Multidisciplinary management of idiopathic intracranial hypertension in pregnancy: case series and narrative review

Abstract Idiopathic intracranial hypertension (IIH) is a neurological condition characterized by raised intracranial pressure of unknown etiology with normal cerebrospinal fluid (CSF) composition and no brain lesions. It occurs in pregnant patients at approximately the same frequency as in general population, but obstetric and anesthetic management of the pregnancy and labor remains controversial. In this article we provide a multidisciplinary review of the main aspects of IIH in pregnancy including treatment options, mode of delivery and anesthetic techniques. Additionally, we report three cases of pregnant women diagnosed with IIH between 2012 and 2019 in our institution.

Bariatric surgery as a treatment for idiopathic intracranial hypertension in a male adolescent: case report / Cirurgia bariátrica como tratamento de pseudotumor cerebral primário em adolescente do sexo masculino: relato de caso

ABSTRACT Objective: To describe a case of a male adolescent with symptomatic idiopathic intracranial hypertension (IIH) associated with obesity treated with bariatric surgery. Case description: A 16-year-and-6-month-old severely obese boy [weight: 133.6 kg; height: 1.74 m (Z score: +0.14); BMI: 44.1 kg/m2 (Z score: +4.4)], Tanner pubertal stage 5, presented biparietal, high-intensity, and pulsatile headaches, about five times per week, associated with nocturnal awakenings, and partial improvement with common analgesics, for three months. Ophthalmologic evaluation evidenced bilateral papilledema. Cranial computed tomography revealed no mass or anatomic abnormalities. Lumbar puncture showed increased intracranial pressure of 40 cmH2O (reference value: <28 cmH2O) with a normal content. After being diagnosed with IIH, the patient was started on acetazolamide. However, after three months, he was still symptomatic. He was diagnosed with obesity due to excess energy intake and, as he had failed to lose weight after a conventional clinical treatment, bariatric surgery was indicated. The patient (at 16 years and nine months) underwent an uncomplicated laparoscopic sleeve gastrectomy. Ophthalmologic evaluation, performed five months after surgery, revealed normal visual acuity in both eyes and improvement of bilateral papilledema. Follow-up at 18 months showed a 67.5% loss of excess weight (weight: 94.5 kg and BMI: 31.2 kg/m2) and complete resolution of IIH symptoms. Comments: IIH is characterized by increased intracranial pressure with no evidence of deformity or obstruction of the ventricular system on neuroimaging. It has been associated with obesity. Bariatric surgery may be a valid alternative approach for morbidly obese adolescent patients with refractory symptoms.

RESUMO Objetivo: Descrever um caso de cirurgia bariátrica como tratamento de pseudotumor cerebral primário (PTCP) em adolescente do sexo masculino com obesidade. Descrição do caso: Adolescente, sexo masculino, 16 anos e 6 meses, com obesidade exógena [peso:133,6 kg; estatura:1,74 m (escore z: +0,14); IMC: 44,1 kg/m2 (escore z: +4,4)], estadiamento puberal de Tanner 5, apresentando cefaleia bi-parietal, pulsátil e de alta-intensidade, cerca de cinco vezes por semana, associada a despertares noturnos, e com melhora parcial com analgésicos comuns, há três meses. A avaliação oftalmológica evidenciou papiledema bilateral e a tomografia computadorizada de crânio não revelou massas ou alterações anatômicas. A punção lombar mostrou pressão intracraniana elevada de 40 cmH2O (Referência: <28 cmH2O) com conteúdo normal. Feito o diagnóstico, o paciente foi iniciou uso de acetazolamida. No entanto, após 3 meses, o paciente mantinha-se sintomático. Ele foi diagnosticado com obesidade devido ao consumo calórico excessivo e, como não havia obtido sucesso na perda de peso com tratamento clínico convencional, a cirurgia bariátrica foi indicada. Aos 16 anos e 9 meses, o paciente foi submetido a gastrectomia vertical laparoscópica sem complicações. A avaliação oftalmológica, cinco meses após a cirurgia, revelou melhora do papiledema bilateral com acuidade visual normal em ambos os olhos. Apresentou perda de excesso de peso de 67,5% (peso: 94,5 kg e IMC:31,2 kg/m2) e resolução completa dos sintomas de PPTC 18 meses após a cirurgia. Comentários: O PTCP é caracterizado pelo aumento da pressão intracraniana, sem evidência de deformidade ou obstrução do sistema ventricular na neuroimagem. Está associado à obesidade. A cirurgia bariátrica pode ser uma alternativa terapêutica válida para pacientes adolescentes obesos graves com sintomas refratários.

