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Objective To determine the long-term outcome of surgically treated Chiari-related syringomyelia. Methods The medical charts of 157 consecutive surgically treated patients with Chiari-related syringomyelia were retrospectively analyzed. Factors predicting outcome, either clinical or radiological, are discussed, and our results are compared with those of other large series in the literature. Results The study included 74 men and 83 women (age range, 16-75 years; mean age at surgery, 38.3 years). Pain and sensory disturbance were the most frequent initial symptoms. The average duration of preoperative symptoms was 8.2 years. The follow-up period ranged from 82 to 204 months (median, 88 months). At the end of the study, 99 patients (63.06%) had improved, 48 (30.58%) had stabilized, 9 (5.73%) had worsened, and 1 (0.63%) had died during the postoperative period. Factors predicting improvement or stabilization were young age at the time of surgery and clinical signs of paroxysmal intracranial hypertension. Factors associated with a poor outcome were older age at the time of surgery, arachnoiditis, and a clinical feature of long-tract impairment syndrome. The presence of arachnoiditis or of basilar invagination was associated with poor clinical presentation (P = 0.05 and 0.0001, respectively). The extent of the cyst on postoperative magnetic resonance imaging was a predictor of poor clinical outcome (P = 0.002). Conclusion Our results confirmed that surgery is an effective and safe treatment of Chiari-related syringomyelia, with a 90% chance of long-term stabilization or improvement on average. Surgery should be proposed as soon as possible in patients with clearly progressing clinical features.

Background Supratentorial hemangioblastomas are rare lesions, occurring either sporadically or in von Hippel-Lindau disease. Objective Following recent advances in our understanding of the natural history of von Hippel-Lindau-associated cerebellar and spinal hemangioblastomas, we conducted a study of the natural history of supratentorial hemangioblastomas in von Hippel-Lindau disease. Methods We reviewed a series of 18 supratentorial hemangioblastomas in 13 patients with von Hippel-Lindau disease. Clinical, genetic, and serial imaging data and operative records were analyzed. Results Hemangioblastomas were most commonly seen in the temporal lobe. Only 6 tumors had a cyst at diagnosis or during follow-up, and only 6 patients had associated symptoms at presentation or during follow-up. The most frequent clinical presentations were intracranial hypertension and visual loss. Of 14 tumors with documented serial imaging, 13 demonstrated tumor growth. Rates and patterns of tumor growth were unique to each patient. The mechanism of cyst formation described in other locations was also demonstrated in the supratentorial region. Patterns of peritumoral edema and rate of cyst formation seemed to be influenced by the presence of anatomic barriers. Germline VHL mutation was identified in all patients, but no specific genotype-phenotype correlation was found, although a familial predisposition is suggested. Conclusion This series illustrates the wide variation in tumor locations, patterns of growth, and edema progression seen in supratentorial hemangioblastomas and adds to our knowledge of the natural history of hemangioblastomas.

Objective Surgery should be considered for patients with intramedullary spinal ependymomas (ISE), particularly those presenting with a neurological deficit preoperatively. In contrast, it is still a debatable matter whether to recommend the same approach for patients with no neurological impairment. To investigate this matter, we analyzed the data of 82 consecutive patients with ISEs treated at our institution. Methods We reviewed the medical charts of all ISE patients undergoing operation at our institution between 1985 and 2000. Particular attention was given to patients without neurological deficit before surgery. Results Eighty-two consecutive ISE patients were included in this study. Preoperatively, a neurological deficit of variable severity was present in 72 patients (Group A, 88%) and absent in 10 patients (Group B, 12%). In the latter group, seven patients had progressive and nonspecific pain as the only presenting symptom; two had arm dysesthesias; and in one patient, ISE was diagnosed incidentally. The mean duration of their symptoms was 21 months. We achieved a total tumor excision in nine patients and subtotal removal in one. At the last follow-up assessment (mean, 45 mo), all Group B patients remained at Grade I of the McCormick classification, except one, who deteriorated to Grade Ib. Furthermore, nonspecific pain diminished in three patients, stabilized in four, and worsened in one. Arm dysesthesias diminished in one patient and stabilized in the other. No surgery-related complication or recurrence was recorded in these patients. Conclusion Surgery should be carefully considered for ISE patients with no objective neurological deficit preoperatively because, in our experience, it resolves their preoperative complaints in 30% of cases, stabilizes them in 60%, and worsens them in 10%.

OBJECTIVE:To assess the effects of Hyaloglide gel (or auto-cross-linked polysaccharide [ACP] gel; Fidia Advanced Biopolymers, Abano Terme, Italy), a hyaluronan-derivative polymer, on peripheral nerve scarring and nerve regeneration.METHODS:We performed two surgical procedures in adult rats: 1) neurolysis of the sciatic nerve and separation of its tibial and peroneal branches, and 2) transection and immediate suture of the sciatic nerve. After nerve manipulation, ACP gel was applied onto the site of operation. We tested two solutions of ACP gel having different viscosities. Additional animals received Adcon-T/N (Gliatech, Inc., Cleveland, OH), an antiadhesive agent currently available for clinical use. No gel was applied on the contralateral side, which served as a control side. Four weeks later, the animals underwent reoperation. We assessed the quality of wound healing, the presence of perineural adherences, and the separability of nerves from surrounding tissues.RESULTS:Significantly fewer perineural adhesions were found in animals treated with ACP gel (high viscosity) and Adcon-T/N compared with controls. Quantitative histological analysis revealed a statistically significant reduction in the amount of scar tissue surrounding the nerves treated with ACP gel. No evidence of toxicity was found, and the gel did not interfere with nerve regeneration (counts of regenerating myelinated axons).CONCLUSION:ACP gel with high viscosity seems to be safe and effective in reducing perineural adhesions and scar formation after peripheral nerve surgery.