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Philippe Pencalet, Christian Sainte-Rose, Arielle Lellouch-Tubiana, Chantal Kalifa, Francis Brunelle, Spiros Sgouros, Philippe Meyer, Giuseppe Cinalli, Michel Zerah, Alain Pierre-Kahn, and Dominique Renier

Object

Choroid plexus tumors are rare intraventricular tumors (1% of all intracranial tumors) that occur mainly in children. The physiopathological characteristics of associated hydrocephalus, surgical management, and oncological issues related to these tumors remain a matter of debate. To understand more about these tumors, the authors have reviewed their experience with the management of 38 children with choroid plexus tumors.

Methods

There were 25 cases of papilloma and 13 of carcinoma. The mean age of the patients at presentation was 22.5 months and one-half of the patients were younger than 2 years of age. Hydrocephalus was present in 33 patients and poorly correlated with the size, site, and pathological characteristics of the tumor. In nine children, a ventriculoperitoneal shunt was required after tumor excision, calling into question the notion that cerebrospinal fluid oversecretion is the only cause of hydrocephalus.

Complete excision was achieved in 96% of the cases of papilloma and 61.5% of the cases of carcinoma. These surgical procedures were complicated by the risks of perioperative hemorrhage, which proved to be fatal in two cases, and postoperative brain collapse, which led to subdural fluid collections requiring subdural shunt placement in six patients. Preoperative embolization was partially successful in four cases and significantly assisted surgery. Preoperative controlled drainage of excessively dilated ventricles and intraoperative gluing of the cortical incision have been used to address the problem of postoperative brain collapse.

Patients with carcinomas were treated postoperatively by chemotherapy alone (seven cases), radiotherapy (one case), or chemotherapy plus radiotherapy (one case). The overall 5-year survival rate was 100% for patients with papillomas and 40% for those with carcinomas.

Conclusions

Total surgical excision is curative in cases of papillomas. For carcinomas, the most effective treatment remains total surgical excision; however, adjuvant treatment in the form of chemotherapy in patients younger than 3 years, supplemented by radiation therapy in older children, can moderately reduce the risk of recurrence.

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Philippe Pencalet, Christian Sainte-Rose, Arielle Lellouch-Tubiana, Chantal Kalifa, Francis Brunelle, Spiros Sgouros, Philippe Meyer, Giuseppe Cinalli, Michel Zerah, Alain Pierre-Kahn, and Dominique Renier

Object. Choroid plexus tumors are rare intraventricular tumors (1% of all intracranial tumors) that occur mainly in children. The pathophysiological characteristics of associated hydrocephalus, surgical management, and oncological issues related to these tumors remain a matter of debate. To understand more about these tumors, the authors have reviewed their experience with the management of 38 children with choroid plexus tumors.

Methods. There were 25 cases of papilloma and 13 of carcinoma. The mean age of the patients at presentation was 22.5 months, and one-half of the patients were younger than 2 years of age. Hydrocephalus was present in 33 patients and poorly correlated with the size, site, and pathological characteristics of the tumor. In nine children, a ventriculoperitoneal shunt was required after tumor excision, calling into question the notion that cerebrospinal fluid oversecretion is the only cause of hydrocephalus.

Complete excision was achieved in 96% of the cases of papilloma and 61.5% of the cases of carcinoma. These surgical procedures were complicated by the risks of intraoperative hemorrhage, which proved to be fatal in two cases, and postoperative brain collapse, which led to subdural fluid collections requiring subdural shunt placement in six patients. Preoperative embolization was partially successful in four cases and significantly assisted surgery. Preoperative controlled drainage of excessively dilated ventricles and intraoperative gluing of the cortical incision have been used to address the problem of postoperative brain collapse.

Patients with carcinomas were treated postoperatively by chemotherapy alone (seven cases), radiotherapy (one case), or chemotherapy plus radiotherapy (one case). The overall 5-year survival rate was 100% for patients with papillomas and 40% for those with carcinomas.

Conclusions. Total surgical excision is curative in cases of papillomas. For carcinomas, the most effective treatment remains total surgical excision; however, adjuvant treatment in the form of chemotherapy in patients younger than age 3 years, supplemented by radiation therapy in older children, can moderately reduce the risk of recurrence.

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José Roberto Tude Melo, Federico Di Rocco, Marie Bourgeois, Stephanie Puget, Thomas Blauwblomme, Christian Sainte-Rose, Philippe G. Meyer, and Michel Zerah

Object

Subdural hematoma (SDH) is the most common finding on cranial CT in pediatric victims of abusive head trauma (AHT). The hematomas are commonly bilateral and sometimes associated with interhemispheric hyperdensity and/or convexity hemorrhages. There is no consensus regarding the best surgical treatment in such cases nor are there standardized surgical protocols. The authors report their experience and discuss the routine surgical options in the management of traumatic SDH at a Level 1 Pediatric Trauma Center.