Bilateral Transverse Sinus Angioplasty for the Treatment of Idiopathic Intracranial Hypertension ­ Case Report and Literature Review

Idiopathic intracranial hypertension (IIH) is a disease characterized by an increase in intracranial pressure, without presence of parenchymal lesions or hydrocephalus that justify it. Over 90% of cases there is association with stenosis of the dural venous sinuses. It is characterized by headache, tinidus, nausea, vomiting and visual disturbances. Initial treatment is clinical and when it fails there is indication of invasive procedures, among them shunts and fenestration of the optic nerve sheath. Angioplasty of dural venous sinuses, when indicated, has shown an alternative with better results and less complications. We report a case of a female patient, with 27 years old, diagnosed with IIH and bilateral transverse sinus stenosis, which was treated by bilateral stenting and total resolution of symptoms. Besides describing the case we review the literature about the subject.

Pseudotumor cerebri in childhood and adolescence: data from a specialized service / Pseudotumor cerebral na infância e adolescência: dados de um serviço especializado

ABSTRACT Objective: To report cases of children and adolescents diagnosed with pseudotumor cerebri associated or not with rheumatic disease. Methods: This was a retrospective study based on medical reports of 29 patients, up to 18 years of age and diagnosed with pseudotumor cerebri, followed up in the Pediatric Rheumatology and Neurology outpatient clinics of a tertiary hospital, until December 2016. Results: Among the 29 patients diagnosed with pseudotumor cerebri, 51.7% were girls and the mean age at the disease onset was 12.3 years. In 18 patients (62%) where an etiology was found, four were associated with a rheumatic disease. The most common symptom was headache (69%) and acetazolamide was the most used medication (69%). Two patients developed blindness and 10 are still being followed up. Conclusion: Although rare, pseudotumor cerebri should be considered in children with headaches, especially in patients with rheumatic disease.

RESUMO Relatar os casos de crianças e adolescentes com diagnóstico de pseudotumor cerebral com ou sem doença reumática. Métodos: Estudo retrospectivo através de revisão de prontuários, 29 pacientes com idade até 18 anos e diagnóstico de pseudotumor, atendidos nos ambulatórios de Reumatologia Pediátrica e Neurologia de um hospital terciário, registrados até dezembro de 2016. Resultados: Dentre os 29 pacientes com diagnóstico de pseudotumor cerebral, 51,7% eram meninas. A média de idade de aparecimento dos sintomas foi de 12,3 anos. Em relação à etiologia do pseudotumor cerebral, em 18 pacientes (62%) foi possível identificar uma causa, sendo o diagnóstico de doença reumática associada em quatro desses casos. Cefaléia foi o sintoma mais frequente (69%), e a medicação mais utilizada foi a acetazolamida (69%). Dois pacientes evoluíram para cegueira e 10 ainda se encontram em seguimento ambulatorial. Conclusão: Concluímos que, apesar de raro, o diagnóstico de pseudotumor cerebral deve ser considerado em crianças com cefaleia, principalmente nos pacientes com doença reumática.

Pseudotumor cerebral asociado a hipovitaminosis A, B6 y D: A propósito de dos casos / Pseudotumor cerebri associated with hypovitaminosis A, B6 and D: About two cases

La hipertensión endocraneana idiopàtica se asocia infrecuentemente con la hipovitaminosis A y D. Se presenta el caso de una paciente femenina de 8 años con visión borrosa de 24 horas y papiledema bilateral. Resonancia magnética nuclear normal. Presión de apertura de líquido cefalorraquídeo: 260 mm^O. Presentó déficit de vitamina A y D, e inició un tratamiento sustitutivo. El segundo caso corresponde a un paciente masculino de 12 años con fiebre y odinofagia de 3 días. Con antecedente de glomerulonefritis y sobrepeso. Presentaba edema bipalpebral y papiledema. Tomografia axial computada de la órbita: aumento de líquido en la vaina de ambos nervios ópticos. Resonancia magnética nuclear: aracnoidocele intraselar. Presión de apertura de líquido cefalorraquídeo: 400 mm^O. Presentó déficit de vitamina D y B6, e inició el tratamiento sustitutivo. La elevación de la presión intracraneal desencadena mecanismos de compensación que, al fallar, pueden comprometer la vida o provocar graves discapacidades neurológicas. Reconocer la causa para un enfoque terapéutico preciso es clave para disminuir la morbimortalidad asociada a esta patología.

