Clinical analysis of 15 pregnant women complicated with moyamoya disease / 中华妇产科杂志

Objective: To explore the effects of pregnancy complicated with moyamoya disease on maternal and fetal outcomes. Methods: The general clinical data and maternal and fetal outcomes of 20 pregnancies of 15 patients with moyamoya disease admitted to the First Affiliated Hospital of Zhengzhou University from January 2012 to October 2022 were retrospectively analyzed. Results: (1) General information: among the 20 pregnancies of 15 clearly diagnosed pregnant women complicated with moyamoya disease, 12 were diagnosed before pregnancy (60%, 12/20), 3 were diagnosed during pregnancy (15%, 3/20), and 5 were diagnosed during puerperal period (25%, 5/20). There were 7 cases of primipara (35%, 7/20) and 13 cases of multipara (65%, 13/20). (2) Pregnancy complications and maternal and infant outcomes: among the 20 pregnancies of 15 pregnant women with moyamoya disease, there were 9 pregnancy complications (45%, 9/20), including 5 gestational hypertension (25%, 5/20), 2 severe pre-eclampsia (10%, 2/20), 1 hyperlipidemia and 1 gestational diabetes mellitus (5%, 1/20). There were 2 case of drug abortion in the first trimester, 3 cases of labor induction in the second trimester, and 15 cases of delivery during the third trimester. All the 15 deliveries were cesarean section, of which 11 (11/15) were cesarean sections with medical indications, and 4 (4/15) were cesarean sections caused by personal factors. General anesthesia was used in 5 cases (5/15), epidural block anesthesia in 7 cases (7/15), and combined spinal and epidural anesthesia in 3 cases (3/15). The median gestational age of 15 neonates was 37.2 weeks (34.0 to 40.8 weeks), with 10 cases (10/15) were full-term infants, and 5 (5/15) were preterm infants (3 of which were associated with hypertensive disorder complicating pregnancy). The birth weight of 15 neonates was (2 853±454) g. Four neonates were admitted to neonatal intensive care unit (NICU), of which 3 cases were admitted to NICU due to premature delivery and 1 case was admitted to NICU due to neonatal jaundice. There was no neonatal asphyxia or death. All neonates were followed up from 4 months to 6 years after birth, and all grew well. (3) Neurological symptoms during pregnancy: 8 cases (40%, 8/20) had neurological symptoms during pregnancy, and 6 cases (30%, 6/20) had hemorrhagic symptoms, of which 3 cases occurred during the puerperal period (3/6). There were 2 cases of ischemic symptoms (10%, 2/20), all of which occurred during the puerperal period (2/2). (4) Analysis of factors related to the occurrence of cerebral hemorrhage: the incidence of cerebral hemorrhage in patients with moyamoya disease diagnosed before pregnancy was significantly lower than that in those without a clear diagnosis, and the incidence of cerebral hemorrhage in women with moyamoya disease was lower than that in primipara (all P<0.01). The incidence of cerebral hemorrhage in moyamoya patients without hypertensive disorder complicating pregrancy was lower than that in patients with hypertensive disorder complicating pregrancy, but the difference was not statistically significant (P>0.05). Conclusions: Pregnancy combined with moyamoya disease has adverse effects on maternal and infant outcomes, and the incidence of pregnancy complications increases. Cerebral hemorrhage occurres in prenatal and puperium, while cerebral ischemia occurres mainly in puperium.

Unusual presentation of moyamoya disease with popliteal involvement: case report and review of the literature / Apresentação incomum de doença de moyamoya com envolvimento poplíteo: relato de caso e revisão da literatura

Abstract Moyamoya disease is a rare disorder that involves the cerebrovascular system. Usually, it leads to occlusion of the arteries of the cerebral system and causes cerebral circulatory complaints. A 48-year-old female patient was admitted to our clinic with intermittent claudication in both legs. Biphasic and monophasic waveform patterns were detected bilaterally in distal (trifurcation arteries) lower extremities with Doppler sonography. The patient therefore underwent systemic vascular examination. Computed tomography angiography revealed bilateral carotid occlusion at the level of supraclinoid segments, and opacifications were detected at the distal segments of the bilateral anterior cerebellar and middle cerebellar arteries. The patient was diagnosed with moyamoya disease, and anticoagulant treatment was started. In conclusion, most previous reports have presented the cerebrovascular involvement of moyamoya disease. However, this disease can involve different peripheral vascular systems and careful and systemic vascular examination is necessary for an exact diagnosis.

