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Background: In non-walking children with severe spasticity, daily care can be difficult and many patients suffer from pain. Selective dorsal rhizotomy (SDR) reduces spasticity in the legs, and therefore has the potential to improve daily care and comfort., Aim: To examine effects of SDR on daily care and comfort in non-walking children with severe spasticity due to different underlying neurological conditions., Methods: Medical history, changes in daily care and comfort and satisfaction with outcome were assessed retrospectively in non-walking children who underwent SDR in our center, with a mean follow-up of 1y 7m (range 11m-4y 3m). All eligible patients (n = 24, years 2009-2014) were included., Results: Mean age at SDR was 12y 4m (SD 4y 3m, range 2y 8m-19y 3m). Associated orthopaedic problems were frequent. Seven patients underwent scoliosis correction in the same session. Most improvements were reported in dressing (n = 16), washing (n = 12) and comfort (n = 10). Median score for satisfaction was 7 on a scale of 10 (range 1-9). SDR resulted in reduction of spasticity in leg muscles. In nine patients dystonia was recorded post-operatively, mainly in children with congenital malformations and syndromes., Interpretation: SDR is a single event intervention that can improve daily care and comfort in non-walking children with severe spasticity, and can safely be combined with scoliosis correction. Despite the improvements, satisfaction is variable. Careful attention is necessary for risk factors for dystonia, which may be unmasked after SDR., (Copyright © 2016 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.)

Purpose: Very long-term follow-up and outcome are rare for pediatric patients with hydrocephalus and shunt operations. The aim of this study was to determine the long-term mortality rates in these patients., Methods: Pediatric patients with first shunt operation between 1982 and 1992 were included. For each patient, time and cause of death were determined. Further, patients with first operation from 1982 to 1987 were compared to those first operated from 1988 to 1992., Results: One-hundred thirty-seven patients were included. Etiologies of hydrocephalus were intraventricular hemorrhage (31.4 %), meningomyelocele (25.5 %), postinfectious (11.7 %), congenital (10.2 %), posterior fossa cyst (8.8 %), aqueductal stenosis (8 %), and others (4.4 %). Overall, 53 patients (38.7 %) died. The percentage of patients surviving 1, 2, 10, and 20 years after first operation were 82.6, 73.6, 69.4, and 65.3 %, respectively. In 23 patients, the cause of death was related to shunt treatment: shunt infection was diagnosed in 18 and acute shunt dysfunction in 5 patients. Mortality was considerably higher for patients with their first operation in time period 1982-1987 compared to time period 1988-1992 (51 versus 25 %). The reduction of mortality was mainly due to an increased survival after shunt infection. Eighty-seven patients survived more than 20 years after initial shunt operation. Of those long-term survivors, three (3.4 %) patients died 22-24 years after first operation., Conclusion: Mortality in hydrocephalic pediatric patients is high especially in the first postoperative years but is even significant in adult patients with pediatric hydrocephalus. As deaths occur even after 20 years, routine follow-up of long-term survivors remains necessary.

Selective dorsal rhizotomy (SDR) surgery is a well-established treatment for ambulatory children with bilateral spastic paresis and is performed to eliminate spasticity and improve walking. The objective of this case report is to describe sudden falls as a persistent complication of SDR. The authors report on 3 patients with bilateral spastic paresis, aged 12, 6, and 7 years at the time of surgery. The percentage of transected dorsal rootlets was around 40% at the L2-S1 levels. Sudden falls were reported with a frequency of several a day, continuing for years after SDR. The falls were often triggered by performing dual tasks as well as occurring in the transition from sitting to standing, during running, after strenuous exercise, or following a fright. Patients also had residual hyperesthesia and dysesthesia of the foot sole. The authors hypothesize that the sudden falls are caused by a muscle inhibition reflex of the muscles in the legs, as an abnormal reaction to a sensory stimulus that is perceived with increased intensity by a patient with hyperesthesia. A favorable effect of gabapentin medication supports this hypothesis.

Purpose: An accessory to suprascapular nerve (XIN-SSN) transfer is considered in patients with obstetric brachial plexus lesion who fail to recover active shoulder external rotation. The aim of this study was to evaluate the quality of extraplexal suprascapular nerve neurotization and to perform a detailed analysis of the infraspinatus muscle (IM) and shoulder external rotation., Methods: A XIN-SSN transfer was performed in 14 patients between 2000 and 2007. Patients had been operated at the age of 3.7 ± 2.8 years. Follow-up examinations were conducted up to 8.5 ± 2.5 years. Magnetic resonance imaging was performed to investigate muscle trophism. Fatty muscle degeneration of the IM was classified according to the Goutallier classification. We conducted nerve conduction velocity studies of the suprascapular nerve and needle electromyography of the IM to assess pathologic spontaneous activity and interference patterns. Active glenohumeral shoulder external rotation and global shoulder function were evaluated using the Mallet score., Results: Postoperatively, growth of the IM increased equally on the affected and unaffected sides, although significant differences of muscle thickness persisted over time. There was only grade 1 or 2 fatty degeneration pre- and postoperatively. Electromyography of the IM revealed a full interference pattern in all except one patient, and there was no pathological spontaneous activity. Glenohumeral external rotation as well as global shoulder function increased significantly., Conclusion: Our results indicate that the anastomosis after XIN-SSN transfer is functional and that successful reinnervation of the infraspinatus muscle may enable true glenohumeral active external rotation.

