Síndrome de Millard-Gubler secundario a angioma cavernoso: reporte de un caso / Millard-Gubler syndrome secondary to cavernous angioma: report of a case

RESUMEN El síndrome de Millard-Gubler es considerado un síndrome protuberancial debido a una lesión pontina inferior y caracterizado, desde el punto de vista clínico, por parálisis facial y del VI par, ipsilateral a la lesión y parálisis braquiocrural contralateral; frecuentemente es de causa vascular, menos frecuente de causa traumática o por efecto de masa secundario a un tumor. Se realiza reporte de un caso de un paciente de 45 años de edad que acude al servicio hospitalario de la Fundación Centro Colombiano y Enfermedades Neurológicas FIRE con cuadro clínico caracterizado en pérdida de la fuerza muscular en hemicuerpo izquierdo, y déficit motor de la hemicara derecha, disartria y deterioro del estado de consciencia. La TAC de cráneo simple al ingreso evidenció sangrado extenso del tallo cerebral. De acuerdo con los hallazgos clínicos se concluye que el paciente presentó un síndrome de Millard-Gubler.

SUMMARY The Millard Gubler syndrome is considered a protuberant syndrome due to a lower pontine lesion and characterized, from the clinical point of view, by facial paralysis and VI pares, ipsilateral to the lesion and contralateral brachiocrural palsy; frequently it is of vascular cause, less frequent of traumatic cause or by effect of mass secondary to a tumor. A case report is presented of a 45-year-old patient who attends the hospital service of the Fundación Centro Colombiano y Neurológicas FIRE with a clinical picture characterized by loss of muscle strength in the left side of the body, and motor deficit of the right side of the face, dysarthria and deterioration of the state of consciousness. The simple skull CT on admission showed extensive brainstem bleeding. According to the clinical findings, it is concluded that the patient presented a Millard Gubler syndrome.

Surgical Treatment of Cavernous Angiomas in the Pineal Gland ­ Case Report

Pineal cavernous angioma is a vascular malformation that has a prevalence lower than 1%. The etiology is debated. It is believed that it is originated from an autosomal dominant inheritance or from radiotherapeutic treatment. Complete resection enables the cure and prevents complications of the natural evolution of the lesion, mainly hemorrhagic events. A female patient, with 67 years of age, presented mental confusion and visual acuity deficit, which evolved to periods of psychomotor agitation. A magnetic resonance imaging (MRI) scan of the head showed a lobulated mass lesion in the pineal region with hypersignal on T1 and hyposignal on the susceptibility weighted imaging (SWI) sequence. Hydrocephalus was also noticed. The patient underwent a microsurgery for complete lesion resection though a supracerebellar/ supratentorial access. The anatomopathology revealed an arteriovenous malformation compatible with cavernous angioma. The pineal cavernous angioma is a rare malformation that should be considered in the differential diagnosis of expansive lesions of the pineal gland.

Cavernomas de tronco encefálico: presentación de 8 casos operados, análisis y revisión del estado del arte / Brainstem cavernous malformations: a series of 8 surgicalcases, analysis and state of the art review

Introducción: Las lesiones del tronco cerebral son uno de los mayores desafíos neuroquirúrgicos. Los angiomas cavernosos de esta localización son lesiones de comportamiento agresivo, con alta morbi-mortalidad asociada, donde el neurocirujano tiene la posibilidad de curar al paciente pero con un nivel de riesgo que hacen que la oportunidad de la cirugía se mantenga en debate. Material y Método: Análisis retrospectivo de 8 casos de cavernomas de tronco operados entre los años 2009-2013 con sus características clínicas, quirúrgicas y de seguimiento. Se realiza además una revisión reflexiva sobre la evolución del manejo de estas lesiones y del estado del arte a nivel en el concierto internacional. Resultados: Los 8 casos presentaron evoluciones inmediatas y mediatas favorables tras la cirugía. No hubo mortalidad ni empeoramiento del status neurológico en relación al estado preoperatorio en ninguno de los 8 casos. Tres de los pacientes de la serie fueron operados tras caer en una condición clínica crítica y son los que registran los mayores déficits durante el seguimiento. El análisis de la evolución del manejo de este tipo de lesiones a nivel mundial revela una tendencia hacia el manejo quirúrgico precoz en lesiones sintomáticas. Conclusiones: Los resultados de esta serie así como la evolución del estado del arte permiten concluir que en pacientes sintomáticos una cirugía precoz ofrece en general mejores expectativas que intervenciones tardías para lesiones relativamente superficiales.

