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Bertrand Baussard, Federico Di Rocco, Matthew R. Garnett, Nathalie Boddaert, Arielle Lellouch-Tubiana, Jacques Grill, Stephanie Puget, Thomas Roujeau, Michel Zerah and Christian Sainte-Rose

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 excision of the tumor, and there has been no tumor progression in three children, two of whom have had no evidence of enhancement of the postoperative residual tumor. The tumor has progressed in six children, requiring further surgery in three, chemotherapy in four, and radiotherapy and second-line chemotherapy in one child to control the tumor.

Conclusions

The imaging of gangliogliomas in the posterior fossa showed patchy enhancement. The patients in whom it was possible to achieve a radical resection, aimed at removing at least the enhancing portion of the tumor, have not required further treatment. A second excision, for progressive tumors, is an effective adjuvant therapy.

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Stéphanie Puget, Charles Kondageski, Alison Wray, Nathalie Boddaert, Thomas Roujeau, Federico Di Rocco, Michel Zerah and Christian Sainte-Rose

✓ The authors describe the case of a 12-year-old girl with Marfan syndrome, sacral dural ectasia, and tonsillar herniation, who presented with headache. Initially, it was hypothesized that the headaches were secondary to the tonsillar herniation, and the patient consequently underwent surgical decompression of the foramen magnum. Postoperatively, the patient's condition did not improve, and additional magnetic resonance (MR) imaging demonstrated evidence of a cerebrospinal fluid (CSF) leak at the level of the dural ectasia. It was surmised that the girl's symptoms were due to spontaneous intracranial hypotension (SIH) and that the tonsillar herniation was caused by the leakage. The patient responded well to application of a blood patch at the level of the demonstrated leak, and her headache resolved. This appears to be the first reported case of a patient with Marfan syndrome presenting with a symptomatic spontaneous CSF leak complicated by tonsillar herniation. In this rare association of SIH and connective tissue disorders, recognition of the clinical signs and typical MR imaging features of SIH may lead to more appropriate and less invasive treatment, potentially avoiding surgery.

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Christian Sainte-Rose, Ricardo Oliveira, Stéphanie Puget, Liana Beni-Adani, Nathalie Boddaert, John Thorne, Alison Wray, Michel Zerah and Marie Bourgeois

Object

The authors’ aim in this study was to review their experience in the use of indirect revascularization alone in a series of 14 children with moyamoya disease, in which numerous bur holes and arachnoid openings were made over each affected hemisphere.

Methods

Revascularization through multiple bur holes and arachnoid openings was performed in 14 children (mean age at diagnosis 6.5 years [range 3–15 years]) who suffered from progressive moyamoya disease. The authors performed surgery in a total of 24 hemispheres during 18 procedures. Ten children underwent bilateral multiple bur hole procedures, three underwent a unilateral procedure in the more severely affected hemisphere, and one child had previously undergone an encephaloduroarteriomyosynangiosis on the contralateral side. Ten to 24 bur holes were made in the frontotemporoparietooccipital area of each hemisphere, depending on the site and extent of the disease. Early postoperative perfusion magnetic resonance imaging studies, performed in the five most recent cases, showed restoration of cortical perfusion as early as 3 months, which was confirmed on subsequent angiography studies (performed between 8 and 12 months postoperatively) that showed excellent revascularization of the ischemic brain by external carotid artery collateral vessels. None of the children sustained further ischemic attacks postoperatively. Motor improvement was noted in those who had presented with paresis. A single seizure episode occurred in two patients at 2 weeks and 5 months after surgery; both children had presented with epilepsy. There were no postoperative deaths, and only one complication (an infected lumbar shunt in the patient who required cerebrospinal fluid [CSF] drainage). Five of the 18 procedures were complicated by subcutaneous CSF collections, which resolved with tapping and compressive head dressings; a transient lumbar drain was necessary in one case.

Conclusions

The results obtained in this series suggest that in children with moyamoya disease this simple technique is both effective and safe. Furthermore, it is effective as a sole treatment without supplementary revascularization procedures.

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Thomas Blauwblomme, Olivier Naggara, Francis Brunelle, David Grévent, Stéphanie Puget, Federico Di Rocco, Kevin Beccaria, Giovanna Paternoster, Marie Bourgeois, Manoelle Kossorotoff, Michel Zerah, Christian Sainte-Rose and Nathalie Boddaert

OBJECT

Arterial spin labeling (ASL)-MRI is becoming a routinely used sequence for ischemic strokes, as it quantifies cerebral blood flow (CBF) without the need for contrast injection. As brain arteriovenous malformations (AVMs) are highflow vascular abnormalities, increased CBF can be identified inside the nidus or draining veins. The authors aimed to analyze the relevance of ASL-MRI in the diagnosis and follow-up of children with brain AVM.

METHODS

The authors performed a retrospective analysis of 21 patients who had undergone digital subtraction angiography (DSA) and pseudo-continuous ASL-MRI for the diagnosis or follow-up of brain AVM after radiosurgery or embolization. They compared the AVM nidus location between ASL-MRI and 3D contrast-enhanced T1 MRI, as well as the CBF values obtained in the nidus (CBFnidus) and the normal cortex (CBFcortex) before and after treatment.