Background: Dystonic cerebral palsy is primarily caused by damage to the basal ganglia and central cortex. The daily care of these patients can be difficult due to dystonic movements. Intrathecal baclofen treatment is a potential treatment option for dystonia and has become common practice. Despite this widespread adoption, high quality evidence on the effects of intrathecal baclofen treatment on daily activities is lacking and prospective data are needed to judge the usefulness and indications for dystonic cerebral palsy. The primary aim of this study is to provide level one clinical evidence for the effects of intrathecal baclofen treatment on the level of activities and participation in dystonic cerebral palsy patients. Furthermore, we hope to identify clinical characteristics that will predict a beneficial effect of intrathecal baclofen in an individual patient., Methods/design: A double blind placebo-controlled multi-center randomized clinical trial will be performed in 30 children with dystonic cerebral palsy. Patients aged between 4 and 25 years old with a confirmed diagnosis of dystonic cerebral palsy, Gross Motor Functioning Classification System level IV or V, with lesions in the cerebral white matter, basal ganglia or central cortex and who are eligible for intrathecal baclofen treatment will be included. Group A will receive three months of continuous intrathecal baclofen treatment and group B will receive three months of placebo treatment, both via an implanted pump. After this three month period, all patients will receive intrathecal baclofen treatment, with a follow-up after nine months. The primary outcome measurement will be the effect on activities of and participation in daily life measured by Goal Attainment Scaling. Secondary outcome measurements on the level of body functions include dystonia, spasticity, pain, comfort and sleep-related breathing disorders. Side effects will be monitored and we will study whether patient characteristics influence outcome., Discussion: The results of this study will provide data for evidence-based use of intrathecal baclofen in dystonic cerebral palsy.

Aim: The aim of this study was to evaluate the long-term effect of selective dorsal rhizotomy (SDR) on the gross motor function of ambulant children with spastic bilateral cerebral palsy (CP), compared with reference centiles., Method: The study used a prospective cohort design and participants comprised 29 children classified using the Gross Motor Function Classification System (GMFCS) in level I (n=7), II (n=4), or III (n=18; 18 males, 11 females; median age at time of surgery 6 y 4 mo; range 2 y 10 mo-12 y 1 mo), who were examined 5 years and 10 years after SDR. We used individual centiles based on Gross Motor Function Measure (GMFM-66) scores and age, corresponding to the GMFCS levels. Individual improvement or deterioration was defined as a change of more than 20 centiles. Side effects experienced and additional treatment received after SDR were also recorded., Results: Five years after SDR, 10 out of 28 children showed improvement, and 10 years after SDR 6 out of 20 children had improved. Spinal side effects were noted in two children and hip subluxation in three. Additional treatments included subtalar arthrodesis (n=13), endorotational osteotomy of the tibia (n=5), and botulinum toxin treatment (n=13)., Interpretation: None of the children showed deterioration of gross motor function based on centile ranking. Five and 10 years after SDR, gross motor function in some children had improved more than would have been expected according to the reference centiles. This suggests, taking the limitations of this study into account, that the applied criteria for selection were adequate. However, the children still required additional treatment after SDR., (© 2013 Mac Keith Press.)

We present a 41-year-old man with severe traumatic brain injury. Cranial imaging studies revealed cerebral contusion and a longitudinal fracture of the temporal bone. Several days later brain herniated into the left external auditory canal. Imaging studies showed the known skull fracture with a direct connection between the external acoustic meatus and the intracranial structures.

Aim: Denervation after obstetric brachial plexus lesion (OBPL) is associated with reduced musculoskeletal growth in the upper arm. The aim of this study was to investigate whether reduced growth of upper arm flexor and extensor muscles is related to active elbow function and humeral length., Method: In this study, 31 infants age less than 6 months (mean age 4.3mo; range 2.1-5.9mo; 17 males; 14 females;) with unilateral OBPL (Narakas class I, 19; II, 3; III, 2; and IV, 7) treated at the VU medical centre, in whom neurosurgical reconstruction was considered were prospectively studied using magnetic resonance imaging of both arms at a mean age of 4.3 months. Humeral length and the cross-sectional area (CSA) of elbow flexor and extensor muscles were measured in both upper arms. Paresis of elbow function was estimated when the infants were a mean age of 4.5 months using the Gilbert score., Results: Both flexor and extensor CSAs were significantly smaller on the affected side than on the unaffected side (88% [SD 32%], p=0.020, and 88% [SD 24%], p=0.001 respectively), as was humeral length (96% [SD 7%], p=0.005) (unaffected side 100% in all cases). There was no relation between the reduction in flexor and extensor CSA and residual muscle function. In 17 out of 31 patients, hypertrophy of flexor and/or extensor muscles was observed. Humeral length was not related to muscle parameters., Interpretation: Denervation has different effects on muscle growth and function as well as bone growth. In young infants with an OBPL, muscle size is not a predictor of muscle function. Flexion contractures of the elbow later in childhood may not be explained by a dominance of flexor muscle mass in infants., (© The Authors. Developmental Medicine & Child Neurology © 2012 Mac Keith Press.)