Introduction: Brainstem lesions are a major neurosurgical challenge. Cavernous angiomas of this location are lesions of aggressive behavior, with high morbidity and mortality associated, where the neurosurgeon has the possibility to cure the patient but with a level of risk that makes that the surgery timing remains under debate. Material and Methods: A retrospective analysis of 8 brainstem cavernomas cases operated in the period 2009-2013 is presented whit its clinical, surgical and follow-up characteristics. A thoughtful review of the evolution of the management of these lesions and the state of art in the international level is also carried out. Results: All 8 patients presented immediate and mediate favorable changes after surgery. There was no mortality or worsening of the neurological condition in relation to the preoperative status in any of the 8 cases. 3 patients in this series underwent surgery after falling in a critical condition and are those who have the highest deficit in the follow-up. The analysis of the evolution of the worldwide management of these injuries reveals a trend towards early surgical management in symptomatic lesions. Conclusions: The results of this series as well as the evolution of the state of the art let us conclude that in symptomatic patients an early surgery offers overall better expectations than later interventions in relatively superficial lesions.

Cavernomatosis cerebral múltiple: primer reporte de caso en Perú / Multiple cerebral cavernous: first case report in Peru

La cavernomatosis cerebral (CC), única o múltiple, es la presencia de conductos vasculares distendidos, angiográficamente ocultos, por lo cual clásicamente eran hallazgos incidentales intraoperatorios o en autopsias. Actualmente la resonancia magnética (RM) cerebral, permite identificar éstas lesiones en un mayor número de pacientes. Las manifestaciones clínicas son hemorragias intracerebrales, crisis epilépticas y cefalea. Se reporta el caso de un varón de 55 años, sin antecedentes de importancia que ingresó por presentar cefalea y crisis de arresto del habla. El examen físico fue normal. Estudios tomográficos previos mostraron una lesión hemorrágica única, al realizarse una RM cerebral con protocolo T2-eco gradiente se evidencia múltiples imágenes compatibles con CC múltiple. Es el primer reporte de CC múltiple, entidad poco diagnosticada, que se debe sospechar en pacientes sin antecedentes de riesgo vascular que debutan con hemorragias intracerebrales, crisis epilépticas y/o cefaleas; constituyendo la RM cerebral en protocolo T2-eco gradiente, el estudio indicado para el diagnóstico.

Cerebral cavernomatosis (CC), single or multiple, is the presence of distended vascular channels, angiographically occult, for that, they are classically incidental intraoperative findings at autopsy. Currently the magnetic resonance imaging (MRI) let us identify these lesions in a larger number of patients. Clinical manifestations are intracerebral hemorrhages, seizures and headache. A male, 55, no history of significance is reported; he was admitted for headache and some months later presents speech arrest crisis. On physical examination, nothing unusual. Previous tomographic studies showed a hemorrhagic lesion, when the brain MRI with T2-eco gradient protocol was performed, multiple images observed, were compatible with multiple CC. This is the first report of multiple CC, unfrequent entity that should be suspected in patients with no history of vascular risk who present intracerebral hemorrhage, seizure and / or headache; being the brain MRI, the study indicated for diagnosis.

