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Abdominal pseudocysts are common complications of ventriculoperitoneal shunt (VPS). We report the case of a 37-year-old woman treated by VPS for congenital obstructive hydrocephalus, who presented shunt dysfunction related to a voluminous abdominal cyst initially diagnosed as cerebrospinal fluid pseudocyst. The cyst was drained and the VPS was removed after endoscopic third ventriculocisternostomy (ETV). A few months later, a large new abdominal cyst appeared and was operated on. Diagnosis was rectified as massive ovarian mucinous cystadenoma. In any intra-abdominal cyst, differential diagnoses need considering to avoid mis-diagnosis in shunted patients, especially if the cyst is very large. The etiology of the hydrocephalus should also be investigated in case of shunt dysfunction. Even in case of longstanding shunt, ETV can be an alternative to shunt revision surgery in obstructive hydrocephalus, enabling VPS withdrawal and treatment of the hydrocephalus.

Introduction Neurofibromatosis type 2 is characterized by the presence of bilateral vestibular schwannomas. However, other nervous system tumors may also occur. Therefore, the management of NF2 patients is complex and requires a multidisciplinary discussion in a specialized center. Materials and methods All recent articles concerning tumors other than vestibular schwannoma in NF2 disease were reviewed, using PubMed databases. Results Intracranial meningiomas occur in 50% of NF2 patients, and are often multiple. Surgery remains the main treatment and should be performed in cases of growing tumors. The role of antiangiogenic therapy is currently under evaluation and the role of radiosurgery still remains to be defined in NF2 disease. Spinal tumors occur in about half of NF2 patients. Surgery should be discussed when radiological tumor progression is demonstrated, even if spinal tumors are asymptomatic, in order to preserve neurological function and good quality of life. As regards lower cranial nerve schwannomas, radiosurgery appears to be a more appropriate treatment for growing tumor with a small volume in order to avoid post-operative complications, especially swallowing disorders. Facial nerve schwannomas may appear, on MRI, like vestibular schwannomas. The diagnosis should be suspected when the facial palsy is an early symptom during cerebello-pontine tumor progression. Trigeminal schwannomas are frequent in NF2 disease and fortunately they are often asymptomatic. Among major neurofibromatosis types, peripheral nerve sheath schwannomas are only present in patients with NF2 disease and schwannomatosis. Surgical resection is required when the cutaneous schwannomas is painful or when tumor progression is observed and causes symptoms. Conclusion Tumors other than vestibular schwannoma are also associated with a poor prognosis in NF2 patients. Surgery remains the main treatment in most cases. Each treatment decision in NF2 disease requires a complete evaluation of all cranial and spinal locations of the disease in order to establish surgical priorities and strategies.

Background and purpose Haemophilia is a well-known X-linked recessive bleeding disorder related to a deficiency of factor VIII. The aim of the paper is to point out the role of an early diagnosis in cases of intracranial bleeding, especially in neonates and to highlight the bad prognosis. Methods Three patients were included in the study from our prospective data. All had severe factor VIII deficiency (less than 1%). Results The first of them was a newborn without familial history of haemophilia which presented with hypotonia and seizures after a delivery with vacuum. CT showed a subdural haematoma and MRI multiple ischemic lesions. Two months later, he died from a new intracranial haemorrhagic episode resistent to infusion of factor VIII related to development of an anti-factor VIII antiboby. The second neonate had no familial history more and experienced hypothermia and areactive mydriasis because of an acute subdural haematoma. He died eight days later of ischemic lesions in spite of a craniotomy. The third was a 5-month-old boy with a familial history. Physical examination revealed lethargy, seizure and a recent cranial perimeter increase. He underwent a craniotomy with a good result. Conclusion The authors stress the need for immediate factor VIII infusion and appropriate neurosurgical work-up. The rapid medical management is mandatory if morbidity and mortality are to be minimized.

