Craniocervical instability in an infant with partial sacral agenesis.

UNLABELLED: Sacral agenesis is an uncommon condition characterised by total or partial absence of the sacrum. The association of this condition with craniocervical junction abnormalities is extremely rare. CASE REPORT: We describe a 3-year-old girl who, at the age of 8 months, while being investigated for short stature, was found to have significant craniocervical instability associated with anomalies of the odontoid peg. In addition to this she had partial sacral agenesis but genetic tests showed a normal karyotype. Due to the inherent difficulty of surgical fixation and immobilisation in an infant of her age, she was managed conservatively in a soft cervical orthosis. At the age of three years, surgery for decompression and stabilisation was deemed necessary due to the onset of neurological morbidity. CONCLUSION: The authors describe this extremely rare association and discuss the difficulties faced while deciding the optimum surgical strategy for managing such young children with craniocervical instability.

New proposals for training in neurosurgery.

The rapidly evolving changes in working patterns, career structure and the regulation of training of doctors have provided an ideal opportunity for proposals to improve the programme for the training of neurosurgeons. The Education and Training Committee of the Society of British Neurological Surgeons (D.G. Hardy, A. J. W. Steers, N. T. Gurusinghe, P. M. Foy, P. van Hille, R. A. Cowie, H. A. Crockard, O. Sparrow and S. Burn) has, in recent months, worked closely with the Specialist Advisory Committee (SAC) in neurosurgery, Department of Health (Modernizing Medical Careers Group, H. A. Crockard, A. Havers, T. Hobbs) and colleagues from the major neuroscience specialties to develop a new programme based on a 'Common Stem' approach. This article describes the principles of the programme. The proposals have received approval by the Council of the SBNS, the Presidents of the four Surgical Royal Colleges and the Senate of Surgery.

Craniovertebral abnormalities in Type VI mucopolysaccharidosis (Maroteaux-Lamy syndrome).

INTRODUCTION: The craniovertebral abnormalities found in patients with Type VI mucopolysaccharidosis (Maroteaux-Lamy syndrome) are described, and the indications for and outcomes of surgery in this group are assessed. METHODS: The clinical histories and radiological findings in all patients with Type VI mucopolysaccharidosis treated at Royal Manchester Children's Hospital during the past 10 years were reviewed. RESULTS: The typical findings in patients with this disease are of canal stenosis at the level of the foramen magnum and upper cervical spine with or without cord compression. The stenosis is secondary to thickening of the posterior longitudinal ligament. Atlantoaxial instability is rare. Of nine patients under regular clinical review, four underwent decompressive surgery for cervical cord compression. Three of the four showed improvement in their neurological symptoms and signs postoperatively. Of the children reviewed, six had radiological evidence of cord compression, although only those with neurological signs or symptoms were treated surgically. DISCUSSION: Despite the often formidable anesthetic challenge, surgery is indicated in those patients who present with progressive neurological deficit due to cervical myelopathy. Surgery can be undertaken safely if the associated medical problems in these children are recognized and managed appropriately.

MRI of the brain and craniocervical junction in Morquio's disease.

We reviewed MRI of the brain and cervical spine in 11 patients with Morquio's disease. No abnormality was seen in the brain. The odontoid peg was abnormal in all patients, with varying degrees of cord compression due to an anterior soft tissue mass and indentation by the posterior arch of the atlas. The degree of cord compression was more marked than suggested by the symptoms and signs. We recommend MRI of the cervical spine in children with Morquio's disease before the development of neurological symptoms, to optimise the timing and type of surgical intervention.

Granular cell tumour of the spinal cord: case report.

A granular cell tumour presenting within the spinal cord of a 17-year-old woman is described. This distinctive tumour has a widespread distribution, but has been reported only rarely in the central nervous system. The literature is reviewed.

Spinal problems in mucopolysaccharidosis I (Hurler syndrome).

