Surgical and functional results of spine fusion in spinal muscular atrophy.

From 1965 to 1987, 84 spinal muscular atrophy patients were followed at Rancho Los Amigos Medical Center (RLAMC). Twenty-seven patients were excluded from this study due to insufficient medical documentation (16), lack of follow-up (5), and death (6); leaving 57 patients in the general study group. Group I (34 patients) had posterior spinal fusion (PSF) with Harrington rod instrumentation (HRI); mean age at surgery was 12 years, average preoperative curve was 57 degrees, average postoperative correction was 42%, with a loss of correction of 9 degrees. The complication rate in this group was 35%. The average follow-up interval was 9 years (range, 4-19 years). Group II (six patients) underwent PSF with Luque segmental spinal instrumentation (SSI); mean age at surgery was 11 years, preoperative curves averaged 37 degrees, average postoperative correction was 42% with a loss of correction of 3 degrees. The complication rate in this group was 16%. Follow-up was 3.5 years. Physical therapy and occupational therapy evaluations were done preoperatively and postoperatively at 2- and 5-year intervals. Information was gathered in three categories: 1) ambulation, 2) equipment use, and 3) functional activities. After fusion, sitting tolerance was maintained but additional use of mobile arm supports, lapboards, and reaching aides was necessary for all patients. The ability to perform activities such as drinking, self-feeding, and self-hygiene declined during the 2 years immediately following surgery but improved by 5 years. Surgical patients never approached their preoperative skill levels. Therapy evaluations further demonstrated that there were no difference in function between either operative group.

Hepatocellular carcinoma presenting as a solitary metastasis to the scapula. Case report and review of the literature.

Although primary hepatocellular carcinoma is uncommon, metastasis to the upper extremity as a presenting symptom is even more rare. Recent case reports and autopsy surveys document that extrahepatic spread of this carcinoma occurs in 30% to 78% of patients, who usually are without regional symptoms involving bone. Although metastatic spread to the lungs and lymph nodes occurs more commonly, the incidence of bone metastases has increased according to previous reports and is now estimated from 2% to 13%. This case report concerns widely disseminated hepatocellular carcinoma presenting initially without any other systemic signs except for shoulder pain and induration. Due to the aggressive nature of this tumor, early detection is crucial. Early diagnosis may offer the only real hope for establishing effective treatment and improving the chances for long-term survival.