Investigation of thunderclap headache in cavernous angioma: when magnetic resonance makes the difference. Case report / Investigação de cefaleia em trovoada em angioma cavernoso: quando a ressonância magnética faz a diferença. Relato de caso

BACKGROUND AND OBJECTIVES: Brain magnetic resonance is a major exam to evaluate thunderclap headache, after excluding subarachnoid hemorrhage. This study aimed at reporting a case of brainstem cavernous angioma (cavernoma) where clinical presentation and computerized tomography have suggested intraventricular hemorrhage. CASE REPORT: Female patient, 55 years, was referred to the hospital with a history of new headache 10 days ago. Pain onset was sudden, pressure-type, severe, located in the occipital region with irradiation throughout the head, followed by severe photophobia, nauseas, diplopia and blurred vision. Neurological evaluation has revealed anisocoria, complete ophthalmoplegia and right eyelid ptosis. Cranial CT has shown blood in the third ventricle. Conventional brain arteriography has not shown aneurysm, arteriovenous malformation or venous sinus thrombosis. At lumbar puncture, an opening water pressure of 45cm was found and liquor analysis was normal. Brain resonance has shown oval lesion (1.0x1.0x0.6cm) of exophytic aspect in the interpeduncular cistern and third ventricle, compatible with brainstem cavernoma. CONCLUSION: In this case, magnetic resonance was essential for the diagnosis, since routine exams (brain tomography, liquor puncture and arteriography) could not define it. Further studies are needed to explain how magnetic resonance impacts investigation. .

JUSTIFICATIVA E OBJETIVOS: A ressonância magnética cerebral é um exame importante na investigação da cefaleia em trovoada, após a exclusão de hemorragia subaracnoidea. O objetivo deste estudo foi relatar um caso de angioma cavernoso (cavernoma) no tronco cerebral, em que a apresentação clínica e tomografia computadorizada sugeriram uma hemorragia intraventricular. RELATO DO CASO: Paciente do gênero feminino, 55 anos, foi encaminhada ao hospital com uma história de cefaleia nova há 10 dias. A dor teve início súbito, do tipo pressão, de forte intensidade, localizada na região occipital com irradiação para toda a cabeça, acompanhada de fotofobia intensa, náuseas, diplopia e visão turva. O exame neurológico revelou anisocoria, oftalmoplegia completa e ptose palpebral à direita. Tomografia computadorizada de crânio mostrou sangue no terceiro ventrículo. Arteriografia cerebral convencional não apresentou aneurisma, malformação arteriovenosa ou trombose de seios venosos. Na punção lombar, uma pressão de 45cm de água de abertura foi encontrada e a análise do líquido cefalorraquidiano foi normal. A ressonância de crânio revelou lesão oval (1,0x1,0x0,6cm) de aspecto exofítica na cisterna interpeduncular e terceiro ventrículo compatível com cavernoma de tronco cerebral. CONCLUSÃO: No caso descrito, a ressonância magnética foi essencial para o diagnóstico, uma vez que os exames de rotina (tomografia de crânio, punção de líquor e arteriografia) não conseguiram defini-lo. Maiores estudos são necessários para esclarecer como a realização de ressonância magnética impacta a investigação. .

Two Cystic Cavernous Angiomas after Radiotherapy for Atypical Meningioma in Adult Woman : Case Report and Literature Review

A correlation between radiation therapy and cavernoma has been suspected since 1994. Since then, only a few cases of radio-induced cavernomas have been reported in the literature (85 patients). Most of them were children, and the most frequent original tumour had been medulloblastoma. The authors report a case of two cystic cavernous angiomas after radiation therapy for atypical meningioma in adult woman. This is the first case of cavernous angioma after radiotherapy for low grade meningioma. A 39-year-old, Latin american woman was operated on for a frontal atypical meningioma with intradiploic component and adjuvant radiotherapy was delivered (6000 cGy local brain irradiation, fractionated over 6 weeks). Follow-up MR imaging showed no recurrences of the tumour and no other lesions. Ten years later, at the age of 49, she consulted for progressive drug-resistant headache. MR imaging revealed two new well defined areas of different signal intensity at the surface of each frontal pole. Both lesions were surgically removed; the histopathological diagnosis was cavernous angioma. This is the first case of cavernous angioma after radiation therapy for atypical meningioma : it confirms the development of these lesions after standard radiation therapy also in patients previously affected by non-malignant tumours.

