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Aymeric Amelot, Kevin Beccaria, Thomas Blauwblomme, Marie Bourgeois, Giovanna Paternoster, Marie-Laure Cuny, Michel Zerah, Christian Sainte-Rose and Stephanie Puget


Arachnoid cysts (ACs) are most frequently located in the middle cranial fossa. Some patients are asymptomatic whereas others exhibit signs of increased intracranial pressure, seizures, or cognitive and behavioral symptoms. When ACs do require treatment, the optimal surgical technique remains controversial. This study was conducted to assess the most effective surgical treatment for these cysts.


The authors retrospectively reviewed 240 temporal intracranial ACs managed over a 25-year period in their pediatric neurosurgical unit. Pre- and posttreatment results were clinically and radiologically assessed.


A majority of male patients (74.6%) with an overall median age of 6.9 years were included. The mean cyst size was 107 cm3; the Galassi classification showed 99 (41.3%) type I, 77 (32.1%) type II, and 64 (26.7%) type III cysts. Forty-four ACs (18.3%) were diagnosed after rupture. Surgical management was performed by microsurgery (28.3%), endoscopic cyst fenestration (14.6%), cystoperitoneal shunting (CPS; 16.2%), or subdural shunting (10%). Furthermore, 74 children (30.8%) did not undergo operations. After a mean follow-up of 4.1 years, the mean percentage decrease in cyst volume and the overall rate of clinical improvement did not significantly differ. The endoscopy group had earlier complications and a shorter event-free survival (EFS) time (EFS at 3 years = 67.7%, vs 71.5% and 90.5% for CPS and microsurgery, respectively; p < 0.007) and presented with more subdural hematomas compared to the microsurgery group (p < 0.005). The microsurgery group also showed a tendency for longer cystocisternostomy permeability than the endoscopy group.


Concerning the management of unruptured symptomatic temporal ACs, microsurgery appears to be the most effective treatment, with longer EFS and fewer complications compared to shunting or endoscopy.

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Senta Kurschel, Stéphanie Puget, Marie Bourgeois, Michel Zerah, Petra Ofner and Dominique Renier


In this study the authors' goal was to identify the complication rate of subduroperitoneal (SDP) shunts for the treatment of subdural hematomas (SDHs) in infants and to determine the influences on and predictive factors for these complications.


The authors present a case series spanning the years 1994 to 2003 and include a statistical analysis of 161 children 2 years of age or younger with SDH who were treated using a unilateral valveless SDP shunt. The patient history, characteristics, and treatment methods including prior therapies, neuroimaging findings, and clinical outcomes were measures of evaluation.

Thirty-six children (22.4%) suffered complications related to SDP shunts: obstruction in 27 (16.8%), infections in eight (5%), disconnection in four (2.5%), migration in three (1.9%), wound complications (leakage and skin ulceration) in two (1.2%), and symptomatic subdural rebleeding in one (0.6%) necessitating bur hole evacuation. Seventeen children (10.6%) underwent placement of a second SDP shunt because of ipsilateral or contralateral persistent fluid collections, or premature shunt removal. With the exception of 12 patients (7.4%), shunt removal was performed systematically and resulted in the following minor complications in 30 children (18.6%): an adherent proximal catheter in 16 (9.9%), transient symptoms of intracranial hypertension in six (3.7%), subcutaneous cerebrospinal fluid accumulation in four (2.5%), local infections in three (1.9%), and hydrocephalus requiring placement of a ventriculoperitoneal shunt in one (0.6%).

Status epilepticus at presentation and neuroimaging findings such as areas of hyperdensity on computed tomography (CT) scans representing fresh blood in the subdural fluid collections before shunt insertion and at follow up were predictors of shunt-related complications. Correlations were also discovered for the following CT findings: ischemic lesions before shunt treatment, cerebral atrophy and ventricular dilation during the last follow up, and residual medium to large collections before shunt removal. Children who attained a good outcome were less affected by shunt-related complications, unlike those who presented with focal deficits and/or visual impairment.


Subduroperitoneal shunt placement for the treatment of SDH in infants is—despite the complication rate—an effective and often inevitable treatment option, especially for most large and symptomatic SDHs; a certain number of complications could be reduced with careful and precise surgical techniques. Close observation for detection of risks is mandatory, and seizure control is essential to prevent further brain injury that may result in large subdural fluid collections that are difficult to treat.

