J. Punt

Neuroendoscopic Third Ventriculostomy in Patients Less than 1 Year Old

A series of neuroendoscopic third ventriculostomies in children less than 1 year old is reported. Twenty-seven patients underwent the procedure with 21 (77%) failing within a mean of 1.36 months of the procedure. Nineteen were subsequently shunted. The presence or absence of flow through the ventriculostomy and the size of the lateral ventricles on post-operative imaging were not an indicator of success or failure. Only 4 (15%) had a complication of the procedure. Although the majority fail, approximately 1/3 are spared the added morbidity and mortality of having a shunt. With such a low morbidity and zero mortality the procedure has many benefits over shunting. Consequently, neuroendoscopic third ventriculostomy is used in this institution, where possible, rather than a shunt.

Primary postoperative chemotherapy without radiotherapy for intracranial ependymoma in children: the UKCCSG/SIOP prospective study

BACKGROUND Over half of childhood intracranial ependymomas occur in children younger than 5 years. As an adjuvant treatment, radiotherapy can be effective, but has the potential to damage the childs developing nervous system at a crucial time-with a resultant reduction in IQ and cognitive impairment, endocrinopathy, and risk of second malignancy. We aimed to assess the role of a primary chemotherapy strategy in avoiding or delaying radiotherapy in children younger than 3 years with intracranial ependymoma. METHODS Between December, 1992, and April, 2003, we enrolled 89 children with ependymoma who were aged 3 years or younger at diagnosis, of whom nine had metastatic disease on pre-operative imaging. After maximal surgical resection, children received alternating blocks of myelosuppressive and non-myelosuppressive chemotherapy every 14 days for an intended duration of 1 year. Radiotherapy was withheld unless local imaging (ie, from the childs treatment centre) showed progressive disease. FINDINGS 50 of the 80 patients with non-metastatic disease progressed, 34 of whom were irradiated for progression. The 5-year cumulative incidence of freedom from radiotherapy for the 80 non-metastatic patients was 42% (95% CI 32-53). With a median follow-up of 6 years (range 1.5-11.3), overall survival for the non-metastatic patients at 3 years was 79.3% (95% CI 68.5-86.8) and at 5 years 63.4% (51.2-73.4). The corresponding values for event-free survival were 47.6% (36.2-58.1) and 41.8% (30.7-52.6). There was no significant difference in event-free or overall survival between complete and incomplete surgical resection, nor did survival differ according to histological grade, age at diagnosis, or site of disease. In 47 of 59 (80%) patients who progressed, relapse resulted from local control only. The median time to progression for the 59 patients who progressed was 1.6 years (range 0.1-10.2 years). The median age at irradiation of the whole group was 3.6 years (range 1.5-11.9). For the 80 non-metastatic patients, the 23 who achieved the highest relative dose intensity of chemotherapy had the highest post-chemotherapy 5-year overall survival of 76% (95% CI 46.6-91.2), compared with 52% (33.3-68.1) for the 32 patients who achieved the lowest relative dose intensity of chemotherapy. INTERPRETATION This protocol avoided or delayed radiotherapy in a substantial proportion of children younger than 3 years without compromising survival. These results suggest, therefore, that primary chemotherapy strategies have an important role in the treatment of very young children with intracranial ependymoma.

Neuroendoscopic third ventriculostomy for hydrocephalus in adults: report of a single unit’s experience with 63 cases

BACKGROUND Neuroendoscopic third ventriculostomy (NTV) is becoming a first line treatment for hydrocephalus in this center. Its use in a consecutive series of adults is reported. METHOD Initially a retrospective data collection after 7 months becoming prospective studying all patients who underwent NTV in this center. The adults (17 years or older) have been studied. RESULTS Sixty-three patients met the criteria for inclusion: 38 male, 25 female. Mean age at first NTV 37.5 years. There was an 80% success rate (i.e., no further therapy for the hydrocephalus required). Follow-up was for a mean of 3.1 years. The largest subgroup were patients with third ventricular tumours (35%), of whom 86% were successfully treated. Mean time to failure for the whole series was 8.5 months (range immediate--30 months). Complications occurred in 17.5%; those deemed serious in 11%. There were three deaths (4.7%) within 30 days of the procedure. There were six other deaths during follow-up, five because of tumour progression and one because of pneumonia. CONCLUSIONS This procedure lends itself to the treatment of hydrocephalus in adults and appears to be more successful than in young children. It is efficacious in both previously shunted and non shunted patients. It is now the first-line treatment for noncommunicating hydrocephalus in this center and also for patients with shunt failure who are anatomically suitable, having cerebrospinal fluid spaces large enough to admit the endoscope. The complication and mortality rates compare favorably with those for shunts.

