Blink Lagophthalmos in Graves Orbitopathy.

PURPOSE: To investigate the relationship between the kinematics of spontaneous blinks and the anterior area of the levator palpebrae superioris muscle in patients with Graves orbitopathy (GO). METHODS: This is a case-control study. The authors measured the margin reflex distance of the upper eyelid (margin reflex distance 1), the kinematics of spontaneous blinks, and the anterior area of levator palpebrae superioris muscle in CT coronal scans of patients with Graves upper eyelid retraction (GO) and a control group. The eye with the greatest margin reflex distance 1 was selected for analysis in each group. RESULTS: A total of 68 participants were included, with 36 in the GO group and 32 in the control group. In the GO group, the mean margin reflex distance 1 measured 6.5 mm, while in the control group, it was 3.9 mm. Almost all parameters related to the closing phase of spontaneous blinking activity, including amplitude, velocity, blinking rate, and interblink time, did not differ between the two groups. However, the effectiveness of the blink's amplitude (ratio of blink amplitude to margin reflex distance 1) and the main sequence (relationship between amplitude and velocity) were significantly reduced in the GO group compared with the control group. The area of the levator palpebrae superioris muscle was significantly larger in GO than in controls, with 71.4% of patients' muscles outside of the maximum range of the controls. CONCLUSIONS: In patients with GO, there is a reduction in blinking effectiveness, also known as blink lagophthalmos, which is a factor in the common occurrence of ocular surface symptoms. The increase in velocity with amplitude is also reduced in GO.

Spontaneous Blinking Kinematics After Frontalis Muscle Flap Advancement.

PURPOSE: To measure the spontaneous blinking metrics after blepharoptosis correction with frontalis muscle flap advancement. METHODS: A video system was employed to measure the amplitude and velocity of spontaneous blinking of 24 eyelids after the frontalis muscle flap surgery for blepharoptosis correction. A control group with no eyelid disorders was also measured. The data of 13 eyelids who had frontalis slings with autogenous fascia, which were previously collected with the same method in another study, were used for comparison. Digital images were used to measure the superior margin reflex distance and the presence of lagophthalmos during a gentle closure of the palpebral fissure. Superficial keratitis was assessed by corneal biomicroscopy. RESULTS: The mean amplitude of spontaneous blinking was 6.3 mm in controls, 2.6 mm in the frontalis flap patients, and 2.1 mm in the fascia sling group. The mean blink velocity was 133.8 mm/second in controls, 39.0 mm/second (3.7 standard error) after the frontalis flap, and 36.3 mm/second in patients with frontalis sling with fascia. For these 2 parameters, there was no statistical difference between the surgical groups, but a significant reduction when compared with the control group. No significant association was found between lagophthalmos and keratitis and the surgical procedure. CONCLUSIONS: There is no difference between the blinking metrics of eyelids operated using the frontalis muscle flap advancement technique or frontalis sling with autogenous fascia. The presence of lagophthalmos and keratitis also does not differ between the 2 procedures.

Apical bleeding: a rare cause of blindness after orbital decompression / Sangramento apical: uma causa rara de cegueira pós descompressão orbitária

ABSTRACT Orbital decompression is widely performed for the management of proptosis for cosmetic and functional cases of Graves orbitopathy. The main side effects include dry eye, diplopia, and numbness. Blindness after orbital decompression is extremely rare. The mechanisms of vision loss after decompression are not well described in the literature. Considering the devastating effect and rarity of this complication, this study presented two cases of blindness after orbital decompression. In both cases, vision loss was provoked by slight bleeding in the orbital apex.

RESUMO A descompressão orbitária é uma cirurgia amplamente empregada para correção da proptose em casos cosméticos e funcionais da orbitopatia de Graves. Os principais efeitos colaterais induzidos pela descompressão são olho seco, diplopia e parestesias. Amaurose pós descompressão é uma complicação extremamente rara e cujos mecanismos são pouco discutidos na literatura. Considerando o efeito devastador representado pela perda visual e a escassez de relatos dessa complicação, os autores apresentam dois relatos de amaurose após descompressão orbitária. Nos dois casos a perda visual ocorreu devido a sangramento de pequena monta no ápice orbitário.

The Relation of the Anterior Ethmoidal Foramen to the Cranial Base.

