Background:The aim of this paper is to report on our surgical strategy and technique and to identify the best management for intracranial dermoids and epidermoids tumors (IDETs).Methods:We retrospectively reviewed 33 consecutive patients (14 males and 19 females; mean age at surgery, 37.9 years) with pathologically confirmed IDETs who underwent surgical resection, with mean follow-up of 7.2 years.Results:Gross total tumor removal was achieved in 24 cases (72.7%) with zero surgical mortality and a recurrence rate of 9%.Conclusions:The surgical strategies used in this group of patients enabled total removal of most tumors without surgical mortality and with low morbidity and recurrence rates, proving to be safe and effective.

-The transoral approach provides a safe exposure to lesions in the midline and the ventral side of the craniovertebral junction. The advantages of the transoral approach are 1) the impinging bony pathology and granulation tissue are accessible only via the ventral route; 2) the head is placed in the extended position, thus decreasing the angulation of the brainstem during the surgery; and 3) surgery is done through the avascular median pharyngeal raphe and clivus. We analyzed the clinical effects of odontoidectomy after treating 38 patients with basilar invagination. The anterior transoral operation to treat irreducible ventral compression in patients with basilar invagination was performed in 38 patients. The patients' ages ranged from 34 to 67 years. Fourteen patients had associated Chiari malformation and eight had previously undergone posterior decompressive surgery. The main indication for surgery was significant neurological deterioration. Symptoms and signs included neck pain, myelopathy, lower cranial nerve dysfunction, nystagmus and gait disturbance. Extended exposure was performed in 24 patients. The surgery was beneficial to the majority of patients. There was one death within 10 days of surgery, due to pulmonary embolism. Postoperative complications included two cases of pneumonia, three cases of oronasal fistula with regurgitation and one cerebrospinal fluid leak. In patients with marked ventral compression, the transoral approach provides direct access to the anterior face of the craniovertebral junction and effective means for odontoidectomy.KEY WORDS: basilar invagination, odontoidectomy, transoral approach. Acesso transoral para a junção craniocervicalRESUMO -O acesso transoral é uma via direta e segura às lesões situadas na linha média e na face anterior da junção craniocervical. As vantagens do acesso transoral são as seguintes:1) a compressão óssea e o tecido de granulação localizam-se anteriormente e são accessíveis pela via anterior; 2) a cabeça do paciente é colocada em extensão, diminuindo a angulação do tronco cerebral durante a cirurgia; e 3) a cirurgia é feita através de um plano avascular na linha média faríngea e clivo. Analisamos os resultados obtidos após odontoidectomia por via transoral em 38 pacientes portadores de invaginação basilar. Trinta e oito pacientes com compressão ventral da junção craniocervical foram submetidos a odontoidectomia por via transoral. A idade dos pacientes variou de 34 a 67 anos. Quatorze pacientes apresentavam associação com malformação de Chiari tipo I e 8 já haviam sido submetidos à cirurgia descompressiva por via posterior. A maioria dos pacientes apresentou nucalgia, mielopatia, déficits dos nervos cranianos baixos, nistagmo, e distúrbio da marcha. Em 24 pacientes foi necessário ampliar o acesso transoral através de miotomia do palato mole, ou osteotomia do palato duro ou maxilotomia. A cirurgia proporcionou melhora dos sintomas na maioria dos pacientes. Um paciente faleceu no pós-operatório imediato por causa de embolia pulmonar. Dois paci...

