Pediatric epilepsy surgery: could age be a predictor of outcomes?

OBJECTIVE Like adults, many children suffering from intractable seizures benefit from surgical therapy. Although various reports indicate that early intervention may avoid severe developmental consequences often associated with intractable epilepsy, surgery is still considered a last option for many children. In this retrospective study, the authors aimed to determine whether pediatric epilepsy surgery, in particular during the first years of life, relates to measurable benefits. METHODS Data from 78 patients (age range 5 months to 17 years) who underwent epilepsy surgery at the Geneva and Lausanne University Hospitals between 1997 and 2012 were reviewed retrospectively. Patients were dichotomized into 2 groups: infants (≤ 3 years of age, n = 19), and children/adolescents (4-17 years of age, n = 59). Compared with children/adolescents, infants more often had a diagnosis of dysplasia (37% vs 10%, respectively; p < 0.05, chi-square test). RESULTS The overall seizure-free rate was 76.9%, with 89.5% in infants and 72.9% in the children/adolescents group. Infants were 2.76 times as likely to achieve seizure-free status as children/adolescents. Postoperative antiepileptic medication was reduced in 67.9% of patients. Only 11.4% of the patients were taking more than 2 antiepileptic drugs after surgery, compared with 43% before surgery (p < 0.0001). The overall complication rate was 15.1% (6.4% transient hemiparesis), and no major complications or deaths occurred. CONCLUSIONS The data show a high seizure-free rate in children ≤ 3 years of age, despite a higher occurrence of dysplastic, potentially ill-defined lesions. Pediatric patients undergoing epilepsy surgery can expect a significant reduction in their need for medication. Given the excellent results in the infant group, prospective studies are warranted to determine whether age ≤ 3 years is a predictor for excellent surgical outcome.

Proteus syndrome revealing itself after the treatment of a bilateral subdural haematoma.

INTRODUCTION: Hypertrophy of the calvarium has different aetiologies, among them the rare Proteus syndrome. CASE REPORT: We report here the case of a young girl initially treated for relapsing right then left large chronic subdural haematoma, who progressively developed craniofacial hypertrophy consistent with the diagnosis of Proteus syndrome. Calvarium hypertrophy was shaved and remodelled combining midface advancement, essentially for cosmetic purposes. During the first calvarium remodelling, important bleeding of the bone required large volume of blood replacement. Haemostasis workup revealed platelets aggregation anomalies. Bleeding issues during subsequent surgeries were controlled with tranexamic acid and desmopressin acetate. DISCUSSION: Other manifestations of Proteus syndrome, such as a right hypertrophy of the face with hypoplasia of its middle third, a pigmented epidermal nevus and asymmetric limbs and scoliosis, appeared progressively over time. Blood and fibroblast phosphatase and tensin homolog mutation was not found. CONCLUSION: Literature review of operated patients with Proteus syndrome did not reveal an association with platelets anomalies. A complete haemostasis workup following this unexpected haemorrhagic complication is recommended for this rare pathology.

Neuropsychological outcome after extra-temporal epilepsy surgery.

BACKGROUND: The neuropsychological results of temporal lobe epilepsy surgery are well reported in the literature. The aim of this study was to analyse the neuropsychological outcome in a consecutive series of patients with extra-temporal epilepsy. METHODS: We retrospectively analysed the data of patients operated between 1996 and 2008 for extra-temporal epilepsy. Standard neuropsychological tests were applied. We assessed the neuropsychological outcome after surgery and the correlation of the neuropsychological outcome with (1) side and localisation of surgery, (2) Engel scale for seizure outcome and (3) timing of surgery. FINDINGS: Patients had a better neuropsychological outcome when undergoing non-frontal resection [χ2 (2) =6.66, p = 0.036]. Subjects who had undergone left or right resection showed no difference in outcome [χ2 (2) =0.533, p = 0.766]. The correlation between the Engel scale for seizure re-occurence and the neuropsychological scores showed only a tendency for better outcome (Spearman ρ = -0.437; p = 0.069). The global measure of change did not correlate significantly with delay of surgery (Spearman ρ = -0.163; p = 0.518). CONCLUSIONS: Resective epilepsy surgery improves neuropsychological status outcome in patients with extra-temporal epilepsy even if the patient did not become seizure free. The outcome is better for non-frontal localisation.

