Frontoethmoidal encephaloceles: reconstruction and refinements.

Frontoethmoidal encephaloceles are herniations of the intracranial contents through a defect in the skull at the junction of the frontal and ethmoidal bones. They are generally classified as nasofrontal, nasoethmoidal, and naso-orbital, although there may be some overlap or multiplicity. The records of 35 patients treated for frontoethmoidal encephaloceles were examined. Of these, 12 cases with complete and accurate medical records were evaluated in detail. The successful correction of frontoethmoidal encephaloceles was shown to depend on the following: a detailed understanding of the pathological anatomy (such as interorbital hypertelorism rather than true orbital hypertelorism and the presence of secondary trigonocephaly), careful planning of the bone movements to correct these deformities, and attention to detail regarding the placement of scars, positioning of the medial canthi, and the nasal reconstruction. Avoiding the "long-nose" deformity often seen after repair should be a priority. In general, the authors recommend a one-stage repair with both a transcranial and external approach.

Acute-onset nontraumatic paraplegia in childhood: fibrocartilaginous embolism or acute myelitis?

Fibrocartilaginous embolus causing acute spinal cord infarction is a rare cause of acute-onset paraplegia or quadriplegia. Few cases of survivors have been reported in the neurosurgical literature, with most reports involving postmortem or biopsy findings. There is little information on MRI findings in such patients. We present the youngest patient ever reported, and discuss the important differences between fibrocartilaginous embolus and acute myelitis of childhood. A 6-year-old girl with a history of back pain presented with sudden-onset nontraumatic paraplegia, with a clinical anterior spinal artery syndrome. Initial MRI scan revealed intervertebral disc disease at L1-2 and an incidental thoracic syrinx, but no cause for her acute-onset paraplegia was identified. Cerebrospinal fluid and other investigations were all negative. Sequential MRI scans revealed development of spinal cord expansion from T10 to the conus medullaris, with increased cord signal in the anterior aspect of the spinal cord. The intervertebral disc disease was unchanged. The imaging and clinical findings were caused by fibrocartilaginous embolus, which meant there was no need for spinal cord biopsy. The report describes the clinical and imaging criteria for diagnosis of fibrocartilaginous embolus, highlighting the case for avoiding an unnecessary biopsy. The clinical pattern in the paediatric group is discussed, with features differentiating it from acute myelitis of childhood.

Delayed presentation of transorbital intracranial pen.

A 13 year old Fijian boy sustained a stab wound to the left orbit 3 years ago. It was not appreciated by the treating physicians in Fiji that the plastic pen had crossed from the left orbit, through the nose, right orbit and right optic nerve, into the right middle cranial fossa and lodged in the right temporal lobe and that the pen remained in situ for the past 3 years. The boy presented to Australia with a discharge from the entry wound in his left lower eyelid. The retained foreign body was not detected on computed tomography imaging, but was detected on subsequent magnetic resonance image. A combined neurosurgery/plastic surgery craniofacial approach was undertaken with successful complete removal of the retained pen, and preservation of vision in his only seeing eye.

Cerebrospinal fluid shunts in the management of behavioural problems in Sanfilippo syndrome (MPS III).

UNLABELLED: Severe behavioural disturbance is a very common feature of Sanfilippo syndrome (mucopolysaccharidosis III, MPSIII), and one of the more difficult aspects of the disease to treat. We describe a series of six patients with MPS III who had cerebrospinal shunts inserted in an attempt to ameliorate behaviour that had proved refractory to conventional treatment. Symptoms improved significantly in all six but removal of the shunt was necessitated in one patient due to shunt blockage and infection. CONCLUSION: Our experience suggests cerebrospinal fluid shunting should be formally evaluated as an adjunct to conventional forms of treatment of extreme behavioural disturbance in MPS III.

The surgical management of osseous cranial base tumours in children.

BACKGROUND: Osseous cranial base tumours in children present as a diverse collection of both benign and malignant pathologies. Concerns raised by the difficulty in accurate diagnosis and local recurrence of benign lesions and by the long-term sequelae of radiotherapy for malignant cranial tumours (marked local growth disturbances, pituitary dysfunction, visual disturbances, late new tumour induction) prompted an evaluation of surgical resection of cranial base tumours in children, with specific regard to safety, efficacy and aesthetic result. METHODS: A retrospective review was performed of 10 consecutive children presenting with tumours either arising from or eroding into bone of the cranial base who were managed by surgical resection in a 10-year period from 1986 to 1996. The patients demonstrated a great variation in both presentation and pathology. All underwent surgical resection of tumour with reconstruction where indicated. RESULTS: There were no postoperative complications or mortality. All patients remained clinically free of disease at follow-up, which ranged from 17 months to 9 years (mean 6 years and 4 months). CONCLUSION: The aggressive surgical resection and craniofacial reconstruction of cranial base tumours in the paediatric population offers a safe and efficacious mode of treatment that obviates problems of diagnosis and local recurrence for benign lesions and of the long-term sequelae of radiotherapy for malignant lesions.

