Frozen Section Histoanatomy of the Peritarsal Eyelid.

BACKGROUND: Mohs surgery of eyelid skin cancers requires detailed knowledge of anatomy for precise surgery and accurate evaluation of histology. OBJECTIVE: To review the histology of the peritarsal eyelid using frozen sections as encountered intraoperatively by Mohs surgeons. METHODS: The authors review the literature describing the anatomy and histology of the peritarsal eyelid from the lens of a Mohs surgeon. Histology from select Mohs cases is used to frame the discussion of the microanatomy of this region. RESULTS: The peritarsal eyelids contain a unique mixture of skin, muscle, tarsus, glandular tissue, and conjunctiva. The histologic appearance of many of these structures differs from skin found outside of this anatomic region. Tumors of the eyelid and periocular region may mimic normal histologic structures found within the peritarsal eyelid. CONCLUSION: The peritarsal eyelids have unique anatomy and associated histologic structures. Knowledge of the detailed histoanatomy is required for confident execution of Mohs surgery in this anatomic region.

Maxillary Ameloblastoma with Local Recurrence, Orbital Invasion, and Systemic Metastases: A Case Report and Review of the Literature.

Introduction: Maxillary ameloblastoma is a rare, slow-growing odontogenic tumor that can recur after surgical excision, be locally aggressive, and rarely develop systemic metastases. We describe the course and management of a patient with recurrent maxillary ameloblastoma with orbital invasion and systemic metastases, the fourth case of its kind to be described in the literature. Case Presentation: A 50-year-old female presented with left hyperglobus. A diagnosis of maxillary ameloblastoma was made based on biopsy and neuroimaging with MRI and CT. Surgical management included partial maxillectomy with orbital floor reconstruction, given the orbital invasion. Three years later, left hyperglobus recurred, and the patient was found to have orbital recurrence and lung metastases on PET imaging. The lung and orbital lesions have responded well to chemoradiation therapy without surgical intervention. Conclusion: Maxillary ameloblastoma is a rare tumor that typically arises from odontogenic tissues. Though considered benign, they can recur and in the case of our patient, metastasize. Complete surgical excision with wide surgical margins is associated with a shorter average time to recurrence and a lower incidence of metastasis. Cases of metastasis are managed with chemotherapy with or without adjuvant radiotherapy. Precision medicine may play a role in managing this entity in the future, given the discovery of differing profiles of maxillary ameloblastoma compared to mandibular. Ophthalmologists should be aware of this tumor as it can invade the orbit, resulting in significant ocular morbidity and mortality.

Changes in retinal nerve fiber layer, ganglion cell complex, and ganglion cell layer thickness in thyroid eye disease: A systematic review.

Thyroid eye disease (TED) is characterized by progressive orbital inflammation. In severe cases, it can lead to dysthyroid optic neuropathy (DON), involving death of retinal ganglion cells and permanent vision loss. Imaging with optical coherence tomography (OCT) has enabled noninvasive examination of the structural impact of the disease, including of the retinal nerve fiber layer (RNFL), the ganglion cell layer (GCL), and the inner plexiform layer (IPL). In this study, we systematically review 20 studies (897 patients, 1430 eyes) to evaluate changes in retinal thickness in patients with TED, stratified by severity. Most studies found a decrease in thickness in peripapillary RNFL (pRNFL), non-pRNFL, combined RNFL/GCL/IPL, or combined GCL/IPL when comparing patients with DON to healthy controls or to patients without DON. However, results were equivocal regarding differences between non-DON TED patients and controls. In addition, several studies reported a postoperative decrease in pRNFL thickness after orbital decompression surgery. Future studies examining OCT changes of retinal thickness along with disease progression are needed to assess the potential of OCT in diagnosis and management of patients with TED.

The Impact of Primary Tumor Site on Survival in Mycosis Fungoides.