The role of neuroendoscopy in the management of brain tumours

Neuroendoscopy is increasingly used in the management of brain tumours and tumour related hydrocephalus and this study reviews the efficacy of neuroendoscopic interventions in this unit in patients with brain tumours. A series of 87 neuroendoscopic operations carried out in 77 patients with brain tumours over a 6-year period is reported. The age range of the patients was from 5 months to 70 years (median 13 years). In 56 cases (64%) presentation was with a newly-diagnosed tumour and hydrocephalus. The majority of the remaining patients had var ying degrees of worsening hydrocephalus on the background of a previously diagnosed tumour. Neuroendoscopic third ventriculostomy (NTV) was successful in relieving hydrocephalus in the short term in 63/66 cases (95%) and in the longer term in 55/66 cases (83%). Neuroendoscopic tumour biopsies were successful in providing a tissue diagnosis in 17/28 cases (61%) and four extensive and three partial resections of tumour were carried out. There were two deaths within 30 days of the procedure with only one of these, secondary to intraventricular haemorrhage, directly related to neuroendoscopy. Few significant complications were noted otherwise. For selected intraventricular and paraventricular tumours neuroendoscopy offers the opportunity to combine relief of hydrocephalus with tumour biopsy and sampling of CSF in a single procedure.

Cerebral contusional tears as a marker of child abuse--detection by cranial sonography

A series of 6 infants subjected to child abuse is presented in whom contusional tears of subcortical white matter were detected during life by intracranial sonography. The sonographic appearances of this highly pathognomonic marker of shaking injury are described for the first time and their significance discussed. On the basis of our experience we suggest that high resolution cranial sonography is an extremely valuable part of the diagnostic work up in cases of suspected non-accidental injury.

Diffuse brain stem glioma. A review of stereotactic biopsies.

Abstract The diagnosis and management of diffuse brain stem gliomas (DBSGs) remain a challenging problem for the neurosurgeon and neuro-oncologist. Opposing views on the necessity for biopsy have emerged over the last decade. Open biopsy, with its prohibitive morbid-ity and mortality, has been replaced by stereotactically guided biopsy, with markedly reduced risk. This has been paralleled by improvements in imaging techniques and diagnostic accuracy, which has created reluctance to endorse diagnostic biopsies coupled with the potential of nonrepresentative samples from a heterogeneous tumour mass. For typical DBSGs biopsy is now accepted as unnecessary. We performed a retrospective analysis of radiologically and histologically proven DBSGs in 18 children to assess both morbidity and reliability of our stereotactically guided biopsy procedure.

Neuroendoscopy in the premature population

Abstract The population born prematurely is particularly prone to hydrocephalus. Shunting techniques, whilst still the gold standard, have considerable failure rates and contribute significant morbidity and mortality. The role of neuroendoscopic techniques in the treatment of such patients is explored, and a series of 19 patients born prematurely and operated on neuroendoscopically before their 1st birthdays is described.

Primary postoperative chemotherapy without radiotherapy for treatment of brain tumours other than ependymoma in children under 3 years: results of the first UKCCSG/SIOP CNS 9204 trial.