Intracranial arteriovenous malformations in childhood: presentation, management and outcome.

Cases of true intracranial arteriovenous malformations (AVMs) presenting over a 25 year period were reviewed in order to achieve a better understanding of the behaviour and management of AVMs in children. There were 69 cases, presenting with haemorrhage (78%), seizures (13%) cardiac failure (3%) and focal signs with or without headache (6%). It was less common to present under six years of age. CT scanning, where performed, always demonstrated an abnormality, but this was suggestive of an AVM in less than one third. By contrast, angiography defined the lesion in 82% of initial studies. 59 patients underwent a surgical procedure directed at their AVM or an associated haematoma. Total AVM excision was obtained in 65%, with none of these later rebleeding. Three patients presenting solely with seizures were not operated upon initially, but underwent successful resections of their lesions after later haemorrhages. There were 6 (9%) deaths in the series, with focal deficits in 52% of survivors at last follow up. In the operative group these figures were 3% and 51%, respectively. None of the eight patients operated upon prior to a clinical bleed suffered a new neurological deficit. The role of stereotactic radiosurgery, although not used in any of our cases, is discussed. We believe that our results support an aggressive surgical approach to childhood AVMs, regardless of presentation, given the significant risk of morbidity from a later bleed, and the lack of a clearly better outcome with expectant management or irradiation.

Cerebrospinal fluid shunt infections in children.

We reviewed cerebrospinal fluid (CSF) shunt infections treated in the Royal Children's Hospital, Melbourne from 1981 to 1991. Forty-one episodes of CSF shunt infection were found after 900 shunt operations, an infection rate of 4.5%. Clinical symptoms were nonspecific in 31.7% of episodes, and in 17.1% of episodes the initial CSF sample was normal on microscopy and biochemistry, although a pathogen was isolated on culture. Most episodes occurred within 4 months of the last operation on the shunt, the exception being infections caused by Haemophilus influenzae. Four patients died during treatment, but none could be attributed to infection alone. Treatment of suspected CSF shunt infection should not be withheld because of lack of firm clinical diagnosis or normal CSF microscopy, and episodes occurring more than 4 months after the last operation on the CSF shunt should receive antibiotic cover for H. influenzae.

Transnasal stereotactic biopsy of a clivus tumor. Technical note.

Computerized tomography-guided transnasal stereotactic tissue diagnosis of a lytic lesion in the clivus was performed successfully using the Cosman-Roberts-Wells frame, thus avoiding a major craniotomy. The authors recommend stereotaxis as the preferred technique for biopsy in this region.

Retinal haemorrhage in accidental head trauma in childhood.

Twenty-five children (age range 1.2-14.5 years) who sustained accidental head injury requiring admission to hospital were prospectively examined for evidence of retinal haemorrhage. In no case were retinal haemorrhages detected. It is suggested that accidental head injury seldom results in retinal haemorrhage and that the finding of retinal haemorrhage in a child with a head injury should prompt suspicion of abuse.

Temporal lobectomy for the treatment of intractable complex partial seizures of temporal lobe origin in early childhood.

Eleven patients with intractable complex partial seizures underwent temporal lobectomy during their first decade. The mean age at onset of epilepsy was two years and at surgery was 5.5 years. On the basis of data from clinical evaluation, CT, MRI in six patients, and pathological examination of excised tissue, the aetiology of the epilepsy was thought to be mesial temporal sclerosis in four children, glioma in five, dysplasia in one and chronic progressive encephalitis in another. At follow-up eight children were seizure-free, two had reduced seizure frequency and only the child with chronic progressive encephalitis had not benefitted from surgery.

Autosomal recessive hydrocephalus with third ventricle obstruction.

Here we report a brother and sister who presented in the neonatal period with hydrocephalus. Ultrasonography showed marked dilatation of the lateral ventricles but not the third ventricle. One child with postnatal onset was shunted and had normal development at 3 years. The other child had severe hydrocephalus at birth and was not treated. Neuropathologic studies demonstrated dilatation of the lateral ventricles and marked narrowing of the posterior part of the third ventricle but no other malformations other than those that result directly from hydrocephalus. The potential for a good prognosis is emphasized.

Middle fossa arachnoid cysts in association with subdural haematomas. A review and recommendations for management.

Subdural haematomas occur in association with arachnoid cysts. Six cases of subdural haematoma are reported in 18 patients with previously asymptomatic middle fossa arachnoid cysts. A review of this patient population and the literature in respect of pathogenesis and treatment of arachnoid cysts was made. Cysto-peritoneal shunt is recommended as the surgical treatment after evacuation of the haematoma. The aetiology of haematoma formation has not been conclusively described, but may partly result from a high pressure intracranial system with decreased compliance.

Subependymal giant-cell astrocytoma in children. An unusual discrepancy between histological and clinical features.