RESULTS

The ASL-MRI correctly demonstrated the nidus location in all cases. Nidal perfusion (mean CBFnidus 137.7 ml/100 mg/min) was significantly higher than perfusion in the contralateral normal cortex (mean CBFcortex 58.6 ml/100 mg/min; p < 0.0001, Mann-Whitney test). Among 3 patients followed up after embolization, a reduction in both AVM size and CBF values was noted. Among 5 patients followed up after radiosurgery, a reduction in the nidus size was observed, whereas CBFnidus remained higher than CBFcortex.

CONCLUSIONS

In this study, ASL-MRI revealed nidus location and patency after treatment thanks to its ability to demonstrate focal increased CBF values. Absolute quantification of CBF values could be relevant in the follow-up of pediatric brain AVM after partial treatment, although this must be confirmed in larger prospective trials.

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Thomas Roujeau, Guilherme Machado, Matthew R. Garnett, Catherine Miquel, Stephanie Puget, Birgit Geoerger, Jacques Grill, Nathalie Boddaert, Federico Di Rocco, Michel Zerah and Christian Sainte-Rose

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 two patients affected the initial treatment after the biopsy.

Conclusions

The findings of this study imply that stereotactic biopsy sampling of a diffuse pontine tumor is a safe procedure, is associated with minimal morbidity, and has a high diagnostic yield. A nonmalignant tumor was identified in two of the 24 patients in whom the imaging findings were characteristic of a malignant infiltrative astrocytoma. With the advent of new treatment protocols, stereotactic biopsy sampling, which would allow specific tumor characterization of diffuse pontine lesions, may become standard.

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Stephanie Puget, Darach W. Crimmins, Matthew R. Garnett, Jacques Grill, Ricardo Oliveira, Nathalie Boddaert, Alison Wray, Arielle Lelouch-Tubiana, Thomas Roujeau, Federico Di Rocco, Michel Zerah and Christian Sainte-Rose

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), and tumor excision greater than 90% at surgery (p = 0.04). The perioperative morbidity and mortality rates were 37 and 4%, respectively. Fifty-four percent of the patients in this group had a long-term and independent survival. The thalamopeduncular tumors were mostly pilocytic astrocytomas, which had a good prognosis following surgery. The bilateral thalamic tumors in this series were mainly low-grade astrocytic lesions, and more than half of the children attained long-term survival (mean follow-up duration 4.5 years).

Conclusions

The majority of tumors arising in the thalamus are astrocytic, of which less than half are high-grade lesions. Histological evaluations should be performed in all patients in whom resection is being considered for discrete lesions. Long-term survival is possible in patients with these tumors.

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Stéphanie Puget, Matthew R. Garnett, Delphine Leclercq, Graziella Pinto-Primard, Dinane Samara-Boustani, Christian Sainte-Rose, David Genevieve, Michel Polak, Pascale De Lonlay, Francis Brunelle, Alim Louis Benabid and Nathalie Boddaert

The association between hypothalamic dysfunction and obesity is well documented in both clinical and experimental models. The authors describe 2 children who developed obesity that could not be explained by endocrinological, genetic, or eating disorders. In both cases, cranial MR imaging revealed the typical appearance of a lipoma in the paramedian hypothalamus. In the absence of other etiologies, the authors hypothesized that in these 2 children obesity was caused by their hypothalamic lipomas. To the authors' knowledge, these are the first cases of hypothalamic lipomas likely to be causing obesity that have been described in children. These cases highlight the importance of performing cranial MR imaging in children with otherwise unexplained obesity.

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Forniceal glioma in children

Clinical article

Thomas Blauwblomme, Pascale Varlet, John R. Goodden, Marie Laure Cuny, Helene Piana, Thomas Roujeau, Federico DiRocco, Jacques Grill, Virginie Kieffer, Nathalie Boddaert, Christian Sainte-Rose and Stéphanie Puget

Object

Five to ten percent of pediatric brain tumors are located in the ventricles. Among them, forniceal lesions are rare and their management has not often been described. The aim of this study was to review the clinical, radiological, and histopathological features as well as the feasibility of surgical excision and the outcomes in these patients.

Methods

From a retrospective analysis of 250 cases of supratentorial pediatric glioma, the records of 8 children presenting with forniceal lesions were selected and reviewed.

Results

The median age of patients in the cohort was 13.5 years. Presenting features included intracranial hypertension (7 cases), hypothalamic dysfunction (2), and memory dysfunction (3). Complete resection was possible in only 1 case, where the lesion was mainly exophytic; the remaining patients had either a partial resection or biopsy. On histological review, the tumors were confirmed as pilocytic astrocytoma (4 lesions), WHO Grade II astrocytoma (3), and ganglioglioma (1). Postoperatively, working and retrograde memory was normal for all patients, but the authors found a mild alteration in verbal episodic memory in 5 patients. Despite fatigability for 5 patients, academic achievement was normal for all but 2, both of whom had preoperative school difficulties. Additional treatment was required for 5 patients for tumor progression, with a median interval of 19 months from surgery. At a median follow-up duration of 4.9 years, all patients had stable disease.

Conclusions

In this series, forniceal gliomas were found to be low-grade gliomas. They are surgically challenging, and only exophytic lesions may be cured surgically. Due to the high rate of progression of residual disease, adjuvant therapy is recommended for infiltrative tumors, and it yielded excellent results.

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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.