Atypical presentation of acute promyelocytic leukaemia.
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Adolescent↗
Biomedical subjects
Jean-Pierre Farmer
Publications and source records attributed to Jean-Pierre Farmer.
Long-term outcome after surgical treatment of temporal lobe epilepsy in children.
OBJECT: Surgery is an accepted treatment for carefully selected patients with focal epilepsy. In the present study, the authors assessed clinical and surgery-related data obtained in a large series of children suffering from intractable temporal lobe epilepsy (TLE). METHODS: Etiological, pathological, and clinical features of possible prognostic significance were studied in 109 children who underwent surgery for TLE at the Montreal Neurological Institute and Hospital and the Montreal Children's Hospital between 1985 and 2000. The mean age of patients at seizure onset was 5.5 years and the duration of epilepsy ranged from 0.1 to 17.6 years. Preoperative magnetic resonance imaging revealed mesial sclerosis in 51 patients, a mass lesion in 45, and no visible abnormalities in 12. In six patients invasive monitoring was required. Cortical amygdalohippocampectomy was performed in 72% of patients, whereas 20% underwent transcortical selective amygdalohippocampectomy. In 23 patients a second surgical intervention was necessary. Low-grade tumors were found in 35% and mesial sclerosis was confirmed on pathological evaluation in 45%. Outcome was excellent (seizure free or > 90% reduction) in 94 patients (86%). The patients were followed prospectively for a median of 10.9 years (range 5-20.2 years). There were no permanent neurological complications and no deaths. CONCLUSIONS: Successful postsurgical outcomes, especially in patients treated for mesial temporal lobe sclerosis and lesion-related epilepsies, can be obtained in pediatric patients suffering minimal complications. Unfavorable outcomes are most likely to occur when epileptiform discharges are bitemporal or multifocal in distribution and in cases involving incomplete resection of mesiotemporal structures.
Adolescent↗
Highly conformal radiotherapy for craniopharyngioma: (potentially) throwing the baby out with the bathwater.
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Child↗
Disconnective hemispherectomy for hemispheric dysplasia.
Twelve patients suffering from intractable epilepsy and presenting with radiological evidence of diffuse hemispheric involvement of a dysplastic process, were treated by disconnective hemispherectomy, either functional hemispherectomy or peri-insular hemispherotomy. The median age at surgery was 4.5 years old and the interval between seizure onset and surgery, 3 years. All patients underwent a presurgical evaluation that led to the suggestion of disconnective hemispherectomy. Over 70% of patients have remained in Engel's seizure outcome class I since surgery and another 18% have had a satisfactory seizure outcome. There was one unexplained death and one case of early hydrocephalus. Hemispherectomy offers the possibility to improve seizure control in the majority of patients undergoing surgery for extensive dysplastic pathology of the hemisphere. Disconnective techniques reduce the rate of complications in this specific pathology.
Adolescent↗
Ophthalmic complications of slit-ventricle syndrome in children.
OBJECTIVE: To present the ophthalmic features and visual prognosis of patients with slit-ventricle syndrome (SVS). DESIGN: Observational case series. PARTICIPANTS: Six patients diagnosed and treated with SVS at the Montreal Children's Hospital between 1985 and 1999. METHODS: Patients were included in this study if they had an appropriate ophthalmologic follow-up and if they fulfilled the criteria for the diagnosis of SVS based on intracranial pressure monitoring and neuroimaging studies. MAIN OUTCOME MEASURES: Features studied included patients' baseline ophthalmologic evaluation, visual outcome, and neurosurgical characteristics. RESULTS: The follow-up ranged from 1 to 14 years. The average number of shunting procedures was 3.7. Ocular examination performed on the initial visit revealed a normal visual acuity in four patients, esotropia in three of six patients, and nystagmus in two of six patients. Cycloplegic refraction was normal in all the subjects. Initial funduscopic evaluation revealed optic atrophy in a 4-month-old infant, whereas two children developed optic atrophy later in the course of the disease. One child developed severe visual field defects. The two children with optic atrophy had moderate to severe loss of visual acuity associated with SVS. CONCLUSIONS: A prompt recognition of patients with SVS is crucial, because these individuals are at an increased risk for significant visual loss.
Cerebral Ventricles↗
Reversible coma in children after improper baclofen pump insertion.
We report the occurrence of delayed postoperative coma in five of nine consecutive children following surgery for insertion of baclofen infusion pumps. We suspect that improper technique resulted in an inadvertent administration of an intrathecal baclofen bolus. We suggest ways to minimize this risk.
Analgesics, Opioid↗
Impact of selective posterior rhizotomy on fine motor skills. Long-term results using a validated evaluative measure.
Suprasegmental effects following selective posterior rhizotomy have been frequently reported. However, few studies have used validated functional outcome measures to report the surgical results beyond 3 years. The authors analyzed data obtained from the McGill Rhizotomy Database to determine the long-term impact of lumbosacral dorsal rhizotomy on fine motor skills. The study population comprised children with debilitating spasticity who underwent SPR and were evaluated by a multidisciplinary team preoperatively, at 6 months and 1 year postoperatively. Quantitative standardized assessments of upper extremity function were obtained using the fine motor skills section of the Peabody Developmental Motor Scales (PDMS) test. Of 70 patients who met the entry criteria for the study, 45 and 25 completed the 3- and 5-year assessments, respectively. Statistical analysis demonstrated significant improvements in grasping, hand use, eye-hand coordination, and manual dexterity at 1 year after SPR. More importantly, all improvements were maintained at 3 and 5 years following SPR. This study supports that significant improvements in upper extremity fine motor function using the PDMS evaluative measure are present after SPR and that these suprasegmental benefits are durable.