Objective: The purpose of this study was to review the epidemiology and the clinical, radiological, pathological, and follow-up data of all surgically treated pediatric meningiomas during the last 35 years in The Netherlands., Methods: Patients were identified in the Pathological and Anatomical Nationwide Computerized Archive database, the nationwide network and registry of histopathology and cytopathology in The Netherlands. Pediatric patients of 18 years or younger at first operation in 1974-2009 with the diagnosis meningioma were included. Clinical records, follow-up data, radiological findings, operative reports, and pathological examinations were reviewed., Results: In total, 72 patients (39 boys) were identified. The incidence of operated meningiomas in the Dutch pediatric population is 1:1,767,715 children per year. Median age at diagnosis was 13 years (range 0-18 years). Raised intracranial pressure and seizures were the most frequent signs at presentation. Thirteen (18 %) patients had neurofibromatosis type 2 (NF2). Fifty-three (74 %) patients had a meningioma World Health Organization grade I. Total resection was achieved in 35 of 64 patients. Fifteen patients received radiotherapy postoperatively. Mean follow-up was 4.8 years (range 0-27.8 years). Three patients died as a direct result of their meningioma within 3 years. Four patients with NF2 died as a result of multiple tumors. Nineteen patients had disease progression, requiring additional treatment., Conclusion: Meningiomas are extremely rare in the pediatric population; 25 % of all described meningiomas show biological aggressive behavior in terms of disease progression, requiring additional treatment. The 5-year survival is 83.9 %, suggesting that the biological behavior of pediatric menigiomas is more aggressive than that of its adult counterparts.

Background: Intrathecal baclofen (ITB) treatment is frequently used for individuals with severe, but non-progressive, spasticity refractory to oral treatment. However, experiences with ITB in patients with progressive neurological disorders of childhood causing spasticity are limited., Aim: To investigate whether ITB is an option in patients with progressive neurological disorders causing spasticity in childhood., Design: A systematic literature search in Embase, Pubmed and the Cochrane Library was performed., Results: We identified six eligible studies considering patients with progressive neurological disease in childhood and receiving ITB treatment. The studies included a total of seven paediatric patients and four adult patients. Improvement was reported in spasticity, spasms, pain, gait, activities of daily life and providing care. High satisfaction is described., Conclusions: ITB has beneficial effects in paediatric patients with progressive neurological disease. However, the level of evidence is limited due to the small number of available studies and due to the poor quality of these studies., (Copyright © 2011 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.)

Aim: Treatment options for dystonic cerebral palsy (CP) are limited. Our aims were to determine whether intrathecal baclofen (ITB) improves daily care, decreases dystonia and decreases pain in patients with dystonic CP., Methods: Patients received randomized blinded treatment with ITB or placebo. Scores on problems of daily care were recorded and dystonia, pain and comfort were assessed., Results: Four patients (three males, average age 12 years 6 months) were included (all Gross Motor Function Classification System level V). During the trial period problem scores and dystonia scores decreased in all four patients., Conclusion: In this pilot study we report positive functional effects of ITB trial treatment in four patients with dystonic CP. A randomized trial with a larger cohort is needed to verify these results., (Copyright © 2011 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.)

Background: Children with an obstetric brachial plexus injury (OBPI) can experience problems in the performance of meaningful activities such as writing, bimanual activities, and participation in sports and leisure activities., Aims: To quantify the everyday functioning and participation of 7-8 year-old children with an OBPI, with special emphasis on writing, and to investigate associated characteristics., Methods: Parents of children with an OBPI were sent a self-report questionnaire regarding the school performance, writing abilities, bimanual hand use, and participation in sports and leisure activities of their child, assessed with the Vineland Adaptive Behavior Scales (VABS sub-scale writing), the ABILHAND-kids, and the Children's Assessment of Participation and Enjoyment (CAPE). Furthermore, questions were asked about socio-demographic variables, medical history, pain, and the use of assistive devices., Results: Fifty three questionnaires were filled in (response 61%). According to the parents, 66% of their children were almost completely recovered, and 58% had a near normal arm function. Most of the children preferred to use their non-involved hand. More than 45% of the children complained about pain, and 39.6% had difficulties with writing, which resulted in a mean developmental delay of 8 months on the VABS sub-scale. Children with writing problems significantly more often had neurosurgery, were living with a single parent, more often received assistance at school, and had a significantly lower ABILHAND-kids score, compared to children with no writing problems., Conclusions: Large percentages of 7-8 year-old children with an OBPI experience difficulties with writing and have musculoskeletal pain. Restrictions in participation were less pronounced., (Copyright © 2011 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.)

