Tuberous sclerosis complex renal disease.

Although not as common as other genetic renal diseases such as autosomal dominant polycystic kidney disease, patients with tuberous sclerosis complex frequently have significant renal involvement. Recent revelations in the cell biology of these renal disease manifestations as well as effective therapies for tuberous sclerosis complex-related renal issues have heralded hope of improved renal survival and improved quality of life for the TSC patient. This review specifically addresses some of the major renal manifestations of this disease.

Resolution of ephedrine stones with dissolution therapy.

A patient with a history of ingesting large quantities of an over-the-counter stimulant developed renal calculi that on further analysis, after stone passage, revealed increased amounts of ephedrine. Over the course of 7 months, all of the patient's ephedrine stones were managed successfully by alkalinization. Similar to previously reported ephedrine calculi, these stones were radiolucent on x-ray imaging, but their course was monitored on serial nonenhanced computed tomography scans. We believe this to be the first reported use of alkaline therapy for the dissolution of renal stones containing ephedrine.

Antegrade periurethral administration of Durasphere for management of a vesicoperineal fistula.

A patient who had developed a persistent fistula between the urethra and bladder neck after its surgical closure in the construction of a continent urinary pouch and Mitrofanoff nipple was successfully treated by antegrade periurethral injection of a newly approved injectable bulking agent for stress urinary incontinence (Durasphere) to occlude the bladder neck. We believe this to be the first reported use of Durasphere for such treatment.

Total endoscopic management of the encrusted ureteral stent under a single anaesthesia.

OBJECTIVE: Stented patients lost to follow up may return with large stone burdens encrusting the stent. The study describes total endoscopic management under one anaesthesia to remove such stents. METHODS: A similar approach was done in all cases. Cystoscopic Holmium: YAG (Ho) or electrohydraulic lithotripsy (EHL) removed the bladder calculus. The bladder portion of stent was cut with endoscopic scissors and removed. The patient was repositioned prone for percutaneous access and nephrolithotomy (PCNY), using either ultrasound, EHL, or Ho. The remaining cephalic portion of stent was removed percutaneously. A universal stent was placed and removed one week later. RESULTS: Eleven patients with 12 ureteral stents left indwelling over one year presented with significant stone burden both in the bladder and kidney. Seven patients had failed extracorporeal shock wave lithotripsy (ESWL) prior to referral. One patient with biolateral encrusted stents had both stents treated under one anaesthesia. The average bladder and kidney stone burdens measured 4 and 6 cm, respectively. The median anaesthesia time was 3.5 hours. No patient required transfusion. Ten of eleven patients were rendered stone-free in one procedure. One patient with a complete staghorn calculus was rendered stone-free by post-PCNL ESWL for a residual calyceal stone. CONCLUSIONS: Total endoscopic management of the encrusted ureteral stent accomplishes safe, effective stone and stent treatment under one anaesthetic. Urologists may wish to consider this strategy when ESWL is unlikely to eradicate large stone burdens on encrusted stents.