Executive Function and Psychosocial Adjustment in Adolescent Survivors of Pediatric Brain Tumor.

Adolescent survivors of pediatric brain tumor (PBT) are a sparsely studied subset of childhood cancer survivors. Sustaining a PBT may complicate the development of executive functions (EFs), which play a vital role in long-term psychosocial adjustment. In this study, 48 adolescent survivors and their parents completed questionnaires assessing EF, psychological symptoms, fatigue, and adaptive functioning, and 26 survivors underwent neuropsychological assessment. Survivors reported significantly more problems with adaptive functioning than a healthy control group, and this was most strongly associated to executive dysfunction, compared to psychological symptoms and fatigue. The findings have important implications for long-term follow-ups.

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.

Midface distraction osteogenesis: internal vs. external devices.

This study compares internal and external distraction devices in the treatment of midface retrusion. 20 patients were treated with midface distraction (12 Crouzon, 4 Apert, 4 others); 12 with internal distraction (MID device), 8 with external distraction (Red or Blue device). The two groups were compared regarding operation time, peroperative blood loss and complications. The groups were comparable regarding patient age, sex, weight and diagnosis. In the MID-group, 7 of 12 patients (58%) underwent Le Fort III, 5 underwent 12 monobloc (32%). In the Blue device group, three of eight patients underwent Le Fort II (38%), three of eight underwent Le Fort III (38%), and two of eight underwent monobloc (25%). Operation time was shorter in the Blue device (mean 298 min) than in the MID group (mean 354 min). Peroperative blood loss and complication rates were similar. The internal distraction device is the 'gold standard' for treating midface retrusion. The use of an external distraction device in midface distraction osteogenesis is associated with a shorter operation time; peroperative blood loss and complications were similar. An external device affords better 3-dimensional control during the distraction process, so external distraction is preferable in patients who will tolerate this treatment.

Is intracranial pressure waveform analysis useful in the management of pediatric neurosurgical patients?

BACKGROUND: We have reported casuistic observations that intracranial pressure (ICP) waveform analysis may be useful in the management of pediatric patients. METHOD: We here report our whole patient material of 65 children undergoing ICP monitoring with storage of their ICP raw data files during the years 2002-2005. We retrospectively explored the clinical symptoms/findings and radiological cerebral ventricular size before ICP monitoring. Mean ICP was the actively treated ICP parameter. Using an algorithm for identification of cardiac-beat-induced pressure waves we retrospectively determined the mean ICP wave amplitude and latency, in addition to mean ICP. Outcome with regard to change in symptoms/findings during a 1-year period was determined in 55 children. RESULTS: The material includes children with hydrocephalus, craniosynostosis, shunt failure, benign intracranial hypertension and others. The ICP recordings gave wrong diagnostic information due to bad signal quality in 5 of 65 patients (7.7%). The mean ICP wave amplitude was increased in those with papilledema, lethargy and nausea. The main observations were that the mean ICP wave amplitude (not mean ICP) was increased in those that improved from clinical symptoms/findings after treatment and in those that were unchanged/worse after not being treated. CONCLUSIONS: Waveform analysis with computation of the mean ICP wave amplitude was more useful by providing information about the quality of the ICP recording, by comparing better with the symptoms/findings at the time of ICP monitoring and by best predicting outcome. Most significantly, 14 of 55 patients (25%) with high amplitudes and left untreated did not recover spontaneously.

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.

[Metotopic craniosynostoses]. / Metopisk kraniosynostose.

BACKGROUND: Premature closure of the metopic suture leads to inhibited growth of the frontal bones, producing a keel-shaped forehead (trigonocephaly). Simple metopic synostosis is usually sporadic. Trigonocephalic patients account for 8-16% of the referrals to craniofacial centers, with a marked male predominance. Intracranial pressure (ICP) may be increased, whereas shunt-dependent hydrocephalus is infrequent. Infrequently, patients have intra- or extracerebral anomalies; one third have varying degrees of neuropsychological problems. The treatment is primarily surgical. MATERIAL AND METHODS: We present two patients who during infancy developed increasingly keel-shaped foreheads, retruded orbital rims, increased biparietal diameter and close-set eyes (hypothelorism). Both had raised ICP, but normal psychomotoric development. They were operated using radical fronto-orbital surgical remodelling. RESULTS: Recovery was uneventful. Three months post-operatively, they had pleasing cosmetic results with no symptoms of increased ICP. INTERPRETATION: Where metopic craniosynostosis is suspected, the infant should be examined clinically with palpation of fontanelles and sutures, evaluated with respect to the shape and development of the facial skeleton, as well as by X-ray of the skull sutures. Radical fronto-orbital surgical remodelling gives a stable correction of the craniofacial deformity and generally a satisfactory cosmetic result.

[Distraction osteogenesis--a new therapeutic principle in complex craniofacial synostosis]. / Distraksjonsosteogenese--et nytt behandlingsprinsipp ved komplekse kraniofaciale synostoser.