Resumo A doença de moyamoya é um distúrbio raro que envolve o sistema cerebrovascular. Normalmente, leva à oclusão das artérias do sistema cerebral e causa problemas circulatórios no cérebro. Uma mulher de 48 anos foi admitida em nossa clínica com claudicação intermitente em ambas as pernas. Na ultrassonografia com Doppler, foram detectados padrões de formato de onda bifásico e monofásico nas extremidades inferiores distais (artérias da trifurcação) de forma bilateral. Portanto, realizou-se o exame vascular sistêmico na paciente. A angiografia por tomografia computadorizada revelou oclusão carotídea bilateral no nível dos segmentos supraclinoides, e opacificações foram detectadas nos segmentos distais das artérias cerebelares anteriores e médias de forma bilateral. A paciente foi diagnosticada com doença de moyamoya, e o tratamento anticoagulante foi iniciado. Em conclusão, a maioria dos relatos anteriores apresentou o envolvimento cerebrovascular da doença de moyamoya. No entanto, essa doença pode envolver diferentes sistemas vasculares periféricos, e um exame vascular sistêmico minucioso é necessário para um diagnóstico exato.

Moyamoya syndrome associated with neurofibromatosis type 1 in a pediatric patient

Abstract: Neurofibromatosis type 1 is a multisystem genetic disease of autosomal dominant transmission that reveals important cutaneous manifestations such as café-au-lait spots, multiple neurofibromas, and ephelides in skin fold areas, as well as hamartomatous lesions in the eyes, bones, glands, and central nervous system. Moyamoya disease is a rare progressive vaso-occlusive disorder that occurs with important ischemic cerebrovascular events. Despite the rarity of this association in childhood, children diagnosed with neurofibromatosis type 1 and focal neurologic symptoms should be investigated for moyamoya syndrome. The present study reports the case of a pediatric patient with a rapidly progressive cerebrovascular accident and a late diagnosis of Neurofibromatosis type 1 associated with moyamoya disease.

Tratamiento neuroquirúrgico de la isquemia cerebral / Neurosurgical treatment of cerebral ischemia

Introducción: El manejo actual de la isquemia cerebral aguda (IA) contempla el uso de r-tPA y terapia endovascular, mientrasque en la isquemia cerebral crónica (IC) la mejor terapia aún no está definida. La revascularización cerebral microquirúrgicaha sido descrita como tratamiento alternativo para pacientes con IA y contraindicación para r-tPA o terapia endovascular, asícomo para pacientes con IC en quienes la terapia médica ha fracasado. Objetivo: Comunicar la experiencia inicial en cirugíade revascularización cerebral, con énfasis en la utilidad de la embolectomía microquirúrgica y del bypass cerebral comoterapia de rescate en IA, así como del bypass cerebral en IC. Pacientes y Método: Serie prospectiva de 5 pacientes conisquemia cerebral tratados con cirugía de revascularización cerebral en el período 2013 a 2016. Se describe la técnica y resultadosquirúrgicos, así como el estado funcional a los 3, 6 y 12 meses de seguimiento según modified Rankin Scale (mRS)...

Background: Tissue Plasminogen Activator (t-PA) and mechanical thrombectomy are today the best treatment approachfor acute ischemic stroke (AIS). However, the best management for chronic cerebral ischemia (CCI) is still debated. Microsurgicalrevascularization has been described as alternative treatment for patients with AIS and contraindication for t-PA orendovascular therapy, and for patients with CCI and failure of maximal medical therapy. Aim: To describe the effectiveness ofmicrosurgical embolectomy and cerebral bypass as salvage therapy in AIS, as well as cerebral bypass in CCI. Methods: Fivepatients were treated by cerebral revascularization between 2013 and 2016. Surgical and functional results were analyzed.Neurological outcomes were assessed by modified Rankin Scale (mRS) at 3, 6 & 12 months...

