Optic pathway ganglioglioma with intraventricular cyst.

Gangliogliomas originating in the optic pathway are rare, with less than 20 cases reported in the literature. Diffuse, bilateral involvement of the entire optico-chiasmatic pathway is exceptional. We report a case of suprasellar ganglioglioma that involved bilaterally the entire pregeniculate optic pathway. The patient presented with visual deficit, nystagmus, papilledema and acute biventricular hydrocephalus secondary to intraventricular cyst that required urgent surgery. Endoscopic fenestration of the tumoral cyst allowed control of hydrocephalus and decompression of the visual pathway. Through microsurgical procedure by pterional approach, partial removal of the tumor and histological diagnosis were accomplished 1 week later. The patient was managed with chemotherapy and radiation therapy. He presents stable residual disease at 4-year follow-up. Embryological origins, histological features, neuroradiological appearance, management and prognosis of optic pathway gangliogliomas are reviewed.

Hydrocephalus in Dandy-Walker malformation.

INTRODUCTION: Even if the first description of Dandy-Walker dates back 1887, difficulty in the establishment of correct diagnosis, especially concerning differential diagnosis with other types of posterior fossa CSF collection, still persists. Further confusion is added by the inclusion, in some classification, of different malformations with different prognosis and therapeutic strategy under the same label of "Dandy-Walker". METHODS: An extensive literature review concerning embryologic, etiologic, pathogenetic, clinical and neuroradiological aspects has been performed. Therapeutic options, prognosis and intellectual outcome are also reviewed. CONCLUSION: The correct interpretation of the modern neuroradiologic techniques, including CSF flow MR imaging, may help in identifying a "real" Dandy-Walker malformation. Among therapeutical strategies, single shunting (ventriculo-peritoneal or cyst-peritoneal shunts) appears effective in the control of both ventricle and cyst size. Endoscopic third ventriculostomy may be considered an acceptable alternative, especially in older children, with the aim to reduce the shunt-related problems. Prognosis and intellectual outcome mostly depend on the presence of associated malformations, the degree of vermian malformation and the adequate control of hydrocephalus.

Pseudotumor cerebri.

INTRODUCTION: Pseudotumor cerebri is a condition characterized by raised intracranial pressure, normal CSF contents, and normal brain with normal or small ventricles on imaging studies. It affects predominantly obese women of childbearing age; however, its incidence seems to be increasing among adolescent and children. While among older children the clinical picture is similar to that of adults, younger children present demographic and clinical peculiarities. Different diagnostic criteria for adults and pre-pubertal children have been proposed. Etiology and pathogenesis are still unclear, particular concerning the role of obstruction to venous outflow. METHODS: An extensive literature review concerning all the aspects of pseudotumor cerebri has been performed, both among adults and pre-pubertal children. CONCLUSION: Pseudotumor cerebri is an avoidable cause of visual loss, both in adults and children. Few diagnostic measures are usually sufficient to determine the correct diagnosis. Since pseudotumor cerebri is a diagnosis of exclusion, the differential diagnosis work out is of special importance. Modern neuroimaging techniques, especially magnetic resonance imaging and magnetic resonance venography may clarify the role of obstruction to venous outflow in each case. Various therapeutic options are available: medical, surgical, and endovascular procedures may be used to prevent irreversible visual loss. Treatment is usually effective, and most patients will experience complete resolution of symptoms without persistent deficits.

Surgical treatment of thalamic tumors in children.

