[Optic nerve glioma in children]. / Gliome du nerf optique chez l'enfant.

Optic pathway gliomas are rare tumors accounting for 3-5% of brain tumors in children; 90% are observed in children. They account for 4% of orbital tumors in children. Association with NF 1 is classical and the incidence of NF 1 in patients with optic pathway gliomas is estimated at 30-58%. Usually these tumors are pilocytic astrocytomas and their regression, particularly in patients with NF 1, has been described. Exophthalmos and loss of visual acuity or blindness are the usualpresentation in progressive disease. CT and above all MRI study of the orbit and the brain are necessary to make a complete evaluation of the disease. Gross total removal of the tumor provides 100% cure, but surgery can be proposed only for growing tumor with severe exophthalmos and no useful field of view or blindness. Chemotherapy must be proposed as first-line treatment for growing tumor with moderate exophthalmos and useful vision when the tumor is strictly confined in the orbit. The place of radiotherapy for pure intraorbital gliomas has not been defined and proton beam therapy has to be evaluated.

[Central nervous system cavernomas in children]. / Cavernomes du système nerveux central chez l'enfant.

UNLABELLED: Cavernomas represent 1.7 to 18% of all vascular malformations in children and 25% are observed in children under 18 years of age. Cases observed in neonates and infants have been published, but the mean age varies from 9.1 to 10.2 years. There is no predominance between boys and girls. CLINICAL PRESENTATION: In children, hemorrhage is a common manifestation with an incidence varying from 27.3 to 78% versus 8 to 37% in adult patients. Isolated headaches occur in 2.8% of patients and elevated ICP is observed in 20.1%. Epilepsy is reported in 16 to 60% of children, depending on the series. Neurological deficits are observed in 22.7% of patients and are more severe for deep-seated and brainstem cavernoma. About 14.2% of the cases are discovered fortuitously in asymptomatic patients. Spinal cord deficits are observed in 5% of the cases. LOCATION: Using data in the literature plus our personal series of 57 cases, 79.4% of lesions are in the supratentorial compartment and 20.6% in the posterior fossa, the majority located in the brainstem, most of them in the pons. Spinal cord cavernomas represent 5% and multiples cavernomas (12.6%) of the reviewed cases. MANAGEMENT: Appropriate management of cavernomas has long been a subject of much debate. Today, a consensus has been reached to favor medical management of asymptomatic and non hemorrhagic lesions and surgical management of symptomatic and/or hemorrhagic cavernomas whatever the localization. Progress in neuroimaging, surgical mapping, intraoperative monitoring and microsurgical techniques has greatly contributed to improved approach to those lesions. RESULTS: Results obtained in 217 cases were reviewed. Near 70% of the children are neurologically intact, 19.3% are improved or stable, 2.7% worsened and 1.13% died. Results for epilepsy are very encouraging, surgery is efficient in almost all the children except for temporal lobe cavernomas where invasive presurgical evaluation is recommended. Deep-seated and brainstem cavernomas can safely be removed in most of the cases. Only two children died from recurrent hemorrhage due to residual lesions.

[Pediatric supratentorial oligodendrogliomas: Marseilles and Lyons experiences]. / Les oligodendrogliomes supratentoriels de l'enfant. A propos des séries arseillaises et lyonnaises.

BACKGROUND AND PURPOSE: The goal of this study was to analyze the main aspects of oligodendrogliomas observed in children. METHOD: The records of 35 children aged 15 years or younger (23 from Marseilles and 12 from Lyons) were reviewed. Clinical signs and symptoms, imaging findings (CT scan and pre- and post-operative MRI), extent of surgical resection, histology according to the WHO and Ste-Anne grading and survival were analysed. Considering all these factors, a statistical analyzis was undertaken in order to identify prognostic factors. DISCUSSION AND CONCLUSION: Oligodendrogliomas are rare tumors in children. The most important differential diagnosis to discuss is dysembryoplastic neuroepithelial tumor. Our study allowed us to distinguish several subgroups of patients with a different prognosis: thalamic tumors with a dismal prognosis versus hemispheric tumors. A group of cortical tumors we called "DNT-like" (hemispheric cortical tumor, isolated epilepsy, without neurological deficit and reased ICP, without edema and mass effect on MRI) with an excellent prognosis like the group with epilepsy. Histological grading (grade A/grade B and grade II/grade III) is also a prognostic factor.

[Hydroxyurea treatment for unresectable meningioma]. / Traitement des méningiomes par hydroxyurée.

