ABSTRACT
ABSTRACT This report describes a case of retinal racemose hemangioma that first presented as a vitreous hemorrhage. The authors present the case of a 47-year-old woman with a sudden 5-day painless visual loss in her left eye. At the first visit, the best-correct visual acuities were 20/20 in the right eye and hand motions in the left eyes. Ultrasonography showed an attached retina and a massive vitreous hemorrhage. Pars plana vitrectomy was performed and a dilatation of large vessels was detected bulging from the optic disc. The best-correct visual acuities on day 30 postoperatively was 20/25 in the left eye. Fundus angiography and spectral-domain optical coherence tomography angiography showed anomalous arteriovenous communications with no intervening capillaries. The diagnosis was racemose hemangioma, an arteriovenous malformation of group 2 retina based on the Archer classification.
RESUMO Este relato descreve um caso de hemangioma racemoso da retina que se apresentou inicialmente como hemorragia vítrea. Os autores apresentam o caso de uma mulher de 47 anos com perda visual súbita e indolor 5 dias antes no olho esquerdo. Na primeira visita, a melhor acuidade visual corrigida foi de 20/20 no olho direito e movimentos das mãos no olho esquerdo. A ultrassonografia mostrou uma retina aderida e uma hemorragia vítrea maciça. Foi realizada vitrectomia pars plana, sendo detectada proliferação de grandes vasos salientes do disco óptico. A acuidade visual no dia 30 de pós-operatório foi de 20/25 no olho esquerdo. A angiografia de retina e a angiotomografia de coerência óptica de domínio espectral mostraram comunicações arteriovenosas anômalas sem capilares intermediários. O diagnóstico foi hemangioma racemoso, uma malformação arteriovenosa da retina do grupo 2 com base na classificação de Archer.
Subject(s)
Humans , Female , Middle Aged , Arteriovenous Malformations/complications , Retinal Vessels/abnormalities , Vitreous Hemorrhage/etiology , Angiography , Hemangioma/complications , Arteriovenous Malformations/surgery , Arteriovenous Malformations/diagnosis , Retinal Diseases , Retinal Vessels/diagnostic imaging , Vitrectomy , Vitreous Hemorrhage/surgery , Vitreous Hemorrhage/diagnosis , Ultrasonography , Tomography, Optical Coherence , Hemangioma/surgery , Hemangioma/diagnosisABSTRACT
Wyburn–Mason syndrome is associated with unilateral retinal racemose hemangioma. Rarely, it presents with bilateral and symmetrical grade of malformation. We describe a 37-year old male, who presented with Wyburn–Mason syndrome presenting with bilateral but asymmetrical retinal hemangioma. The eye with advanced grade of hemangioma was complicated with exudation, intraretinal fluid, neurosensory detachment, and reduced vision. He was treated with one intravitreal injection of bevacizumab, after which both the intraretinal fluid and neurosensory detachment resolved. His vision improved and was maintained till 1 year of follow-up.
ABSTRACT
We present a rare case of retinal racemose angioma complicated with fleeting macroaneurysm (MA). A 50-year-old female presented with diminution of vision in her right eye for 6 years. Fundus examination showed a racemose angioma with hemorrhagic MA temporal to the fovea in the right eye. On subsequent follow-ups, spontaneous thrombosis of MA was noted with the development of new MA inferior to the fovea, with intraretinal hemorrhage extending into the fovea. Focal laser to MA resulted in resolution of MA with improvement in vision. We report optical coherence tomography angiographic features of the fleeting MA in a case of racemose angioma.
