Neuromodulation of the posterolateral hypothalamus for the treatment of chronic refractory cluster headache: Experience in five patients with a modified anatomical target.

INTRODUCTION: Deep brain stimulation (DBS) of the posterior hypothalamus has been found to be effective in the treatment of refractory chronic cluster headache (CCH). METHODS: We report the long-term outcomes of five patients with refractory CCH on whom stimulation of a modified target of approximately 3 mm in radius, which included the posterolateral hypothalamus, the fasciculus mammillotegmentalis, the fasciculus mammillothalamicus and the fasciculus medialis telencephali, was performed. The stereotaxic coordinates were 4 mm from the third ventricle wall, 2 mm from behind the mid-intercommissural point and 5 mm from under the intercommissural line. RESULTS: All patients became pain-free for 1-2 weeks after the procedure, but then needed an average of 54 days to optimize stimulation parameters. After a mean follow-up of 33 months, two remain pain-free, two have an excellent response (>90% decrease in attack frequency) and in one the attacks have been reduced by half. There were no serious adverse events. Permanent myosis and euphoria/well-being feeling were seen in three patients. Other adverse events, such as diplopia, dizziness, global headache of cervical dystonia, were seen transiently related to an increase in stimulation parameters. Attacks reappeared transiently in two patients as a result of cable rupture and when the stimulator was disconnected. CONCLUSIONS: Our results supports the efficacy of DBS in very refractory CCH with a slightly modified hypothalamic target conceived to avoid the lateral ventricle wall so as to extend the stimulated brain area and to decrease the morbidity of potential haemorrhagic complications.

Hemangioblastoma quístico de bulbo raquídeo / Cystic bulbar hemangioblastoma

Introducción. Los hemangioblastomas son neoplasias de naturaleza vascular y de características benignas. Representan entre el 2 y el 3% de los tumores cerebrales, y entre el 7 y el 12% de los procesos neoformativos localizados en la fosa posterior. La primera descripción de esta enfermedad se remonta al año 1904, cuando von Hippel hizo pública la primera descripción del hemangioma retiniano. Caso clínico. Varón de 41 años que acudió a su médico, tras presentar durante tres semanas episodios intermitentes de hipo, autolimitados y de duración variable, en ocasiones relacionados con la ingesta y en otras aparecían de forma espontánea. En la exploración neurológica llamaba la atención la presencia de hipoestesia toracoabdominal izquierda. Mediante técnicas de neuroimagen se diagnosticó una lesión tumoral, bien delimitada, quística, de localización bulbar. El abordaje quirúrgico se realizó mediante craniectomía suboccipital, y se llevó a cabo una extirpación completa de la lesión. El estudio anatomopatológico confirmó el diagnóstico de hemangioblastoma. El paciente evolucionó satisfactoriamente, sin presentar nueva clínica neurológica. Conclusión. La localización más frecuente de los hemangioblastomas es en el vérmix y los hemisferios cerebelosos, siendo infrecuente la localización bulbar, que representa un porcentaje inferior al 5% de los hemangioblastomas cerebrales, así como la forma de presentación clínica mediante hipo persistente

Introduction. Hemangioblastomas are neoplasm of vascular type having benign characteristics. They representbetween 2-3% of brain tumors and 7-12% of neoformative processes in the posterior fossa. The first description of the disease goes back to the year 1904, when Eugene von Hippel made the description of retinal haemangioblastoma. Case report. A male patient of 41 years-old who went to his doctor after three weeks of having intermittent episodes of hiccups. In the neurological examination the presence of left thoracic-abdominal hypoesthesia was shown. Brain tumor was diagnosed by neuroimagetechniques. It was well defined, cystic and placed in bulbar region. Surgical approach was carried out by means of suboccipital craniectomy, with the complete removal of the lesion. The histological study confirmed the hemangioblastoma diagnosis. The patient evolved satisfactory, without presenting new neurological symptoms. Conclusion. The most frecuent localization of hemangioblastomas is in vermix and cerebellum hemispheres. The bulbar localization is infrequent (whichrepresents less percentage than 5% of cerebral hemangioblastomas) likewise the clinical manifestation though hiccups

[Cystic bulbar hemangioblastoma]. / Hemangioblastoma quístico de bulbo raquídeo.

INTRODUCTION: Hemangioblastomas are neoplasm of vascular type having benign characteristics. They represent between 2-3% of brain tumors and 7-12% of neoformative processes in the posterior fossa. The first description of the disease goes back to the year 1904, when Eugene von Hippel made the description of retinal haemangioblastoma. CASE REPORT: A male patient of 41 years-old who went to his doctor after three weeks of having intermittent episodes of hiccups. In the neurological examination the presence of left thoracic-abdominal hypoesthesia was shown. Brain tumor was diagnosed by neuroimage techniques. It was well defined, cystic and placed in bulbar region. Surgical approach was carried out by means of suboccipital craniectomy, with the complete removal of the lesion. The histological study confirmed the hemangioblastoma diagnosis. The patient evolved satisfactory, without presenting new neurological symptoms. CONCLUSION: The most frecuent localization of hemangioblastomas is in vermix and cerebellum hemispheres. The bulbar localization is infrequent (which represents less percentage than 5% of cerebral hemangioblastomas) likewise the clinical manifestation though hiccups.