Doença de Moyamoya: impacto no desempenho da linguagem oral e escrita / Moyamoya disease: impact on the performance of oral and written language

RESUMO A Doença de Moyamoya (DMM) é uma forma incomum de doença cerebrovascular oclusiva que acomete artérias do sistema nervoso central, acarretando alterações adquiridas de linguagem e dificuldades na aprendizagem. O objetivo foi descrever habilidades de linguagem oral/escrita e cognitivas em menina com diagnóstico de DMM de sete anos e sete meses. A avaliação constou de entrevista com pais, Observação do Comportamento Comunicativo, Teste de Vocabulário por Imagem Peabody, Teste de Desempenho Escolar, Perfil de Habilidades Fonológicas, Wechsler Intelligence Scale for Children, Teste Gestáltico Visomotor Bender, Wisconsin Card Sorting Test. Até os seis anos e cinco meses, ocorreram dois episódios de acidente vascular encefálico (AVE) em região temporoparietal esquerda e direita e área frontal esquerda. Realizou cirurgia para revascularização e tratamento medicamentoso. Avaliações audiológicas e oftalmológicas indicaram normalidade. Cursa o segundo ano do ensino fundamental. Apresentou alterações na linguagem oral e escrita (silábico-alfabética); não nomeação de todos os grafemas; escrita e aritmética média-inferior e leitura inferior à primeira série; habilidades do processamento fonológico em nível pré-escolar. A avaliação psicológica indicou nível intelectual satisfatório, porém prejuízo no desempenho cognitivo em tarefas verbais e de execução, limitações nas competências gráfico-percepto-motoras e na organização sequencial lógica. Os episódios de AVEs trouxeram interferências no desempenho dos processos de aprendizagem pelas especificidades das áreas afetadas, interferindo na análise, integração e interpretação de informações auditivas e visuais relevantes para os processos de aprendizagem.

ABSTRACT Moyamoya disease is an unusual form of occlusive, cerebrovascular disorder that affects the arteries of the central nervous system, causing acquired language alterations and learning difficulties. The study aim was to describe the oral/written language and cognitive skills in a seven-year-and-seven-month-old girl diagnosed with Moyamoya disease. The assessment consisted of interviews with her parents and application of the following instruments: Observation of Communicative Behavior, Peabody Picture Vocabulary Test, Academic Performance Test, Profile of Phonological Awareness, Raven’s Progressive Matrices Test, Special Scale, Wechsler Intelligence Scale for Children, Bender Visual Motor Gestalt Test, and Wisconsin Card Sorting Test. Two episodes of stroke in the left and right temporal-parietal and left frontal areas occurred until the age of six years and five months. Revascularization surgery and medication treatment were conducted. The audiologic and ophthalmologic assessments indicated normality. At the time of the study, the girl was attending the second grade of elementary school. She presented changes in oral and written language (syllabic-alphabetic), non-naming of all graphemes, low arithmetic and writing means, reading skill below first grade level and psycholinguistic delay, and pre-school level phonological processing skills. The psychological evaluation indicated satisfactory intellectual level; however, it also showed cognitive performance impairment in verbal and execution tasks and limitations on graphic-perceptual-motor skills and sequential logic organization. The stroke episodes influenced the performance of learning processes, affecting the analysis, integration, and interpretation of relevant visual and auditory information.

Enfermedad moyamoya: presentación de un caso y revisión de la literatura / Moyamoya disease: case report and literature review

La Enfermedad Moyamoya es una arteriopatia oclusiva progresiva de los vasos cerebrales, específicamente de la porción distal de las arterias carótidas internas, por lo general con compromiso bilateral. Esta oclusión trae como consecuencia dilatación de los vasos sanguíneos colaterales con el objetivo de convertirse en vías de circulación colateral, de donde la enfermedad adopta su nombre, debido a la apariencia angiográfica de “nube de humo” de estos pequeños vasos dilatados. Clínicamente se manifiesta como eventos cerebrovasculares isquémicos debido a la oclusión de los vasos mencionados o como eventos hemorrágicos debido a la ruptura de los pequeños vasos sanguíneos dilatados. Presentamos el caso de una paciente femenina de 38 años, que acude al hospital en estado de coma, cuya tomografía computarizada revela hemorragia intraventricular que fue tratada con derivación ventricular externa más activador de plasminógeno tisular. Posterior a su estabilización clínica se realizó angiografía cerebral diagnóstica con hallazgos compatibles con Enfermedad Moyamoya. Se decidió realizar subduro-sinangiosis con el fin de inducir la formación de circulación colateral cerebral. Debido a la baja incidencia de la Enfermedad Moyamoya en Latinoamérica, generalmente no es incluida en el diagnóstico diferencial de los eventos cerebrales vasculares, por lo que recomendamos su sospecha en pacientes jóvenes y sin factores de riesgo que debutan con este tipo de eventos cerebrales.

