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Franck-Emmanuel Roux, Sergio Boetto, Oumar Sacko, François Chollet, and Michel Trémoulet

Object. In an attempt to gain a better understanding of the cerebral functions represented in the angular gyrus and to spare them during surgery, the authors studied patients with brain tumors located close to the angular gyrus and mapped cortical sites by using electrostimulation.

Methods. Before undergoing tumor removal, six right-handed patients (five with left and one with right hemisphere tumors) were studied using cortical mapping with the aid of calculating, writing, finger-recognition, and color-naming tasks in addition to standard reading and object-naming tasks (for a total of 36 brain mapping studies). Strict conditions of functional site validation were applied to include only those cortical sites that produced repetitive interferences in the function tested. Preoperatively, four of the patients exhibited discrete symptoms related to Gerstmann syndrome while performing very specific tasks, whereas the other two patients presented with no symptoms of the syndrome. No patient had significant language or apraxic deficits.

Distinct or shared cortical sites producing interferences in calculating, finger recognition, and writing were repeatedly found in the angular gyrus. Object- or color-naming sites and reading-interference sites were also found in or close to the angular gyrus; although frequently demonstrated, these latter results were variable and unpredictable in the group of patients studied. Finger agnosia and acalculia sites were also found elsewhere, such as in the supramarginal gyrus or close to the intraparietal sulcus. Mechanisms involved in acalculia, agraphia, or finger agnosia (either complete interferences or hesitations) during stimulation were various, from an aphasia-like form (for instance, the patient did not understand the numbers or words given for calculating or writing tasks) to an apparently pure interference in the function tested (patients understood the numbers, but were unable to perform a simple addition).

Conclusions. Symptoms of Gerstmann syndrome can be found during direct brain mapping in the angular gyrus region. In this series of patients, sites producing interferences in writing, calculating, and finger recognition were demonstrated in the angular gyrus, which may or may not have been associated with object-naming, color-naming, or reading sites.

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Oumar Sacko, Sergio Boetto, Valérie Lauwers-Cances, Martin Dupuy, and Franck-Emmanuel Roux

Object

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.

Methods

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.

Results

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.

Conclusions

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.

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Edouard Mazerand, Sandro Benichi, Maxime Taverne, Giovanna Paternoster, Alice Rolland, Pierre Antherieu, Julien Todeschi, Lawrence Kamdem Noumoye, Vianney Gilard, Maxime Bretonnier, Luc Le Fournier, Vincent Jecko, Edouard Gimbert, François Proust, Sergio Boetto, Thomas Roujeau, Syril James, Roman H. Khonsari, Laurent Riffaud, Matthieu Delion, Michel Zerah, and Didier Scavarda

OBJECTIVE

Chiari malformation type I (CM-I) is frequent in children and remains a surgical challenge. Several techniques have been described for posterior fossa decompression. No decision algorithm has been validated, and strategies are highly variable between institutions. The goal of this study was to define therapeutic guidelines that take into consideration patient specificities.

METHODS

The authors retrospectively collected data from patients who were < 18 years of age, were diagnosed with CM-I, and were treated surgically between 2008 and 2018 in 8 French pediatric neurosurgical centers. Data on clinical features, morphological parameters, and surgical techniques were collected. Clinical outcomes at 3 and 12 months after surgery were assessed by the Chicago Chiari Outcome Scale. The authors used a hierarchical clustering method to define clusters of patients by considering their anatomical similarities, and then compared outcomes between surgical strategies in each of these clusters.

RESULTS

Data from 255 patients were collected. The mean age at surgery was 9.6 ± 5.0 years, syringomyelia was reported in 60.2% of patients, the dura mater was opened in 65.0% of patients, and 17.3% of patients underwent a redo surgery for additional treatment. The mean Chicago Chiari Outcome Scale score was 14.4 ± 1.5 at 3 months (n = 211) and 14.6 ± 1.9 at 12 months (n = 157). The hierarchical clustering method identified three subgroups with potentially distinct mechanisms underlying tonsillar herniation: bony compression, basilar invagination, and foramen magnum obstruction. Each cluster matched with specific outcomes.

CONCLUSIONS

This French multicenter retrospective cohort study enabled the identification of three subgroups among pediatric patients who underwent surgery for CM-I, each of which was associated with specific outcomes. This morphological classification of patients might help in understanding the underlying mechanisms and providing personalized treatment.