Síndrome de Tolosa-Hunt una extraña causa de oftalmoplejía dolorosa: reporte de caso / Tolosa-Hunt syndrome a strange cause of painful ophthalmoplegia: case report

RESUMEN INTRODUCCIÓN: El síndrome de Tolosa-Hunt (STH) se caracteriza por una oftalmoplejía dolorosa, de etiología desconocida. De acuerdo con los hallazgos histopatológicos, se ha descrito la formación de un tejido granulomatoso en los senos cavernosos. PRESENTACIÓN DEL CASO: Una mujer de 22 años con cuadro clínico de 3 semanas de evolución caracterizado por cefalea hemicránea derecha, dolor ocular derecho y diplopía. Su examen físico evidenció la existencia de una oftalmoplejía derecha; la resonancia magnética (RM) de silla turca demostró engrosamiento y realce en la región del seno cavernoso derecho. Se presenta el caso clínico de una causa inusual de oftalmoplejía dolorosa. DISCUSIÓN: La oftalmoplejía dolorosa tiene múltiples diagnósticos diferenciales que incluyen causas neoplá-sicas, vasculares, inflamatorias e infecciosas que pueden afectar el seno cavernoso o la fisura orbitaria superior. El STH, que es una causa rara de oftalmoplejía dolorosa, sigue siendo un diagnóstico de exclusión. Por otra parte, se caracteriza por tener una adecuada respuesta al tratamiento con glucocorticoides. CONCLUSIÓN: La negatividad en las investigaciones de las etiologías de oftalmoplejía, los hallazgos imagenológicos en la RM y la adecuada respuesta cínica con el uso de los corticoides permiten confirmar el diagnóstico. No debería ser necesaria la biopsia del seno cavernoso ante la sospecha de STH con adecuada respuesta al manejo corticoide.

ABSTRACT INTRODUCTION: Tolosa-Hunt syndrome (THS) is characterized by painful ophthalmoplegia of unknown etiology, the formation of a granulomatous tissue in the cavernous sinuses has been described in histopatho-logical findings. CASE PRESENTATION: A 22-year-old woman presenting with 3 weeks of right sided headache, right eye pain and diplopia. Physical examination revealed the existence of a right ophthalmoplegia; magnetic resonance imaging (MRI) of the sella turcica showed thickening and enhancement of the right cavernous sinus. A clinical case of an unusual cause of painful ophthalmoplegia is presented. DISCUSSION: Painful ophthalmoplegia has multiple differential diagnoses that include neoplastic, vascular, inflammatory and infectious causes that can affect the cavernous sinus or the superior orbital fissure. STH is a rare case of painful ophthalmoplegia that continues to be a diagnosis of exclusion characterized by an adequate response to treatment with glucocorticoids. CONCLUSION: The negativity of the investigations for the causes of ophthalmoplegia, the imaging findings in the MRI and the adequate response to corticosteroids allows the diagnosis to be made. Biopsy should not be necessary when THS is suspected and there is an adequate response to corticosteroid management.

Trombose séptica do seio cavernoso associada à infecção odontogênica: relato de caso / Septic Cavernous Sinus Thrombosis Associated to Odontogenic Infection: Case Report / Trombosis séptica del seno cavernoso asociada a infección odontogénica: reporte de un caso

Introdução: A Trombose Séptica do Seio Cavernoso é uma condição rara, de difícil diagnóstico e seu tratamento deve ser incisivo e assertivo. Mais frequentemente a etiologia da trombose é a extensão de processos infecciosos no terço médio da face, como sinusites dos seios paranasais. Objetivo: Esse trabalho tem como objetivo apresentar um relato de caso clínico de um paciente de 26 anos acometido por trombose séptica do seio cavernoso odontogênica. Relato de caso: O paciente foi submetido a duas drenagens cirúrgicas dos sítios infectados, assim como remoção das causas (dois molares superiores), seguidas de antibioticoretapia endovenosa e controles imaginológico e laboratorial. Conclusão: O diagnóstico precoce e etiologicamente correto seguido de um tratamento clínico e cirúrgico emergente e incisivo são fundamentais na resolução favorável da trombose séptica do seio cavernoso e na diminuição de suas sequelas... (AU)

Introduction: Septic Cavernous Sinus Thrombosis is a rare condition, hard to diagnose and its treatment must be incisive and assertive. More often the etiology of thrombosis is the extension of infectious processes in the middle third of the face, such as sinusitis of the paranasal sinuses. Objectives: This paper aims to present a case report of a 26-year-old patient with odontogenic Cavernous Sinus Septic Thrombosis. Case Report: The patient underwent two surgical drainage of the infected sites, as well as removal of the causes (two maxillary molars), followed by intravenous antibiotic therapy and imaging and laboratory controls. Conclusion: Early and etiologically correct diagnosis followed by an emergent and incisive clinical and surgical treatment are fundamental in the favorable resolution of septic cavernous sinus thrombosis and in the reduction of its sequelae... (AU)