Moyamoya disease is a progressive occlusive disease of the cerebral vessels, specifically the distal portion of the internal carotid arteries, usually with bilateral involvement. This occlusion results in dilatation of collateral blood vessels in order to form collateral circulation pathways, from which the disease takes its name due to the angiographic appearance of “puff of smoke” of these small dilated vessels. Clinically it manifests as ischemic cerebrovascular events due to occlusion of the vessel mentioned or hemorrhagic events due to rupture of small dilated blood vessels. We report the case of a female patient of 38 years old who was admitted to the hospital in coma state, the CT scan revealed intraventricular hemorrhage that was treated with an external ventricular derivation and tissue plasminogen activator. After clinical stabilization diagnostic cerebral angiography was performed with findings consistent with Moyamoya disease. We decided to perform a subduro-sinangiosis, in order to induce the formation of cerebral collateral circulation. Due to the low incidence of Moyamoya disease in Latin America, generally it is not included in the differential diagnosis of cerebral vascular events, we recommend suspect it in young patients without risk factors who present with this type of brain events.

Ivy Sign on Fluid-Attenuated Inversion Recovery Images in Moyamoya Disease: Correlation with Clinical Severity and Old Brain Lesions

PURPOSE: Leptomeningeal collateral, in moyamoya disease (MMD), appears as an ivy sign on fluid-attenuated inversion-recovery (FLAIR) images. There has been little investigation into the relationship between presentation of ivy signs and old brain lesions. We aimed to evaluate clinical significance of ivy signs and whether they correlate with old brain lesions and the severity of clinical symptoms in patients with MMD. MATERIALS AND METHODS: FLAIR images of 83 patients were reviewed. Each cerebral hemisphere was divided into 4 regions and each region was scored based on the prominence of the ivy sign. Total ivy score (TIS) was defined as the sum of the scores from the eight regions and dominant hemispheric ivy sign (DHI) was determined by comparing the ivy scores from each hemisphere. According to the degree of ischemic symptoms, patients were classified into four subgroups: 1) nonspecific symptoms without motor weakness, 2) single transient ischemic attack (TIA), 3) recurrent TIA, or 4) complete stroke. RESULTS: TIS was significantly different as follows: 4.86+/-2.55 in patients with nonspecific symptoms, 5.89+/-3.10 in patients with single TIA, 9.60+/-3.98 in patients with recurrent TIA and 8.37+/-3.39 in patients with complete stroke (p=0.003). TIS associated with old lesions was significantly higher than those not associated with old lesions (9.35+/-4.22 vs. 7.49+/-3.37, p=0.032). We found a significant correlation between DHI and motor symptoms (p=0.001). CONCLUSION: Because TIS has a strong tendency with severity of ischemic motor symptom and the presence of old lesions, the ivy sign may be useful in predicting severity of disease progression.

Coronary Heart Disease in Moyamoya Disease: Are They Concomitant or Coincidence?

The purpose of this study was to determine the prevalence and characteristics of symptomatic coronary heart disease (CHD) in patients with moyamoya disease (MMD). This retrospective study evaluated 456 patients who received examination for MMD between 1995 and 2012. We reviewed the patients' medical history and coronary imaging, including conventional coronary angiography and coronary computed tomography angiogram (CTA). Among 456 patients with MMD, 21 (4.6%) patients were found to have symptomatic CHD. Ten patients were treated with coronary artery bypass graft or percutaneous coronary intervention for unstable angina or myocardial infarction. Eleven were treated with medication for stable angina (n = 6) and variant angina with mild degree of stenosis (n = 5).The median age of these patients was 44 yr (range, 27-59). The median Framingham score at diagnosing MMD was < 1% (range, < 1%-16%). The old age was associated with CHD in uni- and multivariate analyses (P = 0.021, OR, 1.053; 95% CI, 1.008-1.110). Considering low age of onset and low stroke risk factor, CHD might be a systemic manifestation that is clinically relevant to MMD.

Fatal ischemic stroke in a case of progressive moyamoya vasculopathy associated with uncontrolled thyrotoxicosis

No abstract available.

