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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.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 (R2).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 R2 ranging from 0.896 to 0.999, mean 0.971.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.

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.

Two patients with thyroid eye disease underwent inferomedial orbital decompression complicated by a cerebrospinal fluid (CSF) leak. One of the cases developed a postoperative pneumocephalus resolved with conservative measures. In the second case, the CSF leak was managed intraoperatively. In both patients, a computed tomography (CT) scan revealed a pronounced slope of the lateral lamella of the cribriform plate, forming an obtuse angle with its lateral bony extension. When this anatomical disposition of the olfactory fossa (OF) is present, the course of the anterior ethmoidal artery (AEA) is usually embedded in the skull base, and its foramen should not be taken as the upper limit of the transconjunctival ethmoidectomy. The shape and relative height of the olfactory fossa and fovea ethmoidalis, and the course of the AEA should always be assessed before transconjunctival medial decompressions.

Purpose – To measure the effect of rim-off deep lateral decompression for Graves orbitopathy on the lateral rectus muscle path and oculomotor balance. Methods – Retrospective analysis of the medical records and pre- and postoperative computed tomography scans of 34 orbits of 23 patients who underwent deep lateral decompression alone. The oculomotor balance of these 23 patients was measured with the alternate cover test and prisms before and after surgery. Bezier functions were used to measure the postoperative path of the lateral rectus in all decompressed orbits. Results – Deep lateral decompression induced a curvilinear deformation of the lateral rectus. There was no significant correlation between the position of the point of maximum muscle displacement and the size of the residual lateral wall. The changes in the lateral rectus path had no adverse effects on the oculomotor balance of the patients. Conclusions – The location of the curvilinear deformation of the lateral rectus do not depends on the residual segment of the lateral wall. The changes of the lateral rectus path have no deleterious effect on the oculomotor balance.

The purpose of this article was to report a case of intraconal lacrimal gland tissue and to review the literature on lacrimal gland choristoma. The magnetic resonance imaging findings of a biopsy-proven orbital case are also presented. A PubMed database search was performed using the key terms heterotopic, ectopic, aberrant, choristoma, and lacrimal gland to identify all the previously documented studies on lacrimal gland choristoma, in English, Spanish, and French. We classified the lacrimal gland choristoma cases classified according to the location of the lesions, clinical appearance, management, and outcome. The search targeting the period between 1887 and 2019 returned 79 articles, which were reviewed. We found a total of 113 cases of choristomas with normal lacrimal gland tissue. Only two of them were not associated with the eye or its adnexa while the remaining 111 lesions were found either on the ocular surface (n=46) or in the orbit (n=34). Intraocular choristomas were found in 18 patients, and the rest of the lesions were noted either on the eyelids (n=10) or in the lacrimal drainage system (n=3). Orbital and intraocular choristomas are the most harmful lesions as orbital choristomas are frequently associated with permanent diplopia while intraocular lacrimal gland choristomas have a poor visual prognosis and are a common cause of enucleation of the eye. In one of the reported cases, a corneal lacrimal gland choristoma had been experimentally induced by activating the FGF10 signaling pathway. Lacrimal gland choristomas are not uncommon. This peculiar type of lesion has been experimentally induced and may appear in a variety of locations associated with the globe and its adnexa. RESUMO Descrever um caso de tecido de glândula lacrimal orbitário intracônico e revisar a literatura sobre choristomas lacrimais. Os termos heterotópico, ectópico, aberrante, choristoma e glândula lacrimal foram usados para procurar manuscritos em inglês, espanhol e francês. Todos os artigos encontrados foram revisados. Os choristomas lacrimais relatados foram classificados segundo a localização das lesões, aparência clínica, manejo e desfecho. Imagens de ressonância magnética de um novo caso orbitário comprovado por biópsia também são apresentadas. Foram revisados setenta e nove artigos, de 1887 a 2019, descrevendo 113 casos de choristomas constituídos por tecido de glândula lacrimal normal. Apenas 2 casos (2%) não estavam associados com o olho ou seus anexos. A maioria das 111 lesões restantes foram encontradas na superfície dos olhos (n=46) e nas órbitas (n=34). Os choristomas intraoculares foram encontrados em 18 pacientes. As demais lesões foram diagnosticadas nas pálpebras (n=10) e no sistema lacrimal (n=3). Os choristomas orbitais e intraoculares foram as lesões mais deletérias. Na órbita eles são frequentemente associados com diplopia permanente. Os choristomas lacrimais intraoculares imitam neoplasias e são uma causa comum de enucleação. Os choristomas lacrimais não são incomuns. Este tipo peculiar de lesão já foi induzido experimentalmente uma única vez e pode aparecer em uma variedade de locais associados ao globo e sua anexos. Os choristomas intraoculares têm um prognóstico visual ruim.