BACKGROUND: Surgical treatment of syndromal craniofacial synostosis consists of combined neurosurgical and maxillofacial reconstructions of the neurocranium and midface. Patients often need several operations if they are to achieve a functionally and cosmetically good result. This is, in part, due to limitations in the amount of "acute" correction possible peroperatively with conventional surgical technique. MATERIAL AND METHODS: In recent years, a new method called distraction osteogenesis has been developed, by which the skeletal elements are gradually advanced. The method is based upon a process whereby the reparative callus formed between the surfaces of two bone segments is subjected to traction. This leads to new bone formation parallel to the vector of distraction. RESULTS: Distraction osteogenesis allows for greater mobilizations, hence the promise of fewer reoperations. INTERPRETATION: The method seems to be a valuable treatment modality in syndromal craniofacial synostosis, where major fronto-orbital, midface or maxillary reconstructions are often called for.

Monobloc and midface distraction osteogenesis in pediatric patients with severe syndromal craniosynostosis.

We present 2 children with severe syndromal craniosynostosis who were in need of urgent midface advancement surgery due to recurrent ocular dislocations (Pfeiffer's syndrome type II) or severe upper respiratory obstruction (Crouzon's syndrome). They were operated using distraction osteogenesis, with gradual midface or monobloc advancements. In the Pfeiffer patient, a maxillary distraction of 25 mm achieved effective cessation of ocular dislocations, whereas a 23-mm monobloc advancement in the Crouzon patient achieved cessation of nocturnal arterial desaturations. No major postoperative complications were recorded. Distraction osteogenesis has become a versatile and safe technique that allows for large skeletal advancements.

[Frontoethmoidal meningoencephaloceles]. / Frontoetmoidale meningoencefaloceler.

Frontoethmoidal meningoencephalocele is a rare congenital disease where intracranial masses protrudes through a midline defect at foramen coecum from the anterior cranial fossa into the facial skeleton. The patient presents at birth with a soft-tissue swelling at the nasal bridge. In the majority of cases, the cele is completely covered by skin. The face is elongated, the nasal bridge is broad, the upper part of the nose is long, and the patients have telecanthus. Frequently, the patients have a normal psychomotor development, but may have intracerebral malformations and a shunt dependent hydrocephalus. Spinal fluid leakage may result in meningitis. Anosmia is infrequent. The patients may have a wide range of ophthalmological problems. Newborns with meningoencephalocele should be examined by a neurosurgeon. The investigation and work-up commences with a multidisciplinary approach. We present two patients with meningoencephaloceles.

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.

Efflux of gamma-aminobutyric acid caused by changes in ion concentrations and cell swelling simulating the effect of cerebral ischaemia.

The relationships among ischaemic GABA efflux from brain tissue and extracellular and intracellular concentrations of sodium, chloride and potassium ions were investigated by means of 1) transverse hippocampal slices from rat and 2) functional expression of a high affinity GABA transporter in Xenopus oocytes. Brain slices were incubated for 20 min in medium where extracellular sodium and chloride were substituted with impermeant ions. Isethionate (Iseth) substitution for chloride generated a 7-fold increase in GABA efflux. Choline (Chol) but not N-methyl-D-glucamine (NMDG) substitution for sodium likewise increased GABA efflux. Reducing the osmolarity of the medium by decreasing both sodium and chloride concentrations (Hyp) increased GABA efflux 3-fold. This release was blocked by mannitol (Man). Blocking sodium channels with 1 microM of tetrodotoxin (TTX) also increased the release 3-fold. Energy deprivation (ED) increased the GABA release 50-fold. ED/Iseth left the release unchanged, ED/Chol increased the GABA efflux by 23%, whereas ED/NMDG reduced the release by 41%. Adding mannitol did not block the ED-evoked release, whereas TTX reduced it by 52%. Release of preloaded [3H]-GABA from oocytes expressing the GAT-1 GABA transporter was then examined. Depolarisation by current injection or 100 mM extracellular K+ did not increase GABA release. Sodium chloride injection, however, caused membrane depolarisation and a 100-fold increased GABA efflux from the oocytes. This release was blocked when the osmolarity was increased extracellularly by adding mannitol. These results show that 1) TTX releases GABA from brain tissue but blocks release during ED, 2) the high affinity GABA carrier must be altered in order to reverse, 3) ischaemic GABA release is sodium independent, and is modulated by large cations, 4) mannitol blocks the reversal of high affinity carriers in oocytes, but the release from brain slices during ED is unaffected. Taken together, the results suggest that ischaemic release of GABA from brain tissue does not occur by means of reversed high affinity carriers alone, but rather that it is controlled by more complex mechanisms.

Modified Robinson-Smith procedure for the treatment of cervical radiculopathy.

In the present study, a modified Robinson-Smith procedure was used surgery for cervical radiculopathy in 52 patients. Thirty-one one-level and 21 two-level operations were performed. All patients were followed up between 1 and 3 years (mean 23 months) after surgery with a clinical evaluation by an independent investigator including a radiological examination. In 26 patients the postoperative result was classified as excellent, in 23 the result was good, in 2 satisfactory, while one patient was unchanged compared to the preoperative examination. No patient developed worsening of symptoms after surgery. Of 18 patients with duration of symptoms of more than 4 years, 16 demonstrated markedly improvement. No permanent postoperative complications were seen. A modified Robinson-Smith procedure appears to be safe and reliable and can be recommended in surgery for cervical radiculopathy.