[Vascular lesions of the orbit in children]. / Pathologies vasculaires de l'orbite de l'enfant.

Vascular lesions of the orbit in children are most often hemangiomas and lymphangiomas. Hemangioma is a tumor that tends toward spontaneous regression. Lymphangioma is a malformation that tends to increase in size with growth spurts that can occur until adulthood. The treatment is decided according to the functional impact.

[Ophthalmologic characteristics of spheno-orbital meningiomas: a series of 23 surgical cases]. / Manifestations ophtalmologiques des méningiomes sphéno-orbitaires. A propos de 23 cas chirurgicaux.

PURPOSE: The authors report the ophthalmological characteristics and the postoperative results of patients presenting with spheno-orbital meningioma. DESIGN: A retrospective study of 23 charts of patients, presenting with spheno-orbital meningioma between 1994 and 2007, was performed. The data of preoperative and postoperative ophthalmologic examinations, perimetry and visual evoked potentials were collected. RESULTS: All the 23 patients were female. Diagnostic elements were proptosis (n=18), filling of the temporal pit (n=12) and visual impairment (n=11). Diagnosis was confirmed by orbital imaging. Perimetry and visual evoked potentials enabled to detect asymptomatic impairment of visual function, perimetry being the more sensitive method. Neurosurgery was the gold standard treatment, completed with radiotherapy in some cases of incomplete surgery or recurrence. CONCLUSION: Meningioma without optic nerve compression in the optic canal had better postoperative results with stabilization or improvement of the visual acuity compared with more unpredictable results obtained in cases with preoperative compression in the optical canal. Long term follow up of these patients is necessary because of the risk of relapse (n=3).

[Benign intracranial hypertension: the role of medical treatment]. / Hypertension intracrânienne bénigne: place du traitement médical.

The goal of medical treatment in benign intracranial hypertension (BIH) is to treat intracranial hypertension symptoms as well as to preserve vision. Reducing the production rate of cerebrospinal fluid can be achieved using acetazolamide and/or furosemide (carbonic anhydrase inhibitors), although acetazolamide is the most effective drug. The use of steroids is debatable in BIH. This review focuses on the pathophysiology of these medications, followed by the report of a series of 16 pediatric patients suffering from BIH (1996-2006). BIH was idiopathic for eight children. Depletive lumbar punctures were effective, but this result was often transient. All children were treated with acetazolamide. Doses of acetazolamide (10-20mg/kg per day) must be given every 8h to respect its kinetics. This treatment has to be continued for at least several months and decreasing the dosage must be progressive. Hypokalemia is always prevented with oral potassium. There was only one true treatment failure requiring surgery. The authors therefore advise acetazolamide as a first-line treatment (combined with etiologic treatment, if available, in cases of nonidiopathic situations) in BIH.

[The adolescent to adulthood transition of persons with traumatic-brain injury: the physical-medicine point of view]. / La transition adolescent à adulte du traumatisme crânien: le point de vue de la médecine physique.

Traumatic-brain injury (TBI) is relatively frequent and can involve children and adolescents; it causes not only physical but also important neuropsychological and behavioral problems that can impair familial, social, and professional reintegration. The affected person requires long-term follow-up of medical, psychological, and social problems. The transition from childhood to adulthood, which can cause problems related to the lack of organization and training of specialized TBI medicosocial teams and structures in adult care for both the family, which is reluctant to change, and the patient, who often shows anosognosia, therefore requires organized coordination between pediatric and adult-care teams. This transition must be prepared early and in collaboration with the patient and his family. It first concerns physicians, both pediatric and adult specialists, who need to develop closer collaboration and cooperation. However, the patient should remain the main actor, because the objective is to transfer diversified healthcare, which depends on the TBI patient's individual problems. This relates to medical treatments and, more generally, the life project, which should not be disturbed by divergent practices. The main measures enabling harmonious transfer are training, establishment of specific procedures, and, most particularly, networking.

[The transition from childhood to adulthood in neurosurgery: a description]. / La transition de l'enfant à l'adulte en neurochirurgie: exposition du problème.