Introducción: La Trombosis del Seno Cavernoso Séptico es una condición rara, difícil de diagnosticar y su tratamiento debe ser incisivo y asertivo. Más a menudo, la etiología de la trombosis es la extensión de procesos infecciosos en el tercio medio de la cara, como la sinusitis de los senos paranasales. Objetivos: El presente trabajo tiene como objetivo presentar el reporte de un caso de un paciente de 26 años con Trombosis Séptica del Seno Cavernoso odontogénica. Reporte de caso: El paciente fue sometido a dos drenajes quirúrgicos de los sitios infectados, así como a la extirpación de las causas (dos molares maxilares), seguido de antibioticoterapia endovenosa y controles de imagen y laboratorio. Conclusión: El diagnóstico precoz y etiologicamente correcto seguido de un tratamiento clínico y quirúrgico emergente e incisivo son fundamentales en la resolución favorable de la trombosis del seno cavernoso séptico y en la reducción de sus secuelas... (AU)

A comparative study of the morphology of suboccipital cavernous sinus using magnetic resonance imaging and cast specimens / Un estudio comparativo de la morfología del seno cavernoso suboccipital utilizando imágenes de resonancia magnética y muestras de yeso

SUMMARY: A comparative study of the morphology of suboccipital cavernous sinus (SCS) using MRI and cast specimens was performed. The present retrospective study analysed the craniocervical magnetic resonance venography (MRV) imaging data of 61 patients. Three-dimensional reconstruction was performed using Mimics 19.0. The SCS left-right diameter(d1), distance from the midline (d2), supero-inferior diameter(d3), anteroposterior diameter (d4), distance from posterior diameter to skin (d5), and diameter of the SCS at different parts (d6-d8) were measured. Comparison between MRV images and cast specimens, the SCS, marginal sinus, anterior condylar vein, and vertebral artery venous plexus were symmetrical and could be bilaterally displayed, whereas the presence of extra condylar vein and posterior condylar vein exhibited different types. The adjacency between the SCS and its communicating vessels and changes in its communicating vessels corresponded well with the MRV images and cast specimens. Many types of the presence of left and right lateral condylar and posterior condylar veins were found in the cast specimens, which could be divided into the bilateral presence of posterior condylar and lateral condylar veins, unilateral presence of posterior condylar veins, and unilateral presence of lateral condylar vein. A total of 61 cases analysed using MRV images revealed the bilateral presence of posterior condylar and lateral condylar veins (77.1 %), the unilateral presence of posterior condylar vein (18.0 %), and the unilateral presence of lateral condylar vein (9.8 %), of which the bilateral presence of posterior condylar and lateral condylar veins accounted for the largest proportion. MRV images and cast specimens of the SCS showed its normal morphological structure and adjacency, thus providing accurate and complete Three-dimensional imaging anatomical data of the SCS and its communicating vascular structures. This study enriches the Chinese SCS imaging anatomy data and may be valuable in clinical practice.

RESUMEN: Se realizó un estudio comparativo de la morfología del seno cavernoso suboccipital (SCS) mediante resonancia magnética y muestras de yeso. El presente estudio retrospectivo analizó los datos de imágenes de venografía por resonancia magnética (RNM) craneocervical de 61 pacientes. La reconstrucción tridimensional se realizó con Mimics 19.0. Se midió: el diámetro izquierdo-derecho del SCS (d1), la distancia desde la línea mediana (d2), el diámetro superoinferior (d3), el diámetro anteroposterior (d4), la distancia desde el diámetro posterior hasta la piel (d5) y el diámetro del SCS en diferentes partes (d6-d8). En la comparación entre las imágenes RNM y las muestras de yeso, el SCS, el seno marginal, la vena condilar anterior y el plexo venoso de la arteria vertebral eran simétricos y se observaron bilateralmente, mientras que la presencia de la vena extracondilar y la vena condilar posterior presentaba tipos diferentes. La proximidad del SCS y sus vasos comunicantes y los cambios en sus vasos comunicantes se correspondieron bien con las imágenes de RNM y los especímenes moldeados. Se encontraron muchos tipos de venas condilares laterales y condilares posteriores izquierda y derecha en las muestras de yeso, que podrían dividirse en presencia bilateral de venas condilares posteriores y condilares laterales, presencia unilateral de venas condilares posteriores y presencia unilateral de venas condilares laterales. Un total de 61 casos analizados mediante imágenes MRV revelaron la presencia bilateral de venas condilares posteriores y condilares laterales (77,1 %), la presencia unilateral de venas condilares posteriores (18,0 %) y la presencia unilateral de venas condilares laterales (9,8 %) de los cuales la presencia bilateral de las venas condilar posterior y condilar lateral representó la mayor proporción. Las imágenes de RNM y las muestras de yeso del SCS mostraron su estructura morfológica y adyacencia normales, lo que proporcionó datos anatómicos de imágenes tridimensionales precisos y completos del SCS y sus estructuras vasculares comunicantes. Este estudio enriquece los datos de anatomía de imágenes de SCS chino y puede ser valioso en la práctica clínica.

