Impaired primary hemostasis with normal platelet function in Duchenne muscular dystrophy during highly-invasive spinal surgery.

A defective, normal or enhanced hemostasis has been reported in Duchenne muscular dystrophy (DMD). A retrospective analysis of intra-and postoperative (up to 36 h) estimated blood losses was performed in 156 patients undergoing spinal surgery for: DMD (n = 31), idiopathic scoliosis (IS) (n = 70), poliomyelitis (n = 10), cerebral palsy (CP) (n = 28), spinal muscular atrophy (SMA) (n = 17). Platelet aggregation and bleeding times were also investigated in DMD patients. Immunohistochemistry for dystrophin was performed in platelets, megakaryocytes and blood vessels of normal tissues. DMD patients showed significantly higher intraoperative estimated blood losses (DMD: 3495+/-890 ml; IS: 2269+/-804 ml; poliomyelitis: 2582+/-1252 ml; CP: 2071+/-683 ml; SMA: 2464+/-806 ml; P < 0.05), while postoperative blood losses were similar among different groups. Higher estimated blood losses in DMD were independent of the duration of surgery, body weight, gender, age, vertebral levels or preoperative Cobb angle. DMD children had significantly prolonged bleeding times, but retained normal platelet function. From control samples dystrophin was expressed in vascular smooth muscle cells, but not in platelets. DMD appears to be characterized by immediate bleeding during highly-invasive surgery and increased bleeding time without platelet abnormalities. Considering dystrophin expression in normal vascular smooth muscle cells, these results altogether suggest a selective defect of primary hemostasis in DMD, likely to be due to impaired vessel reactivity.

Aneurysmal bone cysts: treatment with direct percutaneous Ethibloc injection: long-term results.

PURPOSE: To assess the efficacy and long-term results of Ethibloc treatment in aneurysmal bone cysts (ABC). METHODS: Thirteen patients with ABC were treated with direct percutaneous Ethibloc injection. Four patients had only one injection and the other nine patients from two to four injections. No severe complications were observed; in two patients a local leakage of Ethibloc from the cyst into the soft tissues occurred but it was temporary and the consequent inflammation self-healed without residua and sequelae. Imaging follow-up lasted from 6 to 67 months and included conventional radiology (CR) and magnetic resonance imaging (MRI), both used in the presurgical phase. RESULTS AND CONCLUSIONS: All images demonstrated a remarkable shrinkage of the cystic lesion and bone cortex thickening. In all patients, circumscribed areas of lucency persisted at radiography, corresponding to residual cystic areas without fluid-fluid levels at MRI. Pain, which was present in all the patients before treatment, was relieved within a month. According to our experience, direct percutaneous Ethibloc injection is effective in the treatment of ABC and can be recommended as the first-choice treatment. Due to its higher sensitivity MRI must be included either in the pretreatment phase to study the multilocular structure or in the imaging follow-up to evaluate the efficacy of Ethibloc in persistently non-responsive areas.