Luis A. B. Borba

Intravagal Paragangliomas: Report of Four Cases

The intravagal paraganglioma is a very rare tumor of the head and neck that accounts for only 5% of the paragangliomas in that area. A painless mass in the high neck with extension into the peripharyngeal space is the most common characteristic of this tumor. Malignant paragangliomas with invasion of the cervical lymph nodes and carotid artery have been reported, but the presence of metastasis, rather than the histological findings, is the only parameter for classifying them as malignant tumors. Despite the numerous descriptions of the efficacy of radiation therapy, the histological findings of irradiated specimens have shown little effect of radiation therapy on the chief cells. The only curative therapy for intravagal paragangliomas is the total resection of the tumor. Using the supra-adventitia dissection plane, we were able to achieve total resections in four cases of complex intravagal paraganglioma. The surgical management of these cases was complex because of the following: 1) misdiagnosis as a carotid body tumor, 2) previous radiation therapy and surgical procedure, 3) association with glomus jugulare, and 4) a giant tumor with invasion of the temporal bone and encasement of the internal carotid artery. We report the surgical management of intravagal paragangliomas and the role of radiation therapy, hormonal secretion, and rehabilitation care.

Surgical management of glomus jugulare tumors: a proposal for approach selection based on tumor relationships with the facial nerve

OBJECT The goal of this paper is to analyze the extension and relationships of glomus jugulare tumor with the temporal bone and the results of its surgical treatment aiming at preservation of the facial nerve. Based on the tumor extension and its relationships with the facial nerve, new criteria to be used in the selection of different surgical approaches are proposed. METHODS Between December 1997 and December 2007, 34 patients (22 female and 12 male) with glomus jugulare tumors were treated. Their mean age was 48 years. The mean follow-up was 52.5 months. Clinical findings included hearing loss in 88%, swallowing disturbance in 50%, and facial nerve palsy in 41%. Magnetic resonance imaging demonstrated a mass in the jugular foramen in all cases, a mass in the middle ear in 97%, a cervical mass in 85%, and an intradural mass in 41%. The tumor was supplied by the external carotid artery in all cases, the internal carotid artery in 44%, and the vertebral artery in 32%. Preoperative embolization was performed in 15 cases. The approach was tailored to each patient, and 4 types of approaches were designed. The infralabyrinthine retrofacial approach (Type A) was used in 32.5%; infralabyrinthine pre- and retrofacial approach without occlusion of the external acoustic meatus (Type B) in 20.5%; infralabyrinthine pre- and retrofacial approach with occlusion of the external acoustic meatus (Type C) in 41%; and the infralabyrinthine approach with transposition of the facial nerve and removal of the middle ear structures (Type D) in 6% of the patients. RESULTS Radical removal was achieved in 91% of the cases and partial removal in 9%. Among 20 patients without preoperative facial nerve dysfunction, the nerve was kept in anatomical position in 19 (95%), and facial nerve function was normal during the immediate postoperative period in 17 (85%). Six patients (17.6%) had a new lower cranial nerve deficit, but recovery of swallowing function was adequate in all cases. Voice disturbance remained in all 6 cases. Cerebrospinal fluid leakage occurred in 6 patients (17.6%), with no need for reoperation in any of them. One patient died in the postoperative period due to pulmonary complications. The global recovery, based on the Karnofsky Performance Scale (KPS), was 100% in 15% of the patients, 90% in 45%, 80% in 33%, and 70% in 6%. CONCLUSIONS Radical removal of glomus jugulare tumor can be achieved without anterior transposition of the facial nerve. The extension of dissection, however, should be tailored to each case based on tumor blood supply, preoperative symptoms, and tumor extension. The operative field provided by the retrofacial infralabyrinthine approach, or the pre- and retrofacial approaches, with or without closure of the external acoustic meatus, allows a wide exposure of the jugular foramen area. Global functional recovery based on the KPS is acceptable in 94% of the patients.

Intracranial aneurysms presenting with mass effect over the anterior optic pathways: neurosurgical management and outcomes

