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Raphael Guzman, Arjun V. Pendharkar, Michel Zerah, and Christian Sainte-Rose

Endoscopic third ventriculostomy (ETV) has become the procedure of choice for treatment of obstructive hydrocephalus. While patient selection is the most critical factor in determining the success of an ETV procedure, the technical challenge lies in the proper site of fenestration and the successful creation of a patent stoma. Positioning of a single balloon catheter at the level or below the floor of the third ventricle to achieve an optimal ventriculostomy can at times be challenging. Here, the authors describe the use of a double-barrel balloon catheter (NeuroBalloon catheter), which facilitates positioning across, as well as dilation of, the floor of the third ventricle. The surgical technique and nuances of using the NeuroBalloon catheter and the experience in more than 1000 cases are described. The occurrence of vascular injury was less than 0.1%, and the risk of balloon rupture was less than 2%. The authors found that the placement and deployment of this balloon catheter facilitate the creation of an adequate ventriculostomy in a few simple steps.

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Abhaya V. Kulkarni, Alain Pierre-Kahn, and Michel Zerah

✓ Spinal lipomas of the conus (SLCs) are relatively common closed neural tube defects. Many of these lesions are treated surgically at the time of diagnosis and, therefore, little has been documented about their true natural progression. In addition, the changes, if any, of SLCs in pediatric patients have not been widely studied or documented using magnetic resonance (MR) imaging. In this report, the authors describe two asymptomatic infants in whom serial MR imaging revealed substantial spontaneous regression of SLCs, a phenomenon that has been very rarely reported in the literature. These are the first MR imaging—documented cases of near-complete spontaneous regression of SLCs in infants.

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Ricardo Santos de Oliveira, Giuseppe Cinalli, Thomas Roujeau, Christian Sainte-Rose, Alain Pierre-Kahn, and Michel Zerah

Object

The authors of this retrospective review and analysis of the literature cover an institutional series of neurenteric cysts of the central nervous system in children treated in the magnetic resonance imaging era during a 14-year period.

Methods

Sixteen patients 20 days to 14 years of age are described. The most frequent signs and symptoms at presentation were acute spinal cord compression (11 patients), paresis of a cranial nerve (two patients), meningitis or infection (two patients), and intracranial hypertension (one patient). The locations of the cysts were as follows: in the spinal canal in 12 patients (75%), the clivus in two (12.5%), the cavernous sinus in one (6%), and the craniocervical junction in one (6%). The most common location was the ventral aspect of the spinal canal (seven patients). Associated spinal deformities were found in five patients. All patients underwent surgery, with a posterior approach used in all of the spinal cases. Total resection was achieved in 12 of the 16 cases and partial resection in four. Of the four patients who underwent subtotal resection, the cyst recurred in three, requiring further surgery.

Conclusions

Neurenteric cysts are uncommon congenital anomalies that can present acutely in the pediatric population. Total removal is usually possible and is associated with a good prognosis.

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Stéphanie Meuric, Raja Brauner, Christine Trivin, Jean-Claude Souberbielle, Michel Zerah, and Christian Sainte-Rose

Object

This study was performed to optimize the management of craniopharyngiomas, particularly by identifying factors predicting weight changes to prevent obesity.

Methods

A series of 35 patients who had undergone surgery at a mean age of 7.4 ± 3.7 years (standard deviation [SD]) and had been followed up until 14.9 ± 5 years of age by the same endocrinologist were assigned to one of three groups according to their hypothalamic involvement: Group 1 (10 patients) had no involvement, Group 2 (eight patients) had compression without involvement, and Group 3 (17 patients) had severe involvement.

Abnormal height and/or weight evolution indicated the craniopharyngioma in only 17% of the patients, although these elements were present at diagnosis in 85%. Before surgery, 85% of the patients lacked growth hormone, 24% lacked thyroid-stimulating hormone, 15% lacked adrenocorticotropin hormone, and 12% lacked antidiuretic hormone. All had complete hypothalamic—pituitary deficiencies after surgery.

The body mass index (BMI) before surgery (mean SD 1.1 ± 1.6) was positively correlated with BMI 1 year after surgery (mean SD 3.1 ± 2), which correlated with the BMI at the last evaluation (mean SD 3.1 ± 1.9; p < 0.0001 for both). Before surgery, patients in Group 3 had a greater BMI than did Group 1 (p < 0.02). The BMI of Group 1 patients did not change, but those of Groups 2 and 3 patients increased during the 1st year after surgery (p < 0.02 and p = 0.0003, respectively), with no further change. The changes occurred mainly during the first 3 months after surgery in Group 1, during the first 6 months in Group 2, and throughout the year in Group 3.