Methods

In this paper, the authors describe a cross-sectional study with consecutive revision of data described in the medical records of Hôpital Universitaire Necker–Enfants Malades between January 2008 and January 2013. During this period, all children younger than 2 years of age who were admitted with a traumatic SDH identified on CT scans were included in this study.

Results

One hundred eighty-four children who had SDH and were younger than 2 years of age were included. Their median age was 5.8 months (range 5 days–23 months), and 70% of the children were male. On admission CT scans, the SDH was bilateral in 52% of cases and homogeneously hypodense in 77%. Neurosurgical treatment was undertaken in 111 children (60%) with an admission Glasgow Coma Scale score of 12 or less, bulging fontanels, or other signs suggestive of intracranial hypertension. The first surgical option was craniotomy in 1.8% (2) of these 111 cases, decompressive craniectomy in 1.8% (2), transcutaneous subdural puncture in 15% (17), external subdural drainage in 16% (18), subdural-subgaleal shunt placement in 17% (19), and subdural-peritoneal shunt placement in 48% (53). In 82% of the children initially treated with transcutaneous subdural puncture and in 50% of those treated with external subdural drainage, increase or persistence of the SDH, CSF or skin infection, or shunt system malfunction was observed and further surgical intervention was required. There was a 26% rate of complications in patients initially treated with a subdural-peritoneal shunt. Although 52% of the patients had bilateral SDH, bilateral drainage was only required in 9.4%.

Conclusions

The choice of treatment should be determined by the clinical and radiological characteristics of the individual case. Although effective on an emergency basis, subdural puncture and external subdural drainage are frequently insufficient to obtain complete resolution of SDH, and temporary placement of a subdural-peritoneal shunt is needed in most cases.

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Sarah Stricker, Grégoire Boulouis, Sandro Benichi, Florent Gariel, Lorenzo Garzelli, Kevin Beccaria, Anais Chivet, Timothee de Saint Denis, Syril James, Giovanna Paternoster, Michel Zerah, Marie Bourgeois, Nathalie Boddaert, Francis Brunelle, Philippe Meyer, Stephanie Puget, Olivier Naggara, and Thomas Blauwblomme

OBJECTIVE

Hydrocephalus is a strong determinant of poor neurological outcome after intracerebral hemorrhage (ICH). In children, ruptured brain arteriovenous malformations (bAVMs) are the dominant cause of ICH. In a large prospective cohort of pediatric patients with ruptured bAVMs, the authors analyzed the rates and predictive factors of hydrocephalus requiring acute external ventricular drainage (EVD) or ventriculoperitoneal shunt (VPS).

METHODS

The authors performed a single-center retrospective analysis of the data from a prospectively maintained database of children admitted for a ruptured bAVM since 2002. Admission clinical and imaging predictors of EVD and VPS placement were analyzed using univariate and multivariate statistical models.

RESULTS

Among 114 patients (mean age 9.8 years) with 125 distinct ICHs due to ruptured bAVM, EVD and VPS were placed for 55/125 (44%) hemorrhagic events and 5/114 patients (4.4%), respectively. A multivariate nominal logistic regression model identified low initial Glasgow Coma Scale (iGCS) score, hydrocephalus on initial CT scan, the presence of intraventicular hemorrhage (IVH), and higher modified Graeb Scale (mGS) score as strongly associated with subsequent need for EVD (all p < 0.001). All children who needed a VPS had initial hydrocephalus requiring EVD and tended to have higher mGS scores.

CONCLUSIONS

In a large cohort of pediatric patients with ruptured bAVM, almost half of the patients required EVD and 4.4% required permanent VPS. Use of a low iGCS score and a semiquantitative mGS score as indicators of the IVH burden may be helpful for decision making in the emergency setting and thus improve treatment.

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Julius Dengler, Daniel Rüfenacht, Bernhard Meyer, Veit Rohde, Matthias Endres, Pavlina Lenga, Konstantin Uttinger, Viktoria Rücker, Maria Wostrack, Adisa Kursumovic, Bujung Hong, Dorothee Mielke, Nils Ole Schmidt, Jan-Karl Burkhardt, Philippe Bijlenga, Edoardo Boccardi, Christophe Cognard, Peter U. Heuschmann, Peter Vajkoczy, and On behalf of the Giant Intracranial Aneurysm Study Group

OBJECTIVE

Clinical evidence on giant intracranial aneurysms (GIAs), intracranial aneurysms with a diameter of at least 25 mm, is limited. The authors aimed to investigate the natural history, case fatality, and treatment outcomes of ruptured and unruptured GIAs.