OBJECT Intracranial aneurysms may grow closer to anterior optic pathways, causing mass effect over these anatomical structures, including visual deficit. The authors retrospectively reviewed a series of aneurysms in patients presenting with visual field deficit caused by mass effect, to analyze the aneurysms characteristics, the neurosurgical management of these aneurysms, as well as their clinical, visual, and radiological outcomes. METHODS The authors reviewed the medical charts, neuroimaging examination results, and surgical videos of 15 patients presenting with visual symptoms caused by an aneurysms mass effect over the anterior optic pathways. These patients were treated at the Department of Neurosurgery, Center of Neurology and Neurosurgery Associates, Hospital Beneficência Portuguesa de São Paulo, Brazil. Statistical analysis was performed to identify the variables related to partial or total recovery of the visual symptoms. RESULTS All patients underwent microsurgical clip placement and emptying of their aneurysms. After a mean follow-up of 38.5 months, the mean postoperative Glasgow Outcome Scale score was 4.33, and the visual outcomes were as follows: 1 patient (6.6%) unchanged, 7 (46.6%) improved, and 7 (46.6%) experienced complete recovery from visual deficits. The variables that influenced the visual outcomes were the size of the aneurysm (p = 0.039), duration of the visual symptoms (p = 0.002), aneurysm wall calcification (p = 0.010), and intraluminal thrombosis (p = 0.007). Postoperative examination using digital subtraction angiography showed complete aneurysm occlusion in 14 (93.3%) of the 15 patients. CONCLUSIONS Intracranial aneurysms causing mass effect over the anterior optic pathways usually present with complex features. The best treatment option must include not only the aneurysm occlusion but also relief of the mass effect. Microsurgical clip placement with reduction of aneurysmal mass effect achieved improvement in visual ability or recovery from visual impairment, as well as total aneurysm occlusion, in 93.3% of the study group. Therefore, this option is well supported as the first choice of treatment for intracranial aneurysms presenting with mass effect over the anterior visual pathways.

Skull Base Chordomas

Summary:Chordomas are rare tumors originating from the remnants of the primitive notochord. Their deep location and patterns of extension and the proximity of structures such as the brain stem, upper cervical spine, hypophysis, and cranial nerves make their treatment a challenge. Even if gross total

The transcondylar approach to extradural non-neoplastic lesions of the craniovertebral junction

✓ Ventral extradural lesions at the craniovertebral junction are commonly exposed through the transoral or transmaxillary approach. The disadvantages of these approaches include: 1) difficulty in reaching laterally located lesions; 2) ineligibility of patients with an intradental distance of less than 25 mm or severe macroglossia; 3) the need for a separate procedure for stabilization and fusion; and 4) the risk of infection from transgressing a contaminated field. In this report, the authors describe the use of the transcondylar approach to extradural nonneoplastic lesions of the anterior craniovertebral junction for decompression and stabilization. Advantages of this approach include: 1) a short distance to the lesion; 2) a wide surgical envelope; 3) direct visualization of the dural sac, eliminating manipulation of the brainstem or upper spinal cord; 4) easy identification and control of the ipsilateral vertebral artery; 5) direct visualization and preservation of the lower cranial nerves; and 6) a ste...

Primary melanoma of Meckel's cave: case report

We present a case of trigeminal neuralgia with cranial normal magnetic resonance image (MRI) and computed tomography. The pain was not relieved by carbamazepine and microvascular decompression surgery was done. After two months the pain was similar to the condition before surgery. At this time, MRI showed an expansive lesion in Meckels cave that was treated with radical resection by extra-dural approach. The pathologic examination revealed a primary melanoma. The follow-up after six months did not show abnormalities.

Application of genetic testing to define the surgical approach in a sporadic case of multiple endocrine neoplasia type 1

We report the use of a genetic test for therapeutic decision making in a case of primary hyperparathyroidism associated with Cushings disease (CD). A 20-year-old woman was evaluated for gradual weight gain, asthenia, muscle pain, and hypertension. Biochemical and radiologic tests confirmed CD and she underwent transsphenoidal surgery. Immunohistochemistry of the microadenoma was positive for adrenocorticotropic hormone (ACTH). On follow-up, hypercalcemia with high parathyroid hormone (PTH) levels was detected, associated with nephrolithiasis and low bone mineral density in the spine and hip. Parathyroid scintigraphy showed tracer uptake in the inferior region of the left thyroid lobe, and cervical ultrasound showed a heterogeneous nodule in the same area, suggestive of a parathyroid adenoma (PA). Genetic testing detected mutation in the MEN 1 gene and total parathyroidectomy with the implantation of a fragment of one gland in the forearm was performed. Pathology showed a PA and 3 normal parathyroid glands, without hyperplasia, despite the diagnosis of MEN 1. This case illustrates the role of genetic testing in defining the therapeutic approach for sporadic MEN 1.