Moyamoya syndrome associated with neurofibromatosis type I in a pediatric patient / Síndrome de moyamoya associada a neurofibromatose tipo I em paciente pediátrico

CONTEXT: Neurofibromatosis type 1 (NF-1) is the most prevalent autosomal dominant genetic disorder among humans. Moyamoya disease is a cerebral vasculopathy that is only rarely observed in association with NF-1, particularly in the pediatric age range. The present study reports an occurrence of this association in an infant. CASE REPORT: An eight-month-old female presented convulsive seizures with clonic movements. The patient suffered an ischemic stroke with hemiparesis. Magnetic resonance imaging revealed radiological findings compatible with moyamoya disease. The diagnosis of NF-1 was made at the age of 20 months. CONCLUSION: Despite the rarity of this association in childhood, children with focal neurological symptoms and a diagnosis of NF-1 deserve to be investigated for moyamoya syndrome.

CONTEXTO: Neurofibromatose tipo 1 (NF-1) é a doença genética autossômica dominante mais prevalente no ser humano. A doença de moyamoya é uma vasculopatia cerebral que raramente se encontra associada à NF-1, particularmente na faixa etária pediátrica. Este estudo descreve a ocorrência desta associação em um lactente. RELATO DE CASO: Paciente feminina, aos oito meses de idade, apresentou quadro de crise convulsiva com movimentos clônicos. Evoluiu com acidente vascular encefálico isquêmico e com hemiparesia à direita. Ressonância nuclear magnética mostrou achados compatíveis com a doença de moyamoya. O diagnóstico de NF-1 foi realizado aos 20 meses de vida. CONCLUSÃO: Apesar da raridade desta associação na faixa etária infantil, crianças com sintomas neurológicos focais e diagnóstico de NF-1 merecem ser investigadas para síndrome de moyamoya.

Factores asociados a ulceras por presión en pacientes del Hospital Alemán Nicaragüense enero 2007 a diciembre 2009 / Factors associated with pressure ulcers in patients Nicaraguan German Hospital in January 2007 to December 2009

Se realizó un estudio descriptivo de corte transversal en el Hospital Alemán Nicaragüense en el periodo de Enero del 2007 a Diciembre del 2009, con el objetivo de identificar los principales factores asociados con el desarrollo de úlceras por presión en los pacientes que fueron ingresados en los diferentes servicios atención de dicho Hospital, con una muestra total de 40 paciente de los cuales sólo 32 fueron incluidos en el estudio, ya que 8 de ellos no cumplieron los criterios de inclusión. Se analizó la frecuencia de úlceras por presión según los servicios de hospitalizados, encontrándose que el servicio de Medicina Interna fue el servicio donde más se presentaron úlceras por presión seguido del servicio de Cirugía y UCI.El grupo de edad más afectado fue el de 61 a más años, seguido de los pacientes entre las edades de 46 a 60 años; y el sexo donde predominó el problema fue el masculino con un poco más del 50% en relación al femenino. Con respecto a las patologías de bases asociadas con el desarrollo de úlceras por presión se destacaron aquellas patologías relacionadas con algún grado de inmovilidad, entre ellas la paraplejia, siendo esta la más predominante y, en menor grado las incontinencias (urinaria y fecal) y la enfermedad cerebrovascular. Cabe señalar que la mayoría de los pacientes estudiados presentaban algún grado de desnutrición y anemia hasta en un 65% y la mayoría ingresó por patologías de base, entre ellas diabetes (25%), neumonía, hipertensión arterial, enfermedad cerebrovascular, entre las más frecuentes. Un poco más del 53% de los pacientes desarrolló úlcera por presión durante su estancia hospitalaria, el resto las desarrolló extra hospitalariamente. En el 83% de los pacientes las úlceras fueron múltiples y las localizaciones más frecuentes fueron en talones, región sacra, glúteos. El promedio de estancia hospitalaria fue de 7-14 días en el 50% de los pacientes. Sólo en el 53% de los pacientes se usó algunas medidas anti escaras...

A Case of Moyamoya Disease in a Girl with Thyrotoxicosis

Moyamoya disease is a cerebrovascular disorder of unknown cause, characterized by slowly progressive bilateral stenosis or occlusion of the internal carotid arteries and produces collateral vessels. Moyamoya syndrome has rarely been reported in association with Graves' disease, especially in children. Several reports suggest that a cerebral infarction might have occurred in patients with clinical and laboratory evidence of hyperthyroid function. We report a case of Moyamoya disease in a girl with Down syndrome and thyrotoxicosis, and we review the relevant literature. To our best knowledge, this is the first report of Moyamoya disease associated with thyrotoxicosis in a young person in Korea.

