A case of permanent vision loss due to a carotidcavernous fistula: importance of early diagnosis / Relato de caso de perda visual secundária a fístuda carótido-cavernosa: a importância de um diagnóstico adequado

ABSTRACT A 97-year-old female presented with spontaneous acute-onset palpebral hyperemia and edema of the right eye that had progressively worsen over the previous three days. These signs did not suggest possible carotid-cavernous fistula until a second examination 72 h later, during which the patient exhibited significant progression. Despite embolization, the patient exhibited sustained corneal edema, clots, and turbidity in the aqueous humor, which resulted in permanent visual loss. A greater level of clinical suspicion for possible carotid-cavernous fistula is warranted on initial presentation of palpebral hyperemia and edema to prevent possible irreversible vision loss.

RESUMO O objetivo deste relato é apresentar o caso de uma paciente de 97 anos com início agudo e espontâneo de hiperemia e edema palpebral. Estes sinais não levaram a uma suspeita diagnóstica de fístula carótido-cavernosa até um segundo momento, quando a paciente apresentou progressão importante do quadro clínico. Apesar da realização de tratamento efetivo com embolização da fístula, a paciente manteve alterações oculares como edema de córnea, coágulos e turvação no humor aquoso, e manteve perda visual definitiva.

A case of permanent vision loss due to a carotidcavernous fistula: importance of early diagnosis.

A 97-year-old female presented with spontaneous acute-onset palpebral hyperemia and edema of the right eye that had progressively worsen over the previous three days. These signs did not suggest possible carotid-cavernous fistula until a second examination 72 h later, during which the patient exhibited significant progression. Despite embolization, the patient exhibited sustained corneal edema, clots, and turbidity in the aqueous humor, which resulted in permanent visual loss. A greater level of clinical suspicion for possible carotid-cavernous fistula is warranted on initial presentation of palpebral hyperemia and edema to prevent possible irreversible vision loss.

Globe Protrusion and Interorbital Divergence in Syndromic Faciocraniosynostoses.

PURPOSE: The aim of this study was to quantify the relationship between globe protrusion (GP), length of medial and lateral walls, interzygomatic distance, and interorbital angle in syndromic faciocraniosynostoses. METHODS: The axial slices of computed tomography of the orbits of 43 patients with faciocraniosynostosis (31 with Crouzon syndrome and 12 with Apert syndrome) and 23 control subjects were measured with the Image J software. The following 5 variables were quantified: the degree of GP, the length of the medial and lateral walls, the interzygomatic distance, and the interorbital angle. RESULTS: Independent t-tests revealed significant differences between the patients and the controls regarding the mean values of all variables measured. The degree of GP was better correlated with the interorbital angle (r = 0.81) than with the medial wall length (r = 0.73). No correlation was found between GP and lateral orbital wall length. CONCLUSIONS: In syndromic faciocraniosynostoses, GP is highly correlated with the interorbital angle. The increment in the interorbital angle is a natural geometric consequence of the fact that a decrease in the orbital depth is not adequately compensated by an increase in the distance between the lateral orbit rims.

Upper eyelid entropion and dry eye in cicatricial trachoma without trichiasis / Entrópio de pálpebra superior e olho seco no tracoma cicatricial sem triquíase

PURPOSE: to evaluate the position of the upper eyelid margin and eye surface status in cicatricial trachoma without trichiasis (TS). METHODS: Slit-lamp biomicroscopy was employed to evaluate the location of the upper lid mucocutaneous transition of 156 eyes of 78 patients with trichiasis and of 130 eyes of 65 control subjects. For each eye the position of the upper lid mucocutaneous junction was graded with respect to the line of meibomian gland orifices into 3 categories: a) anterior, b) at the line, and c) posterior to the line. Ocular surface dye staining with lissamine green was performed in all eyes. All participants answered a questionnaire with queries on the presence and intensity of dry eye symptoms. RESULTS: In the eyes with trichiasis the location of the mucocutaneous transition was posterior to the meibomian gland line in 55 (35.3%), at the line in 77 (49.4%) and anterior to the line in only 24 (15.4%). In the control group these figures were: 5 (3.8%); 42 (42%) and 83 (63.8%). Lissamine staining and dry eye symptoms were also associated with trichiasis. CONCLUSION: Different degrees of upper lid entropion are already present in cicatricial trachoma even in the absence of trichiasis. Trichiasis is associated with lissamine green staining and dry eye symptoms. Conjunctivalization of the upper lid margin may play a role in the development of trachomatous dry eye.