Síndrome de tolosa hunt: presentación de caso clínico / Tolosa Hunt syndrome: case report

El síndrome de Tolosa-Hunt es una entidad poco frecuente cuya etiopatogenia y mecanismos fi- siopatológicos son controversiales, se caracteri- za por cefalea asociada a parálisis de uno o más nervios craneales, diplopía, estrabismo y ptosis palpebral, ocasionados por el compromiso del seno cavernoso o la fisura orbitaria superior. Su diagnóstico es un reto y se aborda dentro de los diagnósticos diferenciales de las oftalmoplejías dolorosas. Con el objetivo de describir y actua- lizar el conocimiento sobre esta enfermedad se presenta el caso de una paciente de 14 años que acudió a consulta por cefalea intensa, dolor ocu- lar y afección de nervios craneales. Los hallazgos clínicos y la resonancia magnética confirmaron el diagnóstico del síndrome de Tolosa Hunt...(AU)

Bilateral cavernous sinus and left dural sigmoid sinus thrombosis associated with extreme exertion: a case report / Trombose bilateral do seio cavernoso e do seio sigmoide esquerdo associada a exercício extremo: relato de caso

ABSTRACT Septic cavernous sinus thrombosis is a rare but often debilitating and potentially fatal disease. We describe a case of bilateral orbital cellulitis with rapidly progressing cavernous sinus thrombosis and left sigmoidal sinus thrombosis in an immunocompetent 20-year-old military man who had undergone intensive physical training. The patient presented with rapid painful swollen left eye for 2 days. The examination results were gross proptosis with total ophthalmoplegia. He was treated with intravenous antibiotics and corticosteroid. At 1 week, visual acuity improved to 20/20 OU, with a normal intraocular pressure. There was a significant improvement in proptosis. The ocular motility of the right eye was fully restored, with slight residual ophthalmoplegia in the left eye. There was no residual illness or recurrence of illness at 3 months' follow-up.

RESUMO A trombose séptica do seio cavernoso é uma condição rara, mas frequentemente debilitante e potencialmente fatal. Descrevemos um caso de celulite orbital bilateral com progressão rápida para trombose do seio cavernoso e trombose do seio sigmoide esquerdo, em um militar imunocompetente de 20 anos de idade que havia sido submetido a treinamento físico intenso. O paciente apresentou um inchaço rápido e doloroso no olho esquerdo por 2 dias. Os resultados do exame foram proptose macroscópica com oftalmoplegia total. Ele foi tratado com antibióticos intravenosos e costicosteróide. Em 1 semana, a acuidade visual melhorou para 20/20, com pressão intraocular normal. Houve uma melhora significativa na proptose. A motilidade ocular do olho direito foi totalmente restaurada, com leve oftalmoplegia residual no olho esquerdo. Não houve doença residual ou recorrência da doença após três meses de acompanhamento.

Trombosis séptica de seno cavernoso: reporte de caso / Septic thrombosis of cavernosus sinus: case report

La trombosis séptica del seno cavernoso se conoce como una complicación rara y potencialmente mortal de las infecciones en el área de la cabeza y el cuello. Aunque el uso de antibióticos ha mejorado el pronóstico, todavía es conocida por sus altas tasas de mortalidad y morbilidad. Objetivo: Analizar la presencia de la trombosis séptica de seno cavernoso mediante el estudio de un caso único. Metodología: De tipo observacional, cualitativa y de corte transversal, apoyado con sustento bibliográfico. Resultado: Caso de una paciente joven de sexo femenino que presenta una semana después de tener sintomatología de Covid, cefalea holocraneana, oftalmoplejía derecha, disminución de la agudeza visual, dolor, eritema, edema, tumefacción orbitaria, secreción ocular amarillenta supurativa derecha, se le realiza tomografía de cerebro evidenciándose de forma incidental trombosis del seno cavernoso. Conclusiones: Se determinó que la trombosis séptica de seno cavernoso es un diagnóstico de poca frecuencia y rara. Asimismo, la trombosis del seno cavernoso tiene la tasa más alta de mortalidad. En raras ocasiones, la infección del oído medio puede ser una causa de trombosis séptica del seno cavernoso y la respuesta al tratamiento es deficiente(AU)