Object: The primary aim of this prospective cohort study was to evaluate the short-term (1 year) and long-term (mean 6 years) effects of selective dorsal rhizotomy (SDR) on gross motor function and spasticity in ambulatory children with spastic diplegia. Secondary aims were to investigate side effects, additional treatment during follow-up (botulinum toxin type A injections or orthopedic surgery), and parental satisfaction., Methods: Thirty-three children who had undergone SDR at a mean age of 6 years and 7 months (± 2 years) were included. There were 7 children at Gross Motor Function Classification System (GMFCS) Level I, 7 at Level II, and 19 at Level III. Gross motor function was assessed with the Gross Motor Function Measure-66 (GMFM-66). Spasticity was measured according to a modified Tardieu scale. Side effects, additional treatment, and parental satisfaction were recorded using a parental questionnaire and medical records., Results: At 1-year follow-up, mean GMFM-66 scores improved significantly by 4.3 ± 4.1 points. Children at GMFCS Levels I and II showed significantly more improvement (7.2 points) on the GMFM-66 compared with children at GMFCS Level III (2.9 points). On long-term follow-up (mean 6 years ± 22 months), mean GMFM-66 scores improved significantly by 6.5 ± 5.9 points, without a difference between children at GMFCS Levels I and II and Level III. No relapse of spasticity was noted. Ten children (30%) needed orthopedic surgery and 13 children (39%) received botulinum toxin type A treatment after SDR. Twenty (91%) of the 22 parents who answered the questionnaire at long-term follow-up believed that their child's functioning had improved after SDR., Conclusions: Selective dorsal rhizotomy resulted in short- and long-term improvements in gross motor function, without relapse of spasticity. However, the majority of the children still needed additional surgery or botulinum toxin A treatment.

Objectives: The primary aim of this study is to perform an internal quality control of pediatric brain tumor surgery in the neurosurgical department of the VU University Medical Center Amsterdam (The Netherlands). Secondly, this study aims to contribute to the accumulating data concerning outcome in pediatric neurosurgery, in order to establish institutional practice benchmarks., Methods: We report the surgical mortality and morbidity of 121 patients (0-18 years) surgically treated for a brain tumor from January 1999 to August 2007. Patients, in whom only a brain tumor biopsy was performed, were excluded., Results: Mean age at first surgery was 8.2 years. Of the 121 patients, 14 had a second surgery, and two underwent a third surgery (for a total of 137 operations). Of all 121 primary surgeries, 66% were total resections, 26% subtotal resections, and 8% partial resections. The overall surgical morbidity rate in this study was 69% after first surgery, 50% after second surgery, and one out of two after third surgery., Conclusion: These overall morbidity rates are comparable to other published mixed case series. The surgical mortality rate was 0.8%; this is comparable to the lowest rates reported for high-volume neurosurgical centers. We encourage other neurosurgical centers to collect, analyze, and publish their data. These data can then serve as a basis for comparison with other pediatric neurosurgical centers and will eventually lead to an improvement of pediatric neurosurgical practice and patient care.

Purpose: To identify MRI characteristics that may predict the functional effect of selective dorsal rhizotomy (SDR) in children with bilateral spastic paresis., Methods: We performed SDR in a group of 36 patients. The gross motor functioning measure-66 (GMFM-66) was applied before and after SDR. Available cerebral MRIs were retrospectively classified into three diagnostic groups: periventricular leucomalacia (PVL; n = 10), hydrocephalus (n = 2), and normal (n = 6). In patients with PVL, we scored the severity of the MR abnormalities. We compared the changes in the GMFM-66 after SDR in the diagnostic groups. In patients with PVL, we correlated the severity of the MR abnormalities with the changes in the GMFM-66., Results: The mean follow-up period was 5 years and 4 months (range, 1 year and 1 month to 9 years). The best improvement in gross motor function was observed in patients with normal MRI, and the slightest improvement was observed in patients with hydrocephalus. The severity of the PVL did correlate with the GMFM-66 score before SDR but not with the functional effect of SDR., Conclusion: We conclude that with respect to gross motor skills, the improvements after SDR are good in patients with no MRI abnormalities. In the patients with hydrocephalus, the improvements after SDR were insignificant. In patients with PVL, the improvements were intermediate and did not correlate with the degree of PVL.

Selective dorsal rhizotomy is an effective treatment for spasticity in children with cerebral palsy who have a spastic motor disorder. It is hypothesized that muscle shortening is related to spasticity; the lack of stretch of a muscle is thought to be the cause of muscle shortening. If this is true, the treatment for spasticity should prevent the occurrence of muscle shortening during growth. We present the case of 1 child with cerebral palsy and spastic diplegia, for whom the treatment with selective dorsal rhizotomy was successful in improving the walking abilities. She did, however, develop muscle shortening during growth. In conclusion, the development of muscle shortening during growth in children with cerebral palsy and spastic paresis cannot be prevented by treatment for the spasticity alone.

Selective dorsal rhizotomy at the lumbar level is a neurosurgical procedure, which reduces spasticity in the legs. Its effect has mainly been studied in children with spastic cerebral palsy. Little is known about the outcome of selective dorsal rhizotomy in patients with neurodegenerative disorders. We report the clinical course after selective dorsal rhizotomy in 2 patients with progressive spasticity. Leg spasticity was effectively and persistently reduced in both patients, facilitating care and improving sitting comfort. However, spasticity of the arms and other motor disturbances, such as spontaneous extension spasms and the ataxia, increased gradually in time. Selective dorsal rhizotomy leads to a disappearance of leg spasticity in patients with a neurodegenerative disease. Other motor signs are not influenced and may increase due to the progressive nature of the underlying disease.