Objective: Observe whether a microsurgical gross total removal (GTR) of a spinal nerve sheath tumors (SNSTs) is safe and decreases the tumor recurrence. Method: We identify 30 patients with 44 SNSTs. Results: We operated upon 15 males and 15 females patients; mean age 40 years. GTR was achieved in 29 (96.6%) instances. Surgical mortality was 3.3% and the recurrence rate was 3.3%. The median follow-up time was 6.2 years. Conclusion: The surgical approach used in this group of patients afford that the great majority of tumors could be totally removed with low mortality and low recurrence rates, proving to be safe and effective.Keywords: microsurgery, neurofibroma, schwannoma, spinal nerve sheath tumors, surgical treatment. RESUMOObjetivo: Observar se a ressecção microcirúrgica completa dos shwannomas ou neurofibromas raquianos é uma técnica segura e efetiva. Método: Foram operados 30 pacientes com 44 schwannomas ou neurofibromas intrarraquiano. Resultados: A remoção total da lesão ocorreu em 27 casos (96.6%). A taxa de mortalidade cirúrgica observada nesta série foi de 3.3%. O tempo médio de seguimento foi de 6.2 anos. Conclusão: A estratégia microcirúrgica empregada com esses pacientes propiciou a remoção total dos tumores na maioria dos pacientes, com baixa mortalidade e recidiva tumoral, mostrando ser segura e efetiva.Palavras-chave: microcirurgia, neoplasia raquiana, neurofibroma, tumors da bainha dos nervos, schwannoma raquiano.The annual incidence rates for primary spinal neoplasms are reported to be in the range of 1.3 to 10.0 per 100,000 1 . One-third is nerve sheet tumors 1,2,3 . The spinal nerve sheet tumors (SNSTs) comprise the schwannomas and neurofibromas 1,2,3,4 . Nevertheless little attention has been paid to this subject within Brazilian settings, which motivated us to review and present the cases of this pathological condition treated by our medical team, and to assess the safety and efficiency of microsurgical removal of those lesions.Various terms have been used for this tumor: neuroma, neurinoma, neurilemoma, perineurial fibroblastoma, schwannoma gliome peripherique, schwannoma and neurofibroma 2,4 . The SNSTs are benign, usually solitary, slowgrowing, encapsulated neoplasms composed of differentiated neoplastic Schwann cells. The most common origin of these tumors is the posterior spinal nerve root 1,2,3,4,5,6 . METHOD Data collectionThe 30 patients registered in this retrospective study with SNSTs were operated at the Hospital Federal dos Servidores do Estado (RJ) and at Rede D'Or São Luiz between 1986 and 2012. The medical charts, pre and postoperative imaging, and pathological reports were retrospectively reviewed for each patient to confirm the diagnosis of SNSTs; creating a database from which information pertinent to the present study was collected. The intraoperative videos of 11 patients were analyzed for nuances of the microsurgical technique. The need for informed consent was waived due to the retrospective character of the study. Detailed neurological examinations were perfo...

We report our experience on the treatment of tuberculum sellae meningiomas (TSMs) regarding the involvement of the optic canal and clinical outcomes. We reviewed 23 patients who were operated on between January 1997 and December 2008. The surgical approach was unilateral subfrontal supraorbital osteotomy in one piece. Attempts were made to improve visual function via extra/intradural unroofing of the optic canal, which released the optic nerve. Visual symptoms were present preoperatively in 21 patients, and two patients were asymptomatic. Visual acuity remained intact in 6 patients, improved in 10, was unchanged in 5 patients, and worsened in 2 patients. The postoperative visual field was normal or improved in 17 patients, unchanged in four patients, and worsened in two patients. The optic canal and clinoid were drilled extradurally in eight patients and intradurally in nine patients. Total resection of TSMs was achieved in 19 patients. Incomplete resection occurred in two patients. Decompression of the optic canal seemed to increase the visual outcome. Key words: tuberculum sellae meningioma, optic canal, visual symptoms. Meningiomas do tuberculo selar: aspectos cirúrgicos resuMoApresentamos nossa experiência em 23 pacientes operados com meningiomas do tubérculo da sela, com enfoque na descompressão do nervo óptico e nos sintomas visuais do pós-operatório. Vinte e três pacientes com meningiomas do tubérculo da sela foram operados entre janeiro de 1997 e dezembro de 2008, através do acesso subfrontal via ostetomia supraorbital. Remoção do teto do canal óptico por via extra ou intradural foi realizada em 17 pacientes. Sintomas visuais no pré-operatório ocorreram em 21 pacientes, 2 eram assintomáticos. Melhora visual ocorreu em 10, permaneceu inalterada em 5 e piorou em 2 pacientes. No pós-operatório o campo visual normalizou-se em 17 pacientes, permaneceu inalterado em 4 e diminuiu em 2. Descompressão dos nervos ópticos foi realizada em 17 pacientes. Ressecção total dos meningiomas do tubérculo da sela foi possível em 19 pacientes. Abertura do canal óptico permitiu a manipulação do nervo óptico sem novos déficites. Palavras-chave: meningioma, tubérculo selar, nervo óptico, sintomas visuais.