ABSTRACT
PURPOSE: To describe a case of Wyburn-Mason Syndrome, which is a rare phakomatosis, and its associated optical coherence tomography findings. CASE SUMMARY: A 5-year-old boy was referred to our clinic for decreased vision in his right eye. He had nevus flammeus on his right forehead, upper eyelid, and cheek. His best corrected visual acuity was hand motion in the right eye, in which a relative afferent pupillary defect was noted. Fundus examination of the right eye showed marked dilation and tortuosity of retinal vessels, which could not be discriminated between arteries and veins on the posterior pole. Optical coherence tomography showed multiple large intraretinal vessels without involvement of the internal limiting membrane. Cerebral angiography revealed arteriovenous malformations (AVMs) and feeder vessels from the internal carotid artery to the ophthalmic artery, extending to the ethmoidal artery in the nasal cavity. CONCLUSIONS: Wyburn-Mason syndrome is a rare phakomatoses and shows unique retinal AVM. Misdiagnosis or undertreatment of Wyburn-Mason syndrome can lead to serious complications. Patients with facial nevus flammeus should undergo thorough ocular examinations to rule out this disorder. Optical coherence tomography is a useful method for understanding structural features of retinal vascular malformations in Wyburn-Mason syndrome.
Subject(s)
Child, Preschool , Humans , Male , Arteries , Arteriovenous Malformations , Carotid Artery, Internal , Cerebral Angiography , Cheek , Diagnostic Errors , Eyelids , Forehead , Hand , Membranes , Methods , Nasal Cavity , Neurocutaneous Syndromes , Nevus , Ophthalmic Artery , Port-Wine Stain , Pupil Disorders , Retinal Vessels , Retinaldehyde , Tomography, Optical Coherence , Vascular Malformations , Veins , Visual AcuityABSTRACT
The present work aimed to evaluate the pattern of CSF alterations in patients diagnosed with neurocysticercosis (NCC) in racemose form.Method This is a retrospective cohort study of patients with diagnosis of NCC in racemose form. CSF samples from 26 patients were analyzed. After patient-chart analysis was performed descriptive analysis of case studies and comparison between sexes in relation to variables were obtained with CSF by Mann-Whitney and Student’s t-tests.Results The sexes did not differ statistically when compared to pleocytosis in CSF. Eosinophils were present in 31% in samples while the ELISA test presented 80% sensitivity in this case series. Of the patient total, 24 presented a meningitis pattern with lymphocytic predominance.Conclusion There was no difference in inflammatory pattern between the sexes, with predominance of lymphocytic meningitis and 80% sensitivity by ELISA test of CSF patients with racemose form of NCC.
O objetivo deste trabalho foi avaliar o padrão de alterações do LCR de pacientes com diagnóstico de neurocisticercose (NCC) na forma racemosa.Método Trata-se de estudo de coorte retrospectiva, de pacientes com diagnóstico de forma racemosa da NCC. Foram analisadas amostras de LCR de 26 pacientes. Após análise de prontuário foi realizada análise descritiva da casuística e comparação entre sexos em relação às variáveis obtidas com o LCR por meio dos testes de Mann-Whitney e t-Student.Resultados Não houve diferença estatisticamente significante quando comparado à pleocitose no LCR entre os sexos. Houve presença de eosinofilorraquia em 31% das amostras e o teste ELISA apresentou sensibilidade de 80% nesta casuística. Do total de paciente, 24 apresentaram padrão de meningite com predomínio linfocítico.Conclusão Não houve diferença no padrão inflamatório entre os sexos, com predomínio de meningite linfocítica e sensibilidade de 80% ao teste ELISA do LCR de pacientes da forma racemosa de NCC.
Subject(s)
Adult , Aged , Female , Humans , Male , Middle Aged , Young Adult , Neurocysticercosis/cerebrospinal fluid , Cell Count , Cerebrospinal Fluid Proteins/analysis , Enzyme-Linked Immunosorbent Assay , Eosinophils , Glucose/cerebrospinal fluid , Leukocytosis/cerebrospinal fluid , Meningitis/cerebrospinal fluid , Neurocysticercosis/diagnosis , Retrospective Studies , Sex FactorsABSTRACT
Infection of the human central nervous system (CNS) by the larvae of Taenia solium, termed neurocysticercosis (NCC), is endemic in most developing countries, where it is a major cause of acquired seizures and other neurological morbidity, including neuropsychiatric symptoms. However, despite its frequent manifestation, some findings, such as cognitive impairment and dementia, remain poorly understood. Less commonly, NCC may affect the ventricular system and subarachnoid spaces and this form is known as extraparenchymal neurocysticercosis. A particular presentation of the subarachnoid form is called racemose cysticercosis, which has a progressive pattern, frequently leads to hydrocephalus and can be life-threatening. Here we review a case of the racemose variety of cysticercosis, complicated by hydrocephalus and reversible dementia, with remission of symptoms after derivation and that remained stable with use of dexchlorpheniramine. We discuss the challenges in diagnosis, imaging findings, treatment and follow-up of this disease.
