Hinman syndrome: Long term follow up of 14 cases

Purpose: Hinman syndrome is the most severe form of nonneurogenic neurogenic bladder causing damage of the upper urinary tract. Fourteen patients with Hinman syndrome followed at our institution were evaluated for their clinical characteristics and prognosis. Here we report the findings of this series of patients for this poorly understood syndrome. Materials and Methods: The medical records of 14 patients, 8 boys and 6 girls, diagnosed with Hinman syndrome from March 1993 to June 2006 were reviewed. The mean duration of follow up was 69 months. The ultrasonography, 99^m Tc-dimercaptosuccinic acid renal scan (DMSA), voiding cystourethrogram (VCUG), and urodynamic study (UDS) results were retrospectively analyzed and efficacy of each treatment method was evaluated based on the medical records on follow up. Results: Hydronephrosis of grade III or greater on ultrasonography and renal scarring of both kidneys on the DMSA renal scan were observed in 12 and 14 patients, respectively. Severe bladder trabeculation and high grade VUR (IV, V) were observed on the VCUG in 14 and 8 patients, respectively. Decreased bladder compliance on the UDS was noted in 13 and detrussor-sphincter dyssynergia (DSD) was observed in eight. Medical treatment was not effective in all 14 cases and six patients who underwent botulinum injection of the bladder were unresponsive to that treatment as well. In spite of conservative treatments such as clean intermittent catheterization (CIC), seven patients eventually underwent bladder augmentations after a mean period of thirty-seven months from diagnosis because of concern about the loss of bladder capacity and renal function. One patient who did not perform CIC progressed to end-stage renal disease and had to be transferred to pediatric nephrology for dialysis. Conclusions: Patients diagnosed with the Hinman syndrome were treated similar to patients with neurogenic bladder. However, from the longterm follow up data at our outpatient clinic, many patients eventually had bladder augmentation to prevent further loss of bladder capacity and renal function. Therefore, urologists must not hesitate in performing such treatment when necessary.