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.

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.

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.

Paratesticular rhabdomyosarcoma: results of therapy in 18 cases.

The management of paratesticular rhabdomyosarcoma has evolved more slowly than that for testicular germ cell tumors, mainly due to its lower incidence and the lack of standardized treatment protocols. With gradual refinements in staging and therapy the prognosis has improved. Between 1960 and 1988, 18 patients 2 to 18 years old were treated at the Children's Hospital. Management consisted of inguinal orchiectomy, staging evaluation and retroperitoneal lymph node dissection, followed by chemotherapy and radiotherapy according to the stage of the disease. In 11 patients (61%) the disease was confined to the scrotum, while 7 presented with spread to the retroperitoneal lymph nodes, including 3 children with more distant metastases. Histological study revealed predominantly embryonal characteristics except for 1 alveolar cell type. Chemotherapy consisted of actinomycin D in 2 patients, vincristine, actinomycin D and cyclophosphamide in 2, pulse vincristine, actinomycin D and cyclophosphamide in 10, and doxorubicin plus vincristine, actinomycin D and cyclophosphamide in 4. A total of 17 patients remain free of disease (median followup 4 years) with 1 death. The actuarial survival without relapse and over-all survival rate are 89 and 94%, respectively. We conclude that paratesticular rhabdomyosarcoma represents a favorable subgroup of rhabdomyosarcoma.

The urodynamic consequences of posterior urethral valves.

We evaluated urodynamically 41 patients with posterior urethral valves because of signs or symptoms of incontinence (35), frequency (3), hydronephrosis (2) and infection (1). Findings included normal urodynamic evaluations in 3 patients, 2 had high voiding pressures secondary to outlet resistance and 1 had incontinence on the basis of external urethral sphincter damage. In the remainder 3 patterns of bladder dysfunction were identified. Myogenic failure with overflow incontinence occurred in 14 patients. In this group clean intermittent catheterization or Valsalva's voiding was used for emptying. Hyperreflexic bladders were seen in 10 patients. Pharmacological suppression of instability was effective in 5 of 7 patients treated; 1 required bladder augmentation. Eleven children had a small capacity bladder and poor compliance. Post-void residuals were low and these bladders were generally but not always stable. Pharmacological bladder relaxation was successful in 3 patients, 3 underwent augmentation, 1 did well with alpha-agonists and followup is unavailable on the other 4. These 3 patterns of bladder dysfunction represent an overlapping constellation of residual urodynamic abnormalities due to previous bladder outlet obstruction. Individual patients may show facets of several types of dysfunction associated with 1 predominant pattern.

The use of rectus fascia to manage urinary incontinence.

The surgical management of urinary incontinence in children is one of the most difficult challenges facing pediatric urologists today. We have critically looked at our experience using a rectus fascial sling to prevent incontinence. Eleven girls (6 to 22 years old) with urinary incontinence from neurogenic bladder dysfunction (9), surgical injury to the bladder (1) or urogenital sinus abnormality (1) comprise this study. All had failed regimens of pharmacological therapy and intermittent catheterization. Three patients had undergone prior bladder neck reconstruction and 2 an augmentation cystoplasty in an attempt to control the incontinence. Urodynamic studies revealed several reasons for the continued wetting in these individuals: a changing neurological lesion leading to a loss of innervation and concomitantly lowered urethral resistance in 2 patients, adequate urethral resistance at rest but decreasing resistance with bladder filling in 4, no increase in urethral resistance with increases in abdominal pressure in 4 and urethral instability (a decrease in resistance following a cough or Valsalva's maneuver) in 1 apparently neurologically normal girl. Eight patients are dry 3 to 24 months postoperatively on intermittent catheterization. All have demonstrated either an increase in urethral resistance at rest or an adequate level of resistance during filling of the bladder or a sudden increase in abdominal pressure. Of the remaining 3 patients 1 is dry for 2 to 3 hours but then leakage occurs, 1 is improved but damp and 1 patient is wet 3 months postoperatively. The use of rectus fascia to improve outlet resistance seems to be a viable alternative in the management of incontinence in selected female subjects.

The unrecognized neuropathic bladder of infancy.

Four infants presented with hydroureteronephrosis and incomplete emptying of the bladder secondary to detrusor-sphincter dyssynergia. None of the patients had other signs suggestive of neurological dysfunction. It is presumed but not proved that these infants manifested a urodynamic reaction in response to a perinatal insult involving the brainstem area. Long-term followup has demonstrated a persistent but nonprogressive neurourological disturbance.