Conclusions

The degree of hypothalamic involvement by the craniopharyngioma determines the presentation and predicts weight changes after surgery.

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Dominique Renier, Eric Arnaud, Giuseppe Cinalli, Guy Sebag, Michel Zerah, and Daniel Marchac

✓ The factors involved in the mental development of patients with Apert's syndrome were studied by the authors, focusing on the age of the patient at operation, associated brain malformations, and the quality of the family environment. Overall, 32% of patients with significant follow-up review had an intelligence quotient (IQ) greater than 70. Age at operation appeared to be the main factor associated with changes in mental development: final IQ was greater than 70 in 50% of patients operated on before 1 year of age versus only 7.1% in patients operated on later in life (p = 0.01). Malformations of the corpus callosum and size of the ventricles played no role in the final IQ, whereas anomalies of the septum pellucidum had a significant effect, with the proportion of patients with an IQ over 70 increasing more than twofold in patients with a normal septum compared with patients with septal anomalies (p < 0.04). Quality of the family environment was the third factor involved in intellectual achievement: only 12.5% of institutionalized children reached a normal IQ level compared to 39.3% of children from a normal family background.

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Darach William Crimmins, Alain Pierre-Kahn, Christian Sainte-Rose, and Michel Zerah

Object

The authors sought to determine the natural history of and optimal treatment for suprasellar cysts (SSCs).

Methods

Three hundred forty-two patients harboring intracranial cysts presented to the authors’ neurosurgery unit between January 1986 and August 2004. The patients’ records were reviewed to assess symptomatology, results of imaging studies, and outcome according to mode of treatment.

Thirty-three patients (9.6%) were eligible for this study. Nine SSCs were diagnosed prenatally and 24 were identified postnatally (range 0 months–18.2 years, mean 5.6 years). The mean follow-up period was 66.8 ± 44.6 months (standard deviation). Seven cysts were left untreated, six (66%) detected before birth and one (4.5%) after birth. Of the 26 patients who required surgery, three were admitted elsewhere for complications of shunt surgery. A ventriculocystostomy (VC) was performed in all three of these patients, but the treatment failed in two. The primary treatment in the remaining 23 children was: open fenestration in two patients, VC in seven, ventriculocystocisternostomy (VCC) in 13, and cystoperitoneal (CP) shunt in one patient. Both open fenestration procedures were successful, as was the CP shunt insertion. The success rate of primary endoscopic surgery, although not statistically significant, was higher for VCCs (11 [85%] of 13 patients) than for VCs (four [57%] of seven patients). None of the patients’ preoperative endocrine disorders resolved postoperatively. The distribution of intellectual and developmental quotients paralleled the normal range. Intellectual performance was unrelated to patient-specific factors or to treatment modalities.

Conclusions

Most SSCs are of moderate size, are stable and asymptomatic, and have a favorable outcome. Treatment is required when the cyst evolves or the patient is symptomatic, but endocrine disturbances alone are not an indication for surgery. When hydrocephalus is present, endoscopic fenestration is the primary treatment of choice. The goal of the procedure should be to open the cyst into both the ventricles and the cisterns. Intellectual capability after treatment at outcome is not related to age at diagnosis, initial or final cyst size, presence or absence of hydrocephalus, or type of endoscopic treatment.

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Giuseppe Cinalli, Laurent Vinikoff, Michel Zerah, Dominique Renier, and Alain Pierre-Kahn

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Subdural sarcoma associated with chronic subdural hematoma

Report of two cases and review of the literature

Giuseppe Cinalli, Michel Zerah, Michel Carteret, François Doz, Laurent Vinikoff, Arielle Lellouch-Tubiana, Beatrice Husson, and Alain Pierre-Kahn

✓ The authors report the cases of two children who presented in the first months of life with progressive macrocrania related to chronic pericerebral fluid collection. This condition resolved spontaneously without treatment after a few months in the first case, whereas it required several aspirations of blood-stained fluid via the fontanel in the second case. Both patients developed normally without evidence of disease in the earliest years of life and presented at the ages of 3 1/2 and 4 1/2 years, respectively, with symptoms and signs of rapidly progressing intracranial hypertension. In both cases contrast-enhanced computerized tomography and magnetic resonance imaging revealed masses in the subdural space of the skull base and the cranial vault associated with significant subdural fluid collections. In the first case the lesion was misdiagnosed in the initial phase and treated, by means of multiple craniotomies, as an organized subdural hematoma. After a diagnosis of liposarcoma had been made, the patient was treated with chemotherapy, which resulted in a good resolution of the lesions at 3-month follow-up review. In the second case a biopsy allowed the diagnosis of fibrohistiocytic sarcoma and the patient was treated with chemotherapy. The authors review the literature of the few reported cases and discuss the possible pathophysiological association between pericerebral fluid collection and the subsequent development of a subdural sarcoma.