BACKGROUND: Management of unresectable progressive meningioma remains controversial and constitutes a major challenge since therapeutic options including chemotherapy and hormone modulation are limited. Recent data have suggested that hydroxyurea treatment may have an antitumoral effect. The purpose of this prospective phase II study was to evaluate the efficacy of hydroxyurea treatment for unresectable progressive meningioma. METHODS: From 1997 to 1999, consecutive patients presenting unresectable meningioma with clinically and/or neuroradiologically documented progression were considered for entry into this protocol. Previous radiotherapy was not a mandatory inclusion criteria. Treatment consisted of continuous oral administration of hydroxyurea at a dose of 20 mg/kg per day. Follow-up assessment included physical examination, computed tomography (CT), and magnetic resonance imaging (MRI) performed every three months, as well as regular blood testing. The primary endpoint was documentation of objective response by MRI or CT. RESULTS: The intent-to-treat population was 43 patients with at least 18 months follow-up. Median age was 60.4 years. Twenty-eight patients had undergone surgery following initial diagnosis. The meningioma was located in the skull base in 67% of patients. Histology was benign in 18 and atypical in 10. The eligible population included 36 patients with documented progressive disease at the time of inclusion; with progression documented clinically in 29 (67.5%) and/or radiologically in 20 (46%). In 7 patients, clinical or radiological progression could not be confirmed. The intent-to-treat analysis at median 26 months follow-up revealed objective response to hydroxyurea in only 3 patients (7%) including one on the basis of improvement in visual symptoms and two on MRI analysis. Progressive disease was observed clinically or radiologically in 26 patients (60.5%). Of the eligible population (n=36), 2 achieved an objective response and 13 (36%) exhibited stabilization under hydroxyurea therapy, while 21 (58%) progressed under treatment. Overall tolerance was good but anemia (grade I-II) and asthenia (grade I-II) were observed in 28% and 23.5% respectively. Treatment was discontinued in 3 patients because of chronic skin toxicity in one and anemia and asthenia in two. CONCLUSION: Hydroxyurea treatment is of marginal efficacy for meningioma and must not be considered as an alternative if radiotherapy or surgery is feasible. New efficient medical treatments are still required for progressive meningiomas.

[Shaken baby syndrome: improvement of cerebral blood flow velocity after a subdural external derivation in a six-month old infant]. / Le syndrome du bébé secoué: amélioration de la circulation cérébrale après dérivation sous-durale externe chez un nourrisson de six mois.

The shaken baby syndrome is a severe form of child abuse. The intracranial injuries are associated with a high morbidity and mortality rates. A 6 month-old healthy infant presented at home a cardiorespiratory arrest. After a cardiopulmonary resuscitation, radiological survey showed sub-dural haematomas and retinal haemorrhages, without a history of trauma. The diagnosis of shaken baby syndrome was made. Despite medical management and a fontanelle tap, clinical signs of intracranial hypertension worsened. Transcranial Doppler examination found the cerebral blood flow velocities to be decreased while the pulsatility index was increased. A sub-dural-external drainage allowed the cerebral blood flow to increase and the pulsatility index to decrease. We conclude that transcranial Doppler examination can be helpful for the clinician caring children presenting a shaken baby syndrome.

[Craniopharyngioma in children: MRI study of 43 cases]. / Les crAniopharyngiomes de l'enfant.

BACKGROUND AND PURPOSE: Craniopharyngiomas are intra-cranial tumors, relatively frequent in children, expanding in the pituitary stalk axis, from the third ventricle to the sphenoid body. Plain films and CT scan generally show a calcified lesion, deforming the sella turcica. MRI improves tumor description and topographic and structural analysis of the lesion. The aim of this study is to analyze the MRI aspect of craniopharyngiomas in a pediatric population and to correlate findings with surgical data. METHODS: MR and CT studies of 43 pediatric cases of histology-proven craniopharyngiomas were reviewed retrospectively. Tumor emergence, extensions and signal on different sequences were recorded. We searched for radio-surgical correlations. RESULTS: Craniopharyngiomas can be classified into two groups: intra-sellar tumors and third ventricle floor tumors (infundibulum and tuber cinereum). Preferential routes of extension are observed in each group correlated with consistency (cystic and/or solid). Surgical data confirmed these results. CONCLUSIONS: MRI is crucial for the pre-therapeutic evaluation of craniopharyngiomas allowing not only a detailed description of the tumor but also guiding therapeutic decisions. This series demonstrated that craniopharyngiomas exhibit two different types of localization and behavior. Embryonic development of the tumor explains the topographical differences.

[Brain stem cavernomas in children. Nine case reports and literature review]. / Cavernomes du tronc cérébral chez l'enfant.

We report our experience with 9 cases of brain stem cavernoma in children aged 15 years during the period 1987-2000. All patients were admitted with lesion-related symptoms, except in one where the brain stem cavernoma was discovered on a routine MR scan. A female predominance was noted in the present study. In 2 children, brain stem cavernoma was associated with multiples lesions. All patients except two with multiple cavernomas underwent surgery. One child died 2 months after surgery from recurrent hemorrhage. The eight others are alive. Among the 8 survivors, only one has worsened. Children with brain stem cavernoma who develop persistent or progressive deficit are candidates for surgery. When surgery is indicated, total removal is mandatory to avoid the risk of recurrence and rebleeding.

Weaning children from tube to oral feeding.

Most of the children seen by specialists in neonatalogy, neuropaediatrics or neurosurgery do not have any problems in starting with oral feeding after a period of tube feeding lasting between 15 and 20 days. Children who have been tube fed for a longer period, however, can find it very difficult or even impossible to re-establish oral feeding when they have sufficiently recovered from their underlying problem. To cope with this situation we propose a procedure based on the afferentation or re-afferentation of the oropharyngeal cavity by sensory stimulations and by re-establishment of the biological clock (circadian rhythm) by applying these stimulations during tube feeding at regular hours. In 19 children who showed difficulties oral feeding became possible a short time after such a procedure had been applied. If the principles of swallowing neurophysiology and the biological rhythm are respected, this procedure, which also involves a contribution from the family, leads to quicker oral feeding and shorter stay in hospital.