Mycosis fungoides (MF) is the most common cutaneous T-cell lymphoma (CTCL), but little is known about the influence of anatomic location of the primary disease site on overall survival (OS) and disease-specific survival (DSS). The purpose of this study was to examine the significance of primary tumor site on survival in MF. A search of the Surveillance, Epidemiology, and End Results (SEER) database was conducted for patients with a diagnosis of MF with a specified primary site from 2000 to 2019. Prognostic factors including demographic and tumor characteristics were examined using Cox regression models. Further research is needed to fully investigate primary disease site as a prognostic indicator, including a deeper dive into MF of all stages and subtypes.

Cavernous sinus thrombosis with bilateral orbital vein involvement and diffuse ischemic retinopathy / Trombose do seio cavernoso com envolvimento bilateral das veias orbitais e retinopatia isquêmica difusa

ABSTRACT A 53-year-old man with a 3-day history of periorbital swelling and vision loss in the left eye was found to have septic cavernous sinus thrombosis with bilateral orbital vein involvement causing congestive orbitopathy. He was treated with an emergent canthotomy and cantholysis, intraocular pressure-lowering drops, antibiotics, anticoagulation, and serial examinations. Optical coherence tomography ultimately revealed diffuse ischemic destruction of both layers of the retina, which suggested occlusion of the ophthalmic artery or the short posterior ciliary arteries and central retinal artery without intracavernous internal carotid artery involvement. The patient remained without light perception in the left eye after treatment.

RESUMO Um homem de 53 anos, com história de 3 dias de edema periorbital e perda de visão no olho esquerdo, apresentou trombose séptica do seio cavernoso com envolvimento bilateral das veias orbitais, causando uma orbitopatia congestiva. O paciente foi tratado com uma cantotomia e cantólise de emergência, colírios para redução da pressão intraocular, antibióticos, anticoagulantes e exames seriados. A tomografia de coerência óptica finalmente demonstrou destruição isquêmica difusa de ambas as camadas da retina, sugerindo uma oclusão da artéria oftálmica ou das artérias ciliares posteriores curtas e da artéria retiniana central, com ausência de envolvimento do segmento intracavernoso da artéria carótida interna. O paciente permaneceu sem percepção luminosa no olho esquerdo.

Tobacco counseling in the setting of thyroid eye disease / Aconselhamento sobre o uso de tabaco no contexto de doenças oculares associadas à tireoide

ABSTRACT Purpose: To collect data on the rate and efficacy of tobacco counseling sessions delivered by ophthalmologists under the setting of patients with thyroid eye disease. Methods: We analyzed the electronic medical records of a digital cohort of patients who visited ophthalmologists at the University of Pennsylvania Health System from 2012 to 2017 with reference to the International Classification of Disease (ICD) codes for Graves' disease, thyrotoxic exophthalmos, and/or thyroid eye disease. Tobacco histories were recorded at the first and last ophthalmology office visits or the most temporally proximal encounter in packs/day (ppd), and each ophthalmology visit note was analyzed to validate the occurrence of tobacco counseling. Results: A total of 435 patients met our study inclusion criteria, of which 72 (16.6%) were active smokers at the time of their first visit. Only 57 (79.2%) of these active smokers had recorded smoking burdens, 34 (59.6%) of which received at least one form of recorded tobacco counseling session. Nine (26.5%) of the subjects who received tobacco counseling and 1 (4.3%) of those who did not have a recorded counseling, quit smoking (risk difference of 22.1%; 95% CI, [1.7%, 39.1%]; p=0.04). In addition, 17 (50.0%) of the subjects who received counseling and 7 (30.4%) of those who did not have a recorded counseling, reduced their ppd consumption (risk difference of 19.6%; 95% CI [-6.3%, 41.3%]; p=0.18). Overall, 14 (25.5%) out of the 55 ophthalmologists who were active smokers had recorded evidence of tobacco counseling. Conclusions: Our cumulative results provide the consequence of both missed opportunities for tobacco counseling as well as its efficacy in the setting of thyroid eye disease.