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Elizabeth Lajeunie, Uli Barcik, John A. Thorne, Vincent El Ghouzzi, Marie Bourgeois and Dominique Renier

Object. Fetal valproate syndrome affects one in 10 children born to mothers who ingest sodium valproate regularly during pregnancy. It has been described as producing a combination of typical dysmorphic features and major organ system anomalies. Trigonocephaly is caused by premature fusion of the metopic suture and has not previously been described as a typical feature of the syndrome. The authors reviewed the cases of 2220 children with craniosynostosis to examine the effect of maternal sodium valproate use on the fetus.

Methods. Case files of all 2220 children were reviewed. The type and severity of each patient's craniosynostosis was assessed. Information about maternal health and medication use was obtained, and family interviews were conducted. Children underwent mental development assessment performed using standard tests both pre- and postoperatively.

Detailed maternal health information was obtained in 1676 cases. Of these, 17 mothers were found to have undergone regular treatment with sodium valproate monotherapy at the time of their pregnancies. No other antiepileptic medical regimen was found. All 17 children exhibited trigonocephaly. These patients' intelligence quotients (IQs) at the time of the most recent follow-up examination ranged from 45 to 100, with a mean of 75; IQs were significantly higher in patients who underwent surgery before reaching 6 months of age.

Conclusions. Ideally any pregnancy in a woman being treated for epilepsy should be planned, and both an obstetrician and a neurologist should be consulted. In children born with fetal valproate syndrome, it is important to be aware of the possibility of metopic suture synostosis, which we believe should be considered part of the syndrome, because early surgical intervention may improve cognitive outcome.

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Marie Bourgeois, Christian Sainte-Rose, Arielle Lellouch-Tubiana, Conor Malucci, Francis Brunelle, Wirginia Maixner, Giuseppe Cinalli, Alain Pierre-Kahn, Dominique Renier, Michel Zerah, Jean-François Hirsch, Francoise Goutières and Jean Aicardi

Object. Surgery in children with epilepsy is a new, evolving field. The important practical issues have been to define strategies for choosing the most suitable candidates and the type and optimal timing of epilepsy surgery. This study was undertaken to elucidate these points.

Methods. To identify the factors that correlated with outcome, the authors analyzed a series of 200 children (aged 1–15 years (mean 8.7 years) who underwent surgery between 1981 and 1996 at the Hôpital Necker—Enfants Malades. In 171 cases (85.5%) the epilepsy was medically refractory and was associated with focal cortical lesions. Surgery consisted of resection of the lesion without specifically attempting to identify and remove the “epileptogenic area.”

In the group of children whose seizures were medically refractory, the mean follow-up period was 5.8 years. According to Engel's classification, 71.3% of these children became seizure free (Class Ia,) whereas 82% were in Class I. A multivariate statistical analysis revealed that among all the factors studied, the success of surgery in a patient in whom there was a good clinical/electroencephalogram/imaging correlation depended on the patient's having undergone a minimally traumatic operation, a complete resection of the lesion, and a short preoperative seizure duration.

After the surgical control of epilepsy, behavior disorders were more improved (31% of all patients) than cognitive function (25%). The patient age at onset, duration and frequency of seizures, intractability of the disease to therapy, and seizure characteristics were correlated with cognitive, behavioral, and academic performance pre- and postoperatively. Multivariate statistical analysis revealed that cognitive dysfunction correlated highly with the duration of epilepsy prior to surgery, whereas behavioral disorders correlated more with seizure frequency.

Conclusions. These data must be taken into account when selecting patients for surgical treatment and when deciding the timing of surgery. Early surgical intervention allows for optimum brain development.

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Philippe Pencalet, Wirginia Maixner, Christian Sainte-Rose, Arielle Lellouch-Tubiana, Giuseppe Cinalli, Michel Zerah, Alain Pierre-Kahn, Elisabeth Hoppe-Hirsch, Marie Bourgeois and Dominique Renier

Object. Cerebellar astrocytomas are benign tumors of childhood known to be associated with excellent long-term survival in patients in whom complete surgical resection is possible. However, the roles of other factors—clinical, radiological, histological, and therapeutic—in the survival of the patient, tumor recurrence, and long-term patient outcome remain imprecise. The goal of this study was to examine these factors and their relationships.