The transition from childhood to adulthood is a difficult period for neurosurgical patients, who are at risk of interrupted follow-up, causing delayed diagnosis of complications and compromised outcome. Many of these patients harbor chronic diseases that have an impact on their adult life, may present new developments and sometimes decompensate suddenly. The authors review their experience with 601 patients treated for hydrocephalus, myelomeningocele, or tumors, and followed after their 20th birthday. Mortality, morbidity and socioprofessional outcome statistics are provided, showing the magnitude of the health problem in this population. The authors then review the obstacles to the transition from childhood to adulthood and propose possible solutions. Some problems stem from the patient's lack of information, remedied by early education. Others involve physicians, whose skills may be fragmented and who lack clinical data; this requires greater awareness of this entity, inclusion of lectures on this topic in the medical curriculum and increased clinical research in this field. Finally, obstacles in hospital organization and healthcare financing require that patient support groups and medical societies unite to lobby for improvements. The problems related to the transition from childhood to adulthood in neurosurgery is a complex one, requiring all participants to be involved in the possible solutions. This endeavor should be regarded as an integral part of the duties of both pediatric and adult neurosurgeons.

[Follow-up of adult patients treated during childhood for hydrocephalus]. / Suivi à l'âge adulte des patients traités dans l'enfance pour hydrocéphalie.

The hydrocephalic patient is at risk throughout life of developing complications that may be severe or even fatal. The neurological, developmental, social and occupational outcome is affected by the sequelae of the initial disease and the consequences of intracranial hypertension, but also by imaginary obstacles. Unless proved otherwise, the patient with a shunt must be considered shunt-dependent; shunt independence is rare and must be proved following a rigorous protocol. The hydrocephalic patient should therefore be followed regularly and for life in neurosurgery to screen for and prevent complications as much as possible. Follow-up also allows patient education and coordination by the neurosurgeon and other specialists such as the neurologist and the physical therapist. Organizing the follow-up of the hydrocephalic patient into adulthood is the responsibility of the neurosurgeon in charge; the modalities of this follow-up will vary depending on local conditions. The patient should be educated on the need for this follow-up and prepared for this transition long before it occurs.

Unstable upper pediatric cervical spine injuries: report of 28 cases and review of the literature.

OBJECTIVE: Traumatic lesions of upper cervical spine are rare in children. To evaluate their experience with this lesions and factors affecting outcome, authors conducted a retrospective study of 28 cases of upper pediatric cervical spine injuries treated in the last 25 years. MATERIALS AND METHODS: To help in treatment of these lesions, we studied our series and reviewed the literature. Patients were divided into three groups: 0 to 2, 3 to 8, and 9 to 16 years, and managed according to status at presentation and type of injury. RESULTS: Seven patients were managed surgically and 21 nonsurgically (3 halo braces, 18 hard collars or molded braces). Patients in the younger age group sustained more neurological injuries than the others. In group 1, 33% present a fracture/luxation of C0/C1 or odontoid. In group 2, 80% had sustained fracture/luxations of C2. In group 3, 60% present odontoid fractures. At late follow-up review, solid fusions were demonstrated in all patients. Neurological deterioration occur in six patients. The mortality rate was 12%. Compared with other authors' report, incidence of this lesions increased but not the number of those managed surgically. CONCLUSIONS: Management must be tailored to the patient's age, neurological status, type, and level of injury. Compared with other author's experience, fusion and instrumentation procedures were used less frequently. Signs of medullary compression, significant spine deformation, dynamic instability, and age higher than 8 years are the criteria for surgery. The criteria for instability in children are different from those used in adults because the residual spinal growth is a major concern. The best treatment is therefore preventive.

The transition from child to adult in neurosurgery.

The transition from child to adult is a growing concern in neurosurgery. Data documenting long-term follow-up are necessary to define this population's healthcare needs. In order to evaluate the problems posed by the child-to-adult transition in neurosurgery, we have studied the neurological, functional and social outcome of patients treated in our department for tumor of the central nervous system, hydrocephalus or myelomeningocele, and followed beyond the age of eighteen years. A large number of patients suffered from chronic ailments, either sequelae of their initial disease, or delayed complications of their initial treatment, with significant morbidity. The mortality during adulthood was 4.6% in the tumor group, 1.1% in the hydrocephalus group, and zero in the spina bifida group. The proportion of patients employed in normal jobs was 35.6, 18.7 and 11.5% for tumors, hydrocephalus and myelomeningocele respectively. IQ score and performance at school generally overestimated the capacity for social integration. Based on these data and on the available literature, we tried to identify the problems and devise solutions for the management of the transition from child-to-adulthood transition. Many problems present during childhood persist to adulthood, some of which are made more acute because of a more competitive environment, the lack of structures and inadequate medical follow-up. The transition from child to adult must be managed jointly by pediatric and adult neurosurgeons. More clinical research is required in order to precisely evaluate the problems posed by adult patients treated during childhood for the different neurosurgical diseases. Based on these data, a concerted trans-disciplinary approach is necessary, tailored to the specific needs of patients suffering from different diseases.