RESUMO Objetivo: Fornecer informações sobre a ocorrência e a eficácia do aconselhamento sobre o uso de tabaco por oftalmologistas a pacientes com doenças oculares associadas à tireoide. Métodos: Analisamos os prontuários médicos eletrônicos de uma coorte digital de pacientes atendidos por oftalmologistas no Sistema de Saúde da Universidade da Pensilvânia entre o início de 2012 e o final de 2017 com os códigos da Classificação Internacional de Doenças (CID) para a doença de Graves, exoftalmia tireotóxica ou doença ocular associada à tireoide. Os históricos de uso de tabaco foram registrados na primeira e na última visita ao consultório de Oftalmologia, ou na visita mais próxima no tempo. A quantidade de maços/dia (mpd) e todas as anotações feitas nas visitas ao consultório de Oftalmologia foram analisadas para aconselhamento sobre o uso de tabaco. Resultados: Um total de 435 indivíduos preencheram os critérios de inclusão, dos quais 72 (16,6%) estavam fumando ativamente no momento do primeiro encontro. Apenas 57 (79,2%) desses indivíduos que fumam ativamente registraram queixas relacionadas ao tabagismo, sendo que 34 (59,6%) deles receberam alguma forma de aconselhamento sobre o uso de tabaco. Ao todo, 9 (26,5%) indivíduos dentre os que receberam aconselhamento sobre tabaco e 1 (4,3%) que não teve aconselhamento registrado pararam de fumar (diferença de risco de 22,1%; IC 95%, [1,7%, 39,1%]; p=0,04). Dentre aqueles que receberam aconselhamento, 17 (50,0%) reduziram seus mpd, além de 7 (30,4%) daqueles que não tiveram aconselhamento (diferença de risco de 19,6%; IC 95% [-6,3%, 41,3%]; p=0,18). No geral, 14 (25,5%) dos 55 oftalmologistas que tiveram um paciente fumante ativo registraram evidências de aconselhamento sobre o uso de tabaco. Conclusões: Os resultados deste estudo revelam tanto as oportunidades perdidas de aconselhamento sobre o uso do tabaco quanto a eficácia do aconselhamento no contexto de doenças oculares associadas à tireoide.

Assessment of the Abbreviated National Eye Institute Visual Function Questionnaire (NEI VFQ 9) in blepharoptosis and dermatochalasis / Avaliação do Questionário Abreviado de Função Visual do National Eye Institute (NEI VFQ 9) em blefaroptose e dermatocálase

ABSTRACT Purpose: To evaluate the Abbreviated National Eye Institute Visual Function Questionnaire (NEI VFQ 9), which is shorter than those previously published, as a tool for assessing vision-related quality of life in patients with ptosis and dermatochalasis. Methods: This is a cohort study of 46 patients who underwent blepharoptosis and/or upper eyelid blepharoplasty surgery by a single surgeon (CN) in 2013 in a public, academic, ambulatory care referral center. Patients included 29 who underwent blepharoplasty, 11 who underwent ptosis surgery, and 6 who underwent combined surgery. The NEI-VFQ 9 was administered pre- and postoperatively, and the composite scores were compared using Student's t-test. Survey duration was timed in a subset of patients. The hypothesis was that the NEI VFQ 9 could detect a statistically significant improvement in composite score after surgical intervention. Results: The mean pre- and postoperative NEI VFQ 9 composite scores were 74.9 and 86.8, respectively, in the blepharoplasty-only group (P<0.0001), 72.07 and 86.41, respectively, in the ptosis-only group (P=0.004), and 75.8 and 87.2, respectively, in the combined group (P=0.022). There was no correlation between the gain in composite score and the change in upper eyelid margin to reflex distance. Twenty-five patients were timed filling out the survey, and the mean was 2.3 min. Conclusions: The NEI VFQ 9 consistently demonstrates a significant increase in visual function for blepharoptosis and dermatochalasis patients. Thus, it may be a useful tool for assessing vision-related quality of life in patients with ptosis and dermatochalasis.

RESUMO Objetivo: Avaliar o Questionário Abreviado de Função Visual do National Eye Institute (NEI VFQ 9) como uma ferramenta para avaliar a visão relacionada com a qualidade de vida (QoL) em pacientes com blefaroptose e dermatocálase. Método: Estudo de coorte de blefaroptose e dermatocálase pacientes tratados por um único cirurgião (CN) em 2013. O acompanhamento foi de 2-3 meses. Pacientes em um centro de referência acadêmico para a atenção pública ambulatorial com ptose funcional e/ou dermatocálase de pálpebra superior foram avaliados prospectivamente utilizando o NEI-VFQ 9. Quarenta e seis pacientes completaram o estudo: 29 pacientes se submeteram somente à blefaroplastia, 11 apenas à cirurgia de ptose, e 6 pacientes à cirurgia combinada. Foram excluídos pacientes com outra cirurgia simultânea da pálpebra. O teste foi repetido em uma visita pós-operatória. A duração teste foi cronometrada em um subgrupo de pacientes. Escores compostos pré e pós-operatórios foram comparados com o teste T de Student. O desfecho principal foi a pontuação composta. Os autores propõem que a NEI VFQ 9 seria capaz de detectar uma melhora estatisticamente significativa na pontuação composta após a intervenção cirúrgica. Resultados: No grupo de blefaroplastia apenas, o NEI VFQ 9 apresentou a média composta de 74,9 e 86,8 no pré e pós-operatório, respectivamente (p<0,0001). Para os pacientes submetidos apenas à cirurgia de ptose, a pontuação média composta foi 72,07 e 86,41, no pré e pós-operatório, respectivamente (p=0,004). No grupo de cirurgias combinadas combinação, a pontuação composta média pré-operatória foi de 75,8, e a pós-operatória foi de 87,2 (p=0,022). Não houve correlação entre o ganho de pontuação composta e a mudança no MRD1. Vinte e cinco pacientes foram cronometrados ao preencher o teste, a média foi de 2,3 minutos. Conclusões: O NEI VFQ 9 demonstra consistentemente um aumento na função visual para pacientes submetidos à cirurgia de blefaroptose e dermatocálase, sendo um instrumento de pesquisa mais curto do que daqueles publicados anteriormente.

Síndrome de Fraser: relato de caso nas vias lacrimais / Fraser Syndrome: case report in lacrimal system

A síndrome de Fraser é uma condição sistêmica caracterizada por criptoftalmo, sindactilia e anomalia da genitália, podendo se associar com alterações dos rins, do ouvido, do nariz, da laringe e do esqueleto. O criptoftalmo pode representar um achado isolado, representado por herança autossômica dominante, associado a outras anomalias congênitas, relatado como herança autossômica recessiva. Criança do sexo feminino, 9 meses, avaliada no ambulatório de vias lacrimais da Universidade Federal de São Paulo. Filha de pais consanguíneos. Ao exame, foram observados criptoftalmo total à esquerda, epífora em olho direito associada à secreção mucopurulenta, nariz em sela, implantação baixa das orelhas, malformação de conduto auditivo, aumento de grandes lábios e sindactilia de mãos e pés. A tomografia de crânio evidenciou braquicefalia ausência de septo pelúcido, proeminência dos ventrículos laterais, importante falha óssea na calota craniana, presença de afilamento do manto tecidual cerebral, fossa posterior pequena, desorganização do segmento anterior, afacia e descolamento total da retina.

Fraser syndrome is a systemic condition characterized by cryptophthalmos, syndactyly and abnormal genitalia, which may be associated with urinary tract, ear, nose, larynx and skeletal abnormalities. Cryptophthalmos can be an isolated finding (that has been reported as an autosomal dominant trait) or associated with other congenital anomalies (reported as an autosomal recessive disorder). Child, female, nine month of life, evaluated in the lacrimal setor of Federal University of São Paulo. Child of consanguineous parents. Her physical examination showed total unilateral cryptophthalmos (left side), epiphora (right side) with mucopurulent discharge, depressed nasal bridge, low set ears, atresia of the external auditory canal, prominent labia majora and syndactyly of the fingers and toes. Ocular ultrasonography showed brachycephaly, absence of septu pellucidum prominence of the lateral ventricles, a major bone defect in the skull, the presence of thinning of the mantle tissue of the brain,a reduced anterior-posterior ocular diameter, anterior segment disorganization, absence of the lens and total retinal detachment in the left eye.