Outcome and development of symptoms after orthotopic liver transplantation for Wilson disease.

BACKGROUND: Wilson disease (WD) is an autosomal recessive copper storage disease resulting in hepatic and neurologic dysfunction. Liver transplantation is an effective treatment for fulminant cases for patients with chronic liver disease. Reports on the outcome of neuropsychiatric symptoms after orthotopic liver transplantation (OLT) are limited. AIM: To assess the course of neuropsychiatric and hepatic symptoms after liver transplantation for Wilson disease METHODS: Nineteen patients with Wilson disease received liver transplantation and were followed prospectively from 2005 to 2010 for the development of hepatic, neurological and psychiatric symptoms. RESULTS: Eight patients (all female) were transplanted for acute liver failure and eleven patients for chronic liver failure. Patient survival rates one and five yr after transplantation were 78% and 65%, respectively. Of the surviving patients, hepatic symptom scores improved in all patients and neurological symptom scores improved in all but one patient after OLT compared to the time of initial diagnosis and compared to pre-OLT status. Psychiatric symptoms showed moderate improvements. CONCLUSION: Survival after OLT for Wilson disease with end-stage liver disease is excellent. Overall, neuropsychiatric symptoms improved after transplantation, substantiating arguments for widening of the indication for liver transplantation in symptomatic neurologic Wilson disease patients with stable liver function.

Quantitative volumetry of cement leakage in viscosity-controlled vertebroplasty.

STUDY DESIGN: This study was designed as a cohort study comparing a prospective sample to a historic control group. OBJECTIVE: The aim of the actual trial was to compare the rate of cement leakage by quantitative volumetry comparing viscosity-controlled and non-viscosity-controlled vertebroplasty. SUMMARY OF BACKGROUND DATA: Percutaneous vertebroplasty (PVP) is a widespread safe and effective technique in the treatment of osteoporotic compression fractures and vertebral metastatic lesions. However, cement leakage has been identified as a problem of this technique. The leakage rates are reported to range from 7% to 90%. The main influence factor for leakage has been demonstrated to be cement viscosity. Assessment of appropriate injection viscosity is highly subjective and observer dependent. Viscosity-controlled vertebroplasty (Vertecem system) has been developed to objectively measure cement viscosity before injection. It introduces a viscosimeter to measure the actual cement viscosity before injection into the vertebra, and therefore may prevent leakages resulting from low-viscosity cement injections. Despite more than 800 Pubmed citations on PVP, there is only 1 report on distinct measurement of cement leakage by semiquantitative volumetry. METHODS: A total of 111 vertebrae in 68 patients, in which PVP was performed for osteoporotic fractures, were included. Thirty-seven patients (76 operated vertebrae) were assessed prospectively using the viscosity-controlled vertebroplasty. The results were compared with a retrospective group of 31 patients (35 operated vertebrae) undergoing PVP without using a viscosimeter. RESULTS: : There were no significant differences between the 2 groups in the applied volume of cement per fractured vertebra (P=0.73). The frequency of cement leakage in viscosimete-assisted vertebroplasty was 42.1% and 58.3% in the historic group. Cement leakage into the basivertebral vein (type B), was detected in 6.6% with and in 11.1% without viscosimetry. CONCLUSIONS: The use of viscosity-controlled vertebroplasty led to a decrease in the leakage rate from 58.3% to 42.1%. Leakage into the basivertebral vein with the risk of compression of nerval structures was reduced to almost 50% when viscosimetry was performed. It revealed to be a helpful tool for more unexperienced surgeons to assess the appropriate viscosity for vertebroplasty.