Restricted Eye Movements Research Articles

Ossifying Fibroma of the Maxilla in Two Cases and Review of the Literature

Ossifying fibroma is a benign tumor that rarely affects the face bones and exclusively the maxilla. We report two cases of ossifying fibroma of the maxilla treated in our department of maxillofacial surgery and stomatology at IBN SINA university hospital in Rabat. The 1st case is a 38-year-old female, with no particular history, who presented a progressive swelling of the left face since 05 months. Extraoral examination demonstrated a grade I left exophthalmos, without diplopia or limitation of eye movements. Intraoral examination revealed a vestibular expression of the tumor. The 2nd case is also a 45-year-old female, who presented a progressive swelling of the left maxilla which gradually increased in size over a period of 04 months. Intra-orally, the buccal vestibule was completely obliterated with the mass. The two patients presented radiological images of similar density, made of a heterogeneous tissue of the maxillary containing calcifications but with different extension. Surgical excision was performed in both patients and the diagnosis was confirmed by histological study of the resected specimen. The evolution of the ossifying fibroma is insidious; this pathology has a clinical polymorphism and a good prognosis. The etiopathogenesis of this tumor is still unknown, it’s more likely to develop in the mandible and very rare in the maxilla. It differs from other types of fibroma by their clinical, radiological and histological aspects. However, only the pathological examination confirms the diagnosis.

Clinical Features and Outcomes of Patients with Orbital Schwannoma.

To report the clinical features, treatment, and outcome of 11 patients with orbital schwannoma in Korean patients. The medical records of 11 orbital schwannoma patients treated between April 2007 and April 2021 were retrospectively reviewed. The demographic data, clinical characteristics, radiological features, and outcomes were reviewed. The mean age at the time of diagnosis was 49.00 ± 14.45 years. The most common initial symptom was ocular protrusion (n = 7), and other symptoms were decreased visual acuity ( n = 5), restriction of eye movement ( n = 4), swelling ( n = 3), and pain ( n = 2). Locations of tumors were superomedial; followed by the orbital apex and inferolateral. The most common shape seen in our patients was beads like multilobulated appearance; followed by a round, oval, fusiform, and dumbbell shape. MRI of T1-weighted revealed isointense or hypointense, whereas the T2-weighted indicated hyper or isointense lesion. Five patients had optic neuropathy at presentation, and 1 of them showed improved vision after surgery. Complete or incomplete excision was performed for all. Surgical complications include decreased vision and paraesthesia. There has been no recurrence to date. Orbital schwannoma is a rare disease and it is difficult to distinguish it from other orbital tumors because the initial symptoms are nonspecific. Differential diagnosis by combining the shape, location, and contrast enhancement findings seen on computed tomography and magnetic resonance imaging can be helpful in surgical treatment. Complete excision gives the best results without recurrence, but if the patient is in an inaccessible location, only surgery to reduce the volume can satisfy the patient without recurrence.

Thyroid eye disease with concurrent orbital lymphoma: a radiological surprise.

SummaryThyroid eye disease (TED) is the most common extra-thyroidal manifestation in Graves’ disease (GD). Additional/concurrent/synchronous pathologies may be present, especially in elderly patients who present with atypical features such as non-axial (or eccentric) proptosis, absence of lid lag and restricted superior extra-ocular movements. A 70-year-old female presented with progressive proptosis of her left eye and diplopia. She was diagnosed with GD a year prior and initiated on carbimazole. On examination, she had eccentric proptosis, restricted superior extra-ocular movements and a palpable mass in the supero-temporal quadrant of the left eye. Her T3 (1.33 ng/mL) and T4 (8.85 µg/dL) were normal with carbimazole. Thyroid-stimulating hormone (TSH)-receptor antibody was positive (3.15 IU/L, reference range <1.75). MRI revealed an enhancing lesion infiltrating the left superior rectus, with concurrent characteristic muscle belly involvement bilaterally. Orbital biopsy showed atypical lymphoid cells (CD20+), suggesting marginal zone lymphoma. CT thorax and abdomen, fluorodeoxyglucose-positron emission tomography and bone marrow examination were normal. The patient was administered orbital radiotherapy for her localised lymphoma and carbimazole was continued. TED is the most common cause of orbital involvement overall and in GD. However, additional or alternative pathology may be present which requires evaluation. MRI can be a useful adjunct in these patients. Orbital lymphoma needs to be staged with workup for disseminated disease. Radiotherapy is the treatment of choice for localized disease. The index case provides evidence for synchronous presentation of dual pathology and highlights the importance of astute clinical examination as well as keeps a low threshold for MRI in selected cases.Learning pointsThyroid eye disease can co-exist with other ocular pathology, especially in elderly individuals.Eccentric proptosis, absent lid lag and restriction of eye movements (suggesting tendon involvement) should alert towards the presence of alternative pathology.Orbital imaging using MRI not only has greater sensitivity in diagnosing radiologically bilateral disease in patients who have unilateral involvement clinically but is also useful to identify concurrent neoplasms.

