Device outcomes in pediatric sacral neuromodulation: A single center series of 187 patients.

INTRODUCTION: Pediatric sacral neuromodulation (SNM) device duration before revision or removal is not well known. Furthermore, secondary surgeries serve as surrogates for key outcomes including complications and symptom improvement. OBJECTIVE: We sought to study the rate and causes of secondary surgeries in pediatric SNM patients. STUDY DESIGN: We assessed our cohort of pediatric SNM patients for secondary surgeries (revision or removal). Baseline patient characteristics associated with secondary surgery were analyzed with Fisher's exact test. Kaplan-Meier analysis was used to describe secondary-surgery-free device survival. RESULTS: 187 pediatric patients underwent sacral neuromodulation at our institution between 2002 and 2019. 7 (4%) patients did not have a permanent device placed due to poor response during an externalized lead trial period, leaving 180 patients with permanent implanted devices. Over a median follow-up of 3.9 years (IQR 2.0 to 6.3), there were 154 total secondary surgeries. There were 83 device revisions, with 89% of revisions for a non-functioning device, 8% for pain, and 2% for infection. Of the non-functioning devices, 11% were due to battery depletion and the rest were due to lead fracture or dislodgement. Permanent device removal was performed in 71 (39%) patients, with 38% of these for unfavorable reasons (6% infection, 8% pain, 24% no longer effective) and 62% for favorable reasons (symptom improvement or resolution). Of patient baseline characteristics, only device duration was associated with favorable removal (p < 0.01). On Kaplan-Meier analysis, 5 year device secondary-surgery-free survival was 32% for any secondary surgery (favorable or unfavorable) and was 47% for unfavorable secondary surgery. At last follow-up, 74% of patients were in a favorable position (using the device, trialing with device off, or device had been removed for improvement) while 26% of patients were requiring other treatments due to device problem or removal. DISCUSSION: Describing the likelihood of subsequent surgery is an important aspect of patient and guardian counseling concerning pediatric SNM. Our cohort had a 68% 5 year all-cause reoperation rate. However, in a theoretical perfect world, pediatric SNM reoperation rate would be 100% after adequate follow-up (either for battery replacement, or device explantation for improvement). CONCLUSIONS: While sacral neuromodulation in our cohort of children carried a high (68% 5-year) reoperation rate (whether for complication or symptom improvement), the majority (74%) of these patients with previously refractory symptoms either continue to use their device or have had significant symptom improvement to permit device removal at moderate-term (median 3.9 year) follow-up.

Sacral neuromodulation for the dysfunctional elimination syndrome: a 10-year single-center experience with 105 consecutive children.

OBJECTIVE: To evaluate our initial experience using sacral neuromodulation via implanted pulse generator as a treatment for children with dysfunctional elimination syndrome and symptoms refractory to maximum medical therapy. MATERIALS AND METHODS: There were 105 consecutive patients who underwent a 2-stage procedure with a trial period (n = 89) or a single procedure (n = 16) for device implantation. They were followed up prospectively for a median of 2.72 years (average, 3.18 years; range, 0.01-9.63 years) for symptom improvement and resolution. RESULTS: Nearly all children (99 of 105 [94%]) experienced improvement of at least 1 symptom; 12 of 105 patients (11%) had at least 1 symptom worsen. Urinary incontinence, constipation, frequency and/or urgency, and nocturnal enuresis improved in 89 of 101 (88%), 73 of 92 (79%), 54 of 81 (67%), and 59 of 89 (66%) children, respectively, and resolved in 41 of 101 (41%), 37 of 92 (40%), 23 of 81 (28%), and 25 of 89 (28%) children, respectively. Outcomes among patients who did and did not undergo the trial were not significantly different (P = .19-1.00), and only 2 of 88 patients did not undergo permanent implantation. Reoperations (n = 85) occurred in 59 of 105 children (56%), mainly for device malfunction (n = 42), whereas explantation was performed in 36 of 104 children (35%) at an average of 2.68 years since implantation (median, 2.36 years; range, 0.03-9.04 years), mainly for complete symptom resolution (n = 12). Explantation for any reason was less common after single-stage procedures (1 of 16 [6%] vs 35 of 88 [40%] patients; P = .01). CONCLUSION: Sacral neuromodulation should be considered for children with dysfunctional elimination syndrome whose symptoms are refractory to maximum medical therapy understanding that the risk of reoperation is >50%. Elimination of the trial period reduces the number of general anesthetics without sacrificing outcomes.

