Melorheostosis: A Retrospective Clinical Analysis of 24 Patients at the Mayo Clinic.

BACKGROUND: Current understanding of the clinical features of persons with melorheostosis is restricted primarily to individual case reports and small case series. OBJECTIVE: To assess the clinical features of patients with melorheostosis treated at our institution from 1972 through 2010. DESIGN: Chart review. SETTING: Tertiary academic medical center. PARTICIPANTS: Twenty-three patients with "definite" and one patient with "probable" melorheostosis based on radiographic criteria. METHODS: The eligible study cohort was identified through the Rochester Medical Index database. Further diagnostic confirmation of patients with melorheostosis was performed by radiographic review. MAIN OUTCOME MEASUREMENTS: We evaluated age at first visit to our institution, gender, affected body area, number of bones affected, presenting symptoms, surgical evaluation, and therapies provided. RESULTS: The average age at first evaluation at our clinic was 36.5 years (median 41.5 years, range 3-68 years). The female to male ratio was 4:1. The lower extremity was most commonly affected (66.6%), followed by upper extremity (33.3%), spine (16.6%), and head (8.3%). One-third of patients had involvement of a single bone; two-thirds had multiple bone involvement. Pain was the most common presenting concern (83.3%), followed by deformity (54.1%), limitation of movement (45.8%), numbness (37.5%), and weakness (25.0%). Most patients had a physician evaluation (87.5%); patients also underwent orthopedic surgery (45.8%), physical therapy (33.3%), and occupational therapy (12.5%). CONCLUSIONS: Melorheostosis is a rare sclerotic bone disease resulting in pain, deformity, and dysfunction. An interdisciplinary approach to care should include nonoperative and operative evaluation, as well as appropriate therapies. A prospective approach to evaluation, including imaging and physical examinations, would provide valuable longitudinal data. LEVEL OF EVIDENCE: IV.

CT and MRI assessment of the aortic root and ascending aorta.

OBJECTIVE: We provide an outline of aortic anatomy and disease patterns, describe methods of aortic measurement, and highlight measurement thresholds for surgical intervention. We also discuss findings that can assist in preoperative planning and in detecting underlying connective tissue disorders. CONCLUSION: A standardized approach to the measurement of the aorta is needed and features suggestive of an underlying connective tissue disorder should be recognized. Radiologists should be aware of the image limitations and clinical implications of reported measurements.

In vitro thermal profile suitability assessment of acids and bases for thermochemical ablation: underlying principles.

PURPOSE: To measure and compare temperature changes in a recently developed gel phantom for thermochemical ablation as a function of reagent strength and concentration with several acids and bases. MATERIALS AND METHODS: Aliquots (0.5-1 mL) of hydrochloric acid or acetic acid and sodium hydroxide or aqueous ammonia were injected for 5 seconds into a hydrophobic gel phantom. Stepwise increments in concentration were used to survey the temperature changes caused by these reactions. Injections were performed in triplicate, measured with a thermocouple probe, and plotted as functions of concentration and time. RESULTS: Maximum temperatures were reached almost immediately in all cases, reaching 75 degrees C-110 degrees C at the higher concentrations. The highest temperatures were seen with hydrochloric acid and either base. More concentrated solutions of sodium hydroxide tended to mix incompletely, such that experiments at 9 M and higher were difficult to perform consistently. CONCLUSIONS: Higher concentrations for any reagent resulted in higher temperatures. Stronger acid and base combinations resulted in higher temperatures versus weak acid and base combinations at the same concentration. Maximum temperatures obtained are in a range known to cause tissue coagulation, and all combinations tested therefore appeared suitable for further investigation in thermochemical ablation. Because of the loss of the reaction chamber shape at higher concentrations of stronger agents, the phantom does not allow complete characterization under these circumstances. Adequate mixing of reagents to maximize heating potential and avoid systemic exposure to unreacted acid and base must be addressed if the method is to be safely employed in tissues. In addition, understanding factors that control lesion shape in a more realistic tissue model will be critical.