OBJETIVOS: Avaliar a posição da margem palpebral superior e a superfície ocular no tracoma cicatricial sem triquíase (TS). MÉTODOS: A localização da transição mucocutânea da pálpebra superior foi avaliada com lâmpada de fenda em 156 olhos de 78 pacientes com triquíase e de 130 olhos de 65 controles. A posição da transição mucocutânea foi classificada em relação à linha das glândulas de Meibômio em três categorias: a) anterior, b) sobre a linha e c) posterior a linha. A superfície ocular de todos os olhos foi avaliada com verde de lissamina. Todos os participantes responderam ao questionário sobre a presença e intensidade dos sintomas relacionados ao olho seco. RESULTADOS: Nos olhos com triquíase a localização da transição mucocutânea foi posterior à linha das glândulas de Meibômio em 55 (35,3%), sobre a linha em 77 (49,4%) e anterior à linha em somente 24 (15,4%). No grupo controle essa distribuição foi 5 (3,8%); 42 (42%) e 83 (63,8%). A positividade ao corante de lissamina e sintomas de olho seco também foram associados à triquíase. CONCLUSÃO: Diferentes graus de entrópio de pálpebra superior estão presentes no tracoma cicatricial mesmo na ausência de triquíase. Triquíase está associada à positividade ao corante verde lissamine e sintomas de olho seco. A conjuntivalização da margem palpebral pode ser um fator no desenvolvimento do olho seco tracomatoso.

Upper eyelid entropion and dry eye in cicatricial trachoma without trichiasis.

PURPOSE: to evaluate the position of the upper eyelid margin and eye surface status in cicatricial trachoma without trichiasis (TS). METHODS: Slit-lamp biomicroscopy was employed to evaluate the location of the upper lid mucocutaneous transition of 156 eyes of 78 patients with trichiasis and of 130 eyes of 65 control subjects. For each eye the position of the upper lid mucocutaneous junction was graded with respect to the line of meibomian gland orifices into 3 categories: a) anterior, b) at the line, and c) posterior to the line. Ocular surface dye staining with lissamine green was performed in all eyes. All participants answered a questionnaire with queries on the presence and intensity of dry eye symptoms. RESULTS: In the eyes with trichiasis the location of the mucocutaneous transition was posterior to the meibomian gland line in 55 (35.3%), at the line in 77 (49.4%) and anterior to the line in only 24 (15.4%). In the control group these figures were: 5 (3.8%); 42 (42%) and 83 (63.8%). Lissamine staining and dry eye symptoms were also associated with trichiasis. CONCLUSION: Different degrees of upper lid entropion are already present in cicatricial trachoma even in the absence of trichiasis. Trichiasis is associated with lissamine green staining and dry eye symptoms. Conjunctivalization of the upper lid margin may play a role in the development of trachomatous dry eye.

Functional and cosmetic results of a lower eyelid crease approach for external dacryocystorhinostomy.

PURPOSE: External dacryocystorhinostomy is routinely performed through a cutaneous vertical incision placed on the lateral aspect of the nose. The lower eyelid crease approach has been seldom reported. The purpose of this study is to report the cosmetic and functional results of the lid crease approach for external dacryocystorhinostomy in a series of patients. METHODS: Prospective, interventional case series. Twenty-five consecutive patients (17 women) ranging in age from 3 to 85 years (mean ± SD= 44.84 ± 23.67) were included in the study. All patients but one underwent unilateral external dacryocystorhinostomy with a 10 to 15 mm horizontal incision placed on a subciliary relaxed eyelid tension line. The inner canthus was photographed with a Nikon D70S digital camera with a macrolens and resolution of 3008 x 2000 pixels at 1, 3 and 6 months after surgery. The resulting scar was judged from the photographs by 3 observers (ophthalmologist, plastic and head and neck surgeons) according to a four level scale (1= unapparent, 2= minimally visible, 3= moderately visible, 4= very visible). RESULTS: The surgery was easily performed in all patients with a 90.48% success. Three of the elderly patients (ages 61, 79 and 85 yr) developed mild lacrimal punctum ectropion, which resolved with conservative treatment. One patient had a hypometric blink which spontaneously recovered within one month. The mean score for scar visibility was 2.19 (1(st) mo), 1.65 (3(th) mo) and 1.44 (6(th) mo). CONCLUSIONS: The eyelid crease approach is an excellent option for external dacryocystorhinostomy. It leaves an unapparent scar since the first month after surgery, even in younger patients. The functional results are excellent and comparable to other techniques. Care should be taken in elderly patients with lower eyelid laxity in order to prevent lacrimal punctum ectropion.