The authors describe five cases of subependymal giant-cell astrocytoma in children in which many clinical, histological, immunohistochemical, and ultrastructural features typical of this tumor were present. However, prominent focal necrosis and mitoses, features usually associated with high-grade tumors, were seen in all cases. Despite the presence of necrosis and mitoses, clinical follow-up studies have revealed a lack of aggressive tumor behavior after surgery alone. The discrepancy between the histological and clinical features in these cases is emphasized so that excessive treatment of a basically low-grade tumor may be avoided. Mast cells were seen in all five cases, often in large numbers.

Injury profile of pedal and motor cyclist casualties in Victoria.

The injury profiles of 512 pedal and 667 motor cyclist casualties managed during 1977-80 at four Melbourne teaching hospitals have been analysed using the Abbreviated Injury Scale (1980 revision). Additional comparison has been made between cyclist casualties involved in collisions with another vehicle, in single vehicle accidents and between casualties aged 17 years or more. Pedal cyclist casualties sustained significantly more frequent and severe head injury although the maximum level of injury to any part of the body (maximum abbreviated injury score) was, with the exception of casualties involved in single vehicle accidents, significantly greater in motor cyclist casualties. Head injury occurred in 59% of pedal and 26% of motor cyclist casualties (P less than 0.001); severe head injuries occurred in 9% and 4%, correspondingly. These differences may be explained, at least in part, by the fact that virtually all motor cyclists were protected by safety helmets whereas few pedal cyclists were similarly protected. The following countermeasures are recommended: effective promotion of approved safety helmet wearing in all schools; bulk purchase of helmets through the Ministry of Education for low cost distribution; a Government subsidy to reduce the costs of helmet purchase; legislation for compulsory wearing of approved safety helmets by pedal cyclists.

Head injury predominance: pedal-cyclists vs motor-cyclists.

A study of the pattern of head injuries in pedal- and motor-cyclist casualties treated at four teaching hospitals in Melbourne was undertaken to determine whether significant differences occurred between the two groups. The injuries were coded according to the 1980 revision of the Abbreviated Injury Scale of the American Association for Automotive Medicine and the data subjected to statistical analysis. Although motor-cyclist casualties sustained more severe injuries to the body, the results show that pedal-cyclist casualties sustained more frequent and severe head injuries. It is considered that the differences are due, at least in part, to a far lower use of protective helmets among pedal-cyclists. Education to increase community awareness of this safety measure followed by legislation for the compulsory wearing of approved safety helmets is urged.

Neurocutaneous melanosis with hydrocephalus and syringomyelia. Case report.

The authors report the investigation, treatment, and progress of a unique case of neurocutaneous melanosis with syringomyelia. The suspected etiology, the pathology, and the variable clinical manifestations of this rare phakomatosis are recounted. The potential for malignant change and the possible mechanisms of syrinx development in this syndrome are discussed.

Management of carotid-cavernous fistulas by surgery combined with interventional radiology. Report of two cases.

Two cases of carotid-cavernous fistulas were successfully treated by standard interventional radiology techniques after otherwise inaccessible vessels were surgically exposed. In the first case, an internal carotid artery (ICA), which had previously been ligated as part of an attempted surgical "entrapment" procedure, was recanalized to permit passage of a detachable balloon catheter to the fistula, resulting in its obliteration. In the second case, an enlarged superior ophthalmic vein was exposed and isolated to facilitate retrograde catheterization of the cavernous sinus and obliteration of a dural fistula between the ICA and the cavernous sinus by steel Gianturco coils. The methods and complications of both procedures are discussed.

Treatment of caroticocavernous fistulae with detachable balloons introduced by percutaneous catheterization.

Five patients with caroticocavernous fistula were treated by new, interventional angiographic techniques. In four patients, latex balloons filled with a silicone polymer were introduced into the cavernous sinus via the transfemoral route and detached; this resulted in complete obliteration of the caroticocavernous fistula and preservation of the internal carotid artery flow in two of the four patients. In the fifth patient, stainless steel coils were introduced into the cavernous sinus via the superior ophthalmic vein which had previously been surgically exposed. These percutaneous, angiographic techniques of intervention should be considered the initial treatment of choice for caroticocavernous fistulae.

Differences in head injuries of pedal cyclist and motorcyclist casualties in Victoria.

There being no legal requirement in Victoria for pedal cyclists to wear crash helmets (and only few do so), we tested the hypothesis that pedal cyclists are subject to significantly greater risk of head injury than motorcyclists, who are required by law to wear them. Injury data of 1975 to 1980 showed that there were three times more fatalities and reported non-fatal casualities among motorcyclists than among pedal cyclists. Head injuries, including fractured vault of skull, fractured base of skull, concussion and intracranial injury, were significantly more frequent among pedal cyclist casualties (P less than 0.001). The number of pedal cyclist casualties with solely head injury was twice that of motorcycle riders, and those pedal cyclist casualties had a significantly greater incidence of fractured vault of the skull. It is likely that risk of head injury for pedal cyclists would be significantly reduced if they wore protective helmets.