Complications of the anterior approach to the cervical spine

OBJETIVO: Evaluar las complicaciones del acceso anterior a la columna cervical en pacientes sometidos a artrodesis cervical con instrumentacion. METODOS: Estudio prospectivo y descriptivo realizado en el periodo de Enero/2009 hasta Abril/2010. Todos los pacientes sometidos a artrodesis de la columna cervical por via anterior fueron incluidos en el estudio, independientemente del diagnostico. El acceso fue realizado por via anterior, por el lado derecho. Fueron evaluados el numero de niveles operados (1, 2 o 3 niveles) y el tipo de procedimiento realizado: discectomia con colocacion de cage y placa (D+C+P), discectomia con colocacion de cage (D+C), o corpectomia con colocacion de cage y placa (C+C+P). Se informaron todas las complicaciones relacionadas con el acceso quirurgico. RESULTADOS: Fueron estudiados 34 pacientes, siendo 70% del sexo masculino. El promedio de edad fue de 50 anos y el tiempo promedio de seguimiento fue de ocho meses. Dieciocho por ciento de los pacientes tuvieron complicaciones distribuidas de la siguiente manera: disfagia (33%) y disfonia (67%). De los paciente que desarrollaron complicaciones, la mayoria fue sometida a la realizacion de D+C+P (83%) y ninguna complicacion fue relacionada con las tecnicas que no utilizaron placas cervicales. Con relacion a los niveles, las dos complicaciones fueron identificadas en los pacientes operados en uno o dos niveles. Sin embargo, en los operados en tres niveles, solo fue identificada la disfonia. CONCLUSION: La complicacion mas frecuente fue la disfonia. Los pacientes que mas presentaron complicaciones fueron aquellos sometidos a discectomia y artrodesis con cage y placa cervical anterior. Todos los casos de disfonia estaban en este grupo. El numero de niveles operados no parece haber afectado a la incidencia de complicaciones.Resumo Introducao : O acesso a coluna cervical anterior e bastante difundido em nosso meio e pode ser utilizado como opcao cirurgica em diversos tipos de patologias que acomete esse segmento da coluna vertebral. Objetivo : Avaliar as complicacoes do acesso anterior a coluna cervical em pacientes submetidos a artrodese cervical com instrumentacao. Metodos : Estudo prospectivo, descritivo realizado no periodo de janeiro/2009 a abril/2010. Todos os pacientes submetidos a artrodese de coluna cervical via anterior foram incluidos no estudo, independente do diagnostico. O acesso foi realizado via anterior pelo lado direito. Foram avaliados o numero de niveis operados (1, 2 ou 3 niveis), o tipo de procedimento realizado: discectomia com colocacao de cage e placa (D+C+P), discectomia com colocacao de cage (D+C) ou corpectomia com colocacao de cage e placa (C+C+P). Todas as complicacoes relacionadas ao acesso cirurgico foram relatadas. Resultados : Foram estudados 34 pacientes, sendo 70% do sexo masculino. A media de idade foi de 50 anos e o tempo de seguimento medio foi de oito meses. Dezoito por cento dos pacientes apresentaram complicacoes assim distribuidas: disfagia (33%) e disfonia (67%). Dentre os pacientes que evoluiram com complicacoes, a maioria foi submetida a realizacao de D+C+P (83%) e nenhuma complicacao foram encontradas nos pacientes onde nao foram colocadas placas cervicais. Em relacao aos niveis, ambas as complicacoes foram identificadas nos pacientes operados de 1 ou 2 niveis. Ja nos operados em 3 niveis, somente a disfonia foi identificada. Conclusao : A complicacao mais encontrada foi a disfonia. Os pacientes que mais apresentaram complicacoes foram aqueles submetidos a discectomia e artrodese com cage e placa cervical anterior. Todos os casos de disfonia estavam neste grupo. O numero de nivel acessado nao parece ter interferido na incidencia das complicacoes. Palavras-chaves : coluna vertebral, disfagia, disfonia, artrodese, pacientes

Foramen magnum meningiomas: surgical treatment in a single public institution in a developing country

OBJECTIVE To analyze the clinical outcome of patients with foramen magnum (FM) meningiomas. METHOD Thirteen patients (11 Feminine / 2 Masculine with FM meningiomas operated on through lateral suboccipital approach were studied. Clinical outcome were analyzed using survival (SC) and recurrence-free survival curves (RFSC). RESULTS All tumors were World Health Organization grade I. Total, subtotal and partial resections were acchieved in 69.2%, 23.1% and 7.7%, respectively, and SC was better for males and RFSC for females. Tumor location, extent of resection and involvement of vertebral artery/lower cranial nerves did not influence SC and RFSC. Recurrence rate was 7.7%. Operative mortality was 0. Main complications were transient (38.5%) and permanent (7.7%) lower cranial nerve deficits, cerebrospinal fluid fistula (30.8%), and transient and permanent respiratory difficulties in 7.7% each. CONCLUSIONS FM meningiomas can be adequately treated in public hospitals in developing countries if a multidisciplinary team is available for managing postoperative lower cranial nerve deficits.

Displasia fibrosa do clivus: relato de caso

We describe the case of a 43 year-old woman with chronic headache and diplopia. The neurorradiologic exam showed volume increase and alteration in the bone density of the clivus. She was submitted to surgery and the anatomopathologic evidenced fibrous dysplasia. Other 14 of about fibrous dysplasia involving the clivus are related in the literature. We discuss clinical aspects, image exams, histology and treatment of this rare presentation of the disease.