Objective: Intracranial aneurysms are very rare in early childhood. Because the location, morphology as well as the clinical and radiological presentation of these aneurysms seem to be different from those in adults, we performed a systematic review of the literature to discuss the clinical, morphological, and radiological features of intracranial aneurysms in the first year of life., Materials and Methods: A computerized search of both Pubmed and EMBASE from before 1966 to 2005 was performed. Included were all articles that dealt with cases in which an intracranial aneurysm was demonstrated in children under 1 year of age., Results: We found 110 articles in which 131 cases of an intracranial aneurysm in children under 1 year were presented. The mean age at diagnosis of the aneurysm was 4.9+/-3.5 months with a male to female ratio of 1.1. There was a hemorrhagic presentation in 73% (n=96). The patients presenting with a hemorrhage were younger (mean 4.3 vs 6.7 months, P<0.001) and tended to have smaller-sized (i.e.<2.5 cm) aneurysms (P=0.07). The aneurysm was defined as traumatic or infectious in 15 and 13 cases, respectively. In 21% (n=27), there was various vascular or congenital co-morbidity. In 76%, the aneurysm was located in the anterior circulation. The prevalence of aneurysms on the middle cerebral artery (MCA) was nearly three times higher than on any other vessel. The mean aneurysm size was 1.8+/-1.4 cm, with 30 giant aneurysms (>2.5 cm). The giant aneurysms were significantly more often located in the posterior circulation (43 vs 16%, P=0.01). The mean period of follow-up was 13.6+/-24.8 months. The Glasgow Outcome Scale (GOS) could be derived in 106 cases: 50% had an excellent outcome (GOS of 5)., Conclusions: The presentation of arterial aneurysms in children under the age of 1 year differs from that in adults with a significantly higher prevalence of giant aneurysms in the posterior circulation. The prevalence of aneurysms on the MCA is nearly three times higher than on any other vessel. The patients presenting with a hemorrhage were younger and tended to have smaller-sized aneurysms. Our study did not confirm the male predominance that has thus far been associated with pediatric aneurysms. The outcome is comparable or slightly better than in adults.

Objective: A systematic follow-up of infants with an obstetric brachial plexus lesion of C5 and C6 or the superior trunk showing satisfactory spontaneous recovery of shoulder and arm function except for voluntary shoulder exorotation, who underwent an accessory to suprascapular nerve transfer to improve active shoulder exorotation, to evaluate for functional recovery, and to understand why other superior trunk functions spontaneously recover in contrast with exorotation., Methods: In 54 children, an accessory to suprascapular nerve transfer was performed as a separate procedure at a mean age of 21.7 months. Follow-up examinations were conducted before and at 4, 8, 12, 24, and 36 months after operation and included scoring of shoulder exorotation and abduction. Intraoperative reactivity of spinatus muscles and additional needle electromyographic responses were registered after electrostimulation of suprascapular nerves. Histological examination of suprascapular nerves was performed. Trophy of spinatus muscles was followed by magnetic resonance imaging scanning. The influence of perinatal variables and results of ancillary investigations on outcome were evaluated., Results: Exorotation improved from 70 degrees to functional levels exceeding 0 degrees, except in two patients. Abduction improved in 27 patients, with results of 90 degrees or more in 49 patients. Electromyography at 4 months did not show signs of denervation in 39 out of 40 patients. Intraoperative electrostimulation of suprascapular nerves elicited spinatus muscle reaction in 44 out of 48 patients. Histology of suprascapular nerves was normal. Preoperative magnetic resonance imaging scans showed only minor wasting of spinatus muscles in contrast with major wasting after successful operations., Conclusion: An accessory to suprascapular nerve transfer is effective to restore active exorotation when performed as the primary or a separate secondary procedure in children older than 10 months of age. Contradictory spontaneous recovery of other superior trunk functions and integrity of suprascapular nerves, as well as absence of spinatus muscle wasting direct to central nervous changes are possible main causes for the lack of exorotation.

Objective: Preoperative, reliable detection by ancillary investigations of spinal nerve root avulsions in infants with severe obstetric brachial plexus lesions to avoid ineffective operative repair from deceivingly intact but actually avulsed nerve roots., Methods: Ten infants were selected with an infrequent, severe dominant C7 lesion, primarily because of the anatomically distinct supraclavicular course of this spinal nerve. Three-dimensional constructive interference in steady-state magnetic resonance imaging (3D CISS MRI) studies under mild sedation were performed and evaluated for detection of avulsed nerve roots by two experienced neuroradiologists. Preoperative electrodiagnostics (electromyography and somatosensory evoked potentials) as well as intraoperative somatosensory potentials and muscle contractions after electrostimulation were recorded. Preoperative and intraoperative ancillary investigations were correlated with intraoperative findings in eight patients and clinical status in two children who recovered spontaneously., Results: Despite two minor motion artifacts, the quality of the 3D CISS MRI studies was good. In 8 of 10 patients, prediction of root continuity was consistent with operative or clinical findings, and 2 remained doubtful. Preoperative and intraoperative electrodiagnostics tended not to correlate with intraoperative findings in this small, selected group., Conclusion: 3D CISS MRI provides good images of anterior and posterior spinal roots in infants with obstetric brachial plexus lesions. Images seem to allow accurate prediction of root avulsion in the majority of patients. In this study, electrodiagnostics were of limited value.

