Management of cystinuric patients: an observational, retrospective, single-centre analysis.

OBJECTIVE

A critical appraisal of the management of patients with cystine stones treated in our unit in the past 6 years and to analyze the outcome of multimodality therapies.

STUDY DESIGN

An observational, single-centre retrospective study.

METHODS

We reviewed the records of all patients with stones referred to our centre over a 6-year period from 1998 to 2005. Data recorded included demographic details, medical therapies received/prescribed, compliance with medical therapies, mode of treatment, stone clearance and any recurrence during this period of study.

RESULTS

A total of 30 cystinuric patients were treated in our institution over the period of 6 years from 1998 to early 2005. Of these 16 were males and 14 females with an average age at last follow-up of 39 years (range 15-70). Two patients were successfully managed medically. The remaining patients (n = 28) underwent a total of 237 procedures (pre- and postreferral to our unit), with an average of 7.9 procedures per patient for 126 stone episodes (4.2 episodes/patient). The modes of treatment included extracorporeal shockwave lithotripsy (n = 143), ureterorenoscopy and intracorporeal lithotripsy (n = 50), percutaneous nephrolithotomy (n = 28) and open procedures (n = 16). Two patients needed open surgery at our unit. Prior to referral to our dedicated unit, patients had received treatment with extracorporeal shockwave lithotripsy (multiple sessions), ureteroscopy (n = 14), percutaneous nephrolithotomy (n = 4) and open stone removal (n = 14). Most of the stones at our unit were managed using minimally invasive therapies.

CONCLUSION

Compliance of cystinuric patients with medical treatment is often poor and patients experience recurrent stone episodes requiring multiple interventions. Modern management of cystine calculi should be with staged minimally invasive procedures to avoid the complications of multiple open procedures wherever possible along with appropriate medical prophylaxis.