Cavernous sinus septic thrombosis is a rare and life-threatening complication of infections in the head and neck area. Although the use of antibiotics has improved the prognosis, it still known for its high mortality and morbidity rates. Objective: To analyze the presence of cavernous sinus septic thrombosis by studying a single case. Methodology: Observational, qualitative and cross-sectional, supported by bibliographic support. Result: Case of a young female patient who presented one week after having symptoms of Covid, holocranial headache, right ophthalmoplegia, decreased visual acuity, pain, erythema, edema, orbital swelling, right suppurative yellowish eye discharge, was performed brain tomography, incidentally showing cavernous sinus thrombosis. Conclusions: It was determined that cavernous sinus septic thrombosis is an infrequent and rare diagnosis. In addition, cavernous sinus thrombosis has the highest mortality rate. In rare cases, middle ear infection can be a cause of cavernous sinus septic thrombosis and response to treatment is poor(AU)

Microsurgical and Endoscopic Anatomy of the Cavernous Sinus

Objective To describe the endoscopic and microsurgical anatomy of the cavernous sinus (CS) with focus on the surgical landmarks in microsurgical anatomy. Materials and methods Ten formalin-fixed central skull base specimens (20 CSs) with silicone-injected carotid arteries were examined through an extended endoscopic transsphenoidal approach. Fifteen formalin-fixed heads were dissected to simulate the surgical position in CS approaches. Results Endoscopic access enables identification of the anterior and posterior surgical corridors. Structures within the CS and on its lateral wall could be visualized and studied, but none of the triangular areas relevant to the transcranial microsurgical anatomy were fully visible through the endoscopic approach. Conclusion The endoscopic approach to the CS is an important surgical technique for the treatment of pathological conditions that affect this region. Correlating endoscopic findings with the conventional (transcranial)microsurgical anatomy is a useful way of applying the established knowledge into a more recent operative technique. Endoscope can provide access to the CS and to the structures it harbors.

Direct carotid-cavernous fistula in a Filipino female presenting with simultaneous orbital/ocular, cavernous and cortical symptomatology without history of trauma: A case report

@#Carotid-cavernous fistula (CCF) is a rare and dangerous neurological disorder that arises due to an abnormal communication between the internal carotid artery (ICA) or the external carotid artery (ECA) and their branches and the cavernous sinus. It can either be a direct fistula (high-flow with acute symptoms) most commonly resulting from trauma (70-90%) or an indirect fistula (low-flow with insidious symptoms) secondary to hypertension, atherosclerosis and collagen vascular disorders. The shunting of arterial blood into the venous system leads to venous hypertension causing various clinical manifestations depending on the venous drainage patterns and the shunt flow. Increased anterior, posterior and superior venous drainage results to orbital/ocular, cavernous and cortical symptomatology, respectively. This paper aims to present a case of 58-year old Filipino female with a 2-day history of sudden, severe headache, vomiting and blurring of vision followed by decrease in sensorium and sudden proptosis and chemosis of the left eye. Patient had no co-morbidities, history of trauma, surgeries, facial skin infections or prior febrile illness. The left eye had exophthalmos, subconjunctival hyperemia, scleral edema/chemosis and ocular bruit. Neurologic examination showed a stuporous patient with multiple cranial nerve deficits (impaired direct and consensual pupillary reflex left, complete ptosis left, sluggish corneal reflex left, impaired oculocephalic reflex left), right hemiplegia and meningeal signs. Cranial Computed Tomography (CT) Angiogram revealed an acute parenchymal hemorrhage in the left frontotemporal lobe with subarachnoid component, with engorged left cavernous sinus and dilated left superior ophthalmic vein. Digital Subtraction Angiography (DSA) was done revealing a direct type of left carotid-cavernous fistula with massive ICA shunting to the cavernous sinus, superior ophthalmic vein and inferior petrosal sinus. The clinical and radiographic evidence were consistent with a Direct/Type A CCF. Unique in this case was a patient with no history of trauma presenting with simultaneous orbital/ocular, cavernous and cortical symptomatology – a clinical picture of CCF that has never been documented in any literature nor included in any classification system. The presence of all three symptomatology can be explained by a direct/highflow fistula that resulted to increased anterior, posterior and superior venous drainage as documented in the DSA. In addition, spontaneous intracranial hemorrhage in CCF is exceptionally rare and it is the most daunting symptomatology of this disease. With that, this specific case may pave the way to a new classification scheme and determine its corresponding treatment approach.