Functional and cosmetic results of a lower eyelid crease approach for external dacryocystorhinostomy / Resultados cosméticos e funcionais na dacriocistorrinostomia com incisão transpalpebral

PURPOSE: External dacryocystorhinostomy is routinely performed through a cutaneous vertical incision placed on the lateral aspect of the nose. The lower eyelid crease approach has been seldom reported. The purpose of this study is to report the cosmetic and functional results of the lid crease approach for external dacryocystorhinostomy in a series of patients. METHODS: Prospective, interventional case series. Twenty-five consecutive patients (17 women) ranging in age from 3 to 85 years (mean ± SD= 44.84 ± 23.67) were included in the study. All patients but one underwent unilateral external dacryocystorhinostomy with a 10 to 15 mm horizontal incision placed on a subciliary relaxed eyelid tension line. The inner canthus was photographed with a Nikon D70S digital camera with a macrolens and resolution of 3008 x 2000 pixels at 1, 3 and 6 months after surgery. The resulting scar was judged from the photographs by 3 observers (ophthalmologist, plastic and head and neck surgeons) according to a four level scale (1= unapparent, 2= minimally visible, 3= moderately visible, 4= very visible). RESULTS: The surgery was easily performed in all patients with a 90.48 percent success. Three of the elderly patients (ages 61, 79 and 85 yr) developed mild lacrimal punctum ectropion, which resolved with conservative treatment. One patient had a hypometric blink which spontaneously recovered within one month. The mean score for scar visibility was 2.19 (1st mo), 1.65 (3th mo) and 1.44 (6th mo). CONCLUSIONS: The eyelid crease approach is an excellent option for external dacryocystorhinostomy. It leaves an unapparent scar since the first month after surgery, even in younger patients. The functional results are excellent and comparable to other techniques. Care should be taken in elderly patients with lower eyelid laxity in order to prevent lacrimal punctum ectropion.

OBJETIVOS: A dacriocistorrinostomia externa é classicamente realizada por meio de incisão cutânea nasal. Há poucos relatos sobre a incisão transpalpebral, posicionada no sulco infraciliar da pálpebra inferior. O presente estudo tem por objetivo relatar os aspectos cosméticos e funcionais utilizando a técnica transpalpebral. MÉTODOS: Série de casos, intervencional e prospectivo. Foram incluídos 25 pacientes consecutivos (17 mulheres) com idades variando de 3 a 85 anos (média ± dp= 44,84 ± 23,67), que apresentavam dacriocistite crônica. A dacriocistorrinostomia foi unilateral em 24 casos e bilateral em 1 caso. A incisão transpalpebral foi posicionada no sulco infraciliar medial, com extensão de 10 a 15 mm. Foram realizadas fotografias digitais do canto interno (Nikon D70S, lente macro, resolução de 3008 x 2000 pixels) nos tempos pós-operatórios 1, 3 e 6 meses. A visibilidade da cicatriz foi avaliada por 3 observadores (oftalmologista, cirurgião plástico e cirurgião de cabeça e pescoço) utilizando a seguinte escala: 1= imperceptível, 2= minimamente visível, 3= moderadamente visível, 4= muito visível. RESULTADOS: A DCR foi realizada sem dificuldades e com sucesso funcional em 90,48 por cento dos casos. A pontuação média para visibilidade da cicatriz foi de 2,19 (1º mês), 1,65 (3º mês) e 1,44 (6º mês). Houve 3 casos de ectrópio leve do ponto lacrimal que foram tratados conservadoramente. Um paciente apresentou piscar espontâneo incompleto, com resolução no primeiro mês de pós-operatório. CONCLUSÃO: A incisão transpalpebral é uma excelente via de acesso para realização da dacriocistorrinostomia. A cicatriz é pouco visível desde o primeiro mês após a cirurgia, mesmo em pacientes mais jovens. Os resultados funcionais são semelhantes ao das outras técnicas. Em pacientes mais idosos é necessário avaliação cuidadosa da frouxidão palpebral a fim de evitar a indução de ectrópio lacrimal.

Posterior scleral tuberculoma: case report.

Posterior scleral tuberculoma formation is an extremely rare condition. The few reports on scleral involvement in tuberculosis refer to cases of anterior scleritis. In the present manuscript we describe a patient who had rheumatoid arthritis and developed a large posterior scleral tuberculoma. The lesion provoked retinal detachment and visual loss and was diagnosed only after enucleation due to a misdiagnosis of choroidal melanoma.

