Orbital manifestations of granulomatosis with polyangiitis: 12-year experience in Mexico City.

PURPOSE: To present the clinical picture and radiological characteristics of orbital manifestations of granulomatosis with polyangiitis in a Mexican hospital and compare them with worldwide literature. METHODS: Retrospective, observational study from January 2007 to January 2019. An electronic file review was performed. All patients with the diagnosis of granulomatosis with polyangiitis (GPA) in the Oculoplastics department were included. Ophthalmological examination, biopsy, antibodies and tomographical results were included in the data collected. Descriptive statistics were obtained. RESULTS: One hundred and one patients in our institute had a diagnosis of GPA. Only 15 (14.8%) had orbital manifestations and were included in our study. 73.3% were female with a median age of 46.20 years (17-81). Diagnostic delay was on average 6 months. Only 6.7% had bilateral manifestations. No past medical history was found in 40%, 20% had a previous diagnosis of systemic GPA. Pain was reported in 73.3%. Increase of volume (proptosis or diffuse orbital mass) was present in 86.7%. C-ANCA antibodies were positive in seven patients (46.7%). In tomography, lacrimal gland involvement was present in 33.3% and diffuse orbital mass was present in 66.6%. Definite diagnosis was done with biopsy in 93.3%. One patient died from complications of GPA. CONCLUSIONS: Ophthalmologists should consider this rare disease as a differential diagnosis of orbital tumors, as it may have different clinical manifestations, even in non-Caucasian population. When in doubt, biopsy is always valuable. The statistics at our reference center correspond with statistics reported worldwide.

Eyelash hair transplantation with strip composite eyebrow graft: an enhancing technique in tarsoconjunctival flap eyelid reconstruction procedure.

Purpose: To describe and evaluate the eyelash transplantation with strip composite eyebrow graft to improve eyelid anatomy in tarsoconjunctival flap eyelid reconstruction procedure. Methods: Twenty two patients with eyelid tumors managed by local excision and reconstruction using tarsoconjunctival flap reconstruction procedure were included. Eyelash graft was performed during flap division by harvesting a graft from the eyebrow and creating a recipient pocket in the receptor eyelid. Patients had a 1-year follow up. Eyelash orientation and follicle survival rate were recorded. Demographic variables were analyzed with descriptive statistics. For eyelash follicles survival rate assessment, we evaluated the number of follicles found in the first postoperative day with the follicles found at the end of the study using the Wilcoxon signed rank test. P < 0.05 was considered statistically significant. Results: All eyelash grafts showed a good follicle orientation and no cases of eyelashes misdirection toward the eyeball were reported. No statistical differences (p > 0.05) were found in Wilcoxon signed rank test when comparing the number of follicles present at the first postoperative day and the number of follicles present at the 12th postoperative month, meaning that the vast majority of follicles survived at the end of the study. Graft ischemia, necrosis or infection were not observed. Conclusions: Tarsoconjunctival flap is an outstanding option for eyelid reconstruction and eyelash transplantation with an eyebrow composite graft is an excellent procedure to enhance and recreate a normal eyelid.

Management of Ocular Surface Squamous Neoplasia with Topical and Intralesional Interferon Alpha 2B in Mexicans.

INTRODUCTION: Ocular surface squamous neoplasia (OSSN) is the most common nonpigmented ocular surface neoplasm and it can originate from the conjunctiva and/ or corneal epithelium. Since the rate of recurrence after surgical excision is high, chemotherapeutic and immunotherapeutic agents such as interferon alpha-2b (IFN α-2b) have been used for its treatment. OBJECTIVE: Our objective is to describe the demographic variables of patients with OSSN treated with IFN α-2b and to describe the outcomes of its use in Mexican patients, treated between 2011 and 2017 at Instituto de Oftalmología Fundación Conde de Valenciana. MATERIALS AND METHODS: This is a non-randomized retrospective interventional case series. We reviewed the files of patients older than 18 years, with clinical and/or histopathological diagnosis of OSSN, treated with IFN α-2b alone or in combination with surgery, with a minimum of 6 months follow-up. We studied demographic variables, such as gender, age, eye affected and associated risks factors; we also studied the type of therapy with interferon (topical, intralesional and/or associated with surgery), time of treatment, time of follow up, recurrences and complications. RESULTS: A total of 39 patients were included. The mean age of diagnosis was 61 ± 16 years and OSSN was more frequent in males (64.1% of cases). Most of the cases (43.6%) had no identifiable risk factors. The mean time of IFN α-2b treatment was 5 ± 2 months, being higher in the intralesional group (6.5 months) than in the topical group (4.2 months). The clinical resolution with the use of IFN α-2b was evidenced between 0.5 and 10 months, being achieved in 87.1% of the patients. CONCLUSIONS: The use of IFN α-2b is effective for the treatment of OSSN with a high success rate (87.1%) and a low incidence of relapse (5 cases), with no reported complications during follow up.

