Trochlear nerve schwannomas occurring in patients without neurofibromatosis: case report and review of the literature.

OBJECTIVE AND IMPORTANCE: Despite their predilection for sensory nerves, intracranial schwannomas have been reported in a number of mixed and purely motor cranial nerves, especially in association with Type 2 neurofibromatosis. We report the rare occurrence of a trochlear nerve schwannoma in a patient without neurofibromatosis and review 17 other case reports from the literature. CLINICAL PRESENTATION: A 35-year-old woman presented with an 8-week history of evolving left hemiparesis, bilateral bulbar paresis, and out-of-character emotional lability. INTERVENTION: She underwent a left temporal craniotomy and a subtemporal, transtentorial approach to the tentorial hiatus, with complete excision of a cisternal trochlear nerve schwannoma. CONCLUSION: Postoperative complications included temporary oculomotor and abducens nerve palsies and temporary right hemiparesis and mild expressive dysphasia, which were resolved at 23-month follow-up. Preoperative symptoms and signs completely resolved, but a postoperative complete trochlear nerve palsy required inferior oblique myectomy for correction of diplopia. A review of the literature showed no preoperative trochlear nerve involvement in at least 45% of cases. The tumor is isointense on T1- and T2-weighted magnetic resonance images and enhances brightly with gadolinium. The most frequently used approach for surgical excision is the subtemporal approach, and the tumor is almost always totally excised. Long-term follow-up suggests recovery of preoperative deficit, and persisting or new trochlear nerve palsy is the rule.

Brain injury from a spent bullet descending vertically. Report of five cases.

This is a report of five cases in which a bullet penetrated the vertex of the skull and brain, apparently during its vertical descent after having been fired into the air.

Hemifacial spasm in an infant due to fourth ventricular ganglioglioma. Case report.

The case of an infant is reported in which hemifacial spasm due to a ganglioglioma of the fourth ventricle was relieved by surgery. Previously described causes of hemifacial spasm are summarized, and the relevance of this case to theories on the pathogenesis of the condition is discussed.

Phenytoin and postoperative epilepsy. A double-blind study.

A double-blind trial of phenytoin therapy following craniotomy was performed to test the hypothesis that phenytoin is effective in reducing postoperative epilepsy. A significant reduction in the frequency of epilepsy was observed in the group receiving the active drug up to the 10th postoperative week. Half of the seizures occurred in the first 2 weeks and two-thirds within 1 month of cranial surgery. High rates of epilepsy were observed after surgery in patients with meningioma, metastasis, aneurysm, and head injury. Routine prophylaxis with phenytoin (in a dosage of 5 to 6 mg/kg/day) would seem to be indicated, particularly in high-risk patients and, where possible, this treatment should be started 1 week preoperatively. Seizure control is best when therapeutic levels of phenytoin are maintained.

Transcranial correction of orbital neurofibromatosis.

Neurofibromatosis is a systemic disease that often produces striking disfigurement. Orbital manifestations are common and include sphenoid dysplasia with or without infiltration of the periorbital soft tissues. The resultant deficiency of the posterolateral orbital wall may lead to protrusion of the temporal lobe into the orbit, displacement of the globe, and pulsatile exophthalmos. Treatment at our unit has consisted of transcranial orbital reconstruction with bone grafts and periorbital soft-tissue correction. Observation of complete bone graft resorption in one patient prompted an assessment of the Australian Craniofacial Unit's experience with particular attention paid to the stability of operative correction. Of 36 patients with head and neck neurofibromatosis treated during the period from 1981 to 1995, 14 patients underwent transcranial correction of orbital deformities secondary to sphenoid dysplasia. The treatment and outcomes of this transcranial group are reviewed. The most notable finding was that of recurrent globe pulsation in four patients following initial resolution. Computed tomography scans have documented partial to complete bone graft resorption in three of these patients. Titanium mesh is now being utilized to provide a more durable reconstruction.