Posterior scleral tuberculoma: case report / Tuberculoma escleral posterior: relato de caso

Posterior scleral tuberculoma formation is an extremely rare condition. The few reports on scleral involvement in tuberculosis refer to cases of anterior scleritis. In the present manuscript we describe a patient who had rheumatoid arthritis and developed a large posterior scleral tuberculoma. The lesion provoked retinal detachment and visual loss and was diagnosed only after enucleation due to a misdiagnosis of choroidal melanoma.

A formação de granuloma tuberculoso na esclera posterior é um evento extremamente raro. Os poucos relatos de acometimento escleral na tuberculose referem-se a casos de esclerite anterior. No presente trabalho é descrito um caso de granuloma escleral posterior em um paciente portador de artrite reumatóide. A lesão provocou descolamento da retina e perda visual e só foi diagnosticada após enucleação por suspeita de melanoma de coróide.

Orbital abscess secondary to acute dacryocystitis: case report.

Acute dacryocystitis usually induces preseptal infection. In rare instances the infection that is confined to the lacrimal sac can extend to the orbital contents resulting in orbital cellulitis. We present a case of intraconal abscess secondary to acute dacryocystitis and review the literature of orbital cellulitis resulting from acute lacrimal sac infection.

Orbital abscess secondary to acute dacryocystitis: case report / Abscesso orbitário secundário à dacriocistite aguda: relato de caso

Acute dacryocystitis usually induces preseptal infection. In rare instances the infection that is confined to the lacrimal sac can extend to the orbital contents resulting in orbital cellulitis. We present a case of intraconal abscess secondary to acute dacryocystitis and review the literature of orbital cellulitis resulting from acute lacrimal sac infection.

A dacriocistite aguda comumente evolui para infecção pré-septal. Raramente a infecção localizada no saco lacrimal pode estender-se ao conteúdo orbitário resultando em celulite orbitária. Apresentamos um caso de abscesso orbitário intraconal secundário à dacriocistite aguda e uma revisão de literatura de celulite orbitária causada por infecção aguda do saco lacrimal.

The role of major histocompatibility complex alleles in the susceptibility of Brazilian patients to develop the myogenic type of Graves' orbitopathy.

OBJECTIVE: To assess the frequency of the genetic markers HLA-DRB1 and DQB1 in patients with Graves' orbitopathy (GO) with and without extraocular muscle involvement. DESIGN: The frequencies of class II HLA-DRB1 and DQB1 allele groups were determined for 81 Brazilian patients with GO and 161 normal subjects. The patients were divided into myogenic and nonmyogenic groups based on the clinical characteristics of the orbitopathy and quantitative computed tomography analysis of the extraocular muscle (EOM) dimensions. MAIN OUTCOME: Compared to the frequency obtained for samples of normal subjects of the Brazilian population, HLA-DRB1 16 ( pc=0.008) was overrepresented in myogenic and HLA-DRB1 03 ( pc=0.02) in nonmyogenic patients. CONCLUSIONS: The association between the HLA-DRB1 16 and the myogenic subtype of GO suggests that EOM involvement in GO may be genetically predisposed.

Association between thyroid autoimmune dysfunction and non-thyroid autoimmune diseases.

PURPOSE: To assess the prevalence of the association between thyroid autoimmune dysfunction in patients with and without Graves orbitopathy and non-thyroid autoimmune diseases. METHODS: Retrospective review of the medical records of 254 consecutive patients with thyroid autoimmune disease with (n = 150) and without (n = 104) orbitopathy who had been followed at the same institution by ophthalmologists and general clinicians. All medical records contained information on any systemic diseases of the patients and a detailed description of their eye examinations. The mean follow-up period was 5.25 +/- 4.67 years. RESULTS: Non-thyroid autoimmune diseases were detected in 24 (9.4%) patients. Type 1 diabetes was the most prevalent non-thyroid autoimmune disease diagnosed in the patients without orbitopathy (7 patients, 6.7%). For the patients with orbitopathy, vitiligo was the most prevalent condition, affecting 6 patients (4%). Other diseases including systemic sclerosis, systemic lupus erythematosus, myasthenia gravis, Sjögren syndrome, and rheumatoid arthritis were seen in a few patients in both groups. The time intervals between the diagnoses of the orbitopathy and the non-thyroid autoimmune disease were highly variable, ranging from none (concomitance of the 2 conditions) to decades. CONCLUSIONS: The present data show that several non-thyroid autoimmune diseases may be associated with thyroid autoimmune dysfunction. Patients with Graves disease without orbitopathy are likely to develop polyglandular syndrome due to the occurrence of type 1 diabetes. Patients with Graves orbitopathy should be screened for other autoimmune conditions, especially vitiligo.

