Pediatric meningiomas in The Netherlands 1974–2010: a descriptive epidemiological case study

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. 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. 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. 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 counterpart

Preoperative MRI findings and functional outcome after selective dorsal rhizotomy in children with bilateral spasticity

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

Abdominal Pseudocysts and Peritoneal Catheter Revisions: Surgical Long-Term Results in Pediatric Hydrocephalus

Objective: An abdominal pseudocyst (APC) is a distal catheter site-specific failure in patients treated with ventriculoperitoneal shunts. Few studies with more than 10 patients have been reported. The aim of this study was to analyze causes of peritoneal catheter revisions with special emphasis on revisions because of an APC. Methods: Pediatric patients with first shunt operation between 1982 and 1992 were included, and time, cause, and modality of peritoneal catheter revision were determined retrospectively. Results: One hundred thirty-eight patients were treated for hydrocephalus, and 112 patients received a peritoneal catheter during the follow-up. An APC was diagnosed in 14 (12.5%) patients, and 28 revisions were needed for its treatment. The rate of shunt infection in patients with APC was 50%, but bacterial examination of the pseudofluid culture revealed infection in only 3 patients. Age at first surgical procedure, type of first surgical procedure, and etiology of hydrocephalus were not associated with APC diagnosis. APC recurred in 4 patients. These patients had a catheter repositioning directly into the peritoneum as first surgical treatment. No recurrences were observed in patients with shunt externalization or replacement of the peritoneal catheter. Conclusions: An APC is a major long-term complication after ventriculoperitoneal shunt treatment. Although a sterile inflammatory response cannot be excluded completely, our results favor the hypothesis of low-level shunt infection. In both cases, the surgical consequences are the same. An infected APC should be treated as a shunt infection. Uninfected patients can be treated with shunt externalization and replacement of only the peritoneal catheter

Detection of root avulsion in the dominant C7 obstetric brachial plexus lesion: experience with three-dimensional constructive interference in steady-state magnetic resonance imaging and electrophysiology

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

Evolution of gait in adolescents and young adults with spastic diplegia after selective dorsal rhizotomy in childhood: A 10 year follow-up study

Background: Short-term benefit on gait of selective dorsal rhizotomy (SDR) surgery, which relieves spasticity of the lower extremities has been demonstrated in children with cerebral palsy (CP). However very little is known of the evolution of gait when patients become adolescents and young adults. Research question: How does the gait pattern evolve in adolescents and young adults who underwent SDR during childhood? Methods: A longitudinal study was performed including 19 ambulant patients with spastic diplegia due to CP or other causes (mean age at SDR: 6.6 ± 1.6 years) who were assessed four times: pre-SDR, 2 years post- SDR, 5 years post-SDR and at least 10 years post-SDR. From 2D video recordings, Edinburgh Visual Gait Score and lower limb joint kinematic parameters were calculated. Results: Our data show that the improvement in the gait pattern obtained short-term after SDR continues during into adolescence and adulthood. Ten years after SDR all patients improved compared to baseline. Considering the lower limb joint kinematics, most notable improvements were found at knee and ankle joints. Compared to the evaluation before SDR, the range of motion of the knee increased: the knee was more extended at initial contact and knee flexion in midswing improved. Excessive ankle plantar flexion was reduced during the entire gait cycle. Only minor changes were found at hip and pelvis. Eight patients underwent additional orthopaedic surgery in the years after SDR, and the present findings should be considered as a combination of SDR, development and additional treatment. Significance: We demonstrate lasting improvement of gait quality in ambulant patients with spastic diplegia who underwent SDR during childhood when they become adolescents and young adults

Adult Outcome in Shunted Pediatric Hydrocephalus: Long-Term Functional, Social, and Neurocognitive Results

Objective: Very long-term outcomes are rarely reported for patients with shunted pediatric hydrocephalus. This study aimed to determine the functional, social, and neurocognitive outcomes of such patients after transition to adulthood. Methods: Adult patients with pediatric hydrocephalus who underwent their first shunt operation between 1982 and 1992 were included. Functional, social, educational, working aspects, and verbal intelligence were evaluated. In patients with average or above average verbal intelligence, detailed neuropsychological testing was performed and memory, executive functioning, selective attention, and concentration were assessed. Results: Overall, 137 patients underwent primary surgery because of pediatric hydrocephalus, 53 (38.7%) of whom died during the follow-up period. Of the 84 long-term survivors, 65 (77.4%) agreed to participate and were included for further analysis. Forty-five patients (69.2%) had completed secondary school, but only 34 (52.3%) were integrated in the open labor market. Although the verbal intelligence of 31 patients (47.7%) was within the normal range, 19 (29.2%) had a severe mental handicap. Shunt infections (P = 0.0025), epilepsy (P < 0.0001), and the number of shunt operations (P = 0.0082) were associated with reduced verbal intelligence. Most patients with average or above average verbal intelligence had deficits in detailed neuropsychological testing. In 23 patients, detailed neuropsychological testing was performed. Conclusions: The overall long-term outcome of patients with shunted pediatric hydrocephalus is poor. These results highlight the importance of lifelong routine controls to avoid later complications. Further, repeated neuropsychological examinations might be important to understand the patient's special needs to optimize professional support

Accessory nerve to suprascapular nerve transfer to restore shoulder exorotation in otherwise spontaneously recovered obstetric brachial plexus lesions

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

Neurological recovery in obstetric brachial plexus injuries: an historical cohort study

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 goo

Intrathecal baclofen treatment in dystonic cerebral palsy: a randomized clinical trial: the IDYS trial

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. TRIAL REGISTRATION: Nederlands Trial Register, NTR364