OBJECTIVE In the past, the outcome of surgical treatment for thalamic tumor was poor. These lesions were often considered inoperable. However, contemporary microsurgical techniques, together with improvements in neuroimaging that enable accurate presurgical planning, allow resection to be accomplished in a safer way. METHODS The medical records, imaging studies, and operative and pathology reports obtained for pediatric patients who were treated for thalamic tumors at the authors' department were reviewed. Neuronavigation and intraoperative monitoring of motor and somatosensory evoked potentials were used. Preoperative tractography, which helped to identify internal capsule fibers, was very important in selecting the surgical strategy. Postoperatively, an MRI study performed within 24 hours was used to assess the extent of tumor resection as partial (≤ 90%), subtotal (> 90%), or gross total (no residual tumor). RESULTS Since 2002, 27 children with thalamic tumors have been treated at the authors' department. There were 9 patients with unilateral thalamic tumors, 16 with thalamopeduncular tumors, and 2 with a bilateral tumor. These last 2 patients underwent endoscopic biopsy and implantation of a ventriculoperitoneal shunt. Thirty-nine tumor debulking procedures were performed in the remaining 25 patients. Different surgical approaches were chosen according to tumor location and displacement of the posterior limb of the internal capsule (as studied on axial T2-weighted MRI) and corticospinal tract (as studied on diffusion tensor imaging with tractography, after it became available). In 12 cases, multiple procedures were performed; in 7 cases, these were done as part of a planned multistage resection. In the remaining 5 cases, the second procedure was necessary because of late recurrence or regrowth of residual tumor. At the end of the surgical phase, of 25 patients, 15 (60%) achieved a gross-total resection, 4 (16%) achieved a subtotal resection, and 6 (24%) achieved a partial resection. Eighteen patients harbored low-grade tumors in our series. In this group, the mean follow-up was 45 months (range 4-132 months). At the end of follow-up, 1 patient was dead, 12 patients were alive with no evidence of disease, 4 patients were alive with stable disease, and 1 was lost to follow-up. All patients were independent in their daily lives. The outcome of high-grade tumors in 9 patients was very poor: 2 patients died immediately after surgery, 6 died of progressive disease, and 1 was alive with residual disease at the time of this report. CONCLUSIONS This institutional review seems to offer further evidence in favor of attempts at radical resection in pediatric patients harboring unilateral thalamic or thalamopeduncular tumors. In low-grade gliomas, radical resection in a single or staged procedure can be curative without complementary treatment. Recurrences or residual regrowth can be safely managed surgically. In high-grade tumors, the role of and opportunity for radical or partial resection remains a matter of debate.

Interhemispheric and quadrigeminal cysts.

BACKGROUND: Interhemispheric and quadrigeminal cysts are rare lesions, similar in their propensity to present in young babies and to be associated with other central nervous system malformations, such as corpus callosum agenesia, holoprosencephaly, encephalocele, and neuronal heterotopias. Recently endoscopy has become increasingly popular in the treatment of arachnoid cysts, but experience with cysts located in the interhemispheric fissure and in the quadrigeminal cistern is limited. METHODS: This study reviews the specific anatomy of interhemispheric and quadrigeminal cysts and their relationship with the ventricular system and subarachnoid cisterns to select the most appropriated treatment. It also reviews the literature on endoscopic treatment of interhemispheric and quadrigeminal cysts. RESULTS: Interhemispheric and quadrigeminal cysts are not homogeneous, they have different extensions toward surrounding regions. In most cases it is presented as an area of contiguity between the cyst and ventricular system and/or subarachnoid cisterns, making endoscopic treatment feasible. The success rate for endoscopic treatment is not different from that reported in large series of arachnoid cysts elsewhere. CONCLUSIONS: Endoscopic treatment should be considered the first-line option in the treatment of such lesions, even if some complications, such as subdural collections due to thinness of the cerebral mantle or subcutaneous CSF collections due to multifactorial associated hydrocephalus, must be expected.

Neuroendoscopic treatment of arachnoid cysts of the quadrigeminal cistern: a series of 14 cases.

