High-intensity, short-term biofeedback in children with Hinman s syndrome (non-neuropathic voiding dyssynergia)

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1 Journal of Pediatric Urology (006), 344e350 High-intensity, short-term biofeedback in children with Hinman s syndrome (non-neuropathic voiding dyssynergia) L.M. Costa Monteiro a,b, D. Carlson a, A.B. Belman a, H.G. Rushton a, * a Division of Urology, Children National Medical Center, Washington DC, USA b Laboratory of Pediatric Urodynamic, Instituto Fernandes Figueira, Fundaç~ao Oswaldo Cruz, Rio de Janeiro, Brazil Received 18 October 005; accepted 4 March 006 Available online 19 May 006 KEY WORDS Hinman s syndrome; Voiding dysfunction; Biofeedback; Urinary incontinence; Non-neuropathic voiding dyssynergia; Non-neurogenic neurogenic bladder Abstract Objective: To evaluate the long-term response to high-intensity, shortterm biofeedback in children with severe voiding dysfunction. Patients and methods: We retrospectively reviewed patients who underwent shortterm, high-intensity biofeedback therapy from 1996 to 004. Improvement was classified based on clinical and radiographic findings. Patients were categorized as having Hinman s syndrome when, in addition to urinary incontinence, at least four of the following categories were present: sphincter dyssynergia, bladder trabeculation, large post-void residual (PVR), hydronephrosis, vesicoureteral reflux (VUR) and urinary tract infections. There were 14 patients (eight males and six females), 13 of whom had Hinman s syndrome. Age when biofeedback was initiated varied from 5.6 to 1.9 years (m ¼ 8.9.). Before biofeedback, all had large PVRs, bladder trabeculation and sphincter dyssynergia. Nine had hydronephrosis and five had VUR. One patient had renal failure. Results: Before biofeedback, the mean PVR was 9 ml (5e70 ml); after biofeedback, this decreased to 1 ml (0e150 ml), including two patients who eventually failed treatment. All 14 patients were able to relax their external sphincter and reduce the PVR during biofeedback and on short-term follow up. Long-term follow up (m ¼ 59.4 months) in 1 patients established that seven had a durable response with remission of symptoms, reduced PVR and radiographic improvement. In three, symptoms partially recurred over time and two failed treatment completely. * Corresponding author. Tel.: þ ; fax: þ address: hrushton@cnmc.org (H.G. Rushton) /$30 ª 006 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved. doi:.16/j.jpurol

2 High-intensity, short-term biofeedback in children 345 Conclusion: Short-term, high-intensity biofeedback achieves a durable response in the majority of children with Hinman s syndrome. Long-term follow up is needed to assure compliance. ª 006 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved. Introduction Voiding patterns and acquisition of bladder control in healthy children are age and maturation dependent. Although the neurophysiological process by which children acquire urinary control is not completely understood, various developmental stages are recognized. Conscious sensation of bladder fullness is expected between the first and second year, and the ability to control voiding commonly develops in the second to third year of life [1,]. A normal voiding cycle requires a bladder that expands easily during the filling phase and then contracts to empty completely during the voiding phase. Premature detrusor contractions or significant increases in bladder pressure should not occur during filling. Bladder fullness triggers a detrusor contraction, followed by sphincter relaxation, which can be inhibited at will for a certain interval. During the normal voiding phase there should be complete relaxation of the external urethral sphincter muscle so the urine can be completely and smoothly released from the bladder, without interruption. Voiding dysfunction is caused by an abnormality in one or both phases of the micturition cycle. Consequences may be urinary incontinence, incomplete emptying, and interrupted or intermittent flow of urine. Dysfunctional voiding disorders are divided into neuropathic disorders, which are clearly related to identifiable neurological conditions, and non-neuropathic or functional disorders. Voiding disorders in neurologically intact children are still not completely understood, but usually represent a medically benign although socially detrimental condition. The magnitude of these conditions may vary, and they can be classified according to their impact on the upper urinary tract into three main categories: mild, moderate and major dysfunctional disorders [3]. Included among the major dysfunctional disorders is Hinman s syndrome. Described by Hinman and Baumann [4] in 197, Hinman s syndrome is a rare severe dysfunctional disorder associated with voiding disturbances and upper urinary tract damage in the absence of anatomical obstruction or neurological problem. Other terms that have been used to describe this voiding disorder include non-neurogenic neurogenic bladder and non-neuropathic voiding dyssynergia [5e7]. It is characterized by clinical and urodynamic evidence of involuntary bladder outlet obstruction at the level of the external sphincter. Although the etiology of this severe condition remains unclear, it has been related to emotional, behavioral and psychiatric disturbances, and has been predominantly described in boys [5]. Children with Hinman s syndrome manifest overactivity of the external sphincter/pelvic floor while being unable to inhibit detrusor contractions. Eventually these children develop the consequences of bladder outlet obstruction with elevated intravesical filling and voiding pressures with increased post-void residual urine (PVR). Ultimately, these children may develop a decompensated or non-compliant bladder with secondary hydroureteronephrosis in approximately two-thirds of patients and significant VUR in about half. Patients with Hinman s syndrome present with urinary incontinence associated with chronic urinary retention. Related symptoms are an intermittent or staccato urinary stream, elevated PVR, recurrent UTIs, fecal retention and encopresis. Uroflowmetry will confirm an intermittent voiding pattern with reduced flow rates and prolonged voiding times. Further urodynamic evaluation typically reveals detrusor instability and pelvic floor overactivity during filling and voiding, often associated with decreased bladder compliance and high voiding pressure. Radiological abnormalities vary from bladder trabeculation to uretero-vesical junction obstruction or reflux associated with dilatation of the upper urinary tract and renal damage. Treatment is aimed at improving the child s ability to empty the bladder and bowel, and at preventing upper tract deterioration. The aim of this study is to report our short-term and long-term experience with high-intensity, shortterm biofeedback in children with severe voiding dysfunction (Hinman s syndrome). Patients and methods We retrospectively reviewed 14 patients, eight males and six females, diagnosed with severe voiding dysfunction who underwent high-intensity,