Alveolar soft part sarcoma of the orbit, a case report of a rare tumor.

BACKGROUND: Alveolar Soft Part Sarcoma is a rare malignant tumor of uncertain histogenesis, representing 0.5-1% of all soft tissue tumors. It occurs predominantly in head and neck regions, especially the orbit and the tongue, in infants, causing no specific symptoms for an extended period of time. CASE: A four year old girl was brought to consultation because of persistent swelling of her left upper and lower eyelid for the past six months. A 4 mm proptosis was noted on her left eye with significant upwards displacement of the globe. A firm, not pulsating and non tender mass was palpated, and motility examination revealed deficient abduction and infraduction. Visual acuity was 20/40 and 1/200 in the right and left eye, respectively. The computed tomography revealed an extraconal mass on the inferotemporal aspect of the left orbit, with no bony erosion or globe invasion. An excisional biopsy was made, finding a tumor with nests of clusters of large polygonal cells, separated by fibrous septa and a sinusoidal vascular channel. An Alveolar Soft Part Sarcoma was diagnosed. Our patient had a favorable post-operative follow up, and oncologic evaluations have not shown metastasis or local recurrences. Observations and Conclusions: Alveolar Soft Part Sarcoma is a rare malignant tumor of indolent course, but with propensity to distant metastases, making early diagnose and long term follow up necessary.

Congenital upper eyelids ectropion in Down's syndrome.

Congenital bilateral ectropion of the upper eyelids is a rare, benign condition reported in ophthalmic literature. It is more frequently associated with Down's syndrome, ichthyosis, and sporadic cases in newborns from black population. We report three cases of congenital bilateral upper eyelid ectropion associated with Down's syndrome. Management of these patients usually requires medial and lateral canthoplasties, full-thickness pentagonal resection of the upper eyelids and placement of skin grafts. We present herein the evolution of one of these patients and we will discuss the mechanism of the eyelid ectropion and its treatment.

[Periocular foreign body: Two clinical cases with different management short title: periocular foreign body]. / Cuerpo extraño periocular: dos casos clínicos con diferente manejo.

BACKGROUND: In many cases, the presence of periocular foreign bodies continues to be a diagnostic challenge and a dilemma regarding their management in most cases. The key lies in the type of material of the foreign body and its location in the orbit to determine its management. CLINICAL CASES: We present two cases of periocular foreign body; a description of the clinical presentation and their treatment are given in each case. A review and discussion of treatment is also included. CONCLUSION: It is important to know the indications for the medical and surgical management for periocular foreign bodies to offer an individualized and direct treatment for each patient.

Characterization of PABPN1 expansion mutations in a large cohort of Mexican patients with oculopharyngeal muscular dystrophy (OPMD).

Oculopharyngeal muscular dystrophy (OPMD) is an autosomal-dominant, adult-onset disorder defined by blepharoptosis, dysphagia, and proximal muscle weakness. OPMD arises from heterozygous expansions of a trinucleotide (GCN) tract situated at the 5' region of the polyadenylate RNA binding protein 1 (PABPN1) gene. The frequency of a particular (GCN) expansion in a given population of patients with OPMD is largely influenced by the occurrence of founder mutations. Analysis of large groups of patients with OPMD from different ethnic origins will help to estimate the relative contribution of each expanded allele to the disease. The purpose of this study was to characterize the type of PABPN1 expanded alleles in a large cohort of OPMD individuals from Mexico. Molecular analysis procedures included genomic DNA extraction from blood leukocytes in each patient followed by PCR amplification of PABPN1 exon 1, and direct nucleotide sequencing of PCR products. A total of 102 patients with OPMD were included in the study. Expanded PABPN1 gene alleles were demonstrated in all patients: 65% (66 out of 102) had a (GCN)15 expansion while the remaining 35% (36 out of 102) exhibited a (GCN)13 expansion. This is one of the largest series of molecularly confirmed patients with OPMD in a non-Caucasian population. Ethnic-specific differences in the prevalence of specific PABPN1 expansions must be considered for genetic screening of patients with OPMD.