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Jessica Ternier, Alison Wray, Stéphanie Puget, Nathalie Bodaert, Michel Zerah, and Christian Sainte-Rose

Object

The authors characterized the clinical course of tectal plate lesions in a group of pediatric patients to identify the prognostic factors at presentation that predict progression, in an attempt to differentiate tectal hamartomas from tumors.

Methods

A retrospective review was conducted of the management of tectal plate lesions in children since the advent of magnetic resonance (MR) imaging at the authors’ hospital (1984–2003). The lesion volume seen on MR images, the clinical and radiological features at presentation, and the clinical course of the population were analyzed for correlations.

Forty children with tectal lesions presented in the typical delayed fashion (mean 8.5 months) with symptoms referable to hydrocephalus (93%). Fourteen children whose tumors demonstrated radiological progression (enlargement, contrast enhancement, or cystic change) were treated surgically. Histologically, 80% of the surgically treated lesions were low grade (with the other 20% consisting of one dysplasia, one high-grade tumor, and one unidentified tumor). Five patients required a second operation and one required a third. One patient died of a high-grade astrocytoma after undergoing surgery and radiotherapy; the other 39 patients remain clinically stable. The only factor predictive of tumor enlargement was lesion volume at presentation (p = 0.002). Distribution analysis revealed three subgroups based on lesion volume (< 4, 4–10, and > 10 cm3), which correlated with the clinical course of the disease.

Conclusions

Children with tectal lesions should undergo contrast-enhanced MR imaging and volume assessment at the time of presentation. After hydrocephalus has been managed with endoscopic third ventriculostomy, these children require prolonged, close clinical and radiological surveillance. Lesions with a volume less than 4 cm3 were likely to be hamartomas and followed a predominantly benign course, with few atypical cases progressing. All large lesions, defined as having a volume greater than 10 cm3 at presentation, eventually required treatment, and all were histologically determined to be tumors. An argument is made for earlier treatment of larger lesions with the aim of improving outcome.

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Pierre-Aurelien Beuriat, Stephanie Puget, Giuseppe Cinalli, Thomas Blauwblomme, Kevin Beccaria, Michel Zerah, and Christian Sainte-Rose

OBJECTIVE

Hydrocephalus remains one of the more common pathologies managed in pediatric neurosurgical units. Endoscopic third ventriculostomy (ETV) has an advantage over ventriculoperitoneal shunting as it enables patients to remain device free. Multiple shunt devices with various valve designs exist, with no one valve proven to be superior to another. The aim of this study was to describe the management of hydrocephalus and its long-term outcome.

METHODS

The authors retrospectively reviewed the medical records of all patients who had been treated for hydrocephalus at the Hôpital Necker-Enfants Malades in the period from 1985 to 1995.

RESULTS

Nine hundred seventy-five children had been treated for hydrocephalus. The mean follow-up was 11 ± 7.4 years (mean ± standard deviation). The most common cause of hydrocephalus was tumor related (32.3%), followed by malformative (24.5%) and inflammatory (20.9%) causes. Two hundred eighty patients underwent ETV as the first-line treatment. The procedure was effective in controlling hydrocephalus due to posterior fossa tumors and aqueductal stenosis. Six hundred ninety-five children had initial shunt insertion, with the majority receiving an Orbis-Sigma valve (OSV). The overall OSV shunt survival was 70% at 1 year, 58% at 10 years, and 49% at 20 years. The most common cause for mechanical shunt failure was obstruction (50.7%). Overall shunt survival was statistically different between the OSV and the differential-pressure valve (p = 0.009).

CONCLUSIONS

Endoscopic third ventriculostomy is effective in the management of childhood hydrocephalus. Its success is directly related to the underlying pathology. In the long term, the OSV has significantly higher event-free shunt survival than the classic differential-pressure valve systems