Nodular fasciitis presenting as a large mass in the upper eyelid.

A 3-year-old boy was examined for a large nodular fasciitis in the upper eyelid. The lesion was confined to the preseptal plane of the upper eyelid, provoking mechanical ptosis. Excision by an eyelid crease approach was sufficient to restore normal eyelid position.

Melanoma em cavidade anoftálmica secundária a evisceração: relato de 2 casos e revisão da literatura / Malignant melanoma in anophthalmic socket post-evisceration: case reports and literature review

Os autores relatam 2 casos de pacientes com melanoma em cavidade anoftálmica secundária a eviscerações ocorridas há 30 e 60 anos. Em ambos os casos a análise histopatológica mostrou que o tumor estava aderido a remanescentes esclerais. A implicação desses casos foi discutida no contexto das indicações de evisceração e enucleação.

Reversão de amaurose por neuropatia óptica em orbitopatia de Graves após descompressão orbitária: relato de caso / Reversal of blindness due to Graves' optic neuropathy after orbital decompression: case report

OBJETIVO: Descrever o caso de uma paciente portadora de orbitopatia de Graves com baixa visual no olho esquerdo há 9 meses e amaurose no direito há 20 dias secundária à neuropatia óptica. MÉTODOS: Foi realizada descompressão orbitária bilateral ínfero-medial por via transconjuntival. RESULTADOS: Após a cirurgia a paciente evoluiu lentamente com melhora progressiva da acuidade visual, obtendo 20/20 em ambos os olhos ao cabo de 10 meses. CONCLUSÕES: A descompressão orbitária é eficaz em restabelecer a visão em casos de amaurose por neuropatia óptica da orbitopatia de Graves com até 20 dias de instalação.

Sclerosing inflammation in the orbit and in the pterygopalatine and infratemporal fossae.

PURPOSE: To describe two cases with sclerosing inflammation in the orbit and in the pterygopalatine and infratemporal fossae and to review the literature of head and neck idiopathic inflammation. METHODS: Interventional case series. Two clinical case reports of sclerosing inflammation in the orbits and in the pterygopalatine and infratemporal fossae are presented. A review of the literature and discussion was performed. RESULTS: Both patients had histologic findings typical of chronic orbital sclerosing inflammation, including mature lymphocytes associated with dense collagen deposition and occasional lymphoid follicles. Bone erosion was present in one patient. CONCLUSIONS: The clinical findings of this case series concur with the literature that idiopathic inflammation can be found simultaneously in the orbit and in the infratemporal and pterygopalatine fossae. These cases of idiopathic inflammation are often clinically indistinguishable from malignant neoplasms.

Quantitative comparison between upper eyelid retraction induced voluntarily and by Graves orbitopathy.

PURPOSE: To compare 2-dimensional palpebral fissure measurements of normal subjects with voluntary upper eyelid retraction and patients with Graves upper eyelid retraction. METHODS: Three groups of monocular palpebral fissure images were measured. Group 1 consisted of 32 images of healthy subjects with the upper eyelid in a normal position of rest. Group 2 included the same subjects with voluntary upper eyelid retraction. Group 3 included 45 images of patients with Graves upper eyelid retraction. Fissure images were acquired with a digital camera and transferred to a Macintosh computer. For all images, three variables were quantified with NIH Image software: the midpupil-to-upper eyelid margin distance and the nasal and temporal upper areas of the palpebral fissure. RESULTS: Voluntary upper eyelid retraction significantly increases the absolute difference between the temporal and nasal areas of normal subjects. The disproportion between the lateral and medial areas of the palpebral fissure was greater for the patients with Graves than for the control group with voluntary eyelid retraction, even though there was no difference between the midpupil-to-upper eyelid margin distance of these two groups. Overall, there was a positive correlation between the midpupil-to-upper eyelid margin distance and absolute difference between the temporal and nasal areas (r = 0.75, P < 0.0001). CONCLUSIONS: In normal subjects, voluntary upper eyelid retraction increases the disproportion between the lateral and medial aspects of the fissure. However, even for the same amount of eyelid elevation, the mean disproportion of the control group with voluntary eyelid retraction is smaller than the disproportion displayed by patients with Graves. These findings suggest that an enhanced lateral retraction is a normal finding associated with levator muscle contraction.