[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.

[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 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.

[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.

Lipomatous differentiation in ependymomas: a report of three cases and comparison with similar changes reported in other central nervous system neoplasms of neuroectodermal origin.

Three cases of surgically removed ependymomas with lipomatous transformation of tumor cells are reported. Patients' ages were 13, 16, and 48 years at the time of operation. One patient's tumor was located in the third ventricle; the other two occupied paraventricular hemispheric white matter. Histologically all three cases fulfilled the criteria of ependymomas. In case 1, electron microscopy also confirmed this diagnosis, and preoperative radiologic studies (scans) suggested large amounts of lipids to be present in the tumor. Histologically, in all three cases many tumor cells contained fat droplets coalescing into a single large droplet, resulting in an appearance indistinguishable from adipocytes by conventional stains, but maintaining immunohistological positivity for glial fibrillary acidic protein and neuron-specific enolase in the cytoplasmic rims of the affected cells, attesting to their glial nature as opposed to being true adipocytes in a mixed glial/mesenchymal hamartoma. The alterations were also different from the "xanthomatous" changes seen in some gliomas. Lipomatous transformation of neuroectodermal tumor cells has been previously observed in neurocytomas, medulloblastomas, cerebellar and spinal cord astrocytomas, and primitive neuroectodermal tumors. Our three cases represent the first reported ependymomas with such changes. In medulloblastomas of adults, lipomatous changes have been found to signal relatively benign biologic behavior. So far, all three of our patients are doing well, but only more extended follow-up will show whether such benign behavior applies to lipomatous ependymomas as well.

Differential expression between PAX-6 and EN proteins in medulloblastoma.

A collection of 28 medulloblastomas was analyzed for expression of the developmental control genes PAX-6 and EN by immunohistochemical staining. Sixteen medulloblastomas expressed both EN and PAX-6 but, when differentiation could be assessed in the positive areas, PAX-6 is expressed in the less differentiated cells. Since Drosophila en encodes a negative regulator, we overexpressed the chicken en-1 in retinal pigmented epithelium cells. This resulted in Pax-6 down regulation. These results suggest a regulatory loop between PAX-6 and EN, two molecular markers of medulloblastoma.

Usefulness of the orbitofrontomalar approach associated with bone reconstruction for frontotemporosphenoid meningiomas.

Seven cases of frontotemporosphenoid meningioma are reported. The principles of the orbitofrontomalar approach and of reconstruction of the orbital cone and of the frontomalar region with an orbitotemporal prop are discussed.

Subduroperitoneal drainage for subdural hematomas in infants: results in 244 cases.

OBJECT: Subduroperitoneal drainage (SDPD) is commonly used in the treatment of infantile subdural hematomas (SDHs). Few studies have focused on this technique and most series have included SDHs of various origins in children of different ages. The surgical procedure is not standardized and results achieved using this technique have not been well documented. The authors reviewed their cases of traumatic SDH treated with SDPD in infants (< 2 years of age). Their standard technique includes bilateral SDPD whenever the SDH is bilateral, placement of a free shunt, and systematic removal of the drainage unit after a few months. METHODS: The authors performed SDPD in 244 infants with traumatic SDH. The patients' SDHs were controlled by SDPD in 241 cases, and 78.9% of the patients recovered to live a normal life. Complications of SDPD occurred in 38 patients (15.6%): obstruction in 22 cases (9%), infection in eight cases (3.28%), and internal hydrocephalus in eight cases (3.28%). Early complications could be ascribed to surgical technique, delayed complications were associated with the severity of the initial clinical presentation, and late complications were time dependent and unrelated to initial clinical severity. Poor clinical outcome was correlated to the severity of the initial presentation, but not to complications of surgery. CONCLUSIONS: Because of its efficacy and low complication rate, SDPD is the procedure of choice when subdural taps fail to control SDH. The authors prefer bilateral drainage because of the low rate of complications. Drains should be systematically removed after a few months to prevent long-term complications.

