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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.

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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.

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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.

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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.

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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.

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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.

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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.

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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.

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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.

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