Long-term supratentorial brain structure and cognitive function following cerebellar tumour resections in childhood.

The cerebellum is connected to extensive regions of the cerebrum, and cognitive deficits following cerebellar lesions may thus be related to disrupted cerebello-cerebral connectivity. Moreover, early cerebellar lesions could affect distal brain development, effectively inducing long-term changes in brain structure and cognitive function. Here, we characterize supratentorial brain structure and cognitive function in 20 adult patients treated for cerebellar tumours in childhood (mean age at surgery: 7.1 years) and 26 matched controls. Relative to controls, patients showed reduced cognitive function and increased grey matter density in bilateral cingulum, left orbitofrontal cortex and the left hippocampus. Within the patient group, increased grey matter density in these regions was associated with decreased performance on tests of processing speed and executive function. Further, diffusion tensor imaging revealed widespread alterations in white matter microstructure in patients. While current ventricle volume (an index of previous hydrocephalus severity it patients) was associated with grey matter density and white matter microstructure in patients, this could only partially account for the observed group differences in brain structure and cognitive function. In conclusion, our results show distal effects of cerebellar lesions on cerebral integrity and wiring, likely caused by a combination of neurodegenerative processes and perturbed neurodevelopment.

Predictors of headache 22 years after hospitalization for head injury.

OBJECTIVES: To assess predictors of headache in patients who had sustained a head injury 22 years earlier. MATERIALS AND METHODS: A questionnaire about headache was sent to 361 subjects hospitalized for head injury in 1974-1975. RESULTS: A total of 249 patients (69%) responded to the questionnaire. The prevalence of headache >14 days a month last year was 11%. In multivariate logistic regression analysis female sex (OR = 3.4, 95% CI 1.2-9.6), severe headache 3 months after the head injury (OR = 10.6, 95% CI 2.6-43.5) and psychiatric disease (OR = 2.9, 95% CI 1.1-7.7) predicted chronic headache. There was no significant association between chronic headache and post-traumatic amnesia or other trauma-related variables. CONCLUSION: Female sex and headache 3 months after the head injury were the strongest predictors of long-term headache, while there was little association between long-term headache and trauma variables.

Predictable reduction of intracranial hypertension with hypertonic saline hydroxyethyl starch: a prospective clinical trial in critically ill patients with subarachnoid haemorrhage.

BACKGROUND: After head trauma, hypertonic saline lowers intracranial pressure (ICP) and preserves or increases cerebral perfusion pressure (CPP). Hypertonic saline has not been studied in patients with increased ICP due to subarachnoid haemorrhage (SAH). The aim of this study was to evaluate the effects on elevated ICP and on CPP in patients critically ill from SAH. METHODS: Critically ill SAH-patients needing urgent treatment for an elevated ICP, but otherwise stable, were included in this study. We infused 7.2% saline in 6% hydroxyethyl starch (HyperHAES((R)) Fresenius Kabi AG, Bad Homburg v.d.h., Germany) 2 ml kg(-1) during 20 min in 10 episodes of ICP > 20 mmHg in seven patients with SAH. Our primary outcome variables were changes in ICP and CPP during and for 3 h after this infusion. RESULTS: All interventions resulted in decreased ICP and elevation of CPP. The mean value for maximum ICP decrease in percent of baseline was 58% (range 43-83%, P = 0.002), which occurred at mean 40 min (range 25-90 min) after start of infusion. The mean percent peak increase in CPP was 26% (range 16-32%, P = 0.002). After 210 min, ICP was 35% lower than baseline (range 19-39%, P = 0.008). Serum sodium increase was mean 6.6 mmol l(-1) (range 5-9 mmol l(-1)) 30 min after start of infusion. CONCLUSIONS: 7.2% saline in 6% hydroxyethyl starch is an effective and safe therapy for intracranial hypertension after SAH. We demonstrate that an infusion of 2 ml kg(-1) during 20 min has a predictable and clinically significant beneficial effect on ICP and CPP. The effect was still present 3 h after end of infusion. Rebound ICP-increase was not observed within 3 h.

Changes in intracranial pressure after calvarial expansion surgery in children with slit ventricle syndrome.

The effect of calvarial expansion on symptom relief and intracranial pressure (ICP) in three children with slit ventricle syndrome (SVS) and intracranial hypertension despite a functioning ventricular shunt is reported. These children presented with a clinical picture of SVS, accompanied by slit-like ventricles on cranial computer tomography scan and intracranial hypertension. Calvarial expansion was performed by mans of an anterior approach in one case and a posterior approach (modified tiara plastic) in the other two cases. After calvarial expansion, symptoms of intracranial hypertension were abolished in one case and markedly reduced in two cases (observation period 25-36 months). Comparison of ICP before and after surgery was performed by means of new software (Sensometrics Pressure Analyser, version 1.2) that revealed a significant reduction in the number of abnormal ICP elevations after surgery. The results were not accompanied by changes in the size of the cerebral ventricles. This study demonstrates that in children with SVS and intracranial hypertension despite a functioning shunt, calvarial expansion may reduce ICP and produce long-lasting symptom relief. In these cases, we suggest that intracranial hypertension was caused by compromised intracranial volume.