Trombosis de seno cavernoso en paciente SARS-COV2 durante el mes de julio/2020 en el Hospital Militar Central. Reporte de caso / Cavernous sinus thrombosis in a SARS-COV2 patient during the month of July/2020 at the Central Military Hospital. case report

Introducción En diciembre del 2019 se evidencio un nuevo coronavirus en la provincia de Hubei, China, denominada COVID-19. Este nuevo coronavirus cobra importancia clínica a nivel internacional por su alta letalidad decretándose pandemia por COVID-19 en marzo del 2020 por la Organización Mundial de la Salud. El COVID-19, causa principalmente síntomas respiratorios, aunque cada vez más se describen manifestaciones neurológicas con más frecuencia. Además, los estados procoagulantes producto de la infección viral posiblemente guardan relación directa con la aparición de complicaciones trombóticas a nivel arteriales y venosas, dependiendo de la severidad del cuadro. Objetivo: Conocer características clínicas de un paciente eventos trombóticos cerebrales asociados a infección por SARS-COV2. Método: Se realizó una revisión biográfica sobre manifestaciones neurológicas asociadas a infección por SARS-COV2 y su relación con la aparición de manifestaciones neurológicas que desencadenen cuadros isquémicos o trombóticos. Resultados: COVID-19 está asociado a compromiso neurológico, esto dependiendo de la severidad del cuadro, estas manifestaciones se han observado mas en pacientes con alto riesgo cardiovascular, hipertensos, diabéticos y dislipidémicos que se han asociado a peor pronóstico. El conocimiento preciso de la incidencia de complicaciones trombóticas en pacientes con COVID-19 es importante para la toma de decisiones con respecto a la intensidad de la tromboprofilaxis.

Etiology, localization of the lesion, and prognosis for patients firstly diagnosed in ophthalmology department with oculomotor nerve palsy / 中南大学学报(医学版)

OBJECTIVES@#Oculomotor nerve palsy is a kind of disease with many causes, showing eye movement disorders, abnormal eyelid position, and/or damage of the pupil. The etiology of oculomotor nerve palsy in different departments is different. The study discussed the etiology, localization of the lesion, and prognosis for oculomotor nerve palsy firstly diagnosed in department of ophthalmology.@*METHODS@#Clinical data of 137 hospitalized patients with oculomotor nerve palsy at the Department of Ophthalmology, the First Medical Center of PLA General Hospital from 2009 to 2018 were retrospectively collected. The etiology and its distribution characteristics in different age groups, the location of the lesion, and the prognosis of patients were analyzed.@*RESULTS@#In 137 patients, the top 3 causes for oculomotor nerve palsy were head trauma (38.69%), cavernous sinus lesions (12.40%), and orbital inflammation (9.49%). Other causes included intracranial aneurysm, the intracranial space-occupying lesion, cerebral vessel diseases, infection, orbital tumors, diabetes, the operation of nasal cavity. Traumatic oculomotor nerve palsy was more common in young adults aged 20-49 years and in the patients with cerebral vascular disease in elderly people aged 60-69 years, while diabetic oculomotor nerve palsy is common in middle-aged and elderly people aged 50-69 years. The age distribution of other etiological types was relatively balanced. Seventy-five cases of orbital apex lesions were due to trauma, inflammation, infection, and tumor; 40 cases of cavernous sinus lesions were due to inflammation, tumor, and thrombosis; 6 cases of subarachnoid lesions were due to aneurysms, tumors, and trauma; 5 cases were oculomotor nucleus lesions were due to infarction; 11 cases could not be allocated because of unknown etiology. After treatment, the corrected visual acuity of oculomotor nerve palsy side was not significantly improved. The patients with oculomotor nerve palsy caused by intracranial aneurysm, cerebrovascular disease, and diabetes mellitus had the highest proportion of partial or complete recovery from ptosis and ocular dyskinesia.@*CONCLUSIONS@#Oculomotor nerve palsy is a common cause of ophthalmoplegia and diplopia. Head trauma, cavernous sinus lesions, and orbital inflammation are the most common causes for oculomotor nerve palsy first diagnosed in ophthalmology department. Traumatic oculomotor nerve palsy is common in adolescents. Oculomotor nerve palsy caused by diabetes and cerebrovascular disease are common in the middle-aged and elderly people. Most of the lesions locate in the orbital apex and cavernous sinus. The prognosis of corrected visual acuity is poor. The prognosis of ptosis and ocular dyskinesia caused by intracranial aneurysm, cerebrovascular disease, and diabetes is good. Figuring out the cause timely and accurately is the basis and key to treat oculomotor nerve palsy.