Cerebral Palsy↗
Neurofibromatosis type 1 and the pediatric neurosurgeon: a 20-year institutional review.
Children with neurofibromatosis type 1 (NF1) undergo costly surveillance scanning for a variety of asymptomatic central nervous system lesions whose natural history is poorly understood. We performed a 20-year retrospective chart review of 25 patients with clinically proven NF1 who required surgery (group A) and contrasted this cohort with 150 NF1 patients who did not require surgery (group B). In group A, 52% of patients underwent multiple procedures for more than one lesion (p = 0.043). Group A patients were further distinguished from those in group B by exhibiting a greater number of optic gliomas (p = 0.015), nonoptic intracranial tumors (p = 0.006), cranial nerve (p = 0.000), paraspinal (p = 0.0062), craniofacial (p = 0.001) and visceral (p = 0.03) neurofibromas and moyamoya disease (p = 0.00), as well as a higher frequency of seizure disorder, sphenoid wing dysplasia and poor academic performance. Gadolinium enhancement occurred in 43% of optic gliomas, 50% of parenchymal gliomas, 100% of cranial nerve, 100% of plexus, 67% of paraspinal, 50% of craniofacial and 50% of visceral neurofibromas in group A, while only 1 group B tumor enhanced. In group A, radiological progression occurred after a median of 4 years from initial diagnosis for optic gliomas as well as cranial nerve, plexus and visceral neurofibromas, 2 years for paraspinal neurofibromas and brainstem gliomas and 2.7 years for craniofacial neurofibromas. Only 1 tumor progressed in group B. Therefore, a small subgroup of NF1 patients (12.5%) who require treatment are at risk of subsequently needing further surgical attention, whereas a larger group of NF1 patients (87.5%) carry an indolent form of the disease. We recommend imaging for asymptomatic, gadolinium-enhancing lesions every 2 years for optic pathway and parenchymal gliomas and cranial nerve and visceral neurofibromas, and every year for brainstem gliomas and paraspinal as well as craniofacial neurofibromas. Nonenhancing optic pathway lesions could be followed up radiographically much less often since they do not show progression.
Brain Neoplasms↗
Long-term functional outcome after selective posterior rhizotomy.
OBJECT: Selective posterior rhizotomy (SPR) is a well-recognized treatment for children with spastic cerebral palsy (CP). Few investigators have used quantitative outcome measures to assess the surgical results beyond 3 years. The authors analyzed data obtained from the McGill Rhizotomy Database to determine the long-term functional outcome of children who had undergone selective dorsal rhizotomy accompanied by intraoperative electrophysiological monitoring. METHODS: The study population was composed of children with spastic CP who underwent SPR and were evaluated by a multidisciplinary team preoperatively, and at 6 months and 1 year postoperatively. Quantitative standardized assessments of lower-limb spasticity, passive range of motion, muscle strength, and ambulatory function were obtained. Of the 93 patients who met the entry criteria for the study, 71 completed the 3-year and 50 completed the 5-year assessments, respectively. Statistical analysis demonstrated significant improvements in spasticity, range of motion, and functional muscle strength at 1 year after SPR. The preoperative, 1-, 3-, and 5-year values for the global score of the Gross Motor Function Measure were 64.6, 70.8, 80, and 85.6, respectively. The greatest improvement occurred in the dimensions reflecting lower-extremity motor function, where the mean change was 10.1% at 1 year, 19.9% at 3 years, and 34.4% at the 5-year follow-up review in comparison with the baseline value. This was associated with a lasting improvement in alignment and postural stability during developmental positions, as well as increased ability to perform difficult transitional movements. CONCLUSIONS: The results of this study support the presence of significant improvements in lower-limb functional motor outcome 1 year after SPR, and the improvements persist at 3 and 5 years. The authors conclude that SPR in conjunction with intraoperative stimulation is valuable for permanently alleviating lower-limb spasticity while augmenting motor function.
Cerebral Palsy↗
Functional performance following selective posterior rhizotomy: long-term results determined using a validated evaluative measure.
OBJECT: Selective posterior rhizotomy (SPR) may result in considerable benefit for children with spastic cerebral palsy. To date, however, there have been few studies in which validated functional outcome measures have been used to report surgical results beyond 3 years. The authors analyzed data obtained from the McGill Rhizotomy Database to determine long-term functional performance outcomes in patients who underwent lumbosacral dorsal rhizotomy performed using intraoperative electrophysiological monitoring. METHODS: The study population was composed of children with debilitating spasticity who underwent SPR and were evaluated by a multidisciplinary team preoperatively and at 6 months and 1 year postoperatively. Quantitative standardized assessments of activities of daily living (ADL) were obtained using the Pediatric Evaluation of Disability Inventory (PEDI). Of 57 patients who met the entry criteria for the study, 41 completed the 3-year assessments and 30 completed the 5-year assessments. Statistical analysis demonstrated significant improvement in the mobility and self-care domains of the functional skills dimension at 1 year after SPR. The preoperative and 1-, 3-, and 5-year postoperative scaled scores for the mobility domain were 56, 64, 77.2, and 77.8, respectively. The scaled score for the self-care domain increased from 59 presurgery to 67.9, 81.6, and 82.4 at the 1-, 3-, and 5-year postoperative assessments, respectively. CONCLUSIONS: The results of this study support the presence of significant improvements in functional performance, based on PEDI scores obtained 1 year after SPR. The improvements persisted at the 3- and 5-year follow-up examinations. The authors conclude that SPR performed using intraoperative stimulation is valuable in the augmentation of motor function and self-care skills essential to the performance of ADL.
Activities of Daily Living↗