Idiopathic endocranial hypertension is infrequently associated with hypovitaminosis A and D. The case of an 8-year-old female with 24-hour blurred vision and bilateral papilledema is presented. Nuclear magnetic resonance was normal. Opening pressure of cerebrospinal fluid: 260 mm^O. She presented vitamin A and D deficiency and started replacement therapy. The second case corresponds to a 12-year-old male with fever and odynophagia of 3 days. History of glomerulonephritis and overweight. He had bipalpebral edema and papilledema. Computed tomography scan of the orbit: increase of fluid in the sheath of both optic nerves. Nuclear magnetic resonance: intrasellar arachnoidocele. Opening pressure of cerebrospinal fluid: 400 mmH2O. He presented vitamin D and B6 deficiency and started replacement treatment. The elevation of intracranial pressure triggers compensation mechanisms that, when they fail, can compromise life or cause serious neurological disabilities. Recognizing the cause for an accurate therapeutic approach is key to reduce the morbidity and mortality associated with this pathology.

Esclerose múltipla pseudotumoral: relato de caso e revisão de literatura / Pseudotumoral form of multiple sclerosis: case report and review of literature

A forma pseudotumoral de esclerose múltipla é extremamente rara e possui uma grande variedade de diagnósticos diferenciais, o que a torna um desafio na prática neurológica. Neste artigo, apresentamos um caso desta manifestação incomum de esclerose múltipla, que foi avaliado em uma clínica especializada no Rio de Janeiro. O caso é de um individuo do sexo masculino de 21 anos que apresentou apenas crises convulsivas. Apresentou curso benigno da doença.

Pseudotumoral forms of multiple sclerosis are very and have a great variety of differential diagnosis, which poses it as a great challenge in neurological practice. In this paper, we report one case of this uncommon manifestation of multiple sclerosis, which was assessed in a specialized clinic in Rio de Janeiro. The case is a 21 year old male patient that had only seizures. He presented a benign course of the disease.

Pseudotumor cerebral secundario al consumo de minociclina en un paciente pediátrico / Pseudotumor cerebri secondary to consumption of minocycline in a pediatric patient

El pseudotumor cerebral se caracteriza por una elevación de la presión intracraneal mayor de 20 cmH2O, con ventrículos y líquido cefalorraquídeo de características normales. El consumo de minociclina es una de las causas asociadas a este síndrome. Presentamos una paciente de 13 años de edad con antecedentes de acné tratado con minociclina. Comenzó con cefalea intensa, diplopía y visión borrosa. Se constató el diagnóstico de pseudotumor cerebral y se indicó la suspensión inmediata del antibiótico y el inicio del tratamiento con acetazolamida. Aunque la patogénesis de pseudotumor cerebral no es totalmente conocida, se ha observado una asociación con el empleo de minociclina. Este antibiótico es de uso frecuente para el manejo del acné, por lo que es importante considerar sus complicaciones antes de ser prescrito.

Pseudotumor cerebri is a syndrome characterized by an elevated intracranial pressure greater than 20 cmH2O with ventricles and cerebrospinal fluid of normal characteristics. Consumption of minocycline have been described among the causes associated with this syndrome. We present a 13-year old female patient with a history of acne treated with minocycline who began with severe headache, diplopia and blurred vision. The diagnosis of pseudotumor cerebri was made, indicating the immediate antibiotic suspension and the beginning of the treatment with acetazolamide. Although the pathogenesis of pseudotumor cerebri is not fully known, an association with minocycline has been observed. This antibiotic is often used by health professionals for the management of acne, so it is important to consider its complications before being prescribed.