Orbital positron emission tomography/computed tomography (PET/CT) imaging findings in Graves ophthalmopathy.

BACKGROUND: We aimed to describe orbital positron emission tomography/computed tomography (PET/CT) imaging findings, both structural and metabolic, in different clinical stages of Graves ophthalmopathy (GO). This prospective, observational, cross-sectional study examined 32 eyes of 16 patients with GO. METHODS: Patients were assessed with a complete ophthalmological evaluation and assigned a VISA classification for GO. All patients underwent serum thyroid hormone measurement, antibody profile, and 18-fluorodeoxyglucose positron emission tomography/computed tomography (18-FDG PET/CT) of the orbits. The 18-FDG uptake on PET images was expressed in terms of maximum standard uptake value (SUVmax). CT images were analyzed, and orbital structures were measured in millimeters. Vision, inflammation, strabismus, and overall appearance were assessed according to the VISA classification system, thyroid hormone levels, antibody values, 18-FDG uptake, and thickness of orbital structures. RESULTS: Altogether, 32 eyes of 16 patients (10 women, 6 men; mean age 44.31 ± 13 years, range 20-71 years) were included. Three patients were hypothyroid, seven were euthyroid, and six were hyperthyroid. CT measurements of extraocular muscle diameter were elevated (P < 0.05), and muscle 18-FDG uptake values were increased. Eyes with a clinical VISA inflammation score of ≤ 4 had an average extraocular muscle SUVmax of 3.09, and those with a score of ≥ 5 had an average SUVmax of 3.92 (P = 0.09), showing no clear correlation between clinically observed inflammation and 18-FDG uptake. 18-FDG uptake values also did not show a correlation with extraocular muscle diameter as measured by CT (R2 = 0.0755, P > 0.05). CONCLUSIONS: We demonstrated a lack of correlation between 18-FDG extraocular muscle uptake and either clinical inflammation score or muscle diameter. Although 18-FDG uptake has been used as an inflammation marker in other pathologies, inflammation in GO may be clinically detected in PET/CT-negative cases, and cases with negative clinical findings may show inflammation on PET/CT. Clinical evaluation is mandatory but may be insufficient and inaccurate for classifying GO. A larger and homogeneous sample size and further research is needed to define the role of PET/CT in detecting, grading, and follow-up of GO to optimize treatment of the inflammatory stage respect clinical methods currently used.

18-Fluorodeoxyglucose Uptake by Positron Emission Tomography in Extraocular Muscles of Patients with and without Graves' Ophthalmology.

Objective. To compare 18-fluorodeoxyglucose (FDG) uptake by positron emission tomography (PET) in extraocular muscles (EOMs) of patients with Graves' ophthalmopathy (GO) versus patients without GO. Design. Prospective, observational, comparative, and cross-sectional study. Participants. Thirty-two eyes of patients with GO and seventy eyes of patients without GO. Methods. We prospectively included patients older than 18 years of age with and without GO. FDG-PET imaging study was performed; standardized unit value (SUVmax) was quantified in EOMs. Standard deviation and significant statistical difference (P < 0.05) were calculated. Results. Thirty-two eyes of sixteen patients of the GO group were included, with a mean age of 44.31 (20-71) years. Seventy eyes of thirty-five patients of the group without GO were included, with a mean age of 49.20 (24-77) years. EOMs average uptake of the groups with and without GO were 3.38 ± 1.31 and 1.89 ± 0.51 SUVmax (P < 0.05), respectively. Conclusion. FDG uptake was significantly increased in EOMs of patients with GO. PET gives valuable information and may be a helpful tool in detecting, localizing, and quantifying GO inflammation. Further research is needed to define the role of PET in detecting, grading, and following up GO in order to optimize treatment in the inflammatory stage.

A permanent and reversible procedure to block tear drainage for the treatment of dry eye.