Methods. To clarify these issues a retrospective review was conducted of 168 children who were surgically treated for a cerebellar astrocytoma at Hôpital Necker—Enfants Malades between 1955 and 1995. These patients' clinical files were examined, the histological characteristics of their tumors were reviewed, and their outcomes were assessed according to Bloom's scale and the Wechsler intelligence quotient test.

Of the 168 patients in the study, 91 were male and 77 were female with a mean age of 6.9 years and a mean follow up lasting 7.7 years. Tumors were identified as being strictly located in the cerebellum in 76.2% of the patients and as involving the brainstem (referred to as the “transitional form”) in 23.8% of the patients. Complete surgical excision was possible in 88.7% of cases. There was a total mortality rate of 4.2% and a tumor recurrence rate of 9.5%. Fifty-eight percent of the patients had no neurological sequelae at follow-up evaluation.

Pejorative factors that were discovered by multivariate analysis to be important included: a long preoperative duration of symptoms and the transitional form of tumor with respect to survival; incomplete tumor excision with respect to an increased risk of recurrence; and a long preoperative duration of symptoms, an early epoch during which surgery was performed (1955–1974), severe ventricular dilation, and the transitional form of tumor with respect to a poorer long-term patient outcome.

Conclusions. The presence of brainstem involvement (tumor in the transitional form) emerged as a significant negative prognostic factor and should be treated as a distinct nosological entity. The extent of surgical excision has a significant bearing on the risk of tumor recurrence.

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Marie Bourgeois, Christian Sainte-Rose, Giuseppe Cinalli, Wirginia Maixner, Conor Malucci, Michel Zerah, Alain Pierre-Kahn, Dominique Renier, Elisabeth Hoppe-Hirsch and Jean Aicardi

Object. The incidence of epilepsy among children with hydrocephalus and its relation to shunts and their complications, raised intracranial pressure (ICP), and developmental outcome are explored in a retrospective study.

Methods. The authors studied a series of 802 children with hydrocephalus due to varying causes, who were treated by ventriculoperitoneal shunt placement between 1980 and 1990, with a mean follow-up period of 8 years. Patients who had tumoral hydrocephalus and those whose files lacked significant data were excluded. Data extracted from medical records, including history of the hydrocephalus and history of seizures, if any, were analyzed.

Thirty-two percent of the children had epilepsy, the onset of which frequently occurred at approximately the same time that the diagnosis of hydrocephalus was made. The majority of the affected children had severe uncontrolled epilepsy. The incidence of epilepsy was significantly affected by the original cause of the hydrocephalus. The presence of radiological abnormalities was also found to be a significant predictor of epilepsy. Similarly, shunt complications predisposed to epilepsy. Episodes of raised ICP related to hydrocephalus or in association with shunt malfunction may also predispose to epileptic seizures. Furthermore, the presence of a shunt by itself seems able to promote an epileptogenic focus. Finally, epilepsy appears to be an important predictor of poor intellectual outcome in hydrocephalic children with shunts.

Conclusions. A prospective study is needed to identify clearly and confirm avoidable factors predisposing to seizures in these children so that we can strive to reduce the incidence of these seizures and, subsequently, improve these children's quality of life.

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Thomas Blauwblomme, Federico Di Rocco, Marie Bourgeois, Kevin Beccaria, Giovanna Paternoster, Juliette Verchere-Montmayeur, Christian Sainte-Rose, Michel Zerah and Stéphanie Puget


The ideal treatment for subdural hematomas (SDHs) in infants remains debated. The aim of this study was to analyze the safety and efficiency of subduro-subgaleal drainage in SDH.


The authors conducted a single-center open-label study between August 2011 and May 2012. Data were prospectively collected in a database and retrospectively analyzed.


Eighteen patients (male/female ratio 1.25) with a median age of 5 months were surgically treated. All had preoperative symptoms of intracranial hypertension or seizures. The SDH was bilateral in 16 cases, with a median width of 12 mm. Success of the procedure was noted in 14 of the 18 patients. There was no intraoperative complication or postoperative infection. Drainage failure was attributable to suboptimal positioning of the subdural drain in 2 cases and to migration in 1 case.


Subduro-subgaleal drainage is an efficient treatment that could be proposed as an alternative to external subdural drainage or subduroperitoneal drainage.

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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


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.


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.


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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Marie Bourgeois, Darach William Crimmins, Ricardo Santos De Oliveira, Alexis Arzimanoglou, Matthew Garnett, Thomas Roujeau, Federico Di Rocco and Christian Sainte-Rose


The authors sought to analyze the success rate of surgery in the management of medically intractable epilepsy in children with Sturge–Weber syndrome and to determine whether the extent and timing of surgery affected seizure and developmental outcomes.