A infecção do sistema nervoso central (SNC) pela larva da Taenia solium, intitulada neurocisticercose (NCC) é endêmica na maior parte dos países "em desenvolvimento", onde é a principal causa de convulsão adquirida, além de outras morbidades neurológicas, entre elas, sintomas neuropsiquiátricos. No entanto, apesar de manifestações neuropsiquiátricas serem frequentes, alguns achados, tais como comprometimento cognitivo e demência, continuam a ser mal compreendidos. Menos frequentemente, NCC pode afetar o sistema ventricular e espaços subaracnóideos e esta forma é conhecida como NCC extraparenquimatosa. Uma apresentação particular subaracnóidea, chamada cisticercose racemosa, é encontrada mais raramente, evolui de forma progressiva, associada a hidrocefalia e pode levar a morte. Neste artigo revisamos um caso da variedade racemosa de NCC, complicada com hidrocefalia e demência reversível que evoluiu com remissão dos sintomas após derivação ventricular e permaneceu estável com uso de dexclorfeniramina. Discutimos os desafios no diagnóstico, achados de imagem, tratamento e acompanhamento desta forma de doença.
Subject(s)
Humans , Neurocysticercosis , Taenia solium , Dementia , Histamine AntagonistsABSTRACT
Neurocysticercosis is an infection of the central nervous system caused by the larval form of the pork tapeworm Taenia solium. In the brain it occurs in two forms: parenchymal and extraparenchymal or racemose cysts. The clinical presentation of racemose cysts is pleomorphic, and is quite different from parenchymal cysticercosis. The clinical diagnosis of racemose cysts is quite challenging, with neuroimaging being the mainstay. However, the advent of newer brain imaging modalities has made a more accurate diagnosis possible. The primary focus of this article is racemose neurocysticercosis and its multitude manifestations, and includes a discussion of the newer diagnostic modalities and treatment options.
Subject(s)
Brain , Central Nervous System , Cysticercosis , Diagnosis , Neurocysticercosis , Neuroimaging , Taenia soliumABSTRACT
Cysticercosis is an infection caused by the larval stage of the tapeworm Taenia solium. The parasite may infect the central nervous system, causing neurocysticercosis (NCC). The clinical manifestations depend on load, type, size, location, stage of development of the cysticerci, and the host's immune response against the parasite. The racemose variety occurs in the ventricles or basal cisterns and is a malignant form. Mobile ventricular mass can produce episodic hydrocephalus on changing head posture with attacks of headache, vomiting, and vertigo, triggered by abrupt movement of the head, a phenomenon called Bruns' syndrome (BS). We report a patient with racemose NCC and BS.
A infecção por cisticercose é causada pelo estágio larval da Taenia solium. O parasita pode infectar o sistema nervoso central, causando neurocisticercose (NCC). As manifestações clínicas dependem da quantidade, tipo, tamanho, local, estágio de desenvolvimento do cisticerco e resposta imune do hospedeiro contra o parasita. A variedade racemosa ocorre nas cisternas ventriculares ou basais e é considerada uma forma maligna. O cisticerco móvel no ventrículo pode produzir hidrocefalia episódica com ataques de cefaléia, vômitos e vertigem, provocados pelo movimento abrupto da cabeça, fenômeno chamado de síndrome de Bruns (SB). Relataremos o caso de uma paciente com NCC racemosa com SB.