METHODS

In this international observational registry study, patients with a ruptured or unruptured GIA received conservative management (CM), surgical management (SM), or endovascular management (EM). The authors investigated rupture rates and case fatality.

RESULTS

The retrospective cohort comprised 219 patients with GIAs (21.9% ruptured GIAs and 78.1% unruptured GIAs) whose index hospitalization occurred between January 2006 and November 2016. The index hospitalization in the prospective cohort (362 patients with GIAs [17.1% ruptured and 82.9% unruptured]) occurred between December 2008 and February 2017. In the retrospective cohort, the risk ratio for death at a mean follow-up of 4.8 years (SD 2.2 years) after CM, compared with EM and SM, was 1.63 (95% CI 1.23–2.16) in ruptured GIAs and 3.96 (95% CI 2.57–6.11) in unruptured GIAs. In the prospective cohort, the 1-year case fatality in ruptured GIAs/unruptured GIAs was 100%/22.0% during CM, 36.0%/3.0% after SM, and 39.0%/12.0% after EM. Corresponding 1-year rupture rates in unruptured GIAs were 25.0% during CM, 1.2% after SM, and 2.5% after EM. In unruptured GIAs, the HR for death within the 1st year in patients with posterior circulation GIAs was 6.7 (95% CI 1.5–30.4, p < 0.01), with patients with a GIA at the supraclinoid internal carotid artery as reference. Different sizes of unruptured GIAs were not associated with 1-year case fatality.

CONCLUSIONS

Rupture rates for unruptured GIAs were high, and the natural history and treatment outcomes for ruptured GIAs were poor. Patients undergoing SM or EM showed lower case fatality and rupture rates than those undergoing CM. This difference in outcome may in part be influenced by patients in the CM group having been found poor candidates for SM or EM.

Clinical trial registration no.: NCT02066493 (clinicaltrials.gov)

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Sarah Stricker, Grégoire Boulouis, Sandro Benichi, Marie Bourgeois, Florent Gariel, Lorenzo Garzelli, Jean-François Hak, Quentin Alias, Basile Kerleroux, Kevin Beccaria, Anaïs Chivet, Timothée de Saint Denis, Syril James, Giovanna Paternoster, Michel Zerah, Manoelle Kossorotoff, Nathalie Boddaert, Francis Brunelle, Philippe Meyer, Stéphanie Puget, Olivier Naggara, and Thomas Blauwblomme

OBJECTIVE

Rupture of brain arteriovenous malformation (AVM) is the main etiology of intracerebral hemorrhage (ICH) in children. Ensuing intracranial hypertension is among the modifiable prognosis factors and sometimes requires emergency hemorrhage evacuation (HE). The authors aimed to analyze variables associated with HE in children with ruptured AVM.

METHODS

This study was a single-center retrospective analysis of children treated for ruptured AVM. The authors evaluated the occurrence of HE, its association with other acute surgical procedures (e.g., nidal excision, decompressive hemicraniectomy), and clinical outcome. Variables associated with each intervention were analyzed using univariable and multivariable models. Clinical outcome was assessed at 18 months using the ordinal King’s Outcome Scale for Childhood Head Injury.

RESULTS

A total of 104 patients were treated for 112 episodes of ruptured AVM between 2002 and 2018. In the 51 children (45.5% of cases) who underwent HE, 37 procedures were performed early (i.e., within 24 hours after initial cerebral imaging) and 14 late. Determinants of HE were a lower initial Glasgow Coma Scale score (adjusted odds ratio [aOR] 0.83, 95% CI 0.71–0.97 per point increase); higher ICH/brain volume ratio (aOR 18.6, 95% CI 13–26.5 per percent increase); superficial AVM location; and the presence of a brain herniation (aOR 3.7, 95% CI 1.3–10.4). Concurrent nidal surgery was acutely performed in 69% of Spetzler-Martin grade I–II ruptured AVMs and in 25% of Spetzler-Martin grade III lesions. Factors associated with nidal surgery were superficial AVMs, late HE, and absent alteration of consciousness at presentation. Only 8 cases required additional surgery due to intracranial hypertension. At 18 months, overall mortality was less than 4%, 58% of patients had a favorable outcome regardless of surgical intervention, and 87% were functioning independently.

CONCLUSIONS

HE is a lifesaving procedure performed in approximately half of the children who suffer AVM rupture. The good overall outcome justifies intensive initial management.

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Oral Presentations

2010 AANS Annual Meeting Philadelphia, Pennsylvania May 1–5, 2010