Meningioma de nervio óptico y seno cavernoso: Reporte de caso / Optic nerve and cavernous sinus meningiomas: case report

Los meningiomas de nervio óptico y de seno cavernoso son patologías poco frecuentes, y hasta el momento no ha habido ningún reporte de que se presenten ambos en un mismo paciente. Cabe resaltar que cuando llega un paciente a consulta diagnosticado con alguna patología, asumimos que este diagnóstico es adecuado y pertinente. Pero en nuestro caso, el paciente presentó signos y síntomas de etiología desconocida que hicieron que se re-evaluarán los diagnósticos oftalmológicos que traía la paciente, encontrando que había sido tratada por un diagnóstico que no le correspondía y a su vez este hallazgo nos ayudó a encontrar la verdadera causa(AU)

Optic nerve and cavernous sinus meningiomas are uncommon pathologies, and so far there have not been previously reported to occur in the same patient. It should be emphasized that when a patient arrives at a doctor's office diagnosed with pathology, we assume that this diagnosis is appropriate and pertinent. But in our case, the patient presented signs and symptoms of unknown etiology that led to a re-evaluation of the previous ophthalmological diagnoses that the patient brought, finding that she had been treated for a diagnosis that did not match with all her clinical sign and symptoms and this helped us to find the real cause(AU)

Síndrome de Tolosa-Hunt, uma oftalmoplegia dolorosa / Tolosa Hunt Syndrome, a painful ophthalmoplegia

Resumo A síndrome de Tolosa-Hunt (STH) é uma doença rara caracterizada por oftalmoplegia dolorosa unilateral de início súbito causada por uma inflamação granulomatosa inespecífica no seio cavernoso ou fissura orbital superior (ou ambos). A oftalmoparesia ocorre quando os nervos cranianos III, IV e VI são acometidos pela inflamação. Disfunções pupilares podem estar presentes e está relacionado com acometimento das fibras simpáticas que passam pelo seio cavernoso na porção da artéria carótida interna ou fibras parassimpáticas ao redor do nervo oculomotor. O acometimento do primeiro ramo do trigêmeo pode provocar parestesia território correspondente à distribuição desde ramo (testa). Raramente, pode haver extensão da inflamação para além do seio cavernoso ou fissura orbital superior podendo acometer também o nervo óptico. Há uma boa resposta com o uso de corticoides e pode haver remissões espontâneas. Recidivas ocorrem em 40% dos casos. A doença é mais comum após a segunda década de vida. Afeta ambos os gêneros de forma igualitária. O presente estudo trata-se de um relato de caso de um paciente que se apresentou com oftalmoplegia dolorosa de início súbito à direita com 4 dias de evolução seguido de amaurose ipslateral após um dia do início da dor.

Abstract Tolosa-Hunt syndrome (STH) is a rare disease characterized by sudden onset unilateral painful ophthalmoplegia caused by non-specific granulomatous inflammation in the cavernous sinus or superior orbital fissure (or both). Ophthalmoparesis occurs when the cranial nerves III, IV and VI are affected by inflammation. Pupillary dysfunctions may be present and is related to involvement of the sympathetic fibers that pass through the cavernous sinus in the portion of the internal carotid artery or parasympathetic fibers around the oculomotor nerve. The involvement of the first branch of the trigeminal can cause paresthesia corresponding to the distribution from the first branch (forehead). Rarely, there may be extension of inflammation beyond the cavernous sinus or superior orbital fissure and may also affect the optic nerve. There is a good response with the use of corticosteroids and there may be spontaneous remissions. Relapses occur in 40% of cases. The disease is most common after the second decade of life. It affects both genders equally. The present study is a case report of a patient who presented with painful ophthalmoplegia of sudden onset on the right with 4 days of evolution followed by ipsilateral amaurosis after one day of onset of pain.

Trombosis del seno cavernoso derecho secundaria a rinosinusitis esfenoidal: reporte de caso / Right cavernous sinus thrombosis secondary to sphenoidal rhinosinusitis: case report

RESUMEN La rinosinusitis bacteriana aguda es la infección de la mucosa nasal y de senos paranasales, que se manifiesta con congestión nasal, rinorrea purulenta y fiebre, de curso generalmente autolimitado. La trombosis del seno cavernoso (TSC) es una complicación poco frecuente, que se asocia a compromiso del seno esfenoidal y etmoidal, manifestándose clínicamente con cefalea, fiebre, alteración de pares craneanos y síntomas oculares. Ante su sospecha el estudio imagenológico es fundamental. En estos exámenes se debe buscar un defecto de llene en el seno cavernoso comprometido. El tratamiento debe iniciarse de forma precoz y contempla: antibioticoterapia, drenaje quirúrgico del seno afectado y anticoagulación. Se presenta a un paciente pediátrico masculino con TSC derecha posterior a una rinosinusitis esfenoidal no tratada, que respondió favorablemente al tratamiento. Se describe la clínica y manejo de esta patología, que presenta alta morbimortalidad, y que puede disminuirse considerablemente con un tratamiento precoz y agresivo.