Factors affecting visual loss and visual recovery in patients with pseudotumor cerebri syndrome / Fatores que influenciam na perda e na recuperação visual de pacientes com a síndrome do pseudotumor cerebral

ABSTRACT Purpose: To investigate the frequency of visual loss (VL), possible predictive factors of VL, and improvement in patients with pseudotumor cerebri (PTC) syndrome. Methods: We reviewed 50 PTC patients (43 females, seven males) who underwent neuro-ophthalmic examination at the time of diagnosis and after treatment. Demographic data, body mass index (BMI), time from symptom onset to diagnosis (TD), maximum intracranial pressure (MIP), occurrence of cerebral venous thrombosis (CVT), and treatment modalities were reviewed. VL was graded as mild, moderate, or severe on the basis of visual acuity and fields. Predictive factors for VL and improvement were assessed by regression analysis. Results: The mean ± SD age, BMI, and MIP were 35.2 ± 12.7 years, 32.0 ± 7.5 kg/cm2, and 41.9 ± 14.5 cmH2O, respectively. Visual symptoms and CVT were present in 46 and eight patients, respectively. TD (in months) was <1 in 21, 1-6 in 15, and >6 in 14 patients. Patients received medical treatment with (n=20) or without (n=30) surgery. At presentation, VL was mild in 16, moderate in 12, and severe in 22 patients. Twenty-eight patients improved and five worsened. MIP, TD, and hypertension showed a significant correlation with severe VL. The best predictive factor for severe VL was TD >6 months (p=0.04; odds ratio, 5.18). TD between 1 and 6 months was the only factor significantly associated with visual improvement (p=0.042). Conclusions: VL is common in PTC, and when severe, it is associated with a delay in diagnosis. It is frequently permanent; however, improvement may occur, particularly when diagnosed within 6 months of symptom onset. .

RESUMO Objetivo: Investigar a frequência de perda visual (PV) e os possíveis fatores preditivos para perda e para melhora visual em pacientes com a síndrome do pseudotumor cerebral (SPC). Métodos: Foram revisados 50 pacientes com SPC submetidos a exame neuroftalmológico no momento do diagnóstico e após o tratamento. Dados demográficos, índice de massa corpórea (IMC), tempo decorrido entre o início dos sintomas e o diagnóstico (TD), pressão intracraniana máxima (PIM), ocorrência de trombose venosa cerebral (TVC), e as modalidades de tratamento foram revisadas. PV foi graduada em discreta, moderada e grave, baseada na acuidade e no campo visual. Fatores preditivos para perda e melhora visual foram avaliados por análise de regressão linear. Resultados: Quarenta e três pacientes eram do sexo feminino. A média de idade, o IMC e a PIM (± desvio padrão) foram: 35,2 ± 12,7 anos, 32,0 ± 7,5 kg/cm2 e 41,9 ± 14,5 cmH2O, respectivamente. Sintomas visuais estavam presentes em 46 e TVC em 8 pacientes. TD (em meses) foi <1 em 21, 1-6 em 15 e >6 em 14 pacientes. Pacientes receberam tratamento clinico apenas (n=30) ou associado a tratamento cirúrgico (n=20). Na apresentação a PV era discreta em 16, moderada em 12 e grave em 22 pacientes. Vinte e oito pacientes melhoraram e 5 pioraram. PIM, TD e hipertensão arterial correlacionaram significativamente com PV grave. O melhor fator preditivo para PV grave foi o TD>6 meses (p=0,04; razão de chances 5,18). TD entre 1 e 6 meses foi o único fator significativamente associado com melhora visual após tratamento (p=0,042). Conclusões: Perda visual é comum na SPC e quando grave se mostra relacionado a atraso no diagnóstico. É usualmente permanente mas pode haver melhora visual especialmente quando a doença é diagnosticada nos primeiros 6 após o início dos sintomas. .

Pseudotumor cerebral en dos pacientes pediátricos / Pseudotumor Cerebri on two pediatric patients

Antecedentes: El pseudotumor cerebral es una condición que se caracteriza por presión intracraneal elevada en ausencia de manifestaciones clínicas, evidencia radiológica o laboratorial de lesión ocupativa. Es una entidad clínica bien definida en adultos, pero puede afectar a niños de cualquier edad. Casos clínicos: Paciente 1: Femenino de 8 años con cuadro de cefalea generalizada y vómitos dos días después, presenta desviación nasal de la mirada del ojo derecho, paresia bilateral de los rectos externos, edema bilateral de la papila óptica grado III. Campimetría computada reportó discreto escotoma superior en ojo izquierdo y nasal en ojo derecho. Se realizó punción lumbar donde se registró una presión de apertura de 45 cm de agua. Paciente 2: femenino de 11 años, con historia de un año de evolución de cefalea frontal intermitente, pulsátil y disminución de la agudeza visual referida por la paciente;a la evaluación neurológica edema bilateral de la papila óptica grado III. En la punción lumbar se registró inicialmente una presión de apertura de 43 cm de agua. Ambos casos fueron tratados con acetazolamida oral, con buena respuesta clínica y normalización de la presión intracraneal.Conclusión: el pseudotumor cerebral es una enfermedad infrecuente en pediatría que requiere de su identificación y manejo oportuno con el fin de evitar una lesión visual irreversible...