PURPOSE: To describe a technique of canalicular ligation and report observations on 59 consecutive surgeries. METHODS: Retrospective, non-comparative case series of canalicular ligation by 3 surgeons over a 7-year period. RESULTS: Fifty-nine eyelids of 29 patients (2 men and 27 women) underwent canalicular ligation for the treatment of severe dry eyes. Patient age ranged from 34 to 90 years. Average length of follow-up was 20 months. There were no complications. Ninety-one percent of patients noted an improvement in their symptoms. Two patients developed symptomatic epiphora more than 1 year postoperatively and both underwent successful reversal. CONCLUSIONS: Canalicular ligation is an effective technique for punctal occlusion in patients with severe dry eyes. It combines surgical ease with excellent cosmetic outcomes.

Effects of lacrimal occlusion with collagen and silicone plugs on patients with conjunctivitis associated with dry eye.

PURPOSE: To determine whether canalicular occlusion with collagen and silicone plugs reduces the severity of symptoms in patients with conjunctivitis secondary to dry eye. METHODS: This was a prospective, randomized, double-masked study conducted at a single center in Mexico, in which 61 patients with dry eye/conjunctivitis were assigned to progressive lacrimal occlusion with collagen and silicone plugs or a sham procedural group. Outcome variables included total and individual dry eye and conjunctivitis symptom scores, moisturizing agent usage, best-corrected visual acuity, ocular comfort level, visual performance, corneal/conjunctival fluorescein staining, and incidence of adverse events. RESULTS: Total dry eye and conjunctival symptom scores were reduced by 43.7 and 33.7%, respectively 2 weeks after occlusion of all four lacrimal canaliculi with collagen plugs, increasing to 77.4 and 72.1% 2 weeks later following superior canalicular occlusion of both eyes with silicone plugs and inferior placement of collagen plugs. At the 8-week visit (4 weeks after silicone plug implantation of the inferior canaliculi of both eyes), the reduction in total dry eye and conjunctival symptom scores further increased to 94.2 and 93.0%, respectively, accompanied by a marked decline in each of the seven individual symptom scores (dryness, watery eyes, itching, burning, foreign body, fluctuating vision, and light sensitivity). In concert with these changes, moisturizing agent usage and corneal/conjunctival fluorescein staining decreased in a progressive fashion over the 8-week study period, and best-corrected visual acuity, ocular comfort, and visual performance improved in the absence of any treatment-related adverse events except for one case of epiphora. The response of patients to lacrimal occlusion can be sharply contrasted with the sham procedure group, which remained relatively unchanged from baseline at each of the study visits. CONCLUSIONS: Progressive lacrimal occlusion with collagen and silicone plugs was of clinical benefit to patients with dry eye and conjunctivitis.

Prevalencia de Demodex folliculorum en pacientes con blefaritis crónica / Prevalence of Demodex Folliculorum in patients with cronic blepharitis

La etiología de las blefaritis crónicas es diversa. Demodex folliculorum es un ácaro que habita en las glándulas de Meibomio y en los folículos de las pestañas. Objetivo. Conocer la prevalencia y características clínicas de la infestación por Demodex folliculorum en pacientes con blefaritis crónica. Materiales y métodos. Realizamos un estudio prospectivo, transversal, comparativo y observacional. Se incluyeron pacientes con blefaritis crónica realizando cultivo, antibiograma y búsqueda de Demodex folliculorum en folículos de pestañas, comparándose este último parámetro con un grupo de sujetos sanos. Resultados. Evaluamos 100 pacientes por grupo. Encontramos positividad para Demodex folliculorum en 35 por ciento de los casos con blefaritis y en 7 por ciento de los controles sanos. La prevalencia más alta se encontró después de los 60 años (51 por ciento), el sexo femenino fue el mas afectado (85 por ciento). Los collaretes en la base de las pestañas, alteraciones en su dirección, madarosis y obstrucción de glándulas de Meibomio sugieren la presencia del parásito. Conclusiones. Demodex folliculorum muestra mayor prevalencia en pacientes con blefaritis crónica comparada con sujetos sanos siendo más abundante después de los 60 años y predomina en mujeres. Debe considerarse su búsqueda y tratamiento en blefaritis crónicas resistentes al tratamiento.

