9 had myelodysplasia. All segments of the gastrointestinal tract were used for the augmentation and most were detubularized. Surgery to increase bladder outlet resistance was done in 8 cases. At the time of each perforation 9 children had sterile cultures, however, 3 died of overwhelming sepsis. Presenting signs included abdominal pain in 8 cases, septic shock in 4 cases and shoulder pain in 4 older myelodysplastic children with diaphragmatic irritation from escaping urine. Cystography demonstrated a leak in 10 of 11 cases. Urodynamic studies revealed good compliance with low maximum filling pressure in 8 of 10 children. Hyperreflexia was noted in only 5 cases and outlet resistance greater than 85 cm. water was demonstrated in 5. Histological analysis showed changes in the bowel wall consistent with ischemia but suture granulomas were present in areas adjacent to the perforation site or thinned areas in biopsy or autopsy specimens. In addition to the theory that overdistention may cause enterocystoplasty perforation, current detubularization techniques may produce areas of relative ischemia, which become accentuated when the augmented bladder is distended beyond a reasonable volume.

The prophylactic value of clean intermittent catheterization and anticholinergic medication in newborns and infants with myelodysplasia at risk of developing urinary tract deterioration.

OBJECTIVE: To determine if prophylactic use of clean intermittent catheterization and oxybutynin chloride is effective in preventing urinary tract deterioration in myelodysplastic children with high bladder pressure and detrusor-sphincter dyssynergia. DESIGN: Sequential, nonrandomized trial. SETTING: Referral-based urodynamics facility and myelodysplasia program at a major city pediatric hospital. PARTICIPANTS: Twenty-six of 71 consecutive newborns with myelodysplasia who exhibited these urodynamic findings were treated prophylactically over 5 years, whereas 56 of 105 consecutive newborns with the same findings treated during the previous 7 years were treated expectantly. INTERVENTION: Clean intermittent catheterization and oxybutynin therapy were begun when these specific urodynamic findings were detected. MEASUREMENTS AND RESULTS: Follow-up urodynamic studies and roentgenographic assessment of the urinary tract were performed periodically over 5 years. Oxybutynin eliminated uninhibited contractions in two of 14 newborns and lowered peak contractile pressure in the remaining 12. Oxybutynin also lowered bladder-filling pressure at capacity in all 12 additional neonates with only hypertonicity. Twenty-four (92%) of 26 children had normal kidney function and drainage during the observation period, two (8%) developed hydroureteronephrosis, and one of these two had vesicoureteral reflux. In a prior study of children similarly at risk who were treated expectantly, the roentgenographic appearance of the upper urinary tract had changed in 48%. Minimal side effects were noted with oxybutynin, and no adverse effects of clean intermittent catheterization were detected. CONCLUSIONS: Oxybutynin effectively reduces uninhibited contractions and lowers detrusor filling pressure, while clean intermittent catheterization allows bladder emptying at low pressures with no measurable side effects in these neonates. The overall effect maintains the integrity of the upper urinary tract in almost all myelodysplastic children at risk of urinary tract deterioration. Expectant therapy can no longer be advocated when these "at risk" children are identified because prophylactic treatment is so effective.

The long-term results of artificial sphincters in children.

To determine the long-term efficacy of the artificial urinary sphincter, 36 consecutive children in whom a prosthesis was implanted between August 1978 and July 1985 were followed for a minimum of 5 years and re-evaluated within the last 3 months. Of the 36 sphincters 27 (75%) are currently in place; 2 of these 27 sphincters are dysfunctional due to fluid leakage. Of the 25 functioning sphincters 14 have required no further prosthetic operation, 10 needed 1 revision and 1 patient had 5 revisions. Fluid leakage, occurring in 12 patients, was the most common complication requiring reoperation. Twenty patients are completely continent (11 of whom without any medication) and 5 are occasionally damp. The over-all success rate at 2 and 5 years was 84 and 62%, respectively. The mean survival time of the prosthesis was 7.2 years and the mean standard interval was greater than 10.5 years. Patients with higher balloon pressures (81 to 100 cm. water) had more complications, and a decreased rate of success than those with lower pressure balloons (51 to 80 cm. water, p = 0.02). There was no difference in success rates between boys and girls (p = 0.98), although girls who had a prior bladder neck operation tended to have a higher rate of cuff erosion. Sphincters placed after 1980 (model AS800) had a lower reoperation rate than those placed earlier. In selected instances, the artificial urinary sphincter appears to be a viable long-term alternative for management of children with intractable incontinence who have either failed or are unsuitable for other methods of treatment.