Nozar Aghakhani

Intramedullary spinal ependymomas: analysis of a consecutive series of 82 adult cases with particular attention to patients with no preoperative neurological deficit.

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%.

Long-term follow-up of Chiari-related syringomyelia in adults: analysis of 157 surgically treated cases.

OBJECTIVETo determine the long-term outcome of surgically treated Chiari-related syringomyelia. METHODSThe 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. RESULTSThe 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). CONCLUSIONOur 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.

Curable Cause of Paraplegia. Spinal Dural Arteriovenous Fistulae

Background and Purpose— The rarity of spinal dural arteriovenous fistulae makes physicians often overlook this potential diagnosis in patients with progressive gait disturbance and paraparesis. Consequently, patients with spinal dural arteriovenous fistulae can gradually become completely paraplegic if the final diagnosis is delayed considerably. The objective of the current study is to demonstrate that, particularly in patients with paraplegia, surgical treatment of fistula is necessary and often has a favorable outcome. Methods— Of 42 patients with spinal dural arteriovenous fistulae treated in our institution (surgery or endovascular treatment), 6 were paraplegic preoperatively (Grade IV on the McCormick scale and Grade V on the Aminoff scale, Grade 5 of modified Rankin Scale with motor ASIA between 0 and 10 for both lower limbs). Their clinical history revealed that paraplegia appeared progressively within a period of <3 months. All patients were clinically evaluated at 6 weeks, 6 months, and then annually during an average follow-up of 3 years. Patients received at least one spinal angiography and MRI test during the follow-up period. Results— Total exclusion of the fistula was performed surgically in all cases and was confirmed by spinal angiography. No surgical complications were recorded. All patients improved postoperatively. Three patients showed almost normal walking (Grade I on the McCormick scale, I on the Aminoff scale, Grade 1 of modified Rankin Scale) and 3 were able to walk with a cane (Grade II on McCormick, Grade III on Aminoff scale, Grade 2 of modified Rankin Scale). MRI tests were normal in all patients. Conclusions— Our results indicate that treatment of fistula is a necessary intervention, even in patients with complete paraplegia.

Surgical management of unruptured intracranial aneurysms that are inappropriate for endovascular treatment: experience based on two academic centers.

OBJECTIVETo analyze the results of the surgical management of unruptured intracranial aneurysms (UIA) when coil embolization (CE) was considered first but deemed inappropriate by our multidisciplinary groups. METHODIn two institutions, all UIAs recommended for treatment were considered first for a CE procedure if accessibility, neck width, and fundus-to-neck ratio were appropriate. Patients with UIAs considered inappropriate for CE were to undergo a surgical clipping procedure. We reviewed the medical records of all patients who underwent surgical clipping between February 1996 and February 2006. RESULTSA total of 325 patients with 440 UIAs were treated. Of them, 149 patients were selected by our multidisciplinary staff for treatment by CE, and 176 patients with 238 UIAs were treated by 207 surgical procedures. Angiographic studies revealed complete occlusion in 95% and near total occlusion in 2.5% of surgically treated UIAs. No deaths related to surgery occurred. Sixteen patients (9.1%) experienced postoperative complications, four of which persisted 1 year after surgery (two cases of diplopia and two aphasic disorders). The 1-year morbidity rate was 2.2% (four of 176) by patient and 1.7% (four of 238) by aneurysm. For UIAs smaller than 10 mm in patients younger than 65 years old, the morbidity rate was 0.56%. CONCLUSIONOur results gathered from two centers with the same management of UIAs show that SC remains a safe and effective treatment for UIAs even when CE is considered first.

Surgical treatment of posttraumatic syringomyelia.

OBJECTIVEThe present study evaluates the effectiveness of 2 surgical procedures, shunting and untethering, for posttraumatic syringomyelia. METHODSWe retrospectively reviewed the medical charts of all surgical patients with posttraumatic syringomyelia in our department. Shunting was performed before 1997; after 1997, we used arachnoidolysis and untethering. RESULTSShunting was performed in 15 patients, and 19 patients underwent arachnoidolysis. Statistical analysis found that the 2 groups did not differ in age or initial clinical or radiological presentation. All patients suffered from progressively worsening symptoms. Reconstruction of the subarachnoid space by arachnoidolysis and untethering the cord allowed us to improve or stabilize 94% of our patients. Shunting exposed the patients to a higher rate of clinical recurrence and reoperation. Comparisons between the 2 groups found a significant difference (better results) in favor of arachnoidolysis for the McCormick classification (P = .03), American Spinal Injury Association motor score of the lower extremities (P = .02), and subjective grading (P = .001). There was no significant difference in the evolution of pain or the Vaquero index between the 2 groups; however, a tendency appeared in favor of arachnoidolysis for cyst evolution in regard to the extent of the cyst and the Vaquero index (P = .05). CONCLUSIONOur results confirmed that arachnoidolysis is an effective and safe treatment for posttraumatic syringomyelia. Because the majority of patients were stabilized, we concluded that surgery should be performed as soon as possible in patients with clearly progressing clinical features.

Natural history of supratentorial hemangioblastomas in von Hippel-Lindau disease.

BACKGROUNDSupratentorial hemangioblastomas are rare lesions, occurring either sporadically or in von Hippel-Lindau disease. OBJECTIVEFollowing 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. METHODSWe 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. RESULTSHemangioblastomas 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. CONCLUSIONThis 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.

Decompressive craniectomy in children with nontraumatic refractory high intracranial pressure. Clinical article.

OBJECT In this study, the authors investigated the clinical efficacy of decompressive craniectomy treatments for nontraumatic intracranial hypertension in children. METHODS Seven patients with nontraumatic refractory high intracranial pressure (ICP) were enrolled in the study between 1995 and 2005; there were 2 boys and 5 girls with a mean age of 9 years (range 4-14). Decompressive craniectomy was performed in all patients after standard medical therapy had proven insufficient and ICP remained > 50 mm Hg. All patients had a Glasgow Coma Scale score < 8 at admission and a mean Pediatric Risk of Mortality Scale score of 20 (range 10-27). RESULTS One patient died of persistent high ICP and circulatory failure 48 hours after surgery. Six months later, according to their Glasgow Outcome Scale scores, 3 patients had adequate recoveries, 2 patients recovered with moderate disabilities, and 1 patient had severe disabilities. According to the Pediatric Overall Performance Category Scale, 4 patients received a score of 2 (mild disability), 1 a score of 3 (moderate disability), and 1 a score of 4 (severe disability). Five patients returned to school and normal life. CONCLUSIONS The authors found decompressive craniectomy to be an effective and lifesaving technique in children. This procedure should be included in the arsenal of treatments for nontraumatic intracranial hypertension.

Maladie de von Hippel-Lindau : progrès génétiques et cliniques récents

Resume La maladie de von Hippel-Lindau (VHL) est une affection hereditaire due a des mutations germinales du gene suppresseur de tumeur VHL et qui predispose au developpement de tumeurs benignes et malignes richement vascularisees. Les manifestations cliniques majeures comprennent des hemangioblastomes du SNC et de la retine, des tumeurs du sac endolymphatique, des cancers a cellules claires et des kystes renaux, des pheochromocytomes, des kystes et des tumeurs endocrines du pancreas. Le gene VHL joue un role fondamental dans la reponse tissulaire a l’hypoxie en ciblant le facteur de transcription HIF pour sa degradation dans le proteasome. Des alterations acquises du gene VHL sont egalement a l’origine de la majorite des cancers du rein a cellules claires sporadiques et d’une partie des hemangioblastomes sporadiques. Ces decouvertes majeures ouvrent la voie au developpement de nouvelles approches therapeutiques qui pourraient avoir egalement des applications en Cancerologie.

Clampage vasculaire temporaire

Resume Le clampage vasculaire temporaire est une technique qui peut etre d’une aide inestimable en chirurgie anevrismale. L’utilisation du clip temporaire facilite la dissection de l’anevrisme et securise son exclusion. Plusieurs points, concernant les consequences du clampage, les moyens de detection peroperatoire de l’ischemie, les protocoles de neuroprotection associes, sont abordes. Materiel et methodes Revue illustrative de la litterature neurochirurgicale. Discussion Le clampage temporaire reduit le risque de rupture anevrismale peroperatoire. L’analyse des donnees bibliographiques a souleve de nombreuses questions qui sont parfois restees sans reponse. Les consequences du clampage temporaire sont connues. Les methodes de neuroprotection optimales restent a definir. Les differents moyens de monitorage, en plein developpement, sont presentes.