Assessment of intracranial pressure volume relationships in childhood: the lumbar infusion test versus intracranial pressure monitoring.

OBJECT: This study was undertaken to compare the results of two tests that are widely used to assess intracranial pressure-volume relationships in children: the lumbar steady state infusion test providing information about the resistance to cerebrospinal fluid (CSF) outflow (Rout), and continuous intracranial pressure (ICP) monitoring. METHODS: The study included 28 children aged 5-91 months, on whom both tests were performed. The median duration between the tests was 1 month. With the child in general narcosis, the lumbar CSF pressure was recorded before and during infusion of artificial CSF, and the Rout was calculated on the basis of the opening (Po) and plateau (Pp) pressures (Rout=Pp-Po/infusion rate). ICP was recorded every 5 s using a computer-based system. CONCLUSIONS: We found no significant correlation between Rout and mean ICP asleep. There were no significant relationships between abnormal mean ICPs during sleep (defined as either 10 or 15 mmHg) and abnormally high Rout values (defined as either 10 or 12 mmHg/ml/min), and no significant relationships between abnormally high Rout values (10 or 12 mmHg ml(-1) min(-1)) and the presence of abnormal ICP elevations (defined as either 20 or 25 mmHg and lasting 5 min). Therefore the calculation of Rout by the infusion test performed on children under general anesthesia gave no reliable prediction of abnormal ICP.

Choroid plexus tumors in children and young adults: report of 16 consecutive cases.

Choroid plexus tumors are rare intraventricular tumors, and they represent 2-4% of brain tumors in children. This single-institution retrospective study involves 16 consecutive choroid plexus tumors: 13 papillomas and 3 carcinomas. Tumor locations were the lateral ventricles in 13 cases, the third ventricle in 2 cases and the fourth ventricle in 1 case. The mean age at presentation was 3.1 years. Two patients died of perioperative blood loss. Five-year survival was 85% with papillomas and 33% with carcinomas. None of the papillomas recurred after total tumor resection, and the functional outcome in long-term survivors after papilloma surgery was excellent in 92% of the cases. Two of the carcinoma patients had disseminated disease. Fifty percent of the patients had persistent hydrocephalus after tumor resection, and these required cerebrospinal fluid diversion.

Ependymoma in children and young adults (0-19 years): report of 25 consecutive cases.

The objective of this retrospective study was to evaluate the relative effect of surgery and radiotherapy (RT) on the survival of 25 consecutive children and young adults treated for ependymoma [18 in the posterior fossa (PF), 2 in supratentorial locations (ST) and 5 in intraspinal locations (IS)]. Five-year survival rates for patients with PF, ST and IS tumors were 28%, 0% and 100%, respectively. Total tumor resection was a positive prognostic factor in the case of PF tumors. No patients with subtotal removal of a PF tumor survived for longer than 5 years. The effect of RT on survival of patients with PF ependymomas in this series was uncertain. No patients with PF ependymoma had disseminated disease at diagnosis, and all tumor recurrences were local. Based on these observations, we see no indications for craniospinal RT of PF ependymomas, except in rare cases of disseminated disease. If RT is given, it should only be targeted to the tumor site. The two patients with ST ependymoma died within 3.8 years after primary treatment. Our series of ST ependymomas does not allow any specific treatment recommendations. One patient with IS ependymoma was cured by surgery alone. Four patients with IS ependymoma had documented residual tumor after surgery. RT induced remission in these patients. For IS ependymomas we recommend no RT if total tumor removal can be documented. In patients with subtotal removal of IS ependymomas local RT is effective and should be given.

Neurosurgical treatment of meningiomas in children and young adults.

OBJECT: We studied the frequency, functional outcome, association with neurofibromatosis (NF) and relapse in patients operated on for meningiomas at age 0-20 years in three Norwegian centers between 1972 and 1999. METHODS: Information was collected by examining case notes and histology records, conducting telephone interviews and performing new radiological investigations. Twenty-seven patients were identified. Five had NF, and all 5 developed multiple tumors. In non-NF patients, relapse occurred in 2 out of 19 who underwent total tumor resection, and in all 3 whose surgery was non-radical. There was 1 postsurgical death, and no other major complications. Most patients had few complaints or none at all resulting from their previous tumor or treatment. CONCLUSIONS: Meningiomas without NF have a good prognosis in children and young adults if the tumor can be removed radically. However, relapse can occur many years after the primary operation.