Bone-marrow transplantation has increased the survival of patients with mucopolysaccharidosis-I. We describe the spinal problems and their management in 12 patients with this disorder who have been followed up for a mean of 4.5 years since transplantation. High lumbar kyphosis was seen in ten patients which was associated with thoracic scoliosis in one. Isolated thoracic scoliosis was seen in another. One patient did not have any significant problems in the thoracic or lumbar spine but had odontoid hypoplasia, which was also seen in three other children. Four of the eight patients in whom MRI of the cervical spine had been performed had abnormal soft tissue around the tip of the odontoid. Neurological problems were seen in two patients. In one it was caused by cord compression in the lower dorsal spine 9.5 years after posterior spinal fusion for progressive kyphosis, and in the other by angular kyphosis with thecal indentation in the high thoracic spine associated with symptoms of spinal claudication.

Mucopolysaccharidosis type IVA (Morquio syndrome): a clinical review.

Patients with MPS IV have a clinical disorder quite different from other MPS conditions. The major treatment issue revolves around the prevention of cervical myelopathy, although the other aspects of this multisystem disease should not be forgotten. Management is inevitably multidisciplinary and the paediatrician should play a lead role in the coordination of services for affected patients. The timing of occipito-cervical fusion and the selection of patients for this procedure remain unclear. A prophylactic approach to surgery is suggested in this review, although it is by no means clear that all patients will invariably develop cervical myelopathy if left untreated. Parents of affected children need considerable support in dealing with the affected child and the children themselves need help to try to come to terms with their severe physical disabilities. The Society for Mucopolysaccharide Diseases (55 Hill Avenue, Amersham, Bucks HP6 5BX, UK) has offered many parents and affected individuals considerable help as well as raising significant sums for research. Finally, it is important to remember the genetic nature of the disease and the possibility of prenatal diagnosis in subsequent pregnancies.

Osteochondroma of the cervical spine: case report and review of the literature.

Osteochondroma of the spine is a rare condition; we report a patient who presented with a spastic tetraparesis due to such a lesion of the second cervical vertebra. Surgical removal resulted in improvement of the original symptoms, but a transient hemiparesis developed postoperatively. Postoperative magnetic resonance imaging was performed and demonstrated a region of myelomalacia at the level of surgery. Survey of the literature confirms the rarity of this lesion and the use of radiology in diagnosis is discussed.

Oligodendroglioma of the fourth ventricle with intracranial and spinal oligodendrogliomatosis: a case report.

We report a case of oligodendroglioma of the fourth ventricle complicated by disseminated intracranial and spinal oligodendrogliomatosis. This is further evidence that primary oligodendrogliomas arising in close proximity to the cerebrospinal pathway have a predilection for spontaneous dissemination. This condition should be considered in the differential diagnosis of hydrocephalus and myelopathy.

Misleading CT scans: a report of two cases.

Two patients who appeared to have suffered from intracranial haemorrhage are presented. Their clinical histories and computed tomographic scans were supportive of the diagnosis of intracranial haemorrhage. However, both patients were found to have infected cerebrospinal fluid.

The late results of antero-lateral cordotomy for pain relief.

The operation of antero-lateral cordotomy was carried out on 56 patients with intractable pain between 1968 and 1978. The follow-up of these patients was continued for at least three years or until death to determine the late success of this procedure. Of the 33 patients who underwent surgery for malignant pain 95% of survivors had effective relief on discharge from hospital, the success rate falling to 73% at six months, and 55% at one year of follow-up. The operation was judged successful for patients with malignant disease because of short life expectancy. Thirteen patients had chronic pain from non-malignant conditions, and 85% obtained initial relief, the success rate falling to 35% at one year, and 20% at three years of follow-up. Two patients died from respiratory failure, giving an operative mortality of 3.5%. The results and complications of open cordotomy are compared with those of the percutaneous method, and the role of this procedure discussed in relation to alternative stimulatory analgesic procedures.

Spontaneous infected pneumatocoele secondary to chronic otitis media.

A case of spontaneous temporal pneumatocoele secondarily infected by Haemophilus parainfluenzae is reported. Its relation to the lateral ventricle and to a defect in the tegmen tympani was confirmed by computerized tomography (CT). The possible aetiological mechanisms are discussed.