Therapeutic effect of gamma knife on intracranial cavernous angioma / 中南大学学报(医学版)

Objective: To evaluate the therapeutic effect of gamma knife on patients with intracranial cavernous angioma (CA). Methods: The medical records of 122 patients (134 lesions) who underwent radiosurgery were reviewed retrospectively. Results: hT e average follow-up period was 43 months. No patient died. One patient underwent CA resection. In patients with epilepsy, 83% patients showed alleviation of seizures. About 44% of the lesions shrank in size after treatment with gamma knife radiosurgery (59/134). Seven cases had hemorrhage again after radiosurgery, and the overall annual hemorrhage rate was 1.6%. Edema was found in 11.5% patients (14/122) and all patients showed improvement atf er treatment. Conclusion: Gamma knife is a safe treatment for CA, which could obviously improve the symptoms of epilepsy. Gamma knife radiosurgery is the if rst option for the treatment of cavernous sinus angiomas.

Angioma cavernoso espinal extradural dorsal / Spinal extradural cavernous angioma

Se presenta el caso clínico de un paciente de 57 años de edad, de la raza blanca, con cuadro clínico de compresión medular dorsal, por lo cual se le realizó una laminectomía de D5-D7, con resección de una lesión extradural, de color vino, redondeada, de 3x2x2 cm. Los resultados de la biopsia confirmaron el diagnóstico de una malformación angiomatosa (cavernoma). La cirugía es el tratamiento ideal para estos tipos de lesiones y la resonancia magnética de columna, un estudio útil para diagnosticar y tratar a los afectados.

The clinical case of a white 57 year-old patient is presented, with clinical pattern of dorsal medullary compression, reason why a laminectomy of D5-D7 was carried out, with resection of an extradural, rounded, wine colored lesion, of 3x2x2 cm. The results of the biopsy confirmed the diagnosis of an angiomatous malformation (cavernoma). Surgery is the ideal treatment for these types of lesions and the spine magnetic resonance is an useful study to diagnose and to treat those affected.

Optochiasmatic Cavernous Angioma with Rapid Progression after Biopsy Despite Radiation Therapy

We present a rare case of optochiasmatic cavernous angioma (CA) that progressed despite radiation therapy. A 31-year-old female patient presented with sudden loss of left visual acuity and right homonymous hemianopsia. Magnetic resonance imaging (MRI) revealed a suprasellar mass and findings compatible with a craniopharyngioma or an optic glioma with bleeding. An open biopsy was conducted using the transcranial approach, and histological examination revealed gliosis. During the one-year follow-up period, imaging suggested intratumoral bleeding and the mass continued to grow. We recommended re-operation, but the patient refused due to fear of surgery. Consequently, the patient received fractionated radiation therapy (3,000 cGy) to the parasellar area. Despite the radiotherapy, the mass continued to grow for the following 6 years. The final MRI before definitive treatment revealed a multilobulated, multistage hematoma with calcification in the parasellar area, extending into the third ventricle and midbrain. The patient ultimately underwent reoperation due to the growth of the tumor. The mass was completely removed with transcranial surgery, and the pathologic findings indicated a cavernous angioma (CA) without evidence of glioma. As shown in our case, patients may suffer intratumoral hemorrhage after biopsy and radiotherapy. This case places the value of biopsy and radiotherapy for a remnant lesion into question. It also shows that reaching the correct diagnosis is critical, and complete surgical removal is the treatment of choice.

Spinal Intradural Extramedullary Cavernoma Presenting with Intracranial Superficial Hemosiderosis

A case of intradural extramedullary cavernous angioma is presented with headache, dizziness, and bilateral sensorineural hearing loss caused by an intracranial superficial hemosiderosis. It was incidentally found in a patient with a 3-month history of sustained headache, dizziness and a 3-year history of hearing difficulty. The neurological examination was unremarkable in the lower extremity. MR images showed an intracranial superficial hemosiderosis mostly in the cerebellar region. Myelography and MR images of the thoracolumbar spine revealed an intradural extramedullary mass, which was pathologically proven to be a cavernous angioma. T12 total laminoplastic laminotomy and total tumor removal were performed without any neurologic deficits. The patient's symptoms, including headache and dizziness, have been absent for three years. Intradural extramedullary cavernous angioma can present with an intracranial superficial hemosiderosis as a result of chronic subarachnoid hemorrhage.

