The management of bifocal intracranial germinoma in children.

OBJECTIVES: Bifocal intracranial germinoma (BFG) is a tumour of the pineal and suprasellar regions, which is known to be highly radiosensitive. The definitive treatment and outcomes are not well defined, particularly in the paediatric population. We review our series of purely paediatric cases from a single institution and combine them with the limited reports in the literature to determine the results of different management strategies. METHODS: Four patients were treated at our institution with a median age of 15.3 years. A literature search identified a further 38 paediatric cases with a median age of 12.9 years. RESULTS: All four patients had normal serum and CSF tumour markers. One patient had a diagnosis made based on imaging findings of bifocal pineal and suprasellar lesions presenting with diabetes insipidus. Three others underwent biopsy. All had craniospinal radiotherapy, which has led to complete cure with no cases of progression at a mean follow-up of 3 years. The most common treatment modality in published cases is craniospinal irradiation. In the cases reviewed, limited radiation treatments (whole ventricle or focal) combined with chemotherapy regimens yield comparable outcomes where there is no spinal dissemination. Outcomes do not appear to be altered by biopsy in cases with negative tumour markers and characteristic imaging appearances. CONCLUSION: Patients who present with a classic appearance of germinoma, negative tumour markers and diabetes insipidus probably do not require a biopsy to confirm the diagnosis. No evidence of dissemination may obviate the need for craniospinal irradiation, but good quality long-term follow-up data are required to demonstrate the benefits of combined focal radiotherapy and chemotherapy regimes.

Intra-operative 3-T MRI for paediatric brain tumours: challenges and perspectives.

MRI is the ideal modality for imaging intracranial tumours. Intraoperative MRI (ioMRI) makes it possible to obtain scans during a neurosurgical operation that can aid complete macroscopic tumour resection­a major prognostic factor in the majority of brain tumours in children. Intraoperative MRI can also help limit damage to normal brain tissue. It therefore has the potential to improve the survival of children with brain tumours and to minimise morbidity, including neurological deficits. The use of ioMRI is also likely to reduce the need for second look surgery, and may reduce the need for chemotherapy and radiotherapy. Highfield MRI systems provide better anatomical information and also enable effective utilisation of advanced MRI techniques such as perfusion imaging, diffusion tensor imaging, and magnetic resonance spectroscopy. However, high-field ioMRI facilities require substantial capital investment, and careful planning is required for optimal benefit. Safe ioMRI requires meticulous attention to detail and rigorous application of magnetic field safety precautions. Interpretation of ioMRI can be challenging and requires experience and understanding of artefacts that are common in the intra-operative setting.

Infantile myofibromatosis of the craniovertebral junction.

Infantile myofibromatosis (IM) is a rare pathological entity characterized by solitary or multiple nodular skin, soft tissues or bony lesions. Craniovertebral (CV) junction lesions are rare. We report the successful management of a solitary IM involving the posterior elements of the CV junction in a 6-month-old child.

The role of endoscopic third ventriculostomy in the management of hydrocephalus associated with cerebellopontine angle tumours.

BACKGROUND: Progressive hydrocephalus secondary to cerebellopontine angle tumours has been traditionally managed with ventriculo-peritoneal shunting. Endoscopic third ventriculostomy provides an alternative treatment option and the success rate in this patient group has not previously been reported. METHODS: We report a retrospective series of 11 patients with cerebello-pontine angle tumours who presented with symptomatic hydrocephalus, or developed hydrocephalus following radiosurgery, who underwent endoscopic third ventriculostomy. RESULTS: Seven patients (63.6%) remain shunt free. There were no complications following endoscopy in any patient. Where the ventriculostomy failed there was no additional morbidity. CONCLUSIONS: Endoscopic third ventriculostomy is a low morbidity procedure, which avoids the inherent problems of shunts, particularly infection and should be considered for patients with hydrocephalus and cerebello-pontine angle tumours.

Spontaneous resolution of acute obstructive hydrocephalus in the neonate.

Spontaneous resolution of acute hydrocephalus without aspiration of cerebral fluid is rare. In a neonate born at full term this has only been reported once before. We report on one further case that was caused by intraventricular haemorrhage (IVH). The probable mechanism is resolution of the acute haemorrhage in the region of the aqueduct, resulting in resolution of the hydrocephalus itself. The importance of considering conservative management of acute hydrocephalus in the clinically stable neonate is emphasised.

Clinical features and outcomes in patients with non-acoustic cerebellopontine angle tumours.