Etiological profile of stroke and its relation with prothrombotic states.

OBJECTIVE: To study the etiological profile of childhood stroke and its relation with prothrombotic states. METHODS: Children with acute stroke with no evidence of CNS infection or head injury were studied. Stroke was confirmed by CT scan and further evaluated by MRI. Cardiac status was assessed with transthoracic echocardiography. Test for hypercoagulable state (antithrombin III, protein C, protein S, anticardiolipin antibody IgG and IgM and lupus anticoagulant) were done in all patients. RESULTS: A total of 66 children were enrolled--36 cases and 30 controls. Presenting symptoms were motor deficit (72%), seizure (66%), altered sensorium (36%), aphasia (27%). Causes identified were antiphospholipid antibody syndrome (25%), Moya Moya disease (16.6%), cardiac disease (11.1%), vasculitis (5.5%), ATIII deficiency (5.5%), Protein C deficiency (2.7%). Etiology remained unknown in 25% of cases with infarction. Hemorrhage was seen in 8.2% of cases and they had DIC or liver disease as the underlying cause. CONCLUSIONS: Magnetic Resonance Angiography and ELISA for antiphospholipid antibody should be done in all patients with stroke without an obvious cause.

Moyamoya en la población infantil no asiática. Ampliación de casuística / Moyamoya disease in non Asian population

La enfermedad o síndrome de moyamoya es una rara afección que provoca una oclusión de las carótidas intracraneales y determina una red vascular colateral anormal en la base del cráneo y que, por su aspecto arteriográfico, hace ya más de 50 años un grupo de neurocirujanos japoneses denominaron moyamoya, y de la cual persisten muchas interrogantes sin resolver. Se realizó un estudio descriptivo y longitudinal de 19 niños con síndrome o enfermedad de moyamoya. La edad promedio de inicio fue de seis años. Del total de niños incluidos, doce debutaron en forma aguda con infarto cerebral; dos pacientes (con antecedentes de epilepsia y retraso) lo hicieron, uno con infarto y otro con hemorragia cerebral, otros dos con epilepsia y retraso, uno con trastorno del lenguaje, siendo en los dos restantes un hallazgo casual. Entretanto doce pacientes se consideraron idiopáticos (portadores de enfermedad de moyamoya) y siete asociados a enfermedades sistémicas (síndrome de moyamoya). El diagnóstico inicial se confirmó por angiorresonancia en diez casos y por arteriografía convencional en nueve. El seguimiento promedio fue de cinco años, once evidenciaron progresión clínica y ocho estabilidad, con un seguimiento promedio de siete y dos años respectivamente. Todos mostraron progresión angiográfica. En siete niños se realizaron diez cirugías de revascularización sin complicaciones y con buenos resultados clínicos y angiográficos. Dos niños fallecieron (uno por hemorragia cerebral y otro por un infarto expansivo) y catorce presentaron secuelas leves a moderadas. Resulta importante identificar los niños que se pueden beneficiar con la cirugía de revascularización, una de las pocas opciones terapéuticas para evitar la progresión y complicaciones de esta grave enfermedad.

Moyamoya's disease or syndrome is a rare illness wich causes symptoms of cerebral ischemia due to intracranial arteries' stenosis, with secondary abnormal vasculature networks at the base of the brain. Since the first description of moyamoya disease done by a group of Japanese neurosurgeons more than 50 years ago, this rare illness is still considered an intriguing disease. The clinical features, treatment, imaging findings and outcomes of a series of not Asian children with Moyamoya disease are described in this study. A total of 19 six year old patients were analyzed. The initial presentation in 12 patients was infarctions, 2 with epilepsy and mental retardation, another 2 who had personal history of epilepsy and mental retardation developed symptoms of ischemic stroke (one of them), and hemorrhagic stroke the other one; 1experienced language problems, while in the last two the diagnosis was casual. On the other hand, 12 patientes were considered idiopathic cases (moyamoya's disease) and 7 were related to systemic illnes (moyamoya's syndrome). Initial diagnosis was achieved by magnetic resonance angiography in 10 cases, and conventional angiography in 9. All patients were followed up for a mean period of 5 years. While 11 patients experienced clinical worsening of symptoms, 6 asymptomatic. However, all of them showed angiographic progression. Seven patients underwent bypass surgery with no further complications, 2 passed away and 14 remained with moderate handicaps. A number of surgical procedures have been developed for revascularization, improving the outcome of some of these patients by preventing ischemic and hemorrhagic stroke. Due to the fact that moyamoya disease is not a silent disorder and its progress cause complications, surgical revascularization should always be considered in the management of these patients.

