Although endoscopic third ventriculostomy (ETV) has been accepted as a procedure of choice for the treatment of obstructive hydrocephalus, the outcome of this treatment remains controversial with ...regard to age, cause, and long-term follow-up results. The goal of this study was to assess the risk of failure associated with these factors in a retrospective cohort study.
Between 1999 and 2007, 368 ETVs were performed in 350 patients (165 patients < 18 years of age) with hydrocephalus at the University Hospital of Toulouse. Failure of ETV was defined as cases requiring any subsequent surgical procedure for CSF diversion or death related to hydrocephalus management.
Tumors (53%), primary aqueductal stenosis (18%), and intracranial hemorrhage (13%) were the most common causes of hydrocephalus. The median follow-up period was 47 months (range 6-106 months), and the overall success rate was 68.5% (252 of the 368 procedures). Patients < 6 months of age had a 5-fold increased risk of ETV failure than older patients (adjusted hazard ratio HRa 5.0; 95% CI 2.4-10.4; p < 0.001). Hemorrhage-related (HRa 4.0; 95% CI 1.9-8.5; p < 0.001) and idiopathic chronic hydrocephalus (HRa 6.3, 95% CI 2.5-15.0, p < 0.001) had a higher risk of failure than other causes. Most failures (97%) occurred within 2 months of the initial procedure. The overall morbidity rate was 10%, although most complications were minor. Finally, the introduction of ETV in the authors' department reduced the number of shunt insertions and hospital admissions for shunt failures by half and was a source of cost savings.
Endoscopic third ventriculostomy is a safe procedure and an effective treatment option for hydrocephalus. Factors indicating potential poor ETV outcome seem to be very young children and hemorrhage-related and chronic hydrocephalus in adults.
A 21-day-old male infant was admitted with signs of intracranial hypertension. Brain magnetic resonance imaging (MRI) revealed a voluminous mass in the posterior fossa with an intense peripheral ...enhancement on T1 images with gadolinium. The child was treated secondarily by surgical decompression of the posterior fossa and the lesion was biopsied. The pathological findings indicated infantile hemangioma. Treatment with oral prednisolone was initiated at 3 months, given the lack of tumor involution. Six months after corticotherapy was stopped, repeated MRIs indicated a significant reduction in tumor size and then complete disappearance. Psychometric evaluation was performed at the age of 15 years, showing heterogeneous cognitive disabilities, with verbal abilities superior to nonverbal abilities and delayed motor development. Neurological examination was normal with no focal deficit. To our knowledge, this is the first published case reporting the long-term evolution of a patient with neonatal intracerebral hemangioma. We conclude that psychometric evaluations should be part of the long-term follow-up of children who have had an intracranial capillary hemangioma.
We investigated the challenging diagnostic case of a ventricular cystic glioneuronal tumor with papillary features, by RNA sequencing using the Illumina TruSight RNA Fusion panel. We did not retrieve ...the SLC44A1‐PRKCA fusion gene specific for papillary glioneuronal tumor, but an EWSR1‐PATZ1 fusion transcript. RT‐PCR followed by Sanger sequencing confirmed the EWSR1‐PATZ1 fusion. It matched with canonic EWSR1 fusion oncogene, juxtaposing the entire N‐terminal transcriptional activation domain of EWSR1 gene and the C‐terminal DNA binding domain of a transcription factor gene, PATZ1. PATZ1 protein belongs to the BTB‐ZF (broad‐complex, tramtrack and bric‐à‐brac ‐zinc finger) family. It directly regulates Pou5f1 and Nanog and is essential to maintaining stemness by inhibiting neural differentiation. EWSR1‐PATZ1 fusion is a rare event in tumors: it was only reported in six round cell sarcomas and in three gliomas of three exclusively molecular studies. The first reported glioma was a BRAFV600E negative ganglioglioma, the second a BRAFV600E negative glioneuronal tumor, not otherwise specified and the third, very recently reported, a high grade glioma, not otherwise specified. In our study, forty BRAFV600E negative gangliogliomas were screened by FISH using EWSR1 break‐apart probes. We performed methylation profiling for the index case and for seven out of the ten FISH positive cases. The index case clustered apart from other pediatric low grade glioneuronal entities, and specifically from the well‐defined ganglioglioma methylation group. An additional pediatric intraventricular ganglioglioma clustered slightly more closely with ganglioglioma, but showed differences from the main ganglioglioma group and similarities with the index case. Both cases harbored copy number variations at the PATZ1 locus. EWSR1‐PATZ1 gene fusion might define a new type of glioneuronal tumors, distinct from gangliogliomas.