Objectives: The aim of this study is to evaluate the effect of selective dorsal rhizotomy (SDR) on functional abilities in a well-defined group of ambulatory children with spastic diplegia., Methods: Nine children were selected for SDR (mean age 65 months, range 43-82 months). Gross motor function was measured with the Gross Motor Function Measure (GMFM-88). Self-care was assessed with the Pediatric Evaluation of Disability Inventory (PEDI) and gait pattern was measured with the Edinburgh Visual Gait Score (EGS). There were nine single-case research designs with a 12-month follow-up after surgery., Results: After 12 months the mean improvement in the total GMFM-88 scores was 8.8%. On an individual level, all patients improved significantly in comparison with baseline. Functional skills and care-giver assistance measured with the PEDI showed significant improvement. Improvement in gait was also found; in particular, better initial contact and heel-lift resulted in an increased EGS., Conclusion: In this well-defined group of ambulatory children SDR had a small but significant positive effect on gross motor function, self-care and gait pattern.

Objects: A case of a Suriname female occipito-parietal to occipito-parieto-temporal craniopagus twins is described. The girls were transferred to the VU University Medical Center (VUmc) in Amsterdam, the Netherlands, for further diagnostics and to analyze whether surgical separation was feasible and ethically justifiable. The multifactorial aspects of different treatment options are discussed., Methods: The twins underwent multiple investigations by a multidisciplinary team. Advanced imaging techniques with 3D-CT scan, MRI and MRA scans, image fusion techniques and, most importantly, cerebral angiography with balloon occlusion tests were performed., Conclusions: Because of a shared venous ring, with preferential drainage to the left child, and which endovascular balloon occlusion showed could not be separated, surgical separation of the twins with a fair chance of survival without additional neurological damage and with prospects of a good quality of life was regarded as impossible. In accordance with the parents' wishes, the twins were not separated and offered optimal integral conservative treatment.

Object: The authors performed a prospective study in which magnetic resonance (MR) imaging was conducted in 26 consecutive infants (mean age 5.6 months, range 2.7-14.5 months) in whom recovery from an obstetric lesion of the brachial plexus had been inadequate in the first 3 months of life. The purpose was to identify early secondary deformations of the shoulder in obstetrical brachial plexus lesions (OBPLs)., Methods: Features of the shoulders were analyzed according to a standardized MR imaging protocol in patients with OBPLs. Measurements were made of the appearance of the glenoid, glenoid version, and the position of the humeral head. The appearance of the glenoid on the affected side was normal in only 11 shoulders. In the remainder it was convex in eight and biconcave in seven cases. The degree of humeral head subluxation was significantly greater (p = 0.001) in affected shoulders than in normal shoulders (152 and 170 degrees, respectively). The presence of abnormal glenoid retroversion and humeral head subluxation increased with age: there was a statistical difference (p = 0.001) between infants younger than 5 months of age and those who were older., Conclusions: Magnetic resonance imaging demonstrates shoulder-related anatomical and nerve root lesion, allowing evaluation of neural, osseous, and cartilaginous structures in younger children.

In this prospective study of 19 consecutive children, the operative treatment of internal rotation contracture of the shoulder in obstetric brachial plexus lesions by subscapular tendon lengthening and open reduction of the humeral head is evaluated. The average age of the children was 3.7 years and average follow-up was 20 months. Active shoulder function, as measured by the Mallet score, improved significantly in the dimensions of external rotation, hand-mouth movement and hand-neck movement. However, eight of the 19 children developed a severe, functionally disturbing external rotation contracture of the shoulder. This contracture was found predominantly in children who had a lower preoperative Mallet score for abduction and hand-back movement.

An historical cohort study was conducted to investigate the rate and extent of neurological recovery in obstetric brachial plexus injury (OBPI) and to identify possible prognostic factors in a cohort of children with OBPI from birth to 7 years. All children (n=56; 31 females, 25 males) with OBPI were evaluated at fixed time intervals by one examiner. They underwent a final neurological examination at a mean age of 3 years 10 months (range 1 to 7 years). Neurological outcome was not as favourable as is often reported: complete neurological recovery occurred in 37 out of 56 children (66%). In half of these there was delayed recovery, in which case complete neurological recovery was assessed from 1.5 to 16 months of age (median age 6.5 months, SD 4.2 months). External rotation and supination were the last to recover and recovered the least. Although biceps function at three months was considered to be the best indicator for operative treatment, external rotation and supination were found to be better in predicting eventual complete recovery. Initial symptoms directly post partum were not found to be prognostic. Functional outcome was mainly reported to be good.