Subject(s)
Adult , Female , Humans , Cerebral Ventricles/parasitology , Headache/parasitology , Hydrocephalus/parasitology , Neurocysticercosis/diagnosis , Vertigo/parasitology , Vomiting/parasitology , Magnetic Resonance Imaging , Syndrome , Tomography, X-Ray ComputedABSTRACT
PURPOSE: The authors of the present case report observed a bilateral retinal racemose hemangioma which was located within the peripapillary area. CASE SUMMARY: A 17-year-old man presented with floaters in both eyes. Fundus revealed tortuous and anastomosed retinal vasculature around the optic disc. In addition, fluorescein angiography showed a non-leaking retinal arteriovenous anastomosis. Seven years after the initial visit, vitreous hemorrhage occurred in the patient's left eye, and then 1 year later, subretinal hemorrhage was found in his left eye. CONCLUSIONS: Because retinal racemose hemangioma can accompany vitreous hemorrhage and subretinal hemorrhage regardless of size, a routine periodic ophthalmic examination is recommended.
Subject(s)
Adolescent , Humans , Arteriovenous Anastomosis , Eye , Fluorescein Angiography , Hemangioma , Hemorrhage , Retinaldehyde , Vitreous HemorrhageABSTRACT
PURPOSE: To describe a case of Wyburn-Mason Syndrome, which is characterized by arteriovenous malformations in the central nervous system and the retina. CASE SUMMARY: A 13-year-old girl who underwent neurosurgical intervention for intraventricular hemorrhage was referred to our ophthalmic clinic because brain angiogram and MRI finding revealed arteriovenous malformations (AVMs) that extended from the orbit and optic chiasm along the optic pathway. Fundus and fluorescein angiography of the left eye showed marked dilation and tortuosity of the retinal vessels. Fluorescein angiographic findings showed rapid filling of all vessels and no dye leakage. CONCLUSIONS: Retinal racemose hemangioma should be considered for Wyburn-Mason syndrome, and the finding of retinal AVMs should warrant cerebral imaging studies including MRI and angiography.
Subject(s)
Adolescent , Humans , Angiography , Arteriovenous Fistula , Arteriovenous Malformations , Brain , Central Nervous System , Eye , Fluorescein , Fluorescein Angiography , Hemangioma , Hemorrhage , Neurocutaneous Syndromes , Optic Chiasm , Orbit , Retinal Vessels , RetinaldehydeABSTRACT
The so-called racemose cysticercosis, a rare variety of neurocysticercosis occurring in ventricles or basal cisterns, is characterized by abnormal growth of cystic membranes with degeneration of Taenia solium heads (scolex). Although lesions of this type are known to follow a progressive course even after ventricular shunting, there are limitations of case series treated with antiparasitic drugs, and the optimal duration of the treatment is not yet known. We report a case of relapsed racemose cysticercosis in the Sylvian fissure, who has been successfully treated with albendazole and adjunct corticosteroid for 4 weeks. The patient had been previously treated with praziquantel and ventriculoperitoneal shunt, and maintained on the anticonvulsant drug for one year, but returned to the hospital due to seizure recurrence. The patient has been well in seizure-free state for the follow-up 2 years after albendazole therapy. The subarachnoid racemose cysticercosis seems to respond well to treatment of corticosteroid along with prolonged albendazole.
Subject(s)
Humans , Albendazole , Antiparasitic Agents , Cysticercosis , Follow-Up Studies , Head , Membranes , Neurocysticercosis , Praziquantel , Recurrence , Seizures , Taenia solium , Ventriculoperitoneal ShuntABSTRACT
The so-called racemose cysticercosis, a rare variety of neurocysticercosis occurring in ventricles or basal cisterns, is characterized by abnormal growth of cystic membranes with degeneration of Taenia solium heads (scolex). Although lesions of this type are known to follow a progressive course even after ventricular shunting, there are limitations of case series treated with antiparasitic drugs, and the optimal duration of the treatment is not yet known. We report a case of relapsed racemose cysticercosis in the Sylvian fissure, who has been successfully treated with albendazole and adjunct corticosteroid for 4 weeks. The patient had been previously treated with praziquantel and ventriculoperitoneal shunt, and maintained on the anticonvulsant drug for one year, but returned to the hospital due to seizure recurrence. The patient has been well in seizure-free state for the follow-up 2 years after albendazole therapy. The subarachnoid racemose cysticercosis seems to respond well to treatment of corticosteroid along with prolonged albendazole.