Pseudotumor cerebri during Cushing's disease treatment with ketoconazole / Pseudotumor cerebral durante o tratamento de doença de Cushing com cetoconazol

Benign intracranial hypertension (Pseudotumor cerebri) has been described as related to the reduction in steroid levels in Cushing's disease (CD), especially after surgical remission. Ketoconazole is a common and effective adjuvant therapy for hypercortisolism, but the major concern is liver enzyme dysfunction. We describe here the case of a 12-year old girl with CD who developed benign intracranial hypertension during treatment with ketoconazole. She presented headache, vomiting, a black spot on her right temporal visual field, and signs of elevated intracranial pressure. Pituitary image was normal on magnetic resonance image (MRI), and all symptoms improved after treatment with acetazolamide. We call attention to the diagnosis of this disorder in CD patients, especially children on ketoconazole treatment, because it could be confounded with adrenal insufficiency and lead to definitive severe visual impairment.

Hipertensão intracraniana benigna (Pseudotumor cerebral) tem sido descrita relacionada à redução dos níveis de esteroides séricos na doença de Cushing (DC), especialmente após a remissão cirúrgica. O cetoconazol é uma opção efetiva e de uso rotineiro como adjuvante na terapêutica do hipercortisolismo, tendo como paraefeito mais temido a toxicidade hepática. Relatamos o caso de uma menina com 12 anos de idade portadora de DC que desenvolveu hipertensão intracraniana benigna durante tratamento com cetoconazol. Apresentou-se com cefaleia, vômitos, comprometimento do campo visual temporal direito e sinais de hipertensão intracraniana. A ressonância magnética (RM) de hipófise era normal e todos os sinais e sintomas resolveram-se com uso de acetazolamida. Chamamos a atenção para esse diagnóstico nos pacientes com DC, especialmente crianças, em tratamento com cetoconazol, porque ele pode ser confundido com insuficiência adrenal e causar comprometimento visual severo e definitivo.

Clinical profile of cerebral venous sinus thrombosis and the role of imaging in its diagnosis in patients with presumed idiopathic intracranial hypertension.

Retrospective descriptive study reporting the rate of occurrence of cerebral venous sinus thrombosis (CVST), highlighting the role of magnetic resonance imaging (MRI) and magnetic resonance venography (MRV) in patients with presumed idiopathic intracranial hypertension (IIH). Study was conducted in the department of neuro-ophthalmology at a tertiary eye care center in South India. Data from 331 patients diagnosed with IIH from June 2005 to September 2007 was included. Inclusion criteria were: Elevated opening cerebrospinal fluid (CSF) pressure of more than 200 mm of water on lumbar puncture, normal CSF biochemistry and microbiology, and normal neuroimaging as depicted by computed tomography(CT) scan. Exclusion criteria were: Space-occupying lesions, hydrocephalus, meningitis, intracranial pressure within normal range, abnormal CSF biochemistry and microbiology. The remaining patients were evaluated with MRI and MRV. CVST was present in 11.4% of patients who were presumed to have IIH (35/308). MRI alone identified 24 cases (68%) of CVST, while MRI used in combination with MRV revealed an additional 11 cases (32%). Risk factors associated with CVST were identified in nine out of 35 patients (26%). CVST may be misdiagnosed as IIH if prompt neuroimaging by MRI and MRV is not undertaken. Risk factors of CVST may not be apparent in all the cases and these patients are liable to be missed if CT scan alone is used for neuroimaging, hence MRI, combined with MRV should be undertaken to rule out CVST.

