Geoffrey L. Klug

A randomized trial of very early decompressive craniectomy in children with traumatic brain injury and sustained intracranial hypertension.

Abstract Object: The object of our study was to determine, in children with traumatic brain injury and sustained intracranial hypertension, whether very early decompressive craniectomy improves control of intracranial hypertension and long-term function and quality of life. Methods: All children were managed from admission onward according to a standardized protocol for head injury management. Children with raised intracranial pressure (ICP) were randomized to standardized management alone or standardized management plus cerebral decompression. A decompressive bitemporal craniectomy was performed at a median of 19.2 h (range 7.3–29.3 h) from the time of injury. ICP was recorded hourly via an intraventricular catheter. Compared with the ICP before randomization, the mean ICP was 3.69 mmHg lower in the 48 h after randomization in the control group, and 8.98 mmHg lower in the 48 hours after craniectomy in the decompression group (P=0.057). Outcome was assessed 6 months after injury using a modification of the Glasgow Outcome Score (GOS) and the Health State Utility Index (Mark 1). Two (14%) of the 14 children in the control group were normal or had a mild disability after 6 months, compared with 7 (54%) of the 13 children in the decompression group. Our conclusion was that when children with traumatic brain injury and sustained intracranial hypertension are treated with a combination of very early decompressive craniectomy and conventional medical management, it is more likely that ICP will be reduced, fewer episodes of intracranial hypertension will occur, and functional outcome and quality of life may be better than in children treated with medical management alone (P=0.046; owing to multiple significance testing P <0.0221 is required for statistical significance). This pilot study suggests that very early decompressive craniectomy may be indicated in the treatment of traumatic brain injury.

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.

Resective surgery in infants and young children with intractable epilepsy

Despite the relatively high incidence of epilepsy during the first few years of life, and its documented adverse impact on development, few studies have documented the long term outcomes following epilepsy surgery in infants and young children. We report a consecutive series of 11 patients under 3 years of age who underwent excisional surgery for intractable seizures. A total of 16 resective procedures were performed, and comprised functional hemispherectomy, temporal lobectomy, and lesionectomy. The mean follow-up period was 3 years. At follow-up, 73% (8/11) were seizure-free, with over half of these patients able to cease anti-epileptic medications. Acceleration of neurodevelopment was seen in all patients in whom preoperative developmental delay was present. Quality of life was enhanced in all patients. This series confirms that excellent results can be obtained following excisional surgery in well-selected infants and young children with intractable seizures.

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

Abstract 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 post-mortem 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.

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.

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.

Gliomas of the optic nerve and chiasm in children

Intrinsic tumours of the optic nerves and chiasm are uncommon tumours, the incidence in children being approximately 3% of all intracranial tumours (Koos & Miller, 1971). Despite extensive discussion in the literature by ophthalmologists, neurologists, neurosurgeons, and neuropathologists, there is still controversy as to the exact nature of the disease and the best method of treatment. Some authors consider that these tumours are potentially aggressive, and advocate surgical excision (Fowler & Matson, 1957) and radiotherapy (Taveras et ai, 1956), while others have considered the tumours to be benign hamartomas which only demand conservative treatment (Hoyt & Baghdassarian, 1969). In all reported series the association of this tumour with von Recklinghausens disease has been noted, but little comment has been made regarding the natural history of the tumour in patients in whom this association exists. The purpose of this paper is to present the experience of management of this tumour at the Royal Childre...

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.