Four children (two boys aged 1.5 and 10 years and two girls aged 2 and 9 years) vomited for one-half to four weeks. In one child, ataxia was later also noted and another tilted his head constantly to the left, but this was initially not alarming. In all four cases CT revealed a brain tumour, for which they were operated. Postoperatively, one child had residual tumour tissue that caused no further problems, in two children the tumour was completely excised with no further symptoms and no recurrence in the following 2 years, and in one child complete excision was not possible so that chemotherapy and radiotherapy were given, but metastases nevertheless developed 10 months later and the child died. Vomiting is common in children and in most cases the result of infectious or gastrointestinal causes. Intracranial pathology also can cause vomiting, both by increased intracranial pressure and by direct stimulation of the vomiting centre in the brainstem. Brain tumours in children often lack specific neurological signs in their clinical presentation. Intractable or chronic vomiting without nausea or deregulation of the water and electrolyte balance could therefore indicate the presence of an intracranial process, even when other neurological signs are absent.

The features of proliferation in brain tumours are related with clinical prognosis for several types of brain tumours, especially gliomas. For childhood central primitive neuro-ectodermal tumours (cPNET), including medulloblastoma, this relation has previously been unclear. The aim of this study is to investigate the relationship between proliferative features of cPNET and in vitro resistance for cytostatic drugs measured with the 3-4,5-dimethylthiazol-2-yl-2,5-diphenyltetrazolium-bromide (MTT) assay. Tumour material was obtained from 23 surgical specimens of cPNET. The expression of the proliferation markers Ki-67, proliferating cell nuclear antigen (PCNA) and cyclin D1 was determined with immunohistochemistry, while S-phase and DNA ploidy were analysed by flowcytometric analysis cell scan (FACS). The in vitro resistance for 10 cytostatic drugs was determined with the MTT assay. Drug resistance levels were available in 19 (83%) of the 23 samples with a complete profile of 10 cytostatic drugs tested in 14 samples. An excellent correlation in drug resistance scores was found between pharmacologically related drugs. The Ki-67 staining in 20 samples varied from 10 to 60% and from 30 to 100% for PCNA. Cyclin D1 staining was negative in 11 out of 18 samples. The S-phase in 16 samples ranged from 2 to 16%. Increased staining of Ki-67 was related with actinomycin D sensitivity (r -.603; P=0.022), while cells with a higher S-phase percentage were more resistant to ifosfamide (r.952; P<0.0001). In vitro drug resistance testing of central primitive neuro-ectodermal tumours (PNET) is feasible with the MTT assay. Ifosfamide resistance was related with increased Ki-67 and S-phase percentage of the tumour cells, while increased Ki-67 was also related with actinomycin D sensitivity. These findings suggest a cell cycle dependent activity of cytostatic drugs in vitro.

We undertook a prospective MRI study to measure the retroversion of the humeral head in 33 consecutive infants with a mean age of 1 year 10 months (3 months to 7 years 4 months) who had an obstetric brachial plexus lesion (OBPL). According to a standardised MRI protocol both shoulders and humeral condyles were examined and the shape of the glenoid and humeral retroversion determined. The mean humeral retroversion of the affected shoulder was significantly increased compared with the normal contralateral side (-28.4 +/- 12.5 degrees v -21.5 +/- 15.1 degrees, p = 0.02). This increase was found only in the children over the age of 12 months. In this group humeral retroversion was -29.9 +/- 12.9 degrees compared with -19.6 +/- 15.6 degrees in the normal shoulder (p = 0.009), giving a mean difference of 10.3 degrees (95% confidence interval 3.3 to 17.3). This finding is of importance when considering the operative treatment for subluxation of the shoulder in children with an OBPL.

We performed a prospective study using MRI in 16 consecutive infants with a mean age of 5.2 months (2.7 to 8.7) who had shown inadequate recovery from an obstetric lesion of the brachial plexus in the first three months of life, in order to identify early secondary deformities of the shoulder. Shoulders were analysed according to a standardised MRI protocol. Measurements were made of the appearance of the glenoid, glenoid version and the position of the humeral head. The appearance of the glenoid on the affected side was normal in only seven shoulders. In the remainder it was convex in seven and bioconcave in three. The degree of subluxation of the humeral head was significantly greater (p = 0.01) in the affected shoulders than in normal shoulders (157 degrees v 170 degrees). The presence of an abnormal appearance of the glenoid, retroversion of the glenoid and subluxation of the humeral head increased with age. There was a statistical difference (p = 0.05) between infants younger than five months and those who were older.

Despite improving perinatal care the incidence of obstetric brachial plexus lesions (OBPL) has not declined. Most babies recover spontaneously. In 10-20% recovery is incomplete. To prevent lasting functional deficits early referral to specialized centers is necessary. If the biceps shows no function at 3 months, standardized clinical assessment and additional investigations must delineate the extent of a lesion. Detection of root avulsions by myelography and computed tomography combined with electrodiagnostics remains inconclusive in 15% of cases. Plexus reconstruction is performed during the 4th-6th months. Contractures or deformities are treated conservatively or by orthopedic surgery. Long-term rehabilitation is required. In future, aspects of prevention need attention. Improving imaging and neurophysiological techniques are promising for greater precision in detecting root avulsions and even spontaneous recovering nerves. Functional imaging will allow better understanding of central integration and plasticity. New pharmacological agents may promote nerve regeneration.