Pseudotumor cerebri como una manifestación excepcional de la enfermedad de Behçet: caso clínico / Pseudotumor cerebri secondary to Behçet disease: report of one case

The classical manifestations of Behçet disease are mouth ana genital ulcers, cutaneous lesions ana ocular involvement. The central nervous system is affected in 5 to 59 percent of the cases, usually in the form of meningoencephalitis or sinus venous thrombosis. We report a 17-year-old femóle presenting with a two weeks history of progressive headache, nausea and blurred vision. An initial magnetic resonance was normal. Fifteen days later she was admitted to the hospital due to progression of visual impairment. Shegave a history of oral ulcers and arthralgias. A new magnetic resonance was normal. A lumbar puncture showed a cerebrospinal fluid with a protein concentration of 14 mg/dl, aglucose concentration of 64 mg/dl, 20fresh red blood cells and a pressure of 26 cm H(2)0. The diagnosis of a pseudotumor cerebri, secondary to Behçet disease was raised and the patient was treated with colchicine and acetazolamide. The evolution was torpid and an anterior uveitis was alsofound. After discharge, she continued with oral and genital ulcers and was treated with infliximab. Despite treatment, headache persists.

Isolated and silent spinal neurocysticercosis associated with pseudotumor cerebri.

Incidence of spinal neurocysticercosis (NCC) is rare. Isolated spinal NCC is still rarer. We present here a case report where a young lady presented with all the clinical features of pseudotumor cerebri (PTC), where medical treatment for PTC failed and the presence of cysticercous in spinal canal was detected only on the operation table, while doing a lumbo-peritoneal shunt (LP shunt) to save her vision. Diagnosis could be confirmed only after the histopathology report was received. She did not have any direct evidence of spinal involvement, thereby eluding correct diagnosis. In English literature, we could not find any report of isolated and silent spinal NCC associated with PTC. In addition, we could not find any report of recovery of cysticercous larva through the Touhey's needle injury, although this was an incidental finding. In endemic areas, isolated spinal NCC should be suspected in patients presenting with PTC.

Pseudotumor cerebral secundário a trombose venosa dural: relato de caso pediátrico / Pseudotumor cerebri secondary to dural sinus thrombosis: pediatric case report

O pseudotumor cerebral é uma síndrome neurológica relativamente comum na adolescência. Na maioria dos casos, a etiologia é idiopática, mas pode haver complicações graves, como cegueira, relacionadas com a hipertensão intracraniana. O objetivo deste artigo é enfatizar o diagnóstico diferencial do pseudotumor cerebral, com atenção especial às etiologias tratáveis. Relatamos o caso de um adolescente de 12 anos que se apresentou com diplopia e cefaléia 9 dias após otite média e mastoidite à direita. A tomografia computadorizada do crânio foi normal, mas a ressonância magnética do encéfalo detectou trombose dos seios transverso e sigmóideo ipsilaterais, a qual respondeu à anticoagulação precoce. A conclusão é que a ressonância magnética do encéfalo é essencial nos pacientes com diagnóstico clínico de pseudotumor cerebral para exclusão de causas tratáveis, como a trombose venosa dural.

Perda visual bilateral grave como sinal de apresentação de trombose de seio venoso cerebral: relato de caso / Severe bilateral visual loss as the presenting sign of cerebral venous sinus thrombosis: case report

O objetivo é relatar um caso de trombose de seio venoso cerebral causando síndrome de pseudotumor, onde a principal manifestação clínica foi uma perda visual bilateral grave. Homem de 24 anos apresentando perda visual bilateral grave, obscurecimentos transitórios da visão, cefaléia, papiledema bilateral e campos visuais constrictos. Tomografia computadorizada, bem como exame neurológico, apresentavam-se normais. Punção lombar mostrou um aumento da pressão liquórica inicial e o hemograma mostrou aumento do número de plaquetas. A ressonância magnética de crânio apresentava sinais característicos de papiledema crônico e sinais sugestivos de trombose de seio sagital superior. A venorressonância magnética de crânio mostrou trombose de seios sagital superior, transversos e sigmóides. Paciente foi tratado com descompressão de bainha de nervo óptico do olho esquerdo, acetazolamida e anticoagulação. O exame oftalmológico após o tratamento mostrou redução do papiledema, mas com persistência de perda visual acentuada. O pseudotumor cerebral com perda visual severa pode ser um sinal de apresentação de trombose venosa cerebral secundária a doença hematológica grave.