We observed a patient with 48 XXYY Klinefelter syndrome who visited our hospital because of a short penis as chief complaint. The patient was a 21-year-old, tall and obese man. He had gynecomastia. The penis was short and bilateral testes were underdeveloped. Endocrinologically the LH and FSH showed high level and the testosterone was low. A diagnosis of very rare 48 XXYY Klinefelter was made based of the chromosomal analysis.

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We studied 20 cases of neurogenic bladder with vesico-ureteral reflux retrospectively. Seven patients voided with Valsalva's maneuver and 12 patients were managed with clean intermittent catheterization, but one patient required permanent urinary diversion because of uncontrollable urinary tract infection. The patients were followed by intravenous pyelography, radioisotope renogram, voiding cystourethrography, determination of serum creatinine level and urodynamic study. Anti-reflux surgery was performed in 22 ureters and reflux resolved in 19 ureters. Among 12 ureters not treated by anti-reflux surgery, reflux disappeared in 5 ureters and improved in 2 ureters. Of 5 ureters, reflux resolved after clean intermittent catheterization in 2 ureters, and remained stable without recurrent infection or renal deterioration in the remaining 5 ureters. Regardless whether antireflux surgery was done or not, most of the patients who had high grade reflux and obstructive renal damage on radioisotope examinations had marked low compliance bladders. Our experience suggested the necessity of a suitable treatment to improve bladder compliance before considering anti-reflux surgery.

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We experienced transurethral teflon paste injection for 12 refluxing ureters of 7 patients with neurogenic bladder dysfunction. Preoperative assessment of cystometry showed hypoactive bladder function with normal bladder compliance in 4 patients, and low compliance bladder (< 10 ml/cmH2O) in 1. Voiding cystography revealed grade 1 reflux in 2 ureters, grade 2 in 3, grade 3 in 2, grade 4 in 2, and grade 5 in 2. One ureter did not show reflux. Zero point two to 1.6 ml of teflon paste was injected on each ureter under cystoscopic observation. These patients were followed for a mean of 25.1 months. Reflux disappeared immediately after the first operations in all patients, however recurrence was observed in 2 ureters, in which improvement of reflux (grade 5 to 2) was achieved in 1 ureter but no improvement (grade 2 to 2) in another. Pyelonephritis was not encountered in any patients after injection. No complication was observed through the follow up period. In conclusion, we advocate that endoscopic teflon paste injection is a useful alternative to ureteroneocystostomy in the treatment of reflux in patients with neurogenic bladder dysfunction.