Three previously healthy children, two girls aged 2 and almost 5 years and a boy aged 20 months, developed a progressively stumbling gait within days. In two this occurred after a period of weeks during which they complained of, or seemed to have back pain. In all three cases acute spinal cord compression by a malignant tumour was diagnosed. Histological examination revealed Ewing sarcoma, granulocytic sarcoma and T-cell lymphoma. Surgical decompression led to complete neurological recovery. Although rare, acute spinal cord compression during childhood is a medical emergency because of the risk of neurological morbidity. Back pain, weakness and a stumbling gait usually are the first symptoms. Sensory symptoms and sphincter dysfunction may develop later. Early recognition is essential, as prognosis depends on neurological findings and duration of symptoms when treatment is started.

A minimally invasive method of endoscopy-assisted sural nerve (SN) harvest in infants with obstetric brachial plexus lesions requiring nerve grafting procedures was applied to reduce the skin incision size and scarring at the donor site. Endoscopic visualization was achieved using a flexible and steerable Neuroview neuronavigational endoscope (Promedics, NL) 2.3 mm in diameter and 12 or 18 cm long in a peelaway sheath (700-9F) attached to a video camera. Through three 1.5-cm skin incisions the SN could be dissected free using a 2.5-mm-diameter nerve stripper, pituitary curette or pituitary scissors under endoscopic vision from the opposite direction. To prevent any central nociceptive pain behavior the sural nerve was blocked by lidocaine, and sectioned first proximally in the popliteal fossa then distally at the lateral malleolus.

We describe the case - to our knowledge unique - of an 8-year-old boy who presented with acute onset of lower cranial nerve palsy and tetraparesis caused by a hematoma in a dorsal exophytic pilocytic astrocytoma of the medulla oblongata. The boy showed near-complete recovery after neurosurgical management in two stages: first, emergency evacuation of the hematoma with tumor biopsy, and second, complete tumor removal 5 months after the initial event. Intraoperative electrophysiological monitoring techniques for the lower cranial nerves are of value in preserving their functional integrity. Ultrasonography is helpful in assessing the extent of tumor removal. Although the pathological diagnosis of a pilocytic astrocytoma would not justify radiotherapy, local field radiotherapy was added mainly because of the unexpectedly rapid tumor progression during the interval between the two surgical procedures. The literature on brainstem and tumor hematoma in children is reviewed.

In a 25-year-old woman pregnant for the second time after a successful first pregnancy, a locally aggressive, invasive sacrum tumour was diagnosed. The execution of the necessary but potentially mutilating surgical procedures was seriously hampered even during the preparative phase, in spite of the conscious wish of the patient to comply, by her severe psychiatric problems (posttraumatic stress disorder with dissociative symptoms). The Psychiatric Consultation Service took over the case management and an integrated (biopsychosocial) diagnostic investigation was carried out, involving analysis of the problems on four system levels: the biological, the psychological, the social and the health care level. An integrated treatment plan was drafted. By collaboration of the entire multidisciplinary treatment team conditions were secured under which patient would let herself be treated. In this way she was enabled to undergo the necessary procedures, with good results.

A case is presented of a 14-year-old boy with neurofibromatosis who had a 92 degrees dystrophic kyphosis (as measured on radiographs between C3 and C7) of the cervical spine. He was treated successfully by posterior stabilization and anterior fusion using a free vascularized fibula graft. This method appears to be an attractive alternative to an avascular fibula graft and avoids the risk of graft resorption (creeping substitution), weakening (fracture), or nonunion during the process of bony consolidation. It provides a stable and longstanding anterior strut, essential in the management of high grades of kyphosis. At 1-year followup the patient has no symptoms, is fully mobile, and shows radiographically complete incorporation of the graft with no loss of correction.

In patients operated on for herniated lumbar disc, epidural morphine was administered intraoperatively at the end of the procedure. A 10 milligram dose of morphine provided significant pain relief expressed by less requirement for strong analgesics on the day of operation and the day following, as well as for sedatives during the postoperative observation period of four days in comparison to a control group of patients. The only side effect observed was urinary retention.

A case of monochorionic/monoamnionic twin with discordant occipital developmental malformations is presented. One female twin appeared to have an occipital meningocele with cerebellar aplasia and died immediately after birth. The other twin presented with signs and symptoms of raised intracranial pressure at the age of 7 months. Severe hydrocephalus was present due to an infected intracerebellar dermoid tumor with a contiguous occipital dermal sinus. The clinical and pathological characteristics are described and the different theories concerning twinning, embryogenesis, and dysmorphology in relation to neural tube defects are discussed. Analysis of the features of these monozygotic twins indicates that a meningocele is not a post-neurulation disorder but results from deficient neurulation, probably due to mesodermal insufficiency.

A 7.5-month-old girl was admitted with the clinical signs and symptoms of raised intracranial pressure. This was caused by an obstructive hydrocephalus, due to cerebellar abscesses induced by an infected contiguous complete occipital dermal sinus. Staphylococcus aureus was cultured. The patient was treated by radical excision of the dermal sinus and the abscesses after initial external drainage of the lateral cerebral ventricle, followed by systemic antibiotic therapy. The different types of dermoid cysts of the posterior cranial fossa are described. Early detection of congenital dermal abnormalities along the craniospinal axis by routine examination of newborns is prognostically important. Computerised tomography or magnetic resonance imaging are the methods of choice for further investigation of suspect dermal lesions.