The cloverleaf skull anomaly: managing extreme cranio-orbitofaciostenosis.

The cloverleaf skull anomaly represents the most manifestly extreme form of cranio-orbitofaciostenosis with hydrocephalus that has to date been associated with a uniformly poor outcome and frequent death in infancy. Assessment of the primary deformity and the approach to treatment in a consecutive series of 10 patients with the cloverleaf skull anomaly in the one unit are presented. Early predictable surgical correction of the cranio-stenosis is possible by fronto-orbital advancement and lambdoid craniectomy. Less satisfactory correction of hydrocephalus and orbitostenosis is possible with the attendant increased morbidity. Early survival and the potential for a satisfactory long-term outcome depend on the management of the faciostenosis and an airway that may be anomalous at multiple levels. The initial approach was routine tracheostomy, which provided short-term relief but with the morbidity associated with such airway maintenance in any environment other than the most sophisticated health services. More recently, upper airway surgery (uvulopalatopharyngoplasty, adenoidectomy, and soft palatal split) has ensured airway control and avoided the progression to tracheostomy. When utilized later (1 to 10 years), such surgery may alleviate the symptoms of upper airway obstruction and sleep apnea and permit delay of midfacial advancement.

'Splinter in the mind': a case of penetrating periorbital injury.

A case of penetrating orbitocranial injury in a 12-year-old boy is described. The orbital injuries are usually obvious on presentation and receive prompt attention but a high index of suspicion is needed together with specialized imaging to diagnose the frequently coexistent intracranial penetration. Intracranial suppuration is the major complication with combined mortality from all complications reported to be as high as 25%. The value of aggressive antibiotic therapy and formal exploration is emphasized and the importance of wood as a particularly hazardous foreign body is stressed.

Hydrocephalus in pfeiffer syndrome.

A review of the clinical records and CT scan findings of 11 patients with Pfeiffer syndrome showed ventricular dilation in the majority. In 7 cases the ventriculomegaly was sufficiently severe as to be classified as hydrocephalus and warrant ventricular shunting. The common co-existence of hydrocephalus and multiple premature sutural fusion in Pfeiffer syndrome is a further factor in the apparently worse prognosis of this condition when compared to Crouzon and Apert syndrome. Primary cerebral anomalies as a causative factor for the development of hydrocephalus are infrequently recorded. Extensive craniosynostosis with cranial base distortion and constriction would appear to contribute to the production of hydrocephalus.

Multiple cystic brain lesions in a patient with pilocytic astrocytoma.

Pilocytic astrocytomas are usually present as solitary posterior cranial fossa tumours. An unusual case of pilocytic astrocytoma in a 3 year and 8 month old boy is presented. The patient presented over the course of 10 months with intermittent headaches, vomiting, gait ataxia and drowsiness. After extensive investigations magnetic resonance imaging (MRI) revealed widespread lesions throughout the central nervous system, including multiple cystic cerebral grey matter lesions. A brain biopsy was performed and pathological studies revealed pilocytic astrocytoma. The literature pertaining to neuraxis dissemination of pilocytic astrocytomas in the paediatric population is reviewed. There are only a very limited number of reports of pilocytic astrocytoma ca using multiple brain lesions, with no publications of multiple cystic brain lesions. We believe this to be a unique case of pilocytic astrocytoma presenting with widespread cystic lesions throughout the brain.

Choroid plexus papillomas in childhood.

Chroid plexus papillomas account for only 0.4-0.6% of intracranial tumours at all ages and less than 5% in childhood. Two paediatric cases are reported to demonstrate the variability in clinical presentation, location and neurological outcome of this benign tumour. One case has been followed for 13 years.

Brain tumours in infancy: a clinicopathological study.

