Christian Sainte-Rose

Optimal treatment for pediatric craniopharyngioma remains controversial. Although conservative surgery followed by radiation is favored, particularly when hypothalamic infiltration. Proton beam therapy (PT) offers further sparing of normal tissues exposure compared to photons. The objectives of this study were; To assess the efficacy of limited surgery followed by PT in children with craniopharyngioma; To evaluate feasibility of dose escalation while maintaining risk-adapted dose constraints to the chiasm. The trial included 33 patients (17 males), median age 9.0 years (2.1–15.5) at diagnosis, 9.6 years (3.3–15.8) at radiation. All patients underwent surgery (1–5, median: 2) with measurable residue. Median interval between diagnosis and PT was 7.2 months (2.5–73.6). Median PTV was 31.1 cm3 (12.7–136). Imaging studies during radiation identified increase in size of cystic components in 11 patients, including 2 who required re-planning. Total median dose was 54.4 CGE (52.2–56.1). Based on dosimetric parameters, 5 patients underwent slight dose escalation. Median dose to the chiasm was 52.7 CGE (51.8–54.4). At a median follow-up of 4.3 years (1.3–7.0) since radiation, 32 patients are alive. 3 patients experienced progression. All patients are on hormone replacement, 25/32 patients have normal vision in at least one eye, 17/32 have restricted fields. 24 patients have normal academic status, 8 patients require special schooling. Median BMI is 24.1 (18.7–36.3), 11 patients are overweight and 9 obese.

Frontal lobe function is often evaluated with diffuse lesions such as traumatic brain injury, frontal lobe epilepsy. Few cases study has been reported, evaluating frontal lobe dysfunction after focal lesions, such as tumoral lesions. The aim of the study was to evaluate social cognition and theory of mind performances in 21 patients treated for benign or malignant frontal lobe tumours in childhood. To our knowledge; no study to date has directly measured social cognition aspects and theory of mind aspects in frontal benign or malignant lobe tumours in children and adolescents. 1st ans 2nd order false-belief tests of theory of mind tests (Sally & Ann test, Smarties), facial expression recognition, motor imitation tests were performed. 21 patients (mean age at the time of evaluation: 14.3 years old, mean age at the end of treatments: 9.3 years-old) treated for frontal benign (n=4)/malignant (n=17) lobe tumour during childhood were included in our study. A comparison group of 42 patients were matched on gender, age and level education. Whereas statistical analysis of our study revealed no theory of mind disturbances in 1st and 2nd order false belief in our population, specific measures were identified as discriminating the most patients from controls, such as facial expression recognition test and motor imitation tests. The role and impact of neurons mirrors in our population is discussed.

Objective Abusive head trauma (AHT) is defined as a severe, non-accidental traumatic brain injury. Early recognition and treatment are instrumental in limiting the immediate complications and long-term disabilities. The goal of this study was to describe our experience with traumatic head injuries in children younger than 2 years of age. Methods We reviewed the medical records of 195 children aged under 2 years with suspected AHT who presented with a head injury without witnessed accidental trauma, between January 2008 and June 2013. Results AHT was considered in 145 children. Familial problems (ρ = 0.008), cutaneous hematoma/bruising (ρ < 0.001), retinal hemorrhages (ρ < 0.001), and bone fractures (ρ = 0.04), were significantly more frequent in the AHT group. Conclusions The association between the subdural hematoma and retinal hemorrhage, resulting from an unwitnessed and incoherent history of trauma, is a strong argument for AHT, particularly when associated lesions and socioeconomic risk factors are evident. Objetivo O traumatismo craniano por abuso (AHT) é definido como uma grave lesão cerebral traumática não acidental. O reconhecimento e tratamento precoce são fundamentais para limitar as complicações imediatas e sequelas tardias. O objetivo deste estudo foi descrever a nossa experiência em crianças menores de 2 anos de idade, vítimas de trauma craniano. Métodos Foram revisados os prontuários de 195 crianças com idade inferior a 2 anos com suspeita de AHT, sem trauma acidental testemunhado e com diagnostico de hematoma subdural, entre janeiro de 2008 e junho de 2013. Resultados AHT foi considerado em 145 crianças. Problemas socioeconômicos familiares (ρ = 0,008), hematomas e lesões cutâneas (ρ <0,001), hemorragias retinianas (ρ <0,001), e fraturas em ossos longos (ρ = 0,04), foram significativamente mais frequentes no grupo de crianças com suspeita de AHT. Conclusões A associação entre hematomas subdurais e hemorragia retiniana, resultante de uma história incoerente de trauma sem testemunhas, é um forte argumento para a AHT, particularmente quando lesões cutâneas e fatores de risco socioeconômicos forem identificados.