Fibrohistiocitoma orbitario: reporte de un caso y revisión de la literatura / Orbital fibrohistiocytoma: a case report and review of the literature

Los fibrohistiocitomas son lesiones de tejidos blandos que presentan un gran espectro tanto clínica como radiológicamente. Se clasifican como lesiones benignas, tumores localmente agresivos, y neoplasias malignas invasivas. Estos tumores pueden aparecer en cualquier parte del cuerpo, pero presentan una afinidad especial por el área periorbitaria. El fibrohistiocitoma es un tumor raro, ocupa el 1 por ciento de todas las masas orbitarias. Hasta la fecha, no se han identificado criterios clínicos o histológicos que puedan predecir su comportamiento maligno. El análisis histopatológico y diagnóstico no son fáciles de realizar, requieren estudios de microscopia de luz, inmunohistoquímica y, en ocasiones, microscopia electrónica. El pronóstico depende del grado de malignidad. Presentamos el caso de una paciente quien acudió por presentar un tumor de gran tamaño en la órbita izquierda, causando gran destrucción de los tejidos adyacentes. Por no contar con un estudio histopatológico definitivo, se realizó biopsia incisional, ya que intentar resecar la lesión en forma completa hubiera causado numerosas complicaciones. Por microscopia de luz se hizo el diagnóstico de fibriohistiocitoma de la órbita. Se realizó una revisión de la bibliografía.

Phthiriasis palpebral / Phthiriasis of the eye-lid

La phthiriasis palpebral es una causa poco común de blefaritis y conjuntivitis que puede fácilmente ser omitida por el oftalmólogo. Un alto índice de sospecha y un cuidadoso examen en la lámpara de hendidura sobre las pestañas y márgenes palpebrales del paciente lleva a un diagnóstico certero. Aunque los piojos pueden ser difíciles de detectar, la acumulación de liendres (larvas del piojo) y de las heces de los piojos sobre las pestañas, que se aprecian como un material granular rojizo marrón en la base de las pestañas, ayuda a realizar el diagnóstico. La pediculosis se transmite por contacto íntimo con individuo infestado por vestidos y ropas de cama contaminadas, la otra forma de transmisión es típicamente por contacto sexual. En los niños la infestación puede ser debida al contacto íntimo con la madre o niñera. Pestañas y cejas son los sitios de predilección. En los adultos la phthiriasis palpebral es probablemente transmitida del área genital a los ojos mediante la mano. El tratamiento medicamentoso debe acompañarse de una cuidadosa remoción de los piojos y liendres de las pestañas del paciente. La aplicación local de pediculicidas tales como: fluoresceína al 20 por ciento, ungüento de óxido de mercurio amarillo al 1 por ciento, petrolato de fisostigmina, lociones de hexacloruro de gamma benceno, malatión, carbaryl y permetrina, o el uso de crioterapia y láser de argón. Miembros familiares, contactos sexuales, compañeros íntimos deben ser examinados y tratados, vestidos, ropas artículos personales deberán ser desinfectados a una temperatura de 50 grados centígrados por 30 minutos.

Quiste hemático de la órbita / Hematic cyst of the orbit in a two years old patient

Introducción. Los quistes hemáticos de la órbita no son comunes. La historia clínica y las características oftalmológicas son raramente diagnosticadas antes de la cirugía. La etiología del quiste hemático no está aún clara, pero es probablemente similar a las hemorragias órbitarias en general. La hemorragia dentro de la órbita es resultado ocasional de un trauma. Caso clínico, Se reporta el caso de un quiste orbitario de localización intraconal resultado de un trauma en un niño de 2 años. La tomografía axial computada ayudó en el diagnóstico, el cual llevó a un tratamiento quirúrgico exitoso. Conclusión Se reporta el segundo caso en la literatura de un quiste hemático orbitario de localización intraconal secundario a trauma y el primero por debajo de los 5 años de edad.

Blefaritis por Demodex folliculorum / Blepharitis due to Demodex folliculorum

La asociación de blefaritis con la presencia de Demodex folliculorum es probablemente más común de lo que el oftalmólogo supone. Este ácaro parasita los folículos pilosos, las glándulas sebáceas y las pestañas del hombre. Tiene una amplia distribución en todo el mundo. El papel patogénico de Demodex folliculorum es aún materia de controversia. Se han encontrado dos tipos: el Demodex folliculorum longus y el Demodex folliculorum brevis. Presentamos su morfología, ciclo evolutivo, hábitat, patogénesis y tratamiento de este diminuto parásito.

Orbitopatía tiroidea. Primera parte / Thyroid orbitopathy. First part

Se hace una revisión bibliográfica de las principales características de la orbitopatía tiroidea y se menciona la experiencia de nuestro servicio