PURPOSE: To perform a radiological analysis of the relation of the anterior ethmoidal foramen (AEF) to the cranial base and olfactory fossa (OF). METHODS: Retrospective analysis of computed tomography coronal scans of 35 orbits of 35 patients with Graves orbitopathy. Following a standard multiplanar reconstruction of the orbit, the authors measured the vertical and the horizontal distances of the AEF to the cranial base and to the lateral border of the OF, respectively. The height of the OF was measured at the level of the AEF and at the position of Keros classification. The presence of supraorbital ethmoid cells (SOEC) was also recorded. RESULTS: The vertical distance of the AEF to the cranial base ranged from 0.1 to 7.3 mm with a mean = 2.5 mm (SD = 2.17). In 47.5% of the orbits the foramen was adjacent (<1 mm) to the cranial base. The horizontal distance of the AEF to the lateral border of the OF ranged from 2.3 to 9.5 mm (mean = 5.4 mm, SD = 1.98). The presence of an SOEC (n = 12) increased significantly both the distance from the AEF to the cranial base and to the lateral border of the OF. The height of the OF at the level of AEF is not correlated with the depth of the OF at the level of Keros classification. CONCLUSIONS: In the absence of supraorbital pneumatization, the AEF location is a dangerous landmark for the upper limit of the transconjunctival medial wall decompression.

Bilateral Intraorbital Opticmeningoceles in Joubert Syndrome.

Congenital opticmeningoceles was the term coined to describe large pseudocystic lesions of the intraorbital segment of the optic nerve. This extremely rare congenital anomaly was reported unilaterally only in nonsyndromic patients with fully developed eyes. The authors describe here a 10-month-old girl with a previous diagnosis of Joubert syndrome who presented with the same type of optic nerve malformation in OU. Molecular genetic analysis disclosed a pathogenic variant of the TMEM67 gene which is associated with various types of ciliopathies.

Medial Oblique Eyelid Split Orbitotomy.

PURPOSE: The authors describe their experience with a variant of the split orbitotomy with a small medial oblique transmarginal incision for approaching a variety of lesions involving the superonasal aspect of the orbit. METHODS: Retrospective review of medical records and clinical photographs of all patients who underwent an unilateral medial oblique incision to access various intraconal lesions abutting the superomedial quadrant of the orbit. The curvature of the medial contour of the operated and contralateral eyelids were expressed with Bézier functions and compared using the R-squared coefficient of determination (R 2 ). RESULTS: Twenty-three patients were submitted to this surgical technique for approaching various unilateral lesions on the superonasal quadrant of the orbit. Excellent cosmesis was achieved in all eyelids, with almost imperceptible scars, and no ptosis or retraction. There was no significant difference between the postoperative medial contour of the operated and the contralateral eyelid, with R 2 ranging from 0.896 to 0.999, mean 0.971. CONCLUSIONS: The authors' results show that the modified eyelid split approach provides a wide exposure of the superonasal quadrant of the orbit with no risk of eyelid dysfunctions or significant scars.

Effect of Rim-Off Deep Lateral Orbital Decompression on Interpalpebral Fissure Shape.

PURPOSE: The purpose of this study is to compare the palpebral fissure shape after rim-off deep lateral decompression with and without restoration of the lateral orbital rim. METHODS: Retrospective cross-sectional quantitative analysis of the palpebral fissure images of 17 patients (25 eyes) who underwent rim-off deep lateral orbital decompression without repositioning of the rim (G1) and of 20 patients (34 eyes) operated with rim restoration. Quantification of palpebral fissure images included MRD 1 and MRD 2 , two lateral mid-pupil lid margin (MPD) distances, the width of the palpebral fissure, and the height and shape of the lateral canthus. RESULTS: Proptosis reduction did not differ significantly between G1 and G2. In both groups, decompression reduced the mean values of the MRD 1 and MRD 2 , the lateral height of the upper eyelid, and the height and angle of the lateral canthus. The palpebral fissure width and lateral height of the lateral lower eyelid were not affected. There was no significant difference between the mean changes of G1 and G2. Multivariate analysis showed that the lateral canthus decrease was correlated with changes in MRD 1 and MRD 2 . CONCLUSIONS: Small changes in palpebral fissure shape after deep lateral decompression are not dependent on the presence or absence of the lateral rim.

Ophthalmic histiocytic lesions (diseases of the L group): A multicenter clinicopathological study of 18 cases and review of literature.