The authors performed a retrospective review of 27 children who underwent surgery at their institution for medically resistant epilepsy, and they examined the outcomes with regard to epilepsy control and neuropsychological development.

Seventeen children (63%) experienced onset of their epilepsy when they were younger than 1 year of age. These patients were significantly more likely to have hemiparesis (p ≤ 0.001) and status epilepticus (p ≤ 0.001) and be developmentally delayed (p ≤ 0.025) than children whose epilepsy started later in life. Eight patients underwent a hemispherectomy (either anatomical or functional), and complete resolution of epilepsy was noted in all. Of the 19 patients in whom a focal resection was performed, 11 (58%) became seizure free. The 10 children in whom there was residual disease were more likely to have continuing epilepsy than the nine whose lesions were completely excised (p ≤ 0.05). Seventeen children exhibited improvement in their developmental status following surgery. This improvement was significantly affected by completeness of resection (p ≤ 0.05) and age at surgery (p ≤ 0.009). Seizure freedom per se was not affected by the timing of surgery.


Medically intractable epilepsy in children can be treated effectively by surgery. The degree of resection or disconnection of diseased tissue, but not patient age at the time of surgery, is an important factor in achieving epilepsy control. Early surgery is more likely to improve developmental outcome.

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Christian Sainte-Rose, Giuseppe Cinalli, Franck E. Roux, Wirginia Maixner, Paul D. Chumas, Maheir Mansour, Alexandre Carpentier, Marie Bourgeois, Michel Zerah, Alain Pierre-Kahn and Dominique Renier

The authors conducted a study to evaluate the effectiveness of endoscopically guided third ventriculostomy in the pre- and postoperative management of hydrocephalus in pediatric patients who harbored posterior fossa tumors.

Between October 1, 1993, and December 31, 1997, a total of 206 consecutive children with posterior fossa tumors underwent surgery at Hôpital Necker-Enfants Malades in Paris. Ten patients in whom shunts were implanted at the referring hospital were excluded. The medical records and neuroimaging studies obtained in the remaining 196 patients were reviewed. These patients were categorized into three groups: 67 patients with hydrocephalus on admission in whom endoscopically guided third ventriculostomy was performed prior to tumor removal (Group A); 82 patients with hydrocephalus in whom preliminary third ventriculostomy was not performed and who were managed in a “conventional way” (Group B); and 47 patients without ventricular dilation on admission (Group C).

There was no significant difference between Group A and Group B patients with respect to age at presentation, evidence of metastatic disease, degree of tumor resection, or follow up. In the patients in Group A, however, more severe hydrocephalus was present (p < 0.01). Patients in Group C were, in this respect, different from the other two groups.

Ultimately, only four patients (6%) in Group A as compared with 22 patients (27 %) in Group B (p = 0.001) had progressive hydrocephalus requiring treatment following removal of the posterior fossa tumor. Sixteen patients (20%) in Group B underwent insertion of a ventriculoperitoneal shunt, which is similar to the incidence of this procedure reported in the literature and significantly different from that in Group A (p < 0.016). The other six patients in Group B (6%) were treated by endoscopically guided third ventriculostomy after tumor removal. In Group C, two patients (4%) with postoperative hydrocephalus underwent endoscopically guided third ventriculostomy.

In three of the patients who required placement of cerebrospinal fluid shunts several episodes of shunt malfunction occurred; these were ultimately managed by performing endoscopic third ventriculostomy and definitive removal of the shunt.

There were no cases of death and four cases of transient morbidity associated with the ventriculostomy.

Third ventriculostomy is feasible even in the presence of posterior fossa tumors (including brainstem tumors). When performed prior to posterior fossa surgery, it significantly reduces the incidence of postoperative hydrocephalus. Furthermore, it provides a valid alternative to the placement of permanent shunts in cases in which hydrocephalus develops following posterior fossa surgery, and it may negate the need for the shunt in cases in which the shunt malfunctions.

Although the authors acknowledge that the routine application of third ventriculostomy in selected patients may result in a proportion of patients undergoing an “unnecessary” procedure, they believe that because of patients' less complicated postoperative course, the low morbidity rate, and the high success rate of third ventriculostomy, further investigation of this protocol is warranted.