Atlantoaxial rotatory fixation--a cause of torticollis.

BACKGROUND: A rare, but important cause of torticollis in children is atlantoaxial rotatory fixation. If the patient remains undiagnosed for more than three months, surgery is generally the only therapeutic alternative. In this article we present our experiences of surgical treatment of late-diagnosed atlantoaxial rotatory fixation in children. MATERIAL AND METHOD: This article is based on a review of the case notes of patients who underwent surgery for atlantoaxial rotatory fixation in the Department for Neurosurgery at Oslo University Hospital, Rikshospitalet, during the period 2004-10. RESULTS: The material sample consists of six children aged from seven to 14 years. Five had suffered minor trauma to the upper neck region, while one had had an upper respiratory tract infection. The diagnosis was made 5-36 months after the onset of symptoms. In three of the patients, an attempt was made at closed reduction without success. A CT scan one year postoperatively showed a normal position of the atlantoaxial joint in two patients and partial reduction in three. In the sixth patient there was bone fusion at the time of the operation, and open reduction was unsuccessful. All six patients had reduced rotational movement of the neck at the one-year check-up. INTERPRETATION: All our patients were diagnosed more than five months after the onset of symptoms. Full or partial reduction was achieved in five of the six.

The Long-term Outcome After Resection of Intraspinal Nerve Sheath Tumors: Report of 131 Consecutive Cases.

BACKGROUND: The existing literature on recurrence rates and long-term clinical outcome after resection of intraspinal nerve sheath tumors is limited. OBJECTIVE: To evaluate progression-free survival, overall survival, and long-term clinical outcome in a consecutive series of 131 patients with symptomatic intraspinal nerve sheath tumors. METHODS: Medical charts were retrospectively reviewed. Surviving patients voluntarily participated in a clinical history and physical examination that focused on neurological function and current tumor status. RESULTS: Follow-up data are 100% complete; median follow-up time was 6.1 years. All patients (100%) had surgery as the first line of treatment; gross total resection was performed in 112 patients (85.5%) and subtotal resection in 19 patients (14.5%). Five-year progression-free survival was 89%. The following risk factors for recurrence were identified: neurofibroma, malignant peripheral nerve sheath tumor, subtotal resection, neurofibromatoses/schwannomatosis, and advancing age at diagnosis. More than 95% of patients had neurological function compatible with an independent life at follow-up. The rate of tumor recurrence in nonneurofibromatosis patients undergoing total resection of a single schwannoma was 3% (3/93), in comparison with a recurrence rate of 32% (12/38) in the remaining patients. CONCLUSION: Gross total resection is the gold standard treatment for patients with intraspinal nerve sheath tumors. In a time of limited health care resources, we recommend that follow-up be focused on the subgroup of patients with a high risk of recurrence. The benefit of long-term, yearly magnetic resonance imaging follow-up with respect to recurrence in nonneurofibromatosis patients undergoing gross total resection of a single schwannoma is, in our opinion, questionable. 1NF2, neurofibromatosis 2NST, nerve sheath tumorOS, overall survivalPFS, progression-free survivalSTR, subtotal resectionWHO, World Health Organization.