INTRODUCTION: Langerhans cell histiocytosis (LCH) and Erdheim-Chester disease (ECD) are rare histiocytic disorders in the L (Langerhans) group diseases. They range from self-limited benign diseases to lethal disseminated forms. METHODS: This retrospective study was conducted in 3 tertiary hospitals in Saudi Arabia and Brazil. Histopathological records were searched for all patients diagnosed with ocular and periocular histiocytic disorders from January 1993 to December 2018. Histopathological slides and medical files were reviewed for data collection and simple analysis of demographics, clinical manifestations, and management. The relevant literature is reviewed. RESULTS: Twenty-two eyes of 18 patients with biopsy-proven histiocytic disorders in the L group were included. Female-to-male ratio was 1.25:1. Average age at presentation was 14 years (range, 1-54). LCH was diagnosed in 14 eyes, while eight eyes had ECD. All LCH cases were unilateral and confined to the bone as cases of eosinophilic granuloma (EG), while patients with ECD were bilateral. Commonest presentations in EG and ECD were eyelid swelling (85.7%) and periocular xanthomas (75%), respectively. Orbit was involved in 100% of EG cases, with bony erosion in 54.5%. Relevant systemic involvement was found in 100% of ECD and 21% of EG cases. Surgical intervention was needed in 16 of the 22 eyes (72.7%). All EG and 25% of patients with ECD required surgical excision. CONCLUSIONS: Histiocytic disorders are a rare group of diseases, including the L group. Relevant systemic associations require specific and selective therapy. A high clinical index and multidisciplinary collaboration are essential for the proper evaluation and management of these patients.

Computed tomography and magnetic resonance imaging of an unusual intraconal orbital osteoma.

We report the case of a 64-year-old male patient with a 5 month history of proptosis, motility limitation and vision loss in OD. Visual acuity (VA) was 20/200 in OD and 20/20 in OS. CT showed a large, round, intraconal lesion, with bony density and no apparent connection to adjacent orbital walls. MRI showed a T1-weighted hypointense lesion surrounded by a contrast enhancing capsule. The orbital tumor was excised through a lateral orbitotomy revealing a nodular, round, osseous structure. Histological examination disclosed well-formed lamellar bone trabeculae, with no necrosis or mitosis figures. Immunohistochemical staining was negative for MDM2 and CDK4. After 3 years, there was no evidence of tumor recurrence and VA had improved to 20/30. Intraconal osteomas with no clear attachment to orbital walls are extremely rare. We are aware of a few reported cases in the lid, hand, thigh, tongue, pterygopalatine fossa and brain. To the authors' knowledge, this is the first report in English literature of an orbital intraconal osteoma without any visible relation to the orbital walls.

Bézier curves as a total approach to measure the upper lid contour: redefining clinical outcomes in palpebral surgery.

AIMS: To define and quantify the upper lid contour by adapting Bézier curves with a newly developed software in normal subjects, assessing their reliability. METHODS: Fifty eyes of 50 healthy patients with no ocular pathology were included in this study from October 2020 to November 2020. All measurements were performed on Bézier curves adjusted to the upper lid contour. An original software was used to measure the radial and vertical midpupil-to-lid margin distances (MPLD), temporal-to-nasal (T/N) ratios, contour peak location and grade of superposition (GS) and asymmetry (GA) indexes. We calculated differences in the variables measured regarding age, gender or the side of the eye being assessed. RESULTS: The mean Bézier curve showed an excellent level of inter-rater reliability (intraclass correlation coefficient of 0.99). The median GS index of each eyelid to the mean Bézier curve was 95.4%, 8.5 IQR, and the median GA index was 3%, 3.4 IQR. The mean contour peak location was -0.35 mm, SD 0.45. Overall, the mean central MPLD was 4.1 mm, SD 0.6. No significant differences were found between male and female patients in variables derived from Bézier curves. CONCLUSION: Bézier curves may become a very useful tool for the assessment of upper lid contour, contour peak and symmetry. GS and GA indexes, along with the T/N area ratio are potential outcomes for this purpose. All current variables can be obtained just from one single Bézier curve measurement. Our results offer an in-depth exhaustive description of these variables and their distribution in the normal population.

Clinical and histopathological study of a hollow and posteriorly multiperforated polymethylmethacrylate implant in eviscerated rabbit eyes / Análise clínica e histopatológica de um implante de polimetilmetacrilato oco e multiperfurado em sua porção posterior em olhos eviscerados de coelhos