PURPOSE To evaluate the effect of orbital decompression on the upper eyelid contour. METHODS A paired cross-sectional analysis of the upper eyelid contour was performed for 103 eyes of 66 patients who underwent orbital decompression. A control group of 26 normal subjects was also included. The eyelid contour of all participants were measured with Bezier lines adjusted to the eyelid contour and 9 midpupil eyelid margin (MPD) distances from a horizontal line bisecting the pupil. One central, corresponding to the margin reflex distance (MRD 1), and 8 equally distributed medially and laterally at 20% of the interval between the lines. Patients were classified as with flare if the height of the most lateral MPD relative to the MRD 1 was above the upper limit of the controls. RESULTS Preoperatively 63 of the 103 contours were classified as flare + (F+). After decompression MRD1 showed a mean decrease of 0.4 mm and the location of the contour shifted 0.8 mm medially. These changes were not correlated with proptosis reduction. Orbital decompression decreased the lateral curvature of the contours especially for the F+ lids. In 40% of the F+ eyelids the flare sign disappeared after decompression. CONCLUSIONS Orbital decompression affects the lateral eyelid contour and diminishes the amount of lateral eyelid retraction surgery necessary to correct the flare sign. In 40% of the patients, the eyelid contour is normalized with proptosis reduction only.

Congenital optic nerve cystic-like malformations associated with normally developed globes are extremely rare. We describe 3 children who presented since birth with proptosis, and eye motility limitation. MRI showed in all cases that the intraorbital segment of the optic nerves was malformed with large cystic-like lesions in the intraconal segment of the orbit. In all cases, biopsies of the wall of the lesions were positive for glial fibrillary acidic protein. Since this protein is a neurobiomarker that exists only in astrocytes in the central nervous system, nonmyelinating Schwann cells of peripheral nerves, and enteric glial cells, we believe that these lesions represent true opticmeningoceles.

To systematically review the literature on the deep lateral orbital decompression (DLD).The authors searched the MEDLINE, Lilac, Scopus, and EMBASE databases for all articles in English, Spanish, and French that used as keywords the terms orbital decompression and lateral wall. Two articles in German were also included. Data retrieved included the number of patients and orbits operated, types of the approach employed, exophthalmometric and horizontal eye position changes, and complications. The 95% confidence intervals (CI) of the mean Hertel changes induced by the surgery were calculated from series with 15 or more data.Of the 204 publications initially retrieved, 131 were included. Detailed surgical techniques were analyzed from 59 articles representing 4559 procedures of 2705 patients. In 45.8% of the reports, the orbits were decompressed ab-interno. Ab-externo and rim-off techniques were used in 25.4% and 28.8% of the orbits, respectively. Mean and 95% CI intervals of Hertel changes, pooled from 15 articles, indicate that the effect of the surgery is not related to the technique and ranges from 2.5 to 4.5 mm. The rate of new onset of diplopia varied from zero to 8.6%. Several complications have been reported including dry eye, oscillopsia, temporal howling, lateral rectus damage, and bleeding. Unilateral amaurosis and subdural hematoma have been described in only one patients each.The low rate of new-onset diplopia is the main benefit of DLD. Prospective studies are needed to compare the rate of complications induced by the 3 main surgical techniques used.

The IgG4-related disease (IgG4-RD) is a systemic condition defined as a fibro-inflammatory disorder, characterized by the occurrence of tumor-like lesions in multiple organs including the eye adnexa. The main diagnostic criterion is based on histopathological findings, especially on the IgG4

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.PURPOSE: To describe a patient with Graves' orbitopathy who presented with loss of vision of the left eye for 9 months and amaurosis of the right eye for 20 days. METHODS: Bilateral inferomedial transnconjunctival orbital decompression was performed. RESULTS: After orbital decompression, vision slowly improved and ten months after the surgery the vision was normal in both eyes. CONCLUSIONS: Orbital decompression can reestablish optic nerve function at least 20 days after amaurosis.