Vesicoureteral reflux in children with urachal anomalies.

OBJECTIVE: We report the largest known series of vesicoureteral reflux (VUR) in children with urachal anomalies (UA). METHODS: Two institutions' records were reviewed for children with UA (1951‒2007). RESULTS: Of 30 girls and 36 boys with UA (34 urachal cysts, 14 patent urachus, 10 urachal diverticula, 7 urachal sinuses, and 1 unknown), 57 (86%) underwent surgical resection or drainage. A voiding cystourethrogram was obtained in 22 (33%). VUR was demonstrated in 14 of the 22 children (64%), and rates were similar among the various types of UA. The median age with versus without VUR was not different (1.3 vs 1.7 years, P=0.97). Of 24 refluxing renal units, classification was grade≤3 in 71%, 4‒5 in 12%, and unspecified in 17%. Four children (26%) underwent ureteroneocystostomy and 10 observed patients resolved spontaneously. CONCLUSION: To our knowledge, this is the first series of VUR associated with UA. The increased incidence of VUR (64%) in this small subset of patients warrants prospective studies to determine if there is a positive correlation with UA. We believe thorough genitourinary and family histories are important when evaluating children with UA to help detect clinically significant VUR.

Sacral neuromodulation for the dysfunctional elimination syndrome: a single center experience with 20 children.

PURPOSE: Recent advances in neuromodulation have demonstrated promise in treating children with the dysfunctional elimination syndrome refractory to medical management. Sacral nerve stimulation with the InterStim implantable device has been used in adults for management of chronic urinary complaints. However, there are few data regarding the usefulness of sacral nerve stimulation in children. We report our experience with sacral nerve stimulation for severe dysfunctional elimination syndrome. MATERIALS AND METHODS: A total of 20 patients 8 to 17 years old with the dysfunctional elimination syndrome refractory to maximum medical treatment underwent sacral nerve stimulation at our institution. Patients were followed prospectively for a median of 27 months after the procedure. RESULTS: Urinary incontinence, urgency and frequency, nocturnal enuresis and constipation were improved or resolved in 88% (14 of 16), 69% (9 of 13), 89% (8 of 9), 69% (11 of 16) and 71% (12 of 17) of the patients, respectively. Urinary retention requiring intermittent catheterization persisted in 75% of the patients (3 of 4) despite sacral nerve stimulation. Complications requiring operative treatment occurred in 20% of the patients (4 of 20). Following marked symptomatic improvement 2 devices were explanted at 20 and 19 months following placement, and both patients have remained symptom-free. CONCLUSIONS: Sacral nerve stimulation is effective in the majority of our patients, and should be considered in children with severe dysfunctional elimination syndrome refractory to maximum medical treatment.

Familial multilocular cystic nephroma: a variant of a unique renal neoplasm.

Multilocular cystic nephroma (MCN) is a benign renal lesion believed to be unilateral and nonfamilial. We present 2 cases of MCN in 2 brothers. The older brother had a 10-cm MCN and required radical nephrectomy, and the younger brother had bilateral lesions (6 cm and 3 cm) for which nephron-sparing surgery was possible. Both children underwent chest computed tomography to rule out pleuropulmonary blastoma. These cases suggest a genetic link to the pathogenesis of this lesion is possible and that although MCN can involve the collecting system, it can still be treated with partial nephrectomy.

Preliminary results of sacral neuromodulation in 23 children.

PURPOSE: Sacral nerve stimulation with InterStim is approved in the United States for use in adults. Limited data on the effectiveness of sacral nerve stimulation in children are available. We report our experience with patients who underwent InterStim placement for the treatment of severe dysfunctional elimination syndrome, which is defined as a constellation of functional urinary and gastrointestinal symptoms in patients without anatomical anomalies or obvious neurological disease, in whom intensive medical and behavioral therapies have failed to improve symptoms. MATERIALS AND METHODS: A total of 23 patients 6 to 15 years old with presenting symptoms of dysfunctional voiding, enuresis, incontinence, urinary tract infections, bladder pain, urinary retention, urgency, frequency, constipation and/or fecal soiling were followed for a mean of 13.3 months after InterStim placement. RESULTS: Of the 19 patients with urinary incontinence 3 (16%) had complete resolution, 13 (68%) had improvement, 2 (11%) were unchanged and 1 (5%) was worse (sign test, p = 0.002). Among the 16 patients with nocturnal enuresis 2 (13%) had resolution, 9 (56%) improved, 4 (25%) were unchanged and 1 (6%) was worse (sign test, p = 0.0063). Of the 15 patients with urinary retention requiring intervention 9 (60%) had improvement and 1 had worsening symptoms (sign test, p = 0.022), while 2 of 6 patients (33%) on intermittent catheterization were able to stop. One patient had development of new incontinence and enuresis. Bladder pain, urgency, frequency and constipation improved in 67% (8 of 12), 75% (12 of 16), 73% (11 of 15) and 80% (12 of 15) of the patients, respectively. Medications required postoperatively decreased by an average of 3 per patient (p < 0.001). The overall patient satisfaction rate was 64%, while that of the caregiver was 67%. Two leads were explanted from the 23 patients. Complications encountered included 1 seroma, 1 episode of skin sensitivity, 2 device failures and 1 lead revision. CONCLUSIONS: Sacral nerve stimulation in children is an option for carefully selected patients who have failed other therapies. Our results show that sacral nerve stimulation was effective in the majority of patients with the dysfunctional elimination syndrome. However, longer followup is needed.

Contemporary and emerging drug treatments for urinary incontinence in children.

Pediatric incontinence is a bothersome symptom for children and their parents. It can have a profound influence on a child's social and psychologic development and well-being. It is important to understand the different disorders that result in incontinence and also to understand the neural influences and development on urinary control. Urinary leakage can be a functional or organic disorder, with many possible etiologies. The most common group of pediatric patients with incontinence are those with overactive bladder disorder. Pharmacologic therapy centers on the blockage of muscarinic receptors by the tertiary amines such as oxybutynin, tolterodine, trospium chloride, and propiverine. Although most novel anticholinergic medications are effective and well tolerated in children, in our experience oxybutynin extended release provides superior relief for urge urinary incontinence in children. Other agents such as alpha-adrenoceptor antagonists have been used with success to improve bladder empyting and decrease outlet resistance. Night-time voiding disorders such as primary monosymptomatic nocturnal enuresis tend to be symptomatically treated. One of the mainstays of pharmacotherapy is desmopressin, an analog to antidiuretic hormone, which decreases night-time urine production. Tricyclic antidepressants such as imipramine have also been used successfully through a combined mechanism of action believed to be the result of anticholinergic, antispasmodic, and sympathomimetic effects. Often the successful treatment of constipation also treats urinary incontinence or at least the symptoms of urinary leakage are improved. The new non-absorbable, tasteless, and odorless PEG-3350 (polyethylene glycol 3350) powder has quickly become a mainstay of the pharmacologic treatment for constipation because of its ease of preparation and favorable adverse effect profile. A better understanding of the physiologic control, cellular interactions, and second messenger signal transduction pathways has led to the development of many new potential target sites for pharmacologic intervention. The advancement of new uroselective muscarinic antagonists is currently under investigation for agents such as darifenacin and temiverine, which have the potential to improve efficacy without increasing unwanted adverse effects. New pharmacologic delivery systems are also being developed ranging from intravesical to transdermal applications to change biodistribution and improve selectivity. Incontinence is a significant problem for children, their parents, and their physicians. The changing and advancing field of pharmacotherapy has made big strides for symptom control in this patient population.

Neuromodulation in pediatrics.

Several neuromodulatory options are available for selected children who have voiding dysfunction resistant to conventional therapy. Integral to all types of treatment is an educated team that reinforces progress rather than cure and encourages long-term behavioral changes.