The lateral ventricles are the C-shaped cavities of the telencephalon. Embryology of theses cavities is recalled as well as the immediate relationship of the frontal horn, the body, the atrium and the temporal and occipital horns.

Central neurocytoma is the second most frequent tumor in our series of 284 lateral ventricle tumors (12%). Mean age was 32.6 years. There was a male predominance (20/35). Most frequent clinical signs were neuropsychological disturbances and intracranial hypertension. On imaging, these tumors concentrate central calcifications in more than half of cases. Main locations were frontal horn and corpus of lateral ventricle (82%). Uneventful postoperative course was recorded in 52% of cases. Immunoreactivity against neuron-specific enolase (NSE), synaptophysin, S 100 protein, Microtubul Associated Protein de type 2 (MAP 2) and calcineurin confirms the neuronal nature of the neoplasm. This tumor has a good prognosis. Local control after surgery is obtained in 68% of cases. Gamma knife surgery seems to be useful in cases of little volume with tumoral remnants or recurrence, when radiological appearance is well circumscribed and round-shaped.

Twenty-seven cases of ependymoma were collected in the French multicentric study dedicated to lateral ventricle tumors. The mean age of patients was 31,5 years. All patients underwent surgery, with complete resection of the tumor in 76% of cases. Histologic examination disclosed 23 grade 2 ependymomas and four grade 3 ependymomas. Postoperative mortality was 7%. Four patients underwent radiotherapy, and five chemotherapy. Local recurrence of the tumor occurred in seven cases, with a mean delay of 48 months. Disseminated disease within the central nervous system was found in only one patient. Median survival in all the 27 patients was 181 months, and median progression-free survival was 106 months. The relevant series of the literature are reviewed in order to precise the prognostic factors of ependymomas and therapeutic strategies.

Subependymoma is a benign lesion, slow-growing neoplasm, representing 0.2 to 0.7 % of intracranial tumors. They are often clinically silent, incidentally discovered at autopsy. These symptoms are related to big volume. They are attached to the septum pellucidum, leading to hydrocephalus by Monro foramen obstruction. Overall mean age at diagnosis is 39 years with more males than females. At CT-scan, subependymoma shows a slightly low attenuation compared to gray matter. There is no or mild enhancement following contrast injection. On MR T1-weighted imaging, subependymoma is isointense and hyperintense on MR T2-weighted imaging. Intramural calcifications and cystic components are noted in 20 to 30 % of patients. Peritumoral oedema is absent. Immunohistochemicals studies show intense positivity for S-100 and GFAP. The treatment is surgical with an excellent prognosis.

The lateral ventricle is a deep-seated cavity, overlayed by a cortical mantle which contains eloquent areas, especially on the dominant hemisphere, and surrounded by the optic radiations. The surgical approach requires a thorough preoperative reflexion based on magnetic resonance imaging, in order to understand the site of origin and the vascular pedicles of the tumor. Surgical approaches to the frontal horn, temporal horn and atrium are successively described.

The data from 284 cases of lateral ventricle (VL) tumors treated in 18 departments of Neurosurgery in France between 1995 and 2010 were collected in this series. The mean age was 33 years with a large proportion of children (79/284). Clinical presentation The mean duration of symptoms was short in time (2 months). The revelation was incidental in 10% of the cases ; for symptomatic lesions, raised intracranial pressure (ICP, due to hydrocephalus) and neuropsychological signs were the most frequent. Only one third of the patients had no CSF disorder. Histopathology The two most frequent tumors were meningiomas (40 cases, 15%) and neurocytomas (35 cases, 13%). The sub-ependymal giant cells astrocytomas, the ependymomas and sub-ependymomas were also common (11, 10 and 9% respectively). Therapeutic management A surgical procedure was performed in 258 patients, with the help of neuronavigation in one third of cases. The patients were most frequently operated with a transfrontal approach (40%). The resection was complete in 73%, which was a statistical factor of global survival. The postoperative mortality rate was 4.9%. Outcome Neuropsychological deficits were the most frequent sequelae (22.8%) due to raised ICP. Campimetric deficits (19%), seizures (19%), hemiparesis (15%) and language disorders (12%) were then found. The mean follow-up was 52 months with good evolution for 58% of patients. Fifteen percent of patients had a handicap and, finally, 12.9% will die. At last, two third of living patients had no tumoral residue or re-evolution.