An unusual presentation of an intraorbital abscess associated with an extensive dental caries and pulp gangrene in a 14-years old boy with asthma and obesity

We present an unusual case of a 14-year-old patient who was admitted as an emergency to the hospital with oedema of right orbit with restricted eye movements. Severe asthma from the seventh month of age and obesity were reported. Radiologic examination showed the existence of an intraorbital abscess. He also presented an extensive dental caries and pulp gangrene in seventeen teeth and unilateral sinusitis confirmed in the Computed Tomography scan. Emergency treatment including an oral surgeon’s intervention, which is extraction of seventeen teeth, drainage of the abscess, antibiotics and glucocorticoids was introduced resulting in an immediate improvement of patient’s health status, leading to recovery. The objective of this clinical presentation is to highlight associations between dental status and cellulitis. Infection from decayed teeth in children can lead to unilateral sinusitis, resulting in severe complications such as an intraorbital abscess. It is strongly recommended to put more effort into oral hygiene and healthy lifestyle to avoid serious complications – especially in asthmatic patients.

Fibrin glue assisted amniotic membrane graft reconstruction of conjunctival fornix and canthus following excision of conjunctival tumours.

Conjunctival tumors involving non-limbal locations, such as the fornix and canthus, are typically excised using a “non-touch” technique, often with a wide surgical margin. Reconstruction of these large defects can be difficult due to the contour of the ocular surface and are often complicated by shortening of the fornix, symblepharon formation, and restriction of eye movements. In our experience, the use of amniotic membrane grafts combined with the sealant properties of fibrin glue such as Tisseel® has improved our surgical outcomes during the reconstruction phase. We would like to highlight and describe our surgical technique using fibrin glue and squint hooks to aid amniotic membrane graft reconstruction for surgically challenging locations in the fornix and canthus following excision of conjunctival lesions, with excellent surgical outcomes.

Design considerations for the auditory brain computer interface speller

Brain-computer interface (BCI) systems are designed to translate the measured brain signals into a set of instructions, with a purpose of interaction with outside world. Most common application of such systems is to provide aid to patients with limited physical capabilities, where the visual stimulation based BCI are most commonly used. However, in the most extreme situations, e.g. for patients with amyotrophic lateral sclerosis, visual stimulation approach is insufficient for high-level interaction due to the patient's limited eye movement capabilities. To overcome these limitations, we propose a novel design of the two-stage auditory BCI system which exposes a subject to a stream of letter utterances, such that subject is directly stimulated with letter pronunciation, decreasing the mental effort required by the subject. This is contrary to frequently used auditory spellers, which use various non-related sounds representing a specific letter (i.e. various instruments, natural sounds, etc.). Letter discrimination between targets and non-targets is performed with convolutional neural network, with convolutional spatial and temporal filters capable of efficient extraction of event related brain activity features. Second stage of the target letter discrimination is proposed in order to increase the accuracy and usability in the real-world situations, including the reduced stimuli set based on results from the first classification stage. The proposed BCI has been tested on ten healthy subjects, achieving on average spelling accuracy of 30% (max. 100%) and high information transfer rate of 2.38 bits/min (max. 8.14 bits/min), outperforming the state-of-the-art auditory BCI spellers.

A study of otorhinolaryngological diseases with ophthalmic complications: at a tertiary care center

&lt;p class="abstract"&gt;&lt;strong&gt;Background:&lt;/strong&gt; The close proximity of the eyeball and the orbit to the ENT region, makes the involvement of the orbit and there of ophthalmological manifestations common in the practise of Otorhinolaryngology. The diseases of ENT with orbital extension must be considered whenever a patient presents with signs and symptoms of orbital disease such as proptosis, orbital mass, restriction of eye movements, neurological dysfunction of eye, chemosis, vision disturbances or epiphora.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Methods: &lt;/strong&gt;Patients presenting with ophthalmological manifestations due to various ear, nose, throat and head–neck pathologies were constituted in this prospective study which was carried out on 50 patients of the age group 10 years to 60 years with ophthalmological complications along with ENT diseases who came to the department of ENT and department of Ophthalmology of N.S.C.B. Medical College, Jabalpur from August 2019 to August 2021.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Results: &lt;/strong&gt;Males were affected more than female with ophