Object. A multidisciplinary team devised a protocol for long-term care of patients with skull base chordomas. In this study they describe their approach. Methods. Forty-two patients presented between 1986 and 1998 and were treated by maximum surgical cytoreduction and photon radiation therapy. Tumor volume—doubling time determined on the basis of magnetic resonance imaging, immunostaining, and cell proliferation (Ki67 labeling index [LI]) studies indicated growth rates of individual chordomas. The best outlook was associated with the greatest extent of tumor removal achieved during the first operation. There were no deaths associated with patients who underwent first-time surgery, but there was a 7.1% mortality rate associated with those who underwent subsequent operations. Cerebrospinal fluid leaks, additional cranial nerve palsies, and pharyngeal wound problems were the most difficult management problems encountered after second and subsequent surgeries. The time interval between operations was usually between 2 years and 3 years after the first surgery; very few patients required a second surgery, with a quiescent period in excess of 5 years. Life-table 5- and 10-year survival rates were 77% and 69%, respectively. Conclusions. The authors believe that this series of skull base chordomas provides new insights into the management of these lesions, particularly with regard to techniques that increase survival times and studies that aid in formulating prognoses.

The experience gained over the last decade has provided some further understanding of the rare, but potentially fatal problems associated with pathology at the craniovertebral junction. Some original concepts of the disease in the area have been challenged; new evidence has been provided to explain our alternative theories. The transoral procedure as a surgical tool is now well established. It is, however, technically demanding, and requires careful anatomical study and 'hands-on' workshop training before the novice will be competent.

Knowledge of the role and hazards of transoral surgery has expanded rapidly, but the application of this technique in children has been limited. To assess its usefulness, 27 pediatric patients who underwent transoral surgery between 1985 and 1994 were studied. Transoral surgery was performed for irreducible anterior neuraxial compression at the craniovertebral junction caused by basilar impression, atlantoaxial subluxation with pseudotumor, or chordoma. The patients ranged in age from 3 to 17 years. Symptomatic presentation varied widely, but 89% had significant neurological deficits before surgery. No patient with normal strength deteriorated after surgery. Of the 16 patients with a preoperative motor deficit, nine improved rapidly, three were unchanged, and four significantly worsened in the perioperative period. Those with mobile atlantoaxial subluxation were most vulnerable to surgically related neurological morbidity. Twenty-four patients were alive for long-term follow-up study (average 5.7 years, range 1-9.2 years). Of those with preoperative weakness, nine improved one Frankel grade, four remained the same, and one deteriorated from Frankel Grade D to C. Swallowing and speech worsened in five patients; this occurred only after resection of lesions above the foramen magnum (p<0.05) when rostral pharyngeal disruption resulted in velopharyngeal dysfunction. This study, unlike previous reviews of pediatric transoral operations, leads the authors to suggest that although transoral surgery can be effective, it also carries a significant risk of neurological injury in patients with symptomatic spinal cord compression and it is also associated with long-term swallowing and speech difficulties.

The presentation and results of treatment are reviewed for 38 patients with skull base chordoma treated at the National Hospital for Neurology and Neurosurgery between 1958 and 1988. With few exceptions, previous studies have combined results for clival and sacral chordomas, or for chordomas and other similar tumours such as chondrosarcoma, and thus it is difficult to be specific about effects of therapy. This study included histological review using immunohistochemistry to confirm diagnosis. Analysis of the survival data for our patients suggests that there are two subgroups with distinct survival patterns: one group with high mortality within the first 5 years, and a second group with an indolent disease process and near normal life expectancy. The age of the patients at presentation ranged from 7 to 78 years, with a mean of 44.3 years. Male: female distribution was 6:5. The commonest presentation was with cranial nerve palsy (94%) or with headache (60%). The most frequently involved cranial nerve was the VIth (60%), followed by the IXth and Xth (40% each). Comparing our results with those of 50 years ago, there was little improvement in the outlook for these patients, despite improvements in surgical approaches and the use of radiotherapy. The promising results in skull base tumours using proton therapy must be treated with caution until definite criteria for diagnosis and outcome have been established. There is a case for a multicentre prospective study of this disease.

SUMMARYThe influence of the duration of ischemia on the development and resolution of post-ischemic brain edema (SG method) was studied in anesthetized rats. Edema developed during ischemia and the amount of edema was related to the duration of ischemia (r = 0.843, p < 0.001). With recirculation to three hours, the major determinant of the amount of edema was still the duration of the preceding ischemia (p < 0.001). Resolution of brain edema only occurred following fifteen minutes ischemia. Post-ischemic bloodbrain barrier breakdown ( M C-AIB, EB albumin) was greatest following longer ischemia. Where present, the BBB leakage was simultaneously to large and small molecules.