Intra-Root Cavernous Angioma of the Cauda Equina : A Case Report and Review of the Literature

Authors experienced intra-root cavernous angioma which is very rare case among cavernous angiomas of cauda equina. Our intra-root cavernous angioma was confirmed by findings from operating field and microscopic examination. We report this case with review of the literature.

Medullary Hemorrhage after Ischemic Wallenberg's Syndrome in a Patient with Cavernous Angioma

BACKGROUND: The main complication of cerebral cavernous angioma is hemorrhage. Ischemic stroke as a complication of cerebral cavernous angioma has rarely been described, and hemorrhage after ischemic Wallenberg's syndrome has not been reported before. CASE REPORT: A 45-year-old woman presented with perioral numbness, hoarseness, dysphagia, and worsening of her previous sensory symptoms. The patient had been taking aspirin for 3 years after suffering from ischemic Wallenberg's syndrome with left paresthesia as a residual symptom. Brain computed tomography revealed an acute medullary hematoma in the previously infarcted area. Follow-up magnetic resonance imaging revealed a cavernous angioma in the right medulla. CONCLUSIONS: We presume that cerebral cavernous angioma was responsible for both the ischemia and the hemorrhage, and we also cautiously speculate that the aspirin contributed to the development of hemorrhage in the previously infarcted area.

Development of De Novo Cavernous Hemangioma after Radiosurgery for Cavernous Hemangioma

We report a rare case of cavernous hemangioma (CH) which developed in adjacent location to a preexisting CH after gamma knife radiosurgery (GKRS). A 36-year-old woman underwent GKRS for a CH in the left lentiform nucleus. Three-and-half years after radiosurgery, MRI revealed a new CH in the left caudate nucleus. Surgical excision of the new lesion was performed. The pathological examination confirmed the diagnosis of CH. In radiosurgery for CH, it should be noted that a new CH may develop, which is likely to result from the interaction between radiation and predisposing factors of the patient.

Cavernomas pontinos extraventriculares. Parte I. Acesso transtentorial a cavernoma pontino dorsolateral / Extraventricular pontine cavernomas. Part I. Transtentorial route to remove a postero-lateral pontine cavernoma

Objetivo: Descrever a via transtentorial, por meio de craniotomia têmporo-occipital, para remoção de cavernoma pontino de localização dorsolateral, exemplificado pela apresentação de um caso. Conclusão: O acesso transtentorial demonstra-se útil e seguro para remoção de lesões localizadas nessa região da ponte.

Objective: To present the transtentorial route through a temporal-occipital craniotomy for surgical removal of a posterior-lateral pontine cavernomas, exemplified by the presentation of a case. Regional anatomy is discussed. Conclusion: The transtentorial approach may be used safely to remove lesions of highly located in the posterior-lateral region of the pons.

Cavernomas pontinos extraventriculares. Parte II. Acesso pré-sigmoideo transpetroso a cavernoma pontino ventrolateral / Extraventricular pontine cavernomas. Part II. Pre-sigmoid, transpetrosal approach to ventrolateral pontine cavernomas

Objetivo: Descrever a via pré-sigmoidea ampliada para acesso a lesões localizadas na região ventrolateral da ponte, exemplificado com a apresentação de um caso de cavernoma pontino nessa localização. Conclusão: Esse acesso, amplamente revisto pela literatura, é útil para remoção cirúrgica de cavernomas pontinos ventrolaterais.

Objective: To describe the pre-sigmoid transpetrosal approach to the ventrolateral pontine region, exemplified by the presentation of a case of cavernoma in this localization. Conclusion: This approach is useful to remove pontine ventrolateral cavernomas.