ABSTRACT Acute bacterial rhinosinusitis is the infection of the nasal and paranasal mucous membrane, with symptoms such as nasal congestion, purulent rhinorrhea and fever, usually self-limiting. Cavernous sinus thrombosis (CST) is a rare complication, which is associated with sphenoid and ethmoid sinus involvement, clinically manifest with headache, fever, cranial nerve involvement and ocular symptoms. When suspected, imaging studies will confirm the clinical impression. They will show abnormal flow parameters in the compromised cavernous sinus. The treatment has to start as soon as possible and consists in broad spectrum intravenous antibiotics, surgical drainage of the site of the infection and anticoagulation. We present a patient with a right CST secondary to a non-treated sphenoidal sinusitis, that responded favorably to treatment. The clinical and management of this pathology is described, which presents a high morbidity and mortality that can be reduced considerably with an early and aggressive treatment.

Otite externa necrotizante com paralisia facial periférica e trombose do seio cavernoso: relato de caso / Necrotizint external otitis with peripheral facial paralysis and cavernous sinus thrombosis: case report

Otite externa necrotizante (OEN), conhecida antigamente como otite externa maligna (OEM), atinge o conduto auditivo externo, causando otalgia intensa, otorreia fétida, e em casos mais severos, comprometimento dos ossos do crânio e déficit neurológico. O agente etiológico mais comum é a Pseudomonas aeruginosa. Acomete principalmente pacientes idosos, diabéticos e imunodeprimidos. O comprometimento de nervo craniano levando a paralisia facial e trombose dos seios intracranianos são de piores prognósticos. O diagnóstico depende da suspeita clínica inicialmente. Os exames complementares são imprecisos, embora essenciais no seguimento. A abordagem multidisciplinar é sempre recomendada. O objetivo do trabalho é relatar as complicações clínicas de um caso de otite externa necrotizante com paralisia facial que evoluiu para trombose do seio cavernoso, levando ao óbito.

Necrotizing external otitis (NEO), formerly known as malignant external otitis (MEO), strikes the external auditory canal, causing severe otalgia, fetid otorrhea, and in more severe cases, impairment of the skull bones and neurological deficit. The most common etiological agent is Pseudomonas aeruginosa. It mainly affects elderly, diabetic and immunodepressed patients. Cranial nerve impairment leading to facial paralysis and intracranial sinus thrombosis are of bad prognos. The diagnosis depends on the clinical suspicion. Complementary exams are imprecise, although essential in the follow-up. The multidisciplinary approach is always recommended. The objective of this paper is to report the clinical complications of a case of necrotizing external otitis with facial paralysis and cavernous sinus thrombosis, leading to death.

Intradural Transpetrosectomy for Petrous Apex Meningiomas

OBJECTIVE: This study aimed to assess the surgical results of the intradural transpetrosectomy for petrous apex meningiomas (PAMs). In addition, we describe the methods and techniques used to expose and manage superior petrous vein and greater superficial petrosal nerve.METHODS: The authors conducted a retrospective study of 16 patients with PAMs operated by the senior author via the intradural transpetrosectomy between February 2012 to May 2017. We reviewed patient data regarding the general characteristics, surgical technique and surgery-related outcomes and adopted a combined follow-up strategy of clinic and telephone contacts to evaluate postoperative complications.RESULTS: Simpson grade I and II resection was performed in 10 out of 16 cases (62.5%), and grade III resection were reported in the remaining six cases (37.5%) with no resultant mortality. The mean Karnofsky Performance Status score was 85.6 preoperatively and improved to 91.9 postoperatively, with a mean follow-up period of 34.4 months (range, 6–66 months). Tumor recurrence was found in two patients and they underwent the second surgical operation.CONCLUSION: PAMs could be completely resected by the intradural transpetrosectomy with an improved survival rate and postoperative life quality. Superior petrous vein and greater superficial petrosal nerve should be managed properly in avoidance of postoperative complications. Finally, most meningioma inside cavernous sinus or adhered to brainstem could be totally removed without postoperative complications.