Dementia implies the decline of intellectual abilities or cognitive function. It is also characterized by behavioral abnormalities and changes in personality 1 . One unusual cause of dementia, characterized mainly by amnestic syndrome, apathy, speech and language disturbances, hypersomnia, vigilance disorder and vertical gaze palsy, is due to thalamic lesion that led to the term "thalamic dementia" 2-4 . This is related to the known thalamic connections with cortical nucleus concerned with memory process, cognition and behavior, the thalamo-fronto-limbic loops 5,6 . Intracranial dural arteriovenous fistula (AVF) with venous drainage into internal cerebral veins and consequently venous hypertension is uncommon and may be associated with thalamic injury. Dural AVF consists of abnormal shunting of blood between the meningeal branches or rarely cortical branches of the external, internal carotid or vertebral arteries, and the venous system, embedded in the dural leaflets of a venous sinus to form the nidus 7-10 . Capillary beds usually interposed are lacking 7. Intracranial dural AVF represent approximately 10% to 15% of all intracranial vascular malformations 8,9,[11][12][13] . Since the posterior fossa dural AVF and subsequent venous hypertension is important to be considered in the different diagnosis of thalamic lesions 14 , we report an unique case of thalamic dementia associated with venous hypertension in the bilateral thalamus due to a dural AVF that was successfully treated by endovascular procedures. cASEA 43-year-old man, air professional, coursing political science master, presented subacute onset of apathy and memory deficit. Initially, the symptoms were stress misdiagnosed. Nevertheless, his symptoms worsened progressively. On admission, the patient was awake but confuse in time. There was neither headache, neither visual impairment nor vomits. The thorough neurological examination revealed critical sense mild compromised and amnesia, predominately of recent events. No significant medical history was related.The optic fundi were normal. There was no signal of intra-

Background:Primitive neuroectodermal tumor (PNET) is a malignant neoplasm that generally arises from bone and soft tissues, with predilection for young adults. This neural crest origin tumors share biologic and histologic features with Ewing's sarcoma (ES).Case Description:We present a case of a 22-year-old woman with history of severe progressive neck pain, without radiation, associated with paresthesia in the right arm, and palpable right posterior cervical mass. Neurological examination showed increased reflexes in all four limbs, bilateral Hoffman's sign, right Babinski's sign, and right hemi-hypoesthesia. Neuroimaging revealed a right posterior cervical lesion with heterogeneous contrast enhancement extending to the neural foramina of the atlas and axis. Patient underwent microsurgical removal of the lesion, and histopathological and immunohistochemical analysis confirmed the diagnosis of peripheral primitive PNET (pPNET). The patient had adjuvant treatment with radiotherapy and chemotherapy. After twelve months, neuroimaging showed no signs of tumor regrowth and the patient had no neurological deficits. However, three months later, the patient developed hydrocephalus and cerebrospinal fluid (CSF) was positive for neoplastic cells. No other treatment was administered and the patient died.Conclusion:pPNET is a rare malignant tumor with poor prognosis, although promising results with multimodal treatment-surgery, radiotherapy, and chemotherapy. Diagnosis requires immunohistochemical analysis, with identification of neuronal differentiation markers.

The cavernous sinus (CS) has one of the most complex anatomical networks of the skull base and because of the diversity of its contents is involved in many pathological processes. Nevertheless, anatomical literature concerning the CS is still controversial, so a systematic literature review was performed to find out the microanatomy of the medial wall of the CS and its clinical importance on sellar pathologies. Experimental studies from English-language literature between 1996 and 2010 were identified in MEDLINE, LILACS, and Cochrane databases. After analysis, two tables were prepared exhibiting the major points of each article. Fourteen experimental studies were included in the tables. Four studies concluded that the medial wall of the CS is composed of a loose, fibrous structure, and the remaining ten presumed that the medial wall is formed by a dural layer that constitutes the lateral wall of the sella. The lack of definition standards and of methodological criteria led to variation in the results among different studies. Thus, this hindered results comparison, possibly explaining the different observations.

Image-guided transsphenoidal endoscopic surgery is recommended for its safety and minimal invasiveness.