Twelve-year experience with Hinman-Allen syndrome at a single center.

ABSTRACT

OBJECTIVE: To report the long-term follow-up results of patients with Hinman-Allen syndrome (HAS) at our institution. METHODS: The data from 22 children with HAS were retrospectively analyzed. The patients were followed up every 3-6 months with serial physical examinations, voiding charts, urine culture, postvoid residual urine volume determination, serum creatinine measurement, and urinary imaging. The follow-up time was calculated from the day of the first visit to the day of the latest dimercaptosuccinic acid scan. Urotherapy, pharmacotherapy, clean intermittent catheterization, biofeedback therapy, and surgery were performed sequentially and/or combined, depending on the disease course. Renal deterioration was defined as any presence of a new scar or cortical thinning compared with the findings from the first dimercaptosuccinic acid scan. Upper urinary tract deterioration was defined as the persistence or progression of hydronephrosis on ultrasonography. RESULTS: The mean age at referral was 9.18 ± 3.36 years (range 2-14), and the mean follow-up period was 80.90 ± 19.57 months (range 54-144). Conservative therapy resulted in improvement of the bladder function in 14 patients; however, 8 patients required surgery owing to failure of this approach. Asymptomatic bacteriuria developed in one half of the children (n = 11, 50%), and in 6 (22.7%), ≥1 febrile urinary tract infection developed. None of the patients had upper urinary tract deterioration; however, renal deterioration developed in 3 patients (13.6%). The mean creatinine levels had remained stable at the end of the follow-up. CONCLUSION: Close follow-up at a single institution and proactive treatment resulted in successful stabilization of HAS in most of our children with HAS.