Traumatic extradural hematomas in infancy and childhood. Experience with 144 cases.

The authors report a series of 144 children with traumatic extradural hematomas operated on at the Lille Department of Neurosurgery between 1969 and 1982. The patients are divided into different groups according to age, and clinical findings were recorded for each age group. The overall mortality rate was 9%. The authors demonstrate that prognosis is related to age, neurological status at time of surgery, and duration of postoperative coma.

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.

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.

Cerebellar medulloblastoma: results of multidisciplinary treatment. Report of 120 cases.

The authors report a series of 120 cerebellar medulloblastomas. All patients were operated on between 1953 and 1982. Among them, 88 completed the treatment with radiotherapy, and 32 had additional chemotherapy. The operative mortality was 22.5%. The 5-year survival rate was 30% in the whole series. Recurrences occurred in 35 patients, associated with supratentorial or spinal metastasis in 60% of cases.

Strategy of craniofacial reconstruction after resection of spheno-orbital "en plaque" meningiomas.

Surgical resection of spheno-orbital "en plaque" meningiomas should be as complete as possible to prevent tumor recurrence and therefore requires a bone reconstruction. We report a series of 20 patients operated on for spheno-orbital "en plaque" meningioma between 1981 and 1993. The surgical treatment included a resection of the involved dura and a wide resection of tumoral bone using a fronto-temporal craniotomy extended to the orbitozygomaticomalar bone ridge. The craniofacial reconstruction was performed in the same operative procedure using iliac bone autograft in 11 patients, internal cortical bone from the bone flap in 8 patients, and a coral graft in 1 patient. The cosmetic result was scored according to the following criteria: superior frontal paralysis, appearance of the orbitomalar bone ridge, shape of the external temporal fossa, and projection of the eyeballs. The cosmetic result was scored as excellent or good in 17 patients, average in 2 patients, and poor in 1 patient. The iliac bone autograft appeared to be the best material for craniofacial reconstruction because it could be modeled easily to the desired shape. However, the reconstruction technique was modified as necessary according to the extent of tumor removal, clinical presentation, and age of the patient.

Peritoneal pseudocyst--ventriculo-peritoneal shunt complications.

Pseudocyst formation is a rare complication of ventriculo-peritoneal shunt, occurring in only 22 cases of 1300 shunts from 1968 to 1992. The most common presentation is that of abdominal signs rather than neurological or infectious signs. The diagnosis is easy with ultrasonography. The difficulty is to evocate the cyst and to correlate symptoms and cyst. Treatment of the cyst was by aspiration (21 cases) and excision in 7 cases. The therapeutic choice is made according to the ultrasound findings. When the cyst was infected, an external ventricular shunt was used temporary, but this type of shunt must be avoided where possible. Three patients died.

Craniofacial dysmorphism in Mozart's skull.

Mozart's craniofacial dysmorphism shown in his portraits and in the skull held by the Mozarteum in Salzburg (Austria) helps to document the role of pathology in human identification. The specific syndrome is formed by a premature synostosis of the metopic suture (PSMS) in association with an abnormally shaped skull.

[Postero-medial bulbar hematoma with a favorable outcome. Study of dysautonomia]. / Hématome bulbaire postéro-médian d'évolution favorable. Etude de la dysautonomie.

A case of postero-medial medullary hematoma in a 21 year-old woman is reported. An initial neurovegetative severe disregulation had a favorable outcome after surgery. Late deficits, characterizing the postero-medial medullary syndrome have been studied: paresis of the tongue, suppression of the nauseous reflex, instability with retropulsion, and downbeating nystagmus in primary position of the eyes were noted. Heart rate and arterial blood pressure analysis showed explosive tachycardic reactions and transitory disappearance of oculo-cardiac reflexes, suggesting the involvement of the efferent parasympathetic system coming from the dorsal motor nucleus of the vagus nerve.

[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.

[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.