Background Our objective was to analyze the relevance, potential prognostic factors, and complications of endoscopic third ventriculostomy (ETV) in patients with shunt failures. Methods Among 721 ...ETVs performed between 1999 and 2013, we studied 53 patients with shunts (31 men, 21 less than 18 years of age) who had an ETV performed for shunt failures as the result of various causes. We included all initial causes of hydrocephalus except adult chronic (i.e., “normal pressure”) and pediatric communicant hydrocephalus. The mean duration between initial shunting for hydrocephalus and the ETV procedure was more than 11 years (137 months; range, 1 month to 34 years). Successful ETV procedure was defined as clinical improvement and shunt independence extending until the last follow-up visit. Results The success rate of the ETV procedure was 70% (37 of the 53 cases) with a mean follow-up of 51 months (from 3 to 157 months) and was not related to the age of the patient ( P = 0.922), to the cause of hydrocephalus ( P = 0.622), or to the number of shunt failures ( P = 0.459). We also found no statistical difference ( P = 0.343) between patients whose shunt had been in place for less than 5 years and those shunted more than 5 years. The presence of an infected shunt was not predictive of ETV failure ( P = 0.395). No significant intraoperative or postoperative complications were noted. Conclusion This study confirms that ETV should be considered as the first therapeutic option before shunt revision in cases of initial obstructive hydrocephalus.
The objective of this study was to analyze survival and prognostic factors for children, adolescents, and young adults treated with postoperative radiation therapy (RT) for intracranial ependymoma.
...Between 2000 and 2013, 202 patients aged ≤25 years were treated in the 13 main French pediatric RT reference centers. Their medical records were reviewed for information, treatments received, and survival rates. All children had received postoperative RT- conformal, intensity modulated, or proton beam. In 2009, the prescribed standard dose in France rose from 54 Gy to 59.4 Gy.
Median follow-up was 53.8 months (95% confidence interval CI 47-63.5). Median age at RT was 5 years (range 1-22), and 32% of the children treated were aged <3 years. Regarding treatment, 85.6% of patients underwent gross total resection, 62% of patients received conformal RT (vs 29% for intensity modulated RT and 8% for proton beam RT), 62.4% of patients received a dose >54 Gy, and 71% received chemotherapy. Of the 84 relapses, 75% were local. The cumulative incidence of local relapse was 24.4% (95% CI 18.2-31.2) at 3 years and 31.3% (95% CI 24-38.9) at 5 years. The 5-year disease-free survival (DFS) and overall survival rates were 50.4% (95% CI 42.2-58) and 71.4% (95% CI 63.1-78.2). Tumor grade was the only prognostic factor for local relapse and DFS. Tumor grade, age, and extent of resection were independent prognostic factors for overall survival.
We confirmed several clinical and tumoral prognostic factors in a large French multicenter study. DFS for intracranial ependymoma remains low, and new biological and imaging markers are needed to distinguish among different subtypes, adapt treatments, and improve survival.
Severe traumatic brain injury (TBI) is the most common cause of disability in children. Refractory increased intracranial pressure can be a therapeutic challenge. Decompressive craniectomy can be ...proposed when medical management is insufficient, but its place is not clearly defined in guidelines. The aim of this study was to identify prognostic factors in children with TBI.
We performed a retrospective, multicenter study to analyze long-term outcomes of 150 children with severe TBI treated by decompressive craniectomy and to identify prognostic factors.
A satisfactory neurologic evolution (represented by a King's Outcome Scale for Childhood Head Injury score >3) was observed in 62% of children with a mean follow-up of 3.5 years. Mortality rate was 17%. Prognostic factors associated with outcome were age, initial Glasgow Coma Scale score, presence of mydriasis, neuromonitoring values (maximal intracranial pressure >30 mm Hg), and radiologic findings (Rotterdam score ≥4).
This study in a large population confirms that children with severe TBI treated by decompressive craniectomy can achieve a good neurologic outcome. Further studies are needed to clarify the use of this surgery in the management of children with severe TBI.
Abstract Decompressive craniectomy (DC) is used for the management of refractory raised intracranial pressure, but the impact of DC on surgical outcome is still controversial. We report a 21-year-old ...man admitted to our hospital after a road traffic accident. The brain CT scan revealed a left hemispheric acute subdural hematoma. After DC, he developed a brainstem hemorrhage. Recovery was, however, good.
Abstract Background Prevalence of cerebral cavernomas in the general population is close to 0.5%. In contrast, SCCs are rare. The aim of this study was to determine the outcome of SCC in a large ...sample of patients. Methods Clinical and neuroradiologic findings were retrospectively collected in a multicentric study. Diagnosis was based on pathologic criteria or MR findings. Results Fifty-three patients were included (26 males, 27 females). Mean age at onset of symptoms was 40.2 years (11-80 years). Initial symptoms were progressive (32) or acute myelopathy (20). One case was asymptomatic. Triggering factors were found in 14 of the patients (26%). Clinical symptoms were related to spinal cord compression (27) and hemorrhage (22). Spinal cord cavernoma was thoracic in 41 cases and cervical in 12. Mean size of the lesions was 16.3 mm (3-54 mm). In the 40 surgical patients, long-term follow-up was available in 37 cases for a mean time of 7.3 years (0.4-50 years). During the follow-up period, 20 patients improved, 6 remained on their preoperative baseline, and 11 got worse. Surgical improvement was more often found in posterior rather than anterior location. Using McCormick classification, 22 patients were autonomous (grades 1-2), 12 handicapped (grade 3), and 3 bedridden (grade 4) at the end of the follow-up. Conclusions This study has defined clinical and MR patterns of spinal cavernomas. Surgery lastingly improved more than half of the patients.
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