[Neurosurgical shunt treatment of children with hydrocephalus]. / Nevrokirurgisk shuntbehandling av barn med hydrocephalus.

BACKGROUND: The aims of this study were to determine the ten-year outcome of hydrocephalic children, both in terms of academic results, social skills, physical functioning and surgical morbidity and mortality. MATERIAL AND METHODS: A cohort of 128 children shunted for hydrocephalus at the National Hospital in Oslo between 1985 and 1988 were retrospectively analysed by registering their medical records and in a questionnaire survey. RESULTS: We found that 23 (18%) children died during the ten-year observation period. Six patients died from shunt system failure, ten died from their malignant tumour, six died of their complex cerebral malformations and one died of unknown cause. Of the 105 (82%) children still alive, 104 returned our questionnaire. 92 (88%) reported to be attending a normal school, while 77 (74%) are in the same grade as children of similar age. Of those children at same grade level, 39 (51%) needed extra tutoring, while 35 (45%) reported speech and writing difficulties. Among children not in their adequate grade level, 25 (93%) (p < 0.01) needed extra tutoring, and 23 (85%) (p < 0.01) reported speech and writing difficulties. 50 (48%) participate in physical exercise along with the other children, while 53 (51%) report having normal social relations with children of the same age. 34 (33%) has suffered from epileptogenic seizures, while 24 (23%) daily use prescribed antiepileptic drugs. During the ten-year follow-up period, 108 (84%) patients had a total of 342 shunt revisions. We found no correlation between high incidence of revisions and negative long-term outlook. 99 out of the 105 children alive are considered to be life-long dependent of their shunt system. INTERPRETATION: The vast majority of the children shunted for hydrocephalus that are still alive, have a good or satisfactory level of functioning ten years after their first shunt insertion. This view seems to be shared by the parents of these children, of whom 50 (48%) state that the development of their child has been "very good" compared to what they initially feared.

[Long-term survival following surgery and radiotherapy of brain tumors in childhood]. / Langtidsoverlevelse etter kirurgi og strålebehandling av hjernesvulster i barnealder.

BACKGROUND: Brain tumours are seen in about one third of children with neoplastic disease. Treatment usually includes surgery and/or radiotherapy. Radiotherapy may have serious late effects, especially in children under the age of three; but is necessary for survival in children with medulloblastomas or high-grade gliomas. MATERIALS AND METHODS: We report ten and 20 years survival rates in 115 children with primary brain tumours (58 medulloblastoma, 14 high-grade gliomas, and 43 low-grade gliomas) operated at the National Hospital and given radiotherapy at the Norwegian Radium Hospital during the years 1970-1995. RESULTS: No patients with medulloblastomas or high-grade gliomas relapsed after ten years. Overall ten and 20 years survival in children treated with radiotherapy to tumour doses > 50 Gy for medulloblastoma was 51.5% and for high-grade gliomas 20%. Median survival for patients with low-grade gliomas was not reached at 20 years, but these patients were still at risk for late deaths. INTERPRETATION: Long-term survival in children with high-grade gliomas or medulloblastoma equals cure, while late relapses may occur in low-grade gliomas.

Posterior fossa medulloblastoma in children and young adults (0-19 years): survival and performance.

The objective of this study was to present survival data and outcome status in the long-term survivors of a consecutive series of 111 children and young adults treated for posterior fossa medulloblastoma in our departments from 1960 to 1997. The total surgical mortality was 13%. The surgical mortality rate declined significantly during the time period overall, from 23% before 1970 to 0% after 1990. The 5-year survival rate for patients treated between 1960 and 1973 was 0%, while 5-year survival for patients treated after the introduction of systematic craniospinal radiation in 1974 was 53%. Thirty-four patients were alive at the close of this study, with a mean observation time of 13.5 years. Over half, 61%, of the patients had one or more major deficits/problems with respect to learning ability, power of locomotion, sociability, hobbies and relationships with the opposite sex. A younger age at the time of treatment was correlated with larger deficits/problems in these variables. The correlation between young age at the time of treatment and short final height was significant. The frequency of a second neoplasm was 14%. In all but 3 cases the major cause of permanent deficits/problems was radiation therapy.

A tribute to Helge Nornes.