ABSTRACT Purpose: The study aimed to evaluate the clinical and tissue response to a hollow polymethylmethacrylate orbital implant with a multiperforated posterior surface in an animal model after evisceration. Methods: Sixteen New Zealand rabbits had their right eye eviscerated. All animals received a hollow polymethylmethacrylate implant 12 mm in diameter that is multiperforated in its posterior hemisphere. The animals were divided into four groups, and each one had the eye exenterated at 7, 30, 90, and 180 days post-evisceration. Clinical signs were assessed daily for 14 days post-evisceration and then every 7 days until 180 days. Inflammatory pattern, collagen structure, and degree of neovascularization generated with implant placement were analyzed with hematoxylin-eosin, picrosirius red, and immunohistochemistry staining. Results: There were no signs of infection, conjunctival or scleral thinning, or implant exposure or extrusion in any animal during the study. On day 7, the new tissue migrated into the implant and formed a fibrovascular network through the posterior channels. Inflammatory response reduced over time, and no multinuclea­ted giant cells were found at any time. Conclusion: Hollow polymethylmethacrylate orbital implants with a multiperforated posterior surface enable rapid integration with orbital tissues by fibrovascular ingrowth. We believe that this orbital implant model can be used in research on humans.

RESUMO Objetivo: Avaliar a resposta tecidual e clínica a um implante orbitário de polimetilmetacrilato, oco e multiperfu­rado em sua porção posterior em modelo animal após evisceração. Métodos: Dezesseis coelhos da raça Nova Zelândia foram submetidos à evisceração do globo ocular direito. Todos receberam implante oco de polimetilmetacrilato de 12 mm de diâmetro, multiperfurado em sua semiesfera posterior. O estudo foi dividido em avaliação clínica e histopatológica. A avaliação clínica foi diária até 14 dias pós-evisceração e, a cada sete dias, até completar 180 dias. Os animais foram divididos em grupos de quatro animais e cada um foi submetido à exenteração com 07, 30, 90 e 180 dias e depois à eutanásia. A análise histopatológica teve por fim caracterizar o padrão inflamatório, a estrutura do colágeno e o grau de neovascularização. Para isso, além da tradicional coloração pela hematoxilina-eosina, utilizou-se o corante Picrosirius Red (PSR) e imuno-histoquímica com o marcador CD 34. Resultados: Não houve sinais de infecção, afinamento conjuntival ou escleral, exposição ou extrusão do implante em nenhum animal durante o estudo. Já no sétimo dia, o tecido neoformado migrou para dentro do implante formando uma rede fibrovascular através dos canais posteriores. A resposta inflamatória diminuiu ao longo do tempo avaliado e não foram encontradas células gigantes multinucleadas. Conclusão: O implante analisado permite a sua integração aos tecidos orbitários pelo crescimento fibrovascular em seu interior. Os autores acreditam que este modelo de implante orbital pode fazer parte de testes com humanos.

Bilateral Dacryoadenitis and Central Nervous System Involvement in a Child With Kimura Disease.

Kimura's disease (KD) is a systemic inflammatory condition characterized by lymphadenopathy and subcutaneous nodules in the head and neck region. The lesions have a distinctive histopathological pattern formed by follicular hyperplasia, eosinophilic infiltrates, fibrosis, and vessel proliferation. The disease may occur at all ages but predominates among young males with autoimmune dysfunctions. Visceral and orbital involvement is uncommon. We report a girl with KD who developed bilateral enlargement of the lacrimal glands and a lesion in the left lateral ventricle of the brain indistinguishable from a central nervous system neoplasia. A biopsy of both the lacrimal gland and the lateral ventricle was consistent with KD.

Ophthalmic Rosai-Dorfman disease: a multi-centre comprehensive study.

BACKGROUND: To provide basic demographic information and clinicopathologic features of ophthalmic Rosai-Dorfman disease (RDD) with a literature review. METHODS: A multi-centre retrospective case series reviewing all patients with histopathologically confirmed ophthalmic RDD at three tertiary eye care centres between January 1993 and December 2018. RESULTS: Eleven eyes of eight patients with histopathologically confirmed ophthalmic RDD were included, with equal numbers of males and females. The median age was 40.25 years (range: 26.6-72.4). Two patients had familial RDD. The orbit was the most commonly involved site (90.9% eyes). One patient (one eye) presented with a scleral nodule, anterior uveitis and cystoid macular oedema. Visual acuity ranged from 20/25 to light perception. Six patients had an extra-nodal ophthalmic disease, and the remaining two had an associated submandibular lymphadenopathy (nodal RDD). CONCLUSIONS: Ophthalmic RDD can be the only manifestation of this systemic disease, with the orbit being the most commonly involved site, exhibiting bone destruction, intracranial and/or sinus involvement and variable degree of visual loss. Ophthalmic familial RDD represent a severe form with a malignant course. Steroid monotherapy may be inadequate to control orbital RDD; thus, combined treatment is usually necessary. A comprehensive approach to assessment and management is recommended.