[Role of lipo-filling in the treatment of sequelae in craniosynostosis surgery]. / Intérêt du lipofilling dans le traitement des séquelles de chirurgie des craniosténoses.

AIM: This study presents an innovative technique of correction of cranioplasty sequelae by lipo-filling in 9 patients treated for cranioplasty in childhood with a long-term follow-up. PATIENTS AND METHODS: A prospective study was conducted from January 2001 to February 2005, including all patients treated with lipo-filling technique in the Plastic Surgery department of Lille. For a period of 4 years and 5 months, 144 patients were operated on with 206 lipo-filling procedures: 30 patients for cosmetic purposes and 114 patients for reconstructive surgery. In the current investigation, we examined the 9 cases of lipo-filling which were performed in complement to craniofacial surgery. All patients presented with a sub-cutaneous fronto-temporal deformation which in 3 of the cases was associated to a frontal medial defect. The assessment of results was performed in the context of post-operative follow-up visits by an observer. RESULTS: Nine patients, with a mean age of 16,7 years and a ratio of 1 female for 2 males underwent a total of 17 lipo-filling procedures. The mean time lapse was 15,06 months. 9 patients judged the result to be good. No complication was observed. CONCLUSION: The S.R. Coleman technique of fat grafting seems to offer several advantages, namely the simplicity of the procedure itself, its apparent innocuity, its reproducibility, as well as patients and surgeons satisfaction. It appears to be an alternative of choice in the corrective surgery of craniosynostosis.

[Functional status, scholastic placement after rehabilitation and follow-up of children with traumatic brain injury: a retrospective study of 48 patients]. / Statut fonctionnel, orientation scolaire en fin de rééducation et organisation du suivi d'enfants traumatisés crâniens: étude rétrospective de 48 patients.

OBJECTIVE: To evaluate the follow-up of children with traumatic brain injury (TBI). POPULATION AND METHODS: A retrospective study during 4.5 years of 48 children with TBI. We measured GOS during admission and after rehabilitation and where children were placed after hospitalisation. RESULTS: Most patients were males; the mean age was 9.4 years (range 14 months to 16.5 years). The rehabilitation length was 11.4 months. Most children (77%) had a good functional outcome. For 23%, placement was in establishments for severely handicapped people, for 35.4%, an environment with specific support, and for 37.5%, an environment without specific support. After 2 years, 43.7% of infants were lost to follow up. CONCLUSION: Rehabilitation and functional results have improved for children with TBI, but some improvements must be made.

Large-cell medulloblastoma with arrestin-like protein expression.

We report a case of a cerebellar large-cell medulloblastoma in a 12-year-old patient. Despite a gross-total resection followed by a radiation therapy and then a chemotherapy, the death occurred 6 months later. The cyogenetic analysis showed an isochromosome 17q. Immunoreactivity for synaptophysin, neurofilaments, chromogranin and arrestin-like proteins was detected, whereas rhodopsin, vimentin, EMA and PAX-6 were negative. In this study, we demonstrate that large-cell medulloblastoma with translocation in chromosome 17q is a neuronal differentiated medulloblastoma with non-photoreceptor characterization. By reverse transcription and polymerase chain reaction (RT-PCR) method, using primers for beta1, beta2 and visual arrestin, we demonstrate corresponding mRNA for beta1, beta2 arrestin but not for visual arrestin. These results suggest that arrestin immunoreactivity in this tumor corresponds to non-visual arrestin. This case corresponds to a new entity of large-cell medulloblastoma. The potential role of a new marker linked to a beta2 adrenergic receptor needs further molecular characterization to be useful.