The lateral ventricle (LV) has a deep position within the cerebral hemisphere. The LV is covered by white matter with important functional role in the dominant hemisphere. Lateral wall of the frontal horn is covered by the inferior occipitofrontal fasciculus (IOFF) and its roof by the corpus callosum (CC). The body of the LV has the same cranial relationship and is covered laterally by fibers of internal capsula and arcuate fasciculus; its lower part is in relationship with the body of the fornix. The atrium of the LV is covered by the arcuate fasciculus and its lower part is covered by the IOFF and optic radiations. The inferior horn or temporal horn is covered by optic radiations in depth of middle temporal gyrus (T2). The auditive radiations crossed the optic radiations at the level of the roof of the inferior horn.

Tumors of the lateral ventricle are not as rare in children as in adults and present complex problems which are to be dealt with often in a context of emergency, on account of their large size and the risk of life-threatening bleeding in a small child. Their diagnostic features are different in children, especially in infants and even more so in the fetus. The main surgical problems are the risk of bleeding and of hydraulic complications. From an oncological perspective, the main tumor types are choroid plexus tumors, subependymal giant-cell astrocytoma, and low-grade gliomas. The developmental outcome of these patients is often poor, on account of severe intracranial hypertension and young age at diagnosis.

Metastases of lateral ventricle (LV) are attached to choroidal plexus. Primary source is cancer of kidney. Two thirds of patients are male. Oligodendrogliomas occur in young females. Signs of increased intracranial pressure are a constant feature. These tumors are found in anterior portion of the LV with severe enhancement and clumped calcifications. Treatment is surgical. Cavernomas have a preponderance of rapid growth with a bleeding revelation. Seizures are rare. Rebleeding is frequent and justifies a surgical treatment. Schwannomas are a rare entity in which the majority of patients are very young. MRI shows calcifications, cystic components and a strong enhancement. They are limited to sporadic cases, never associated with neurofibromatosis. Arachnoid cysts are located in the atrium and/or in the occipital horn. Patients are young (mean age < 40 years). MRI demonstrates an intracystic lesion with signal intensity similar to the CSF. Best treatment is an endoscopic fenestration. Epidermoid cysts occur in third decade. These pearly tumors appear isointense or a little hyperintense on T1-weighted imaging, very characteristic. They are enhanced after gadolinium injection and appear strongly hyperintense on T2-weighted imaging. An incomplete removal with a thorough long-term follow-up is necessary. Cavernomas of LV are hyperintense on T1- and T2-weighted imaging. They have a bleeding risk of 25 to 45%. Therefore, they must be operated.

Tumors of the frontal horn of the lateral ventricle (LV) are only supplied by the posteromedial choroidal artery. Tumors of the body of the LV are supplied by the same artery. Tumors of the atrium of the LV with anterior extension are supplied by both posteromedial choroidal and posterolateral arteries. Tumors of the atrium with inferior extension are supplied by both anterior choroidal artery and posterolateral choroidal arteries. Tumors of the inferior horn are only supplied by anterior choroidal artery. The tumoral venous drainage is organized with three main groups of veins: a medial group, a lateral group and a choroidal group.

This series reports 40 cases of meningiomas of the lateral ventricle treated in France between 1995 and 2010. The mean age was 52 years. The revelation was incidental in 23% of cases. 36 patients underwent surgery with uneventful postoperative course in 75% of them. Visual field deficits were the most frequent sequelae. In six cases, recurrences occurred during the follow-up period. The relevant series of the literature were discussed in order to precise the therapeutic strategies and the outcome.