BACKGROUND Radiotherapy is an effective adjuvant treatment for brain tumours arising in very young children, but it has the potential to damage the childs developing nervous system at a crucial time - with a resultant reduction in IQ leading to cognitive impairment, associated endocrinopathy and risk of second malignancy. We aimed to assess the role of a primary chemotherapy strategy in avoiding or delaying radiotherapy in children younger than 3 years with malignant brain tumours other than ependymoma, the results of which have already been published. METHODS Ninety-seven children were enrolled between March 1993 and July 2003 and, following diagnostic review, comprised: medulloblastoma (n=31), astrocytoma (26), choroid plexus carcinoma [CPC] (15), CNS PNET (11), atypical teratoid/rhabdoid tumours [AT/RT] (6) and ineligible (6). Following maximal surgical resection, chemotherapy was delivered every 14 d for 1 year or until disease progression. Radiotherapy was withheld in the absence of progression. FINDINGS Over all diagnostic groups the cumulative progression rate was 80.9% at 5 years while the corresponding need-for-radiotherapy rate for progression was 54.6%, but both rates varied by tumour type. There was no clear relationship between chemotherapy dose intensity and outcome. Patients with medulloblastoma presented as a high-risk group, 83.9% having residual disease and/or metastases at diagnosis. For these patients, outcome was related to histology. The 5-year OS for desmoplastic/nodular medulloblastoma was 52.9% (95% confidence interval (CI): 27.6-73.0) and for classic medulloblastoma 33.3% (CI: 4.6-67.6); the 5-year EFS were 35.3% (CI: 14.5-57.0) and 33.3% (CI: 4.6-67.6), respectively. All children with large cell or anaplastic variants of medulloblastoma died within 2 years of diagnosis. The 5-year EFS for non-brainstem high-grade gliomas [HGGs] was 13.0% (CI: 2.2-33.4) and the OS was 30.9% (CI: 11.5-52.8). For CPC the 5-year OS was 26.67% (CI: 8.3-49.6) without RT. This treatment strategy was less effective for AT/RT with 3-year OS of 16.7% (CI: 0.8-51.7) and CNS PNET with 1-year OS of 9.1% (CI: 0.5-33.3). INTERPRETATION The outcome for very young children with brain tumours is dictated by degree of surgical resection and histological tumour type and underlying biology as an indicator of treatment sensitivity. Overall, the median age at radiotherapy was 3 years and radiotherapy was avoided in 45% of patients. Desmoplastic/nodular sub-type of medulloblastoma has a better prognosis than classic histology, despite traditional adverse clinical features of metastatic disease and incomplete surgical resection. A subgroup with HGG and CPC are long-term survivors without RT. This study highlights the differing therapeutic challenges presented by the malignant brain tumours of early childhood, the importance of surgical approaches and the need to explore individualised brain sparing approaches to the range of malignant brain tumours that present in early childhood.

Liliequist's membrane in minimally invasive endoscopic neurosurgery

Liliequists membrane, an arachnoid condensation extending from the upper border of the dorsum sellae to the anterior edge of the mammillary bodies and formerly a relatively insignificant structure, has been found to be extremely important in the neuroendoscopic management of hydrocephalus. Failure to open this membrane can lead to the failure of third ventriculostomies. Clin. Anat. 11:187–190, 1998.

Clinical management of brain stem glioma.

Brain and spinal tumours in childhood (0–15 years) account for 20–25% of childhood cancer, affecting one in 2500 children. In the UK, this means that about 350 new cases are diagnosed each year, of which 55% and 50% survive five and 10 years, respectively.1 For those who are survivors, about 60% have cognitive deficits and 20–30% have difficulties with mobility and chronic pain.2 Deaths from this group of tumours in England and Wales account for the loss of over 10 000 life years each year (C Stiller, personal communication, 1995).1 Within the group of brain and spinal tumours there are a number of well defined categories with characteristic clinical presentations, biological behaviour, and suitability for specific treatment approaches. These factors combine to predict a range of outcomes from the highly curable tumours (> 80% 10 year survival rate) such as germinoma and cerebellar astrocytoma, through to the virtually incurable brain stem glioma.1 Age at presentation is also a crucial factor affecting both prognosis and treatment selection, especially for those who develop tumours early in life before the brain has completed growth and development.3 Brain stem gliomas account for about 10% of all brain and spinal tumours.1 The selection of brain stem glioma for this article was prompted by difficulties surrounding its clinical diagnosis, both professional and lay uncertainty about optimal surgical management, ineffectiveness of non-surgical treatment, clinical difficulties with good palliative care, and a historical lack of clear guidance about optimal referral patterns to children’s cancer centres, where appropriate resources have already been centralised for children’s cancers of other organs.4 5 We define the brain stem as extending from the midbrain (tectal plate) to the medullary cervical junction (fig 1A). Therefore, brain stem glioma is a term describing a collection of anatomically related tumours …