OBJECTIVES: Non-acoustic tumours of the cerebellopontine angle differ from vestibular schwannomas in their prevalence, clinical features, operative management, and surgical outcome. These features were studied in patients presenting to the regional neuro-otological unit. METHODS: A retrospective analysis of clinical notes identified 42 patients with non-acoustic tumours of the cerebellopontine angle. Data were extracted regarding presenting clinical features, histopathological data after surgical resection, surgical morbidity and mortality, and clinical outcome (mean 32 months follow up). RESULTS: The study group comprised 25 meningiomas (60%), 12 epidermoid cysts/cholesteatomata (28%), and five other tumours. In patients with meningiomas, symptoms differed considerably from patients presenting with vestibular schwannomas. Cerebellar signs were present in 52% and hearing loss in only 68%. Twenty per cent of patients had hydrocephalus at the time of diagnosis. After surgical resection, normal facial nerve function was preserved in 75% of cases. In the epidermoid group, fifth, seventh, and eighth nerve deficits were present in 42%, 33%, and 66% respectively. There were no new postoperative facial palsies. There were two recurrences (17%) requiring reoperation. Overall, there were two perioperative deaths from pneumonia and meningitis. CONCLUSIONS: Patients with non-acoustic lesions of the cerebellopontine angle often present with different symptoms and signs from those found in patients with schwannomas. Hearing loss is less prevalent and cerebellar signs and facial paresis are more common as presenting features. Hydrocephalus is often present in patients presenting with cerebellopontine angle meningiomas. Non-acoustic tumours can usually be resected with facial nerve preservation.

Constructive interference in steady-state 3D Fourier-transform MRI in the management of hydrocephalus and third ventriculostomy.

We describe the use of three-dimensional Fourier transform constructive imaging in the steady state (CISS) MRI in the assessment of patients with hydrocephalus. We have found it of value both as a diagnostic investigation and in the follow-up of patients treated by third ventriculostomy.

Idiopathic syringomyelia and the importance of occult arachnoid webs, pouches and cysts.

Syringomyelia is the condition in which longitudinal cavities are found within the spinal cord. The use of drainage procedures has been widely practised with good short term results. However, the long-term results in some large series have been less favourable. There are many associated conditions and in most forms a blockage to the normal flow of CSF, either at the foramen magnum or in the spinal canal, can be identified. Most surgeons would now direct their efforts to the establishment of normal CSF flow rather than a shunting procedure. In a certain group of patients, even with the advent of sophisticated MRI, no associated abnormality or CSF block is easily identified. This type of syringomyelia is often termed idiopathic. We report 10 patients with symptomatic syringomyelia without readily recognized predisposing factors. In eight patients preoperative myelography revealed a block to the flow of contrast compatible with subarachnoid obstruction. Eight patients underwent laminectomy and division of the obstructing arachnoid webs. Five experienced good improvement and three only moderate improvement. Two of the patients underwent syrinx shunting procedures only, which resulted in a worsening of their symptoms. At operation one patient was found to have an arachnoid cyst. We believe that patients with idiopathic symptomatic syringomyelia may need myelography to identify such arachnoid abnormalities. Subsequent surgery should be directed at the establishment of normal CSF flow by laminectomy and excision of the offending lesion.

Paediatric meningeal tumours.

We review our experience of meningeal tumours in patients under 16 years who were treated between 1957 and 1993. These were 16 with meningiomas (median age 12 years) and 5 with poorly differentiated malignant meningeal tumours (median age 3 years). The incidence of meningeal tumours in this period was 1.62% of all CNS tumours. There were 19 supratentorial tumours (5 intraventricular), 1 tentorial and 1 cervical. Surgical excision was complete in 12 patients and incomplete in 6; in 2 patients a biopsy only was undertaken. The remaining patient had radiological diagnosis only. All 5 patients in the group with malignant poorly differentiated tumours died (median survival 5 months), confirming the aggressive nature of these lesions. Of the 16 patients with meningiomas, 4 died during followup but 11 of the 12 meningioma patients remaining alive had complete resections. There is a strong tendency for paediatric meningeal tumours to have a supratentorial origin and intraventricular location. The female preponderance found in adults is not evident in children. Incomplete surgical excision or focal neurological deficit at presentation was associated with poor outcome.

Successful removal of large pineal region meningiomas: two case reports.

BACKGROUND: Pineal region meningiomas are extremely rare tumors and comprise about 8% of tumors of this region. Two cases of large pineal region meningiomas in young males are presented. METHODS: Computed tomography (CT) scan and cerebral angiography were used to evaluate the patients preoperatively. Both patients were operated on through an occipital transtentorial approach either as a single- or two-stage procedure. RESULTS: Preoperative work-up revealed that both tumors received their main blood supply from the posterior lateral choroidal arteries and were therefore originating from the velum interpositum cerebri (superior tela choroidea). Complete tumor removal was effected in the first patient using a right occipital transtentorial approach. In the second patient, after partial removal using the same approach, complete resection was achieved at a second stage via the left occipital transtentorial approach 3 months later. CONCLUSION: Cerebral angiography, which must include vertebral angiography, is important in the preoperative surgical planning of these tumors. The occipital transtentorial approach provides good access. However, a two-stage procedure using right and left sides may be necessary for large meningiomas receiving a bilateral blood supply from both posterior lateral choroidal arteries. Diagnosis, the surgical approach, and methods of tumor excision are discussed.