[Outcome and quality of life after three years in a cohort of patients with severe traumatic brain injury]. / Devenir et qualité de vie à trois ans dans une cohorte de patients traumatisés crâniens graves.

OBJECTIVES: The late outcome of patients with severe traumatic brain injury (STBI) has been investigated by measuring deficits, disability and social handicap, but their quality of life (QOL) has been less evaluated, and not by a direct analysis of the subjective patient "and relatives" QOL. The aim of this study was to investigate this outcome and the QOL, with its predictive factors, 3 years after STBI, in a homogeneous cohort of patients. MATERIAL AND METHODS: We selected all adult patients from the Lille area (north of France) admitted in the CHU in 1995 following STBI. Each was evaluated at home, in the presence of a close relative, using the EBIS document. This one investigates the medical history, initial status and late outcome, as well as the subjective QOL of patients (evaluated by the patient and by a close relative: 0-10 on an analogical visual scale) and close relatives. Relationships between possible explanatory factors and QOL were analysed using correlation tests. RESULTS: Among the 33 patients, 23 survived at three years. The mean initial GCS score was of 5,6/15 and the mean coma duration of 18.5 days. At three years, physical deficits were usually discrete, intellectual deficits more important and the emotional and behavioural problems even more severe. The GOS was of 6 in one patient, 4-5 in seven, 2-3 in seven and 0-1 in eight. Dependence in advanced activities was more sever than in elementary activities. The subjective QOL of patients was discretely lower (m = 5,48/10) than that estimated by close relatives (m = 5,91). The relatives QOL was similarly reduced (m = 5,45). The factors most influencing the patients QOL were the cognitive and behavioural problems and the dependence in the advanced activities and the GOS for the patients QOL, and the behavioural problem and the dependence in advanced activities for the relatives QOL. CONCLUSION: The reduction of the patients "and relatives" QOL was parallel at three years. Emotional and behavioural problem as well as the dependence in advanced activities mainly explained these QOL.

Intracerebral medulloepithelioma with a long survival.

This report concerns a 3-month-old boy where neuroimaging examination showed a large, well-circumscribed, mildly heterogeneous tumor arising in the left ventricle. Pathological findings were compatible with a medulloepithelioma. A survey of published cases of medulloepitheliomas showed this tumor to be highly malignant, possibly displaying the entire range of differentiation from embryonal primitive neuroepithelium to mature cells and usually involving the cerebral hemispheres with a very poor prognosis in this location. On the other hand, medulloepitheliomas occurring in the eye or the orbit generally benefit from a gross-total resection and may present a good prognosis. Curiously, the patient reported here is doing well 7 years after the resection without any postoperative treatment. The exclusive intraventricular location of the tumor and its gross-total resection clearly seems to have contributed to this unusual recovery.

Late shunt infection: incidence, pathogenesis, and therapeutic implications.

Shunt infections (SI) are a major concern in pediatric neurosurgery. Although SI occurs generally shortly after surgery, it can be very delayed in a number of cases. The incidence of late shunt infection (LSI) is not established, and the sources of contamination are poorly understood. We reviewed 1,793 pediatric cases from our database, with a mean follow-up of 9.12 years. We selected 40 cases of SI occurring more than one year after the previous shunt operation. These represented 12.7 % of SI, and the annual incidence of LSI was 0.28 % in our series. Peritonitis, generally due to appendicitis, was the cause of LSI in 11 cases. Hematogenous contamination was diagnosed in eight cases, because the germ was Haemophilus,Pneumococcus, or Listeria, or an ENT infection had preceded SI; the incidence of purulent meningitis was significantly higher in shunted patients than in the general population. LSI was due in seven cases to bowel perforation, and in four to direct inoculation, after abdominal surgery or traumatic exposure of the shunt. In the remaining 10 cases, no potential cause of infection was identified, and persistence of a germ since the previous shunt operation was suspected. SI represents a life-long threat after shunting, and may be unrelated to shunt surgery.

[Chiari type I malformation in children: presentation in 34 cases]. / Malformation de Chiari de type I de l'enfant: modes de révélation de 34 observations.