This supplement of the Acta Neurochirurgica is dedicated to professor Helge Nornes on the occasion of his retirement. Helge Nornes started his neurosurgical training in Oslo in 1965. In 1980 he was offered the neurosurgical chair of Bern, Switzerland, where he stayed until 1983 when his old university called him back to the chair at the National Hospital in Oslo, a position he filled until he retired last year. The present paper briefly reviews examples of his contributions to neurosurgery and to the understanding of intracranial pathophysiology, including the transcranial doppler, the miniature transducer for intracranial pressure monitoring, his observations on intracranial pressure and internal carotid blood flow during subarachnoid haemorrhage, intracranial arterial blood flow in patients undergoing aneurysm surgery, his studies of the pathophysiology of arteriovenous malformations, the introduction of intraoperative Doppler recordings during surgery for aneurysms and arteriovenous malformations, and his methods for evaluating collateral circulation prior to internal carotid artery occlusion.

[Continuous intrathecal infusion of baclofen. A new therapeutic method for spasticity]. / Kontinuerlig intratekal infusjon med baklofen. Ny behandlingsmetode for spastisitet.

Intrathecal administration of baclofen is now generally accepted as a powerful treatment of spasticity caused by spinal lesions. 35 patients with severe spasticity, 29 of spinal origin and six of supraspinal origin resistant to conservative treatment, had a programmable pump (Synchromed, Medtronic) for continuous intrathecal baclofen infusion implanted. The patients were followed-up for an average of 29 months (0-68). The initial effect of the treatment was positive for all patients; spasms were less frequent, there was remission of pain caused by cramps, and in some cases improved ambulation. In five patients, however, the pump was later removed: in two patients the pump ceased to be effective, two patients became infected, and one experienced multiple catheter problems. Problems with the catheter was the most common complication experienced, and this was seen in nine patients. Three patients died of the underlying disease. The majority of patients became accommodated to intrathecal baclofen and it was necessary to administer increasingly larger doses to maintain the clinical effect. Long-term control of spinal spasticity by intrathecal baclofen can be achieved in most patients, but close follow-up is necessary for assessing efficacy and refilling the pump.

[Spinal puncture in suspected purulent meningitis]. / Spinalpunksjon ved mistanke om purulent meningitt.

Today, CT scan of the brain is performed as a routine a the first diagnostic procedure in cases of suspected acute subarachnoid haemorrhage. Subarachnoid blood is demonstrated in a high percent of these cases provided that the scan is carried out within the first few days after the bleeding. Furthermore, the scan may also disclose localised intracranial haematoma or other expansive lesions with brain shift. In such cases, lumbar puncture may be dangerous, due to the risk of herniation. In cases of suspected acute bacterial meningitis or other infectious disease of the central nervous system, performance of a CT scan before lumbar puncture is not a well established procedure. Two case histories are described. Both patients had brain abscesses and deteriorated dramatically after lumbar puncture. The role of brain CT scanning in the management of such patients is discussed.

Shunt removal or replacement based on intraventricular infusion tests.

In 14 children with indwelling ventriculoatrial or ventriculoperitoneal shunts, the need for continued shunt treatment was judged to be uncertain based on clinical symptoms and signs and CT scans. Ventricular outflow resistance (R0) was determined by implantation of a ventricular catheter and steady state infusion of artificial cerebrospinal fluid (CSF) according to the formula R0 = (Pp - P0)/Infusion rate, where P0 is the opening pressure in the lateral ventricle and Pp the plateau pressure recorded at that particular infusion rate. R0 was determined during general anesthesia and steady state ventilation was ensured by mechanical ventilator. Ventricular fluid pressure (VFP) and arterial blood pressure (ABP) were recorded by standard fluid pressure transducers. The cerebral perfusion pressure (CPP = ABP - VFP) was kept above 30 mmHg by reducing the infusion rate in cases of unacceptable increase in VFP. R0 was determined with the shunt clamped. During steady state infusion at the plateau pressure the shunt was unclamped to test shunt patency. Four children had normal R0 values with the shunt clamped. Their shunts were removed. They have done well clinically, and control CT scans have not demonstrated increased ventricular size. Three children demonstrated pathologic R0 values (above 12 mmHg/ml per min) that normalized after shunt unclamping; i.e. each had a well-functioning shunt. Seven children demonstrated increased R0 values even after shunt unclamping. Their shunts were replaced, and clinical improvement has been observed in 6 of them. Ventricular infusion tests appear useful to evaluate shunt dependence and function in difficult cases.

Tissue expansion for the treatment of myelomeningocele. Case report.

A baby boy who had been born with a large myelomeningocele had a ventriculoperitoneal shunt inserted at the age of 6 months. Two months later it was revised, after which he developed satisfactorily both mentally and physically. He presented at the age of 13 months because the epithelialisation of the myelomeningocele was both fragile and incomplete. Enough soft tissue was obtained to cover the defect, which was 13 x 10 cm, by the use of two 600 ml expanders placed subcutaneously one on either side of the deformity.