Off-Pump Coronary Artery Bypass Grafting in Moyamoya Disease

Moyamoya disease is an occlusive intracranial arteriopathy owing to intimal hyperplasia with formation of abnormal cerebrovascular collateral networks; however, the etiology remains unclear. Although this disease is known to be associated with renovascular hypertension, it is extremely rare for it to be associated with stenoses of the coronary arteries. We herein described a case of a 56-year-old female with angina and asymptomatic moyamoya disease. We performed off-pump coronary artery bypass grafting (OPCAB) to avoid cardiopulmonary bypass and the risk of intraoperative hypotension. Conventional coronary artery bypass grafting has a potential risk of brain ischemia in moyamoya patients, but OPCAB may avoid this perioperative cerebral ischemic complication.

Moyamoya disease and Down syndrome.

Moyamoya disease is an obstruction of the internal carotids and of the afferent and efferent channels of Willis polygon, which causes a collateral circulation, responsible for the typical angiographic image of a "puff of smoke" (Moyamoya, in Japanese). Its etiology is unknown, and it might be congenital or acquired. The clinical features are cerebral ischemia, recurrent transient ischaemic attacks, sensorimotor paralysis, convulsions and migraine-like headaches. We report a 2 years and 9 months old boy with Down syndrome and Moyamoya disease who presented with focal convulsions.

Moyamoya syndrome in a child with down syndrome.

Secondary Moyamoya disease, also known as Moyamoya syndrome has been rarely associated with Down syndrome. In pediatric patients, the usual presentation is that of ischemic stroke. Here is reported a 4-year-old child with Down syndrome and Moyamoya syndrome who presented with acute-onset right hemiplegia. A high index of suspicion is necessary to make the diagnosis.

Anomalias congênitas do disco óptico associadas à doença de Moyamoya: relato de caso / Congenital anomalies of the optic disc associated with Moyamoya disease: case report

As anomalias congênitas do disco óptico podem estar associadas a anormalidades vasculares intracranianas. Relatamos o caso de um paciente de 9 anos com anomalia do disco óptico tipo morning glory em um olho e coloboma do disco óptico e coróide infrapapilar no outro. O paciente apresentava história pregressa de ataque isquêmico transitório e angioressonância cerebral consistente com doença de moyamoya. O presente relato ilustra a importante associação das anomalias congênitas do disco óptico com a doença de moyamoya. O exame fundoscópico de pacientes suspeitos pode trazer subsídios ao diagnóstico clínico, abrindo a possibilidade do reconhecimento precoce das anormalidades vasculares intracranianas encontradas no moyamoya, aumentando a perspectiva de eventual intervenção profilática frente às potenciais complicações.

Anestesia em paciente portadora de doença de moyamoya: relato de caso / Anesthesia in patient with moyamoya disease: case report

JUSTIFICATIVA E OBJETIVOS: A doença de moyamoya é uma vasculopatia cerebral progressiva rara, mais freqüentemente diagnosticada em populações asiáticas, mas que também vem sendo identificada no Brasil. Durante a sua vida, pacientes portadores desta doença podem ser submetidos aos mais variados tipos de procedimentos cirúrgicos. O anestesiologista deve entender a fisiopatologia da doença e instituir as medidas peri-operatórias mais adequadas, no intuito de melhorar o prognóstico destes pacientes. RELATO DO CASO: Paciente do sexo feminino, 22 anos, com insuficiência renal crônica, portadora da doença de moyamoya, submetida à intervenção cirúrgica para instalação de fístula arteriovenosa. A anestesia foi induzida com fentanil, propofol e atracúrio e mantida com sevoflurano. Durante o procedimento, a paciente foi mantida em normocapnia e normotermia. A extubação foi realizada e a paciente transferida à sala de recuperação pós-anestésica sem complicações. CONCLUSÕES: Este artigo apresenta os cuidados anestésicos dispensados a uma paciente portadora da doença de moyamoya.