UNLABELLED: Incidental diagnoses of Chiari I malformation are more and more frequent in pauci or asymptomatic children. The value of neurophysiological investigations for surgical indications is discussed. OBJECTIVE: To determine clinical presentations of Chiari I malformation and relative frequencies of severe and incidental forms, and to evaluate the usefulness of neurophysiological investigations for surgical indications. METHODS: This retrospective study included 34 patients admitted from 1984 to 2000, with Chiari I malformation diagnosis in different pediatric (intensive care, neurology and neurosurgery) units of a university hospital. RESULTS: The clinical presentation was severe (two children died) in four cases (12%). Signs of brain stem compression were found in 41% of cases, scoliosis in 21%, and incidental diagnosis in 26%. The polysomnography, performed in 12 cases, showed sleep-apneas in six cases. One child with an incidental diagnosis of Chiari I malformation had many sleep-apneas which required a surgical treatment. A surgical decompression was performed in 69% of cases, with clinical improvement in 77%. CONCLUSION: The high incidence (12%) of severe forms revealing Chiari I malformations justifies systematic neurophysiological investigations for the initial evaluation and the follow-up of Chiari 1 malformations, including the less symptomatic forms.

[Surgery for craniosynostosis: timing and technique]. / Quand et comment faut-il opérer une craniosténose?

Craniosynostoses are a group of diseases, the presentation of which differs markedly on account of the cranial suture involved. Their impact is cosmetic, cerebral, and ophthalmologic. Syndromic craniosynostoses associate a more or less pronounced faciostenosis, which requires surgical correction as well, because of cosmetic, ophthalmologic or airway problems. Surgical treatment depends on the type of the craniosynostosis and the patient's age; ideally, the child should be operated between 3 and 12 months. This surgery requires a perfect collaboration between neurosurgeon, plastic surgeon, and anaesthesiologist. Surgical correction allows in large measures the preservation of intellect, sight, and body image.

[Follow-up study of head-injured children]. / Devenir de l'enfant traumatisé crânien.

The authors reviewed in a retrospective study 61 cases of severely head injury children who where admitted in the rehabilitation centre to evaluate the outcome 1 and 5 years after the brain injury. Neurologic and neuropsychologic status of children was assessed ad admission, 1 and 5 years later. Duration on intubation and age at time of head trauma were the worst functional prognosis.

[Mid-facial distraction without osteotomy with a trans-facial pin. Report of 4 clinical cases]. / Distraction médiofaciale sans ostéotomie par l'intermédiaire d'une broche transfaciale. A propos de quatre cas cliniques.

After the publications of Mulhbaüer, Raposo do Amaral and mainly the one of Toth and Chin, it has been proved that midfacial distraction was an effective procedure for the treatment of cranio-facial dysostosis. First attempts to distract (in human) the midface without osteotomies has given poor results because devices acting on the zygomatic bone had failed to draw forward the maxilla through a thin maxillo-zygomatic suture which had been dislocated. With the help of a transfacial pin and an external device, we have obtained in four patients an important advancement of the maxilla. We report here the surgical procedure and preliminary results.

[Cerebellar pilocytic astrocytomas in children. Report of 72 cases]. / Les astrocytomes pilocytiques du cervelet de l'enfant. A propos de 72 cas.

BACKGROUND AND PURPOSE: Cerebellar pilocytic astrocytoma (CPA) bears a well-deserved reputation of benignity. However, these tumors infiltrate the brainstem in a number of cases, and total removal may cause morbidity, whereas subtotal removal can lead to recurrence. MATERIAL AND METHODS: To define guidelines for tumor removal, management of tumor remnants and recurrence, we reviewed 72 cases of CPA of childhood operated on in our department since the introduction of CT. RESULTS: Surgical removal was total in 57 cases, had to be withheld because of brainstem infiltration in nine cases, and was revealed subtotal by postoperative imaging in six cases. Two of the latter were reoperated on; six had only radiological controls and did not progress. Early in the series, eight children had external irradiation because of subtotal removal or recurrence. Three children were reoperated on for tumor recurrence, between six and eight years after complete removal. We had no tumor-related mortality; long-term disability could be related to previous mental retardation, the severity of clinical state at presentation, and complications of surgery and irradiation. CONCLUSION: The goal of surgery is to cure the patient with minimal morbidity, however, total removal is not always possible. In our experience: i) tumor remnants can be surveyed radiologically, and reoperated only in case of progression; ii) the indications for radiotherapy should be the exception; iii) clinical and radiological follow-up should be protracted beyond entry into adulthood.