3 346 L.M.C. Monteiro et al. short-term biofeedback therapy from 1996 to 004. Age when biofeedback started varied from 5.6 to 1.9 years (m ¼ 8.9. years). Patient records, imaging studies and urodynamic evaluations were reviewed. Imaging studies included preand post-void renal sonograms, contrast voiding cystograms and renal scans, when appropriate. All patients were evaluated with an MRI of the spine to exclude spinal cord pathology. Highintensity, short-term biofeedback therapy was recommended in a highly select group of patients with severe voiding dysfunction, including 13 (93%) with Hinman s syndrome. Patients were categorized as having Hinman s syndrome when, apart from urinary incontinence, at least four of the following six categories were presented: UTIs, large PVR, sphincter dyssynergia, bladder trabeculation, hydronephrosis and VUR. Before biofeedback, all patients were noted to have sphincter dyssynergia and bladder trabeculation, and 13 (93%) had diurnal urinary incontinence. The average PVR was 9 ml (5e70 ml). Ten patients (71%) presented with repeated episodes of UTI. Nine patients (64%) had hydronephrosis and five (36%) VUR. One patient presented with renal insufficiency. Constipation was an associated symptom in patients (71%). After the technique had been discussed and consent given by patient and family, cystourethroscopy was performed under general anesthesia to allow the percutaneous suprapubic placement of a Dewan dual-lumen catheter and to confirm the absence of anatomical urethral obstruction. After a resting period of 4e48 h, a cystometrogram/ electromyogram (CMG/EMG) urodynamic evaluation was performed via the Dewan catheter, using perianal patch electrodes. The EMG bladdersphincter dyssynergia pattern was demonstrated to the patient and family. All of the patients were found to have some degree of detrusor overactivity. Multiple sequential bladder fillings were performed in a single day, stressing the need to relax the sphincter during voiding while monitoring the patient s EMG response and measuring PVRs. The main goal of each session was to re-educate the patient to relax the sphincter while voiding. Bladder cycling was repeated as many times as needed, often up to e15 fillings, until the patient was able to completely relax the sphincter during the voiding and empty the bladder to completion. Home monitoring of PVRs by the parents was continued for the following week, via suprapubic tube. The patient then returned for follow-up analysis of the PVR diary. Urodynamic evaluation was repeated to re-assess the voiding pattern. Based on the response, the catheter was removed or the session repeated. Complementing the treatment, a bowel program and anticholinergic therapy were recommended, when appropriate. Short-term (up to 1 year) and long-term (up to 8 years) responses were analyzed, based on the clinical and radiographic outcomes. Assessment include urinary continence, urinary infections, constipation, hydronephrosis, VUR, bladder trabeculation and PVR, monitored both during the first week via suprapubic tube and subsequently with pre-/post-void sonograms at 6e1-month intervals. Improvement was classified based on clinical and radiographic findings. Results All 14 patients were able to relax their external sphincters and reduce their PVR during and shortly after the biofeedback sessions. All patients responded to one or two sessions separated by a week of home monitoring of PVR by the parents via the suprapubic catheter. Improvements in clinical and radiographic outcomes after biofeedback are illustrated in Figs. 1 and. Urinary incontinence, number of episodes of UTI and constipation reduced significantly after treatment. After biofeedback, the average PVR decreased from 10 ml to 1 ml (Fig. 3). If the two patients who failed treatment are excluded, average PVR among good and partial responders was 1 ml. The number of patients with bladder trabeculation, hydronephrosis and VUR also diminished considerably (Fig. 4). Long-term follow up (from to urinary incontinence Clinical Symptoms (number of patients) 4 urinary tract infection before BIOF after BIOF constipation Figure 1 Clinical symptoms before and after treatment with high-intensity, short-term biofeedback therapy (BIOF) in children with severe voiding dysfunction. Fourteen patients were analyzed, 13 with Hinman s syndrome. Of 13 patients who presented severe urinary incontinence before biofeedback, 11 (85%) improved after treatment. Ten patients initially had UTI episodes and six (60%) were cured. Constipation improved in 80%.