A reminder for a very rare entity: massive tongue swelling after posterior fossa surgery.

OBJECTIVE: The purpose of this study is to report a case of presumably neurogenic macroglossia that occurred after surgical trapping of a vertebral artery (VA)-posteroinferior cerebellar artery aneurysm, and to analyze its potential pathogenesis. CLINICAL PRESENTATION: A 53-year-old woman who suffered from headaches and intermittent loss of consciousness but without evidence of subarachnoid hemorrhage was admitted. Magnetic resonance imaging and angiography showed an irregular aneurysm in the fourth segment of a left dominant VA. INTERVENTION: Surgical treatment was indicated after discussion with the neuroradiology team. During surgery, in the prone position the aneurysm ruptured. The patient became hemodynamically instable. On the first postoperative day, macroglossia appeared and remained for 3 weeks until spontaneous regression. CONCLUSION: Macroglossia is a rare complication following neurosurgical procedures with very few cases reported so far. It has been attributed to the sitting position and venous flow congestion. We illustrate a case of macroglossia, which occurred following surgery in the prone position. Its etiology remains speculative, but a neurogenic explanation seems most plausible.

Primary leptomeningeal melanocytosis in a 10-year-old girl: a challenging diagnosis with a poor prognosis.

Primary leptomeningeal melanocytosis is rarely encountered in the pediatric population. Despite being considered a nonmalignant condition, it uniformly carries a poor prognosis given the ineluctable progression of meningeal infiltration. The case of a previously healthy 10-year-old girl who was first thought to suffer from recurrent hemiplegic migraine is reported. She later developed insidious subacute hydrocephalus. Meningeal biopsy performed during shunt insertion demonstrated an unexpectedly large number of melanocytes consistent with meningeal melanocytosis. Subsequently, the child developed recurrent shunt dysfunction and showed evidence of malignant transformation. The steps to reaching the proper diagnosis are discussed, and the current literature on this rare clinical entity as well as on related central nervous system melanocytic lesions that can occur in the pediatric population is reviewed.

How I do it: cervical lateral mass screw fixation.

BACKGROUND: Cervical lateral mass screw fixation is indicated for the treatment of cervical subaxial C3-C7 lesions associated with instability. METHOD: The authors first describe the surgical anatomy of the subaxial cervical posterior approach. Then the Magerl technique is detailed. In particular, tricks to avoid complications are presented. The ideal screw entry point, direction, size and exit point are mentioned. A surgical video, artist's drawings and a radiological case report are included. CONCLUSION: The Magerl technique is a safe and effective lateral mass fixation technique. Respecting anatomical landmarks is crucial to avoid nerve root, vertebral artery and facet joint injury.

[Hemorrhagic strokes in children: etiology and management]. / Accidents vasculaires cérébraux hémorragiques spontanés chez l'enfant: etiologies et prise en charge.

Pediatric particularities and management of pediatric hemorrhagic strokes are reviewed. Etiologies of hemorrhagic strokes in children are quite different than in adults. Arterio-venous malformations are much more frequently encountered than aneurysms, cavernous malformations and other non structural causes. Modem imagery allows to diagnose the cause of the hemorrhage with a good security and the management is based on the association of neurosurgery, interventional neuroradiology and radiosurgery. These cases must be handled in specialized tertiary care hospital where these three modalities of treatment are available 24 hours/day.

Symptomatic syringomyelia occurring as a late complication of posterior fossa medulloblastoma removal in infancy in a boy also suffering from scaphocephaly.

INTRODUCTION: The association of a medulloblastoma and a syringomyelia has been already described in rare instances albeit without symptoms related to the syrinx. CASE REPORT: The case of a 23-year-old man operated in infancy for a medulloblastoma and then treated solely with adjuvant chemotherapy is reported. He was also operated in infancy for a scaphocephaly. With a very long time delay, he has developed a Chiari I and a symptomatic cervico-dorsal syringomyelia. The symptoms attributed to the syrinx consisted of a unilateral prurigo over the left arm which was so severe to lead to self-mutilation. DISCUSSION: Clinical and magnetic resonance imaging follow-up after cervico-dorsal decompression shows a significant improvement of the symptoms together with a reduction of the size of the syrinx. This case is discussed in the light of the presumed pathophysiology of the syrinx and its exceptional clinical presentation.