To investigate the clinicopathological features of brain tumours occurring in the first year of life, the records of the Department of Histopathology at the Adelaide Children's Hospital were examined for cases where the initial diagnosis of intracranial neoplasm had been made in infancy. Surgical material was available for review from 1972 to 1993 and autopsy cases were reviewed for an additional 12 years from 1962 to 1993. Twenty-four infants with intracranial neoplasms were diagnosed ranging in age from 5 days to 1 year (average = 7 months). There were 23 surgical cases and 1 autopsy case. The male to female ratio was 17:7. Fifty-eight percent of the tumours were located in the supratentorial region. Although the incidence is relatively low, this study demonstrates that a wide range of brain tumours, which differ significantly in both clinical presentation and location from those found in the older child, do occur during the first year of life. The location of the primary tumour may be affected by associated congenital malformations, and metastatic malignancy, although rare, may occur. Antenatal ultrasound examination may be useful in identifying congenital intracranial tumours.

Childhood glioblastoma multiforme of the spinal cord.

Astrocytoma accounts for more than 50% of all central nervous system tumours diagnosed, with particular prevalence in the 15- to 34-year-old age bracket, rarely arising in younger children. In 1995, a 7-year-old boy presented in Emergency with a 3-day history of severe radicular back pain and associated muscle spasms, exacerbated by lying on his back. Both bone scan and plain X-rays were unremarkable; however, MRI showed a 3-cm space-occupying lesion at the level of T5-T6. The patient proceeded to biopsy and partial excision of the tumour through laminectomy, histology confirming an anaplastic astrocytoma (glioblastoma multiforme), St Anne Mayo grade 4. Treatment consisted of a radical course of radiotherapy alone, delivering a total dose of 44.8 Gy at 1.6 Gy per fraction. The treatment comprised of three phases using two oblique wedged fields on a 6 MV linear accelerator. The patient remains disease free 7 years post treatment, with the only effect noted being a slight kyphoscoliosis at the site of the laminectomy and radiation. This report highlights the efficacy of combined surgery and radiation therapy in the management of spinal cord glioblastoma multiforme in preventing tumour recurrence, with acceptable morbidity. Further evaluation of the treatment efficacy would be difficult because of the scarcity of such cases.

Meningioma of the third ventricle.

A case of meningioma of the third ventricle is described. The tumor was completely removed using a transcallosal approach.

Apert's syndrome.

This paper discusses 33 cases of Apert's syndrome which were treated in the Australian Craniofacial unit at the adelaide Children's Hospital. The main features were discussed. We found that mild ventricular dilatation is common in Apert's syndrome but without associated raised intracranial pressure. Severe ventricular dilatation was seen in only one case. No shunt procedures were performed. We also studied the changes in the ventricular size after transcranial corrective procedures. There was no significant change in the ventricular size, the increase in the skull volume was compensated by expansion of the brain tissue and to some extent by increase in the subarachnoid space. Two cases with unusual features are also described.

Cerebrospinal fluid spaces before and after infant fronto-orbital advancement in unilateral coronal craniosynostosis.

Computed tomographic scan-based examination of the cerebrospinal fluid-filled spaces before and after fronto-orbital advancement in infants with unilateral coronal synostosis was performed. In infants who had not undergone surgery, pronounced compression of the ipsilateral frontal brain substance, frontal subarachnoid space, and frontal horn of the lateral ventricle were evident, with deviation of the interhemisphere fissure. After infant fronto-orbital advancement, frontal dead-space is obliterated early by expansion of the frontal subarachnoid space. Asymmetries interhemispheric fissure are slower to adapt and may be persistent some years later.

Syringomyelia in children with primary scoliosis.

The clinical notes of 35 children presenting with scoliosis were reviewed; all 35 had been investigated with MRI. Seven were found to have syringomyelia, and six of these had Chiari malformation. Correction of the syrinx resulted in improvement or stabilisation of the spinal curvature. We recommend that all cases presenting with primary scoliosis should have MRI and should be treated if a syrinx is found.