[Anterior craniofacial resection: oncologic outcome and complications in a series of 111 cases]. / Resección craneofacial anterior: resultados oncológicos y complicaciones en una serie de 111 casos.

INTRODUCTION: Anterior craniofacial resection is a standardized procedure for the treatment of ethmoid and frontal orbital tumors with intracranial invasion. METHODS: A retrospective review of 111 patients with sinonasal tumors involving the anterior skull base who underwent combined craniofacial surgery. RESULTS: The most frequent pathological entity was adenocarcinoma (54 cases) and other epithelial tumors (29 cases). Five year actuarial survival according to the Kaplan-Meier method was 40%. Survival was affected by the histology of the tumor, brain involvement, and deep soft tissue involvement of the orbit. The UICC staging system did not show statistical prognostic significance. Complications occurred in 39 (35.1%) patients, resulting in 4 (3.6%) postoperative deaths. Major complications included cerebrospinal fluid leak in 18 patients, meningitis in 10, infection in 9, stroke in 4, and pneumocephalus in 4. The extent of the craniofacial resection was the most important factor associated with major complications. CONCLUSIONS: Despite the advanced stage of most of the patients, anterior craniofacial resection succeeded in terms of an acceptable survival rate. Nevertheless, significant complications were observed although in most patients were not life-threatening and had no negative impact on the quality of life.

Resección craneofacial anterior: resultados oncológicos y complicaciones en una serie de 111 casos / Anterior craniofacial resection: oncologic outcome and complications in a series of 111 cases

Introducción: La resección craneofacial anterior es un procedimiento bien establecido para el tratamiento de los tumores etmoidales y fronto-orbitarios con invasión intracraneal. Material y métodos: Se realizó una revisión retrospectiva de 111 pacientes con tumores nasosinusales con invasión intracraneal sometidos a una resección craneofacial. Resultados: El tipo histológico más común fue el adenocarcinoma (54 casos) y otros tumores epiteliales (29 casos). La supervivencia actuarial a los 5 años fue del 40 por ciento. La supervivencia se afectó negativamente por la histología del tumor, invasión del cerebro y la invasión profunda de la órbita. El estadio tumoral no influyó de forma significativa en las expectativas de supervivencia. Se presentaron complicaciones en 39 (35,1 por ciento) pacientes, resultando en 4 (3,6 por ciento) muertes postoperatorias. Las complicaciones más importantes incluyeron fístula cerebroespinal en 18 pacientes, meningitis en 10, infección en 9, infarto cerebral en 4 y neumocéfalo en 4. La extensión de la resección fue el factor más importante asociado a complicaciones. Conclusiones: A pesar del estadio avanzado de la mayor parte de los pacientes, la resección craneofacial anterior consiguió unas aceptables tasas de supervivencia. Sin embargo, se observaron complicaciones importantes, aunque en la mayoría de los casos no pusieron en peligro la vida de los pacientes ni tuvieron un impacto negativo en la calidad de vida (AU)

INTRODUCTION: Anterior craniofacial resection is a standardized procedure for the treatment of ethmoid and frontal orbital tumors with intracranial invasion. METHODS: A retrospective review of 111 patients with sinonasal tumors involving the anterior skull base who underwent combined craniofacial surgery. RESULTS: The most frequent pathological entity was adenocarcinoma (54 cases) and other epithelial tumors (29 cases). Five year actuarial survival according to the Kaplan-Meier method was 40%. Survival was affected by the histology of the tumor, brain involvement, and deep soft tissue involvement of the orbit. The UICC staging system did not show statistical prognostic significance. Complications occurred in 39 (35.1%) patients, resulting in 4 (3.6%) postoperative deaths. Major complications included cerebrospinal fluid leak in 18 patients, meningitis in 10, infection in 9, stroke in 4, and pneumocephalus in 4. The extent of the craniofacial resection was the most important factor associated with major complications. CONCLUSIONS: Despite the advanced stage of most of the patients, anterior craniofacial resection succeeded in terms of an acceptable survival rate. Nevertheless, significant complications were observed although in most patients were not life-threatening and had no negative impact on the quality of life (AU)

Infratemporal approaches to nasopharyngeal tumors.

Twenty patients with neoplasms originating in the nasopharynx were operated using the infratemporal fossa approach with facial translocation (15 cases), the subtemporal-preauricular infratemporal approach (2 cases), and the transmandibular approach (3 cases). A craniectomy was also required in 14 cases. Fifteen tumors were malignant, while 5 were juvenile angiofibromas with infratemporal and intracranial extensions. Most of the lesions were large and involved multiple areas of the skull base. Tumor excision was total in all but 3 patients. Local flaps were utilized in all patients to seal the operative cavity and consisted of temporalis muscle flaps. The most frequent postoperative complications were wound infections and cerebrospinal leaks. Two patients died as a result of postoperative complications. To date, 1 patient has died from disease and 3 are alive with local or distant disease.

Aggressive fibromatosis of the sphenoid.

A fibromatosis located primarily in the sphenoid, with secondary invasion of the maxilla, orbit, and subtemporal region, was extirpated by means of a combined craniofacial approach.