An Unusual Case of a Thrombosed Giant Distal PICA Aneurysm Simulating a Large Cavernous Angioma

A 64-year-old woman was referred to our hospital with a one-month history of progressive headache. Magnetic resonance imaging (MRI) showed a hemorrhagic mass adjacent to the left inferior cerebellar hemisphere associated with a peripheral rim of signal void. Angiography demonstrated an avascular mass and the provisional diagnosis was a large cavernous angioma in the cerebellum. Intraoperative findings revealed a thrombosed giant aneurysm of the left distal posterior inferior cerebellar artery (PICA). We report an unusual case of a completely thrombosed giant aneurysm simulating a large cavernous angioma in the cerebellum. The cerebellar cisternal location of the mass may be a clue for the pre-operative diagnosis of an aneurysm.

Gamma Knife Radiosurgery of The Brain Stem Cavernous Angioma / 대한뇌혈관외과학회지

From December 2001 to December 2007, twenty one patients with symptomatic brainstem cavernous angioma were treated using the Leksell gamma knife. The median marginal dose was 15 Gy (range 13~18 Gy) at 50% isodose (range 45~80%). Mean follow up period was 33 months (range 4~67 months). Symptomatic improvement was achieved in 14 (66.6%) patients. There were 5 (23.8%) patients with neurological deterioration during follow-up period. Among them one patient aggravated due to overt bleeding and radiation effect was thought be the cause of deterioration in the others. Two patients needed surgical treatment subsequently and the other three patients improved with conservative management. Digital volumetry was performed before and after GKS with mean interval of 17 months (range 5~51 months). Mean volume of the lesions was 1155.6 mm3 (range 130.7~3800 mm3) before GKS and 724.8mm3 (range 36.8~1322 mm3) at the last follow-up. Volume of the lesion decreased in 17 patients (81.0%). GKS is an effective treatment modality for brainstem cavernous angioma in surgically unfavorable location. However, neurological deterioration after GKS was not rare (23%), particularly within 1 yr after GKS. It is necessary to be cautious in dose prescription.

Spinal Epidural Cavernous Angioma: A Case Report

Spinal extradural cavernous angiomas(CAs) are apparently rare lesions. The author report a 67 year old man with an epidural CA at T9-10 space presenting with progressive leg weakness. Magnetic resonance imaging showed a thoracic epidural lesion appearing as isointense on T1-weighted with strong contrast enhancement and hyperintense on T2-weighted images. A moderate hyperdensity and foraminal widening was found on computed tomography. Hemilaminectomy was performed to relieve the spinal cord compression. A highly vascular tumor without bony involvement was observed in the epidural space. Histopathology of the lesion revealed a typical CA. The patient's symptoms resolved after treatment. Clinical aspect, radiological features, and surgical treatment of this rare entity will be reviewed.

Cavernous Angioma in the Falx Cerebri: A Case Report

Intracranial cavernous angiomas are benign vascular malformations and can be divided into intra-axial and extra-axial lesions. Extra-axial cavernous angiomas are relatively rare and usually arise in relation to the dura mater and mimick meningiomas. We report a case of cavernous angioma that occured in the falx cerebri of a 22-yr-old female patient with the special focus on neuroradiologic findings. This is the fourth case of cavernous angioma in the falx cerebri reported in the literature to our knowledge.

Isolated Abducens Nerve Palsy Caused by De Novo Pontine Cavernous Angioma

Cavernous angiomas are considered to be congenital in origin. Patients under age of 14 years usually does not require imaging because they are likely to have a benign abducens nerve palsy, unless they develop additional signs or symptoms of neurologic disease during observation. Here we report a case of an isolated abducens nerve palsy caused by overt hemorrhage from de novo formation of cavernous angioma in the pons. Cavernous angiomas of the brain stem should be considered as a possible cause of isolated abducens nerve palsy in young adult and MRI, including gradient-echo sequences should be performed.