Subject(s)
Humans , Albendazole , Antiparasitic Agents , Cysticercosis , Follow-Up Studies , Head , Membranes , Neurocysticercosis , Praziquantel , Recurrence , Seizures , Taenia solium , Ventriculoperitoneal ShuntABSTRACT
OBJECTIVE: The authors report a case of racemose neurocysticercosis presented with myelopathy and hydrocephalus. The cllinical symtoms were not relieved even after the operations with albendazole medication. METHODS: A 44 year-old man was transferred due to headache, quadriparesis and epileptic fit. Preoperative magnetic resonance image(MRI) on brain and computed tomographic cisternogram on brain and upper cervical cord showed racemose cysticercosis on vermis, medulla and upper cervical cord. Two times operations(vesicles removal and adhesiolysis on cerebrospinal fluid pathway) were done with albendazole medication(15mg/kg/day, 30 days). The clinical symtoms were not relieved. Ventriculoperitoneal(V-P) shunt procedure was done and the symtoms were improved. CONCLUSION: The cause of aggravated hydrocephalus might be inflammatory reaction of the host to acute destruction of parasites due to albendazole in the brain.
Subject(s)
Adult , Humans , Albendazole , Brain , Cerebrospinal Fluid , Cysticercosis , Headache , Hydrocephalus , Neurocysticercosis , Parasites , Quadriplegia , Spinal Cord DiseasesABSTRACT
PURPOSE: Racemose hemangiomas of the retina are rare developmental vascular anomalies with abnormal arteriovenous anastomoses. We report our observations of a retinal racemose hemangioma with marked dilation and tortuosity of the retinal vessels in the entire left retina. METHODS: In a 9-year-old boy with loss of vision and mild esotropia who presented with hugely dilated and tortuous retinal vessels in left retina, we examined fundus, fluorescein angiography, cerebral angiography, brain CT and MRI. RESULTS: Fundus and fluorescein angiography of the left eye showed marked dilation and tortuosity of the retinal vessels. Angiographic finding showed rapid filling of all vessels and no dye leakage. MRI finding revealed small tortuous vessels around left optic nerve, optic chiasm and tract. Brain CT and cerebral angiography showed nothing abnormal.
Subject(s)
Child , Humans , Male , Arteriovenous Anastomosis , Brain , Cerebral Angiography , Esotropia , Fluorescein Angiography , Hemangioma , Magnetic Resonance Imaging , Optic Chiasm , Optic Nerve , Retina , Retinal Vessels , RetinaldehydeABSTRACT
Cysticercosis is a relatively common disease in Korea. Racemose cysticercosis, a peculiar form of the neurocysticercosis usually occurs as meningeal forms and rarely involves the cerebral parenchyme. The authors present a case of cerebral parenchymal racemose cysticercosis in a 55 year-old man with generalized seizure. Brain CT and MRI showed a large multilobulated cyst in the right frontal lobe. At surgery, we removed cyst covered by milkish white, multiple septated membranes completely. Histological dignosis of surgical specimen was racemose cysticercosis without scolex.
Subject(s)
Humans , Middle Aged , Brain , Cerebrum , Cysticercosis , Frontal Lobe , Korea , Magnetic Resonance Imaging , Membranes , Neurocysticercosis , SeizuresABSTRACT
Racemose cysticercosis, which is almost exclusively found in intracranial cavity in vary rare occasions, was recognized in a 54-year-old Korean woman. Brain CT revealed a large lobated cystic mass without marginal enhancement, in the right frontotemporal lobe, together with irregularly distributed calcified spots in the parietal area. A 5x6x7cm sized irregularly lobated mass in the subarachnoid space, containing 38ml of xanthochromic fluid was removed. Pathologically the cyst was a racemose cysticercus without scolex. The serum and CSF of the patient showed positive reaction with antigen of Cysticercose celluosae by micro-ELISA for their specific IgG antibody. After the surgery, the patient was treated with Praziquantel for remaining worms(as revealed by calcified spots on brain CT). Follow-up examinations showed improvement in both clinical symptoms and brain CT findings, but Cysticercus-specific IgG antibody level did not fall to normal for 1 year. The rarity of racemose cysticercus infection, together with evidences of concomitant occurrence with C. cellulosae warranted one's case report.