OBJECT: In this paper, the authors' goal was to evaluate the role of neuroendoscopy in the treatment of arachnoid cysts of the quadrigeminal cistern. METHODS: Between March 1995 and February 2008, 14 patients affected by arachnoid cysts of the quadrigeminal cistern were treated endoscopically. The cysts were classified according to their anatomical and radiological appearance. The most frequent form (Type I) extended infratentorially and supratentorially with a dumbbell shape. Type II cysts were confined to the infratentorial space and were associated with the most severe and acute form of hydrocephalus. Type III cysts presented a significant asymmetric expansion toward the temporal fossa. Ten patients underwent an endoscopic procedure as primary treatment and 4 as an alternative to shunt revision. In 6 cases, the first endoscopic procedure was ventriculocystostomy (VC) together with endoscopic third ventriculostomy (ETV). In the other 8 cases, the first endoscopic procedure was VC alone. RESULTS: In the 6 cases in which VC was performed with an ETV, the procedure was successful, and the patients did not require further surgery. Of the 8 cases in which the first endoscopic procedure performed was VC without ETV, 7 underwent reoperation. Four of these patients underwent endoscopic procedures (by reopening the obstructed VC and performing ETV or cystocisternostomy) 2, 4, 4, and 5 months later with final success in all cases. Three patients (all of whom were previously treated using ventriculo- or cystoperitoneal shunts) required shunt reimplantation (complete failure). Subdural collection developed in 1 case, which was managed by transient insertion of a subduroperitoneal shunt. Neurological and developmental outcomes were good except for 1 patient who did not show improvement in preoperative developmental delay. No transient or permanent morbidity or mortality was observed. CONCLUSIONS: The analysis of this series suggests that arachnoid cysts of the quadrigeminal cistern and the associated hydrocephalus can be effectively treated by endoscopy; this approach allows the patient to be shunt independent in more than 78% of the cases. If endoscopy is used as first option, the success rate of endoscopic procedures observed in this series was 90%. Endoscopic third ventriculostomy should be associated with a VC to offer the highest success rate with a single procedure.

Complications following endoscopic intracranial procedures in children.

BACKGROUND: The significant technological improvement of endoscopic instrumentation has allowed, in the last 10 years, a widespread diffusion of neuroendoscopic procedures. Nevertheless, severe, sometimes life-threatening, complications may occur during neuroendoscopic surgery, and the incidence and age specificity of complications in children have been underdescribed so far. MATERIALS AND METHODS: Complications recorded in a prospectively collected database of pediatric patients undergoing neuroendoscopic procedures were analysed; the medical histories of the patients and the surgical procedures were reviewed. RESULTS: Complications occurred in 32 out of 231 (13.8%) procedures performed for the management of obstructive hydrocephalus (137), multiloculated hydrocephalus (53), arachnoid cysts (29) and intraventricular tumors (12). Subdural hygroma occurred in 11 cases, seven requiring subdural shunting. In one of these cases, infection of the subdural space occurred and required a craniotomy. Cerebrospinal fluid (CSF) infection occurred in 11 cases. In one case, a frontal abscess developed and was managed with craniotomy. CSF leak occurred in nine cases, intraventricular haemorrhages in two, technical failures in seven, subcutaneous CSF collection (managed with lumbo-peritoneal shunt) in one, thalamic contusion and post-operative transient akinetic mutism in one. This patient suddenly died 6 months later, probably as a consequence of closure of the stoma. Two patients developed secondary compartmentalisation of the ventricles after complicated endoscopic third ventriculostomy. In nine cases, these complications were associated. Overall, no patient died after the procedure (operative mortality 0), one patient died 6 months after the procedure for unexplained events (sudden death rate 0.4%), and three patients presented permanent disability as a consequence of surgical complication (permanent morbidity 1.3%). CONCLUSIONS: Complication rate of neuro-endoscopic procedures is not negligible even in experienced hands. The majority are minor complications which do not affect the final outcome, but sporadically major events may occur, leading to significant problems in surgical management and, occasionally, to permanent disabilities. Careful selection of patients on pre-operative imaging studies and intensive training of surgeons are mandatory to improve results.