Objective The aim of this prospective study was to analyze the changes in cerebrospinal fluid (CSF) distribution after endoscopic third ventriculocisternostomy (ETV). Methods Twenty-two hydrocephalic children (eight boys, aged 3 months to 17 years) candidates for ETV were studied by preoperative brain magnetic resonance (MR) and repeated post-ETV MRs at established time intervals. A volumetric analysis of CSF distribution after ETV was performed using a specific software. Results Fifteen children had an uneventful follow-up, whereas four required a second ETV due to a secondary closure of the stoma, one died of acute intracranial hypertension, and two needed an extrathecal shunt. A progressive reduction in the volume of the ventricles was found in case of successful ETV during the follow-up period. The ventricular volumes were reduced in average of 76 % of the initial volume at day 3, 69 % at 2 weeks, 42 % at 2 months, and 40 % at 6 months. This finding was associated with an enlargement of subarachnoid spaces which increased in case of successful ETV (192 % of initial volume at day 3; 210 % at day 15; 428 % at 2 months; and 468 % at 6 months). In case of secondary closure of the stoma, the distribution of intra- and extraventricular CSF tended to go back to the preoperative status. Conclusion Volume variations of the ventricles and the subarachnoid spaces are a good indicator of the efficacy of the ETV.

Object The aim of this study was to retrospectively review the clinical presentation, the roles of surgery and adjuvant therapy, and the treatment-related morbidity in children with a ganglioglioma in the posterior fossa and to try and determine the prognostic factors. Methods Between 1991 and 2006, 10 children were treated for a posterior fossa ganglioglioma at the authors' institution. The mean age of the children, the duration of symptoms prior to diagnosis, and the follow-up were 8.2, 2.4, and 3.9 years, respectively. Nine of the children presented with symptoms of raised intracranial pressure. Preoperative imaging showed enhancement in all patients; in eight it was in a patchy distribution. The operations consisted of radical resection (> 75%) in seven children, biopsy in two, and a total macroscopic excision in one. Results The surgical procedure did not cause deterioration in the neurological condition in any of the children. There was no recurrence in the child who underwent total macroscopic exci...

Object Empirical radiotherapy is the current treatment for children with diffuse pontine lesions that have imaging characteristics of an infiltrative malignant astrocytoma. The use of chemotherapeutic agents is, however, currently under investigation in the treatment of these tumors. To be included into a trial, patients need a definitive histological diagnosis. The authors present their prospective study of the stereotactic biopsy of these lesions during a 4-year period. Methods A suboccipital, transcerebellar approach was used to obtain biopsy samples in 24 children. Results Two patients suffered deficits. Both had a transient (< 2 months) new cranial nerve palsy; one of these patients also experienced an exacerbation of a preoperative hemiparesis. No patient died during the perioperative period. A histological diagnosis was made in all 24 patients as follows: 22 had a malignant infiltrative astrocytoma, one had a low-grade astrocytoma, and one had a pilocytic astrocytoma. The diagnosis of the latter tw...

Object Two to five percent of pediatric brain tumors are located in the thalamus. The optimal management for these tumors remains unclear. The aim of this study was to determine whether clinical and neuroimaging features could guide treatment, and to what extent these features, together with histological diagnosis and treatment modalities, influenced survival. Methods The records of 69 children who presented with a thalamic tumor between 1989 and 2003 were retrospectively reviewed. Three groups of tumors were analyzed separately: 1) unilateral thalamic tumors (54 lesions); 2) thalamopeduncular tumors (six); and 3) bilateral thalamic tumors (nine). In the patients in whom a unilateral thalamic tumor was diagnosed, 33 had an astrocytic tumor. Of the 54 patients, 32 had a low-grade and 22 had a high-grade tumor. The survival rate was significantly better for patients with the following characteristics: symptom duration longer than 2 months (p < 0.001), lesions with low-grade histological features (p = 0.003)...

Craniopharyngiomas are benign slow growing tumours that are located within the sellar and para sellar region of the central nervous system. The point prevalence of this tumour is approximately 2/100,000. The onset of symptoms is normally insidious with most patients at diagnosis having neurological (headaches, visual disturbances) and endocrine (growth retardation, delayed puberty) dysfunctions. Craniopharyngiomas are thought to arise from epithelial remnants of the craniopharyngeal duct or Rathke's pouch (adamantinomatous type) or from metaplasia of squamous epithelial cell rests that are remnants of the part of the stomadeum that contributed to the buccal mucosa (squamous papillary type). The neuroradiological diagnosis is mainly based on the three components of the tumour (cystic, solid and calcified) in the characteristic sellar/para sellar location. Definitive diagnosis is made following histological examination of a surgical specimen. The differential diagnosis includes other tumours in this region (pituitary adenoma), infectious or inflammatory processes (eosinophilic granuloma), vascular malformations (aneurysm) and congenital anomalies (Rathke's cleft cyst). The current treatment is gross total excision of the tumour, if there is no hypothalamic invasion or, in the presence of hypothalamic invasion, a sub-total resection with post-operative radiotherapy. Endocrine disturbances are normally permanent and need careful replacement. Overall, there is an 80% 5 year survival, though this can be associated with marked morbidity (hypothalamic dysfunction, altered neuropsychological profile).