Clinical Significance of Radical Surgery in the Treatment of Silent Corticotroph Adenoma

OBJECTIVE: Silent corticotroph adenomas (SCA) are endocrine-inactive pituitary adenomas with positive immunohistochemistry staining for adrenocorticotropic hormone (ACTH). We investigated whether SCA-associated clinical profiles were more aggressive than hormonally negative adenomas (HNA).METHODS: Among 627 patients with pathologically proven endocrine-inactive pituitary adenomas between 2004 and 2013, positive immunohistochemistry revealed 55 SCAs and 411 HNAs. Surgical outcomes and radiological and endocrinological characteristics were compared.RESULTS: Strong female predominance was observed in the SCA group (p<0.001). Cavernous sinus invasion was identified in 22 (40%) SCA patients and 72 (17.6%) HNA patients (p<0.001). There were no differences in ACTH or cortisol levels between the two groups. The incidence of preoperative hypopituitarism and postoperative hormonal outcome did not differ between two groups. Total resection was achieved in 35 patients (63.7%) with SCA and 332 patients (80.8%) with HNA (p=0.007). When tumors were completely removed, recurrence rates were not statistically different between two groups (p=0.60). When complete resection was not achieved, tumors regrew from these remnants in seven patients (35.0%) with SCA and 12 patients (15.2%) with HNA (p=0.05).CONCLUSION: Total surgical resection for SCA is often challenging as these tumors frequently invade a cavernous sinus. Early remnant tumor intervention is justified, because untreated residual pituitary tumors regrow when patients were followed up for a long time. Prophylactic radiotherapy is not warranted for completely resected SCAs as tumor recurrence is uncommon.

Intradural Transpetrosectomy for Petrous Apex Meningiomas

OBJECTIVE: This study aimed to assess the surgical results of the intradural transpetrosectomy for petrous apex meningiomas (PAMs). In addition, we describe the methods and techniques used to expose and manage superior petrous vein and greater superficial petrosal nerve. METHODS: The authors conducted a retrospective study of 16 patients with PAMs operated by the senior author via the intradural transpetrosectomy between February 2012 to May 2017. We reviewed patient data regarding the general characteristics, surgical technique and surgery-related outcomes and adopted a combined follow-up strategy of clinic and telephone contacts to evaluate postoperative complications. RESULTS: Simpson grade I and II resection was performed in 10 out of 16 cases (62.5%), and grade III resection were reported in the remaining six cases (37.5%) with no resultant mortality. The mean Karnofsky Performance Status score was 85.6 preoperatively and improved to 91.9 postoperatively, with a mean follow-up period of 34.4 months (range, 6–66 months). Tumor recurrence was found in two patients and they underwent the second surgical operation. CONCLUSION: PAMs could be completely resected by the intradural transpetrosectomy with an improved survival rate and postoperative life quality. Superior petrous vein and greater superficial petrosal nerve should be managed properly in avoidance of postoperative complications. Finally, most meningioma inside cavernous sinus or adhered to brainstem could be totally removed without postoperative complications.

Clinical Significance of Radical Surgery in the Treatment of Silent Corticotroph Adenoma

OBJECTIVE: Silent corticotroph adenomas (SCA) are endocrine-inactive pituitary adenomas with positive immunohistochemistry staining for adrenocorticotropic hormone (ACTH). We investigated whether SCA-associated clinical profiles were more aggressive than hormonally negative adenomas (HNA). METHODS: Among 627 patients with pathologically proven endocrine-inactive pituitary adenomas between 2004 and 2013, positive immunohistochemistry revealed 55 SCAs and 411 HNAs. Surgical outcomes and radiological and endocrinological characteristics were compared. RESULTS: Strong female predominance was observed in the SCA group (p<0.001). Cavernous sinus invasion was identified in 22 (40%) SCA patients and 72 (17.6%) HNA patients (p<0.001). There were no differences in ACTH or cortisol levels between the two groups. The incidence of preoperative hypopituitarism and postoperative hormonal outcome did not differ between two groups. Total resection was achieved in 35 patients (63.7%) with SCA and 332 patients (80.8%) with HNA (p=0.007). When tumors were completely removed, recurrence rates were not statistically different between two groups (p=0.60). When complete resection was not achieved, tumors regrew from these remnants in seven patients (35.0%) with SCA and 12 patients (15.2%) with HNA (p=0.05). CONCLUSION: Total surgical resection for SCA is often challenging as these tumors frequently invade a cavernous sinus. Early remnant tumor intervention is justified, because untreated residual pituitary tumors regrow when patients were followed up for a long time. Prophylactic radiotherapy is not warranted for completely resected SCAs as tumor recurrence is uncommon.