4 High-intensity, short-term biofeedback in children Radiographic findings (number of patients) 5 bladder trabeculation 9 90 months, m ¼ 59 months) was achieved in 1 (86%) patients, six males and six females. Two female patients, aged 6.6 and 11.9 years, failed treatment. The elder one has significant developmental delay. She improved during the short-term evaluation, but symptoms returned after years. Both patients had persistent urinary incontinence, UTI, constipation and an average PVR of 150 ml on long-term follow-up. Ten patients (83%) sustained a long-term positive response to biofeedback, with clinical and radiographic 1 hydronephrosis 5 VUR before BIOF after BIOF Figure Radiographic findings before and after treatment with high-intensity, short-term biofeedback therapy (BIOF) in children with severe voiding dysfunction. All patients presented bladder trabeculation before treatment; only five (36%) maintained it after biofeedback. Hydronephrosis improved in 89% and VUR in 60% of patients after treatment. improvement. During a mean period of 59 months, all patients maintained a low PVR (m ¼ 1 ml, compared to m ¼ 10 ml before treatment). Among them was the 6-year-old patient who had renal insufficiency when he started biofeedback. At that time, he had bilateral hydronephrosis and a very trabeculated bladder, with a PVR of 40 ml. After 67 months of follow up he sustained the clinical and radiological improvements, with almost no PVR. In three of the patients who responded long term, some symptoms gradually recurred. The first, a 1-year-old female with hydronephrosis and grade IV VUR before biofeedback, developed recurrent episodes of UTI. She was treated with a ureteral reimplant and is doing well, with a 64-month follow up. Symptoms of daytime and nighttime incontinence reappeared in the second patient, who was 9 years old when treatment started. Although he was able to improve again after biofeedback retraining, he was immature and unable to comply with therapy for a longer period. Therefore, after 30 months of follow up, he was treated with Botox sphincter injections with only moderate results. The third was an 8-year-old female, who presented with bacteriuria and urinary incontinence after 3 years of follow up. The voiding and bowel programs were reinforced during consultations and her continence improved. She had no bladder trabeculation and had a small PVR after 80 months follow up. The other seven responders (58%) maintained a full long-term response average PVR Average Post Void Residual (ml) 10 1 excluding fail before BIOF after BIOF Figure 3 Measurements of PVR in 14 children with severe voiding dysfunction, before and after treatment with high intensity, short-term biofeedback therapy (BIOF). Thirteen were diagnosed with Hinman s syndrome. Mean PVR dropped from 10 ml to 3 ml after biofeedback, including two patients who failed treatment because of maintained PVR of 150 ml. When these two patients were excluded, the average PVR after biofeedback dropped to 1 ml during long-term follow up. Discussion The management of children with Hinman s syndrome is difficult [6]. Hinman divided the management of this condition into four categories: (1) suggestion therapy, including hypnosis; () retraining and bladder drill (timed voiding); (3) drug administration; and (4) biofeedback [7]. Most patients require a combination of these measures. Currently, initial treatment includes antibiotic prophylaxis for infection, intensive bowel management with enemas and/or laxatives followed by long-term use of stool softeners and fiber supplementation, a strict timed and multiple voiding schedule, and anticholinergics to reduce detrusor instability. Pharmacological efforts to relax the striated muscle sphincter (e.g., diazepam) and smooth muscle sphincter (alpha-adrenergic blockade) have met with anecdotal success, as have psychotherapy and hypnotism [8e1]. Biofeedback therapy may be particularly effective in older

348 L.M.C. Monteiro et al. Figure 4 A 9-year-old boy with Hinman s syndrome presented with recurrent pyelonephritis, urinary incontinence and constipation.

5 348 L.M.C. Monteiro et al. Figure 4 A 9-year-old boy with Hinman s syndrome presented with recurrent pyelonephritis, urinary incontinence and constipation. (A) IVP (left) shows bilateral hydroureteronephrosis and a distended bladder. VCUG demonstrates right Grade IV VUR (right top) and a trabeculated bladder (right middle). The voiding image (right bottom) shows a dilated proximal urethra. Cystoscopy did not reveal a PUV. (B) Tc-99m DMSA renal scan demonstrates right upper pole renal scarring best seen in right posterior oblique view (arrow). (C) Renal bladder sonogram after biofeedback therapy shows complete resolution of bilateral hydroureteronephrosis. The pre- and post-void images of the bladder reveal excellent emptying with a minimal PVR. individuals when they can be made to appreciate the necessity and technique for relaxation of the voluntary pelvic floor musculature during the bladder contraction [13e15]. Although our approach of short-term, highintensity biofeedback to treat this select group of difficult patients involves a more invasive technique requiring general anesthesia for initial placement of the Dewan percutaneous suprapubic dual-lumen urodynamic catheter, it offers several advantages. These include the ability to perform urodynamic evaluation in sensate children via the suprapubic catheter without the need for urethral catheterization, which in some children will produce artifactual contraction of the external urethral sphincter during the voiding phase of the

6 High-intensity, short-term biofeedback in children 349 Figure 4 (continued) study. This approach also affords the opportunity for multiple sequential treatment cycles during 1 day, allowing for a more rapid, condensed period of treatment and a shorter learning curve for the patient and family. Rather than taking several weeks and multiple follow-up biofeedback sessions, this approach usually only required one full day of biofeedback therapy to achieve successful results. Furthermore, suprapubic measurements of PVR during the follow-up week are more reliable and provide for ongoing feedback at home, making the patient and family more confident of the results. It is important to stress that these patients still need long-term follow up to reinforce the therapy, to insure compliance with ancillary measures including timed voiding and a bowel regimen, and to monitor for the early recurrence of symptoms, infections, and/or elevated PVRs. Conclusion Short term, high intensity biofeedback achieves a rapid and durable response in the majority of children with Hinman s syndrome. Long-term follow up is needed to assure compliance and long-term success. References [1] Yeates WK. Bladder function in normal micturition. In: Kolvin I, MacKeith RC, Meadow SR, editors. Bladder control and enuresis. London: Heinemann; p. 8. [] Stein ZA, Susser MW. Social factors in the development of sphincter control. Dev Med Child Neurol 1967;9:69. [3] Homsy YL, Austin PA. Dysfunctional voiding disorders and nocturnal enuresis. In: Belman AB, King LR, Kramer SA, editors. Clinical pediatric urology. 4th ed. London: Martin Dunitz; 00. p. 345e69. [4] Hinman Jr F, Baumann FW. Vesical and ureteral damage from voiding dysfunction in boys without neurologic or obstructive disease. Trans Am Assoc Genitourin Surg 197;64: 116e1. [5] Perez LM, Rushton HG. A pragmatic approach to the evaluation and management of non-neuropathic daytime voiding disorders, Hinman s syndrome. In: Gearhart JP, Rink RC, Mouriquand PDE, editors. Pediatric urology. Philadelphia: WB Saunders; 001. p. 488e9. [6] Philips E, Uehling DT. Hinman s syndrome: a vicious cycle. Urology 1993;4:317e9. [7] Hinman Jr F. Non-neurogenic neurogenic bladder (the Hinman s Syndrome) e 15 years later. J Urol 1986;136:769e77.

7 350 L.M.C. Monteiro et al. [8] Hanna MK, Di Scipio W, Suh KK, Kogan SJ, Levitt SB, Donner K. Urodynamics in children II. The pseudoneurogenic bladder. J Urol 1981;15:534e7. [9] Hinman Jr F, Baumann FW. Vesical and ureteral damage from voiding dysfunction in boys without neurologic or obstructive disease. J Urol 1973;9:77e3. [] Smey P, King LR, Firlit CF. Dysfunctional voiding in children secondary to internal sphincter dyssynergia: treatment with phenoxybenzamine. Urol Clin North Am 1980;7: 337e47. [11] Austin PF, Homsy YV, Masel JL, Cain MP, Casale AJ, Rink RC. Alpha-adrenergic blockade in children with neuropathic and nonneuropathic voiding dysfunction. J Urol 1999;16:64e7. [1] Bogaert G, Beckers G, Lombaerts R. The use and rationale of selective alpha blockade in children with non-neurogenic neurogenic bladder dysfunction. Int Braz J Urol 004;30:18e34. [13] McKenna PH, Herndon CDA, Connery S, Ferrer FA. Pelvic floor muscle retraining for pediatric voiding dysfunction using interactive computer games. J Urol 1999;16: 56e63. [14] Combs AJ, Glassberg AD, Gerdes D, Horowitz M. Biofeedback therapy for children with dysfunctional voiding. Urology 1998;5:31e5. [15] McGuire EJ, Savasano JA. Urodynamic studies in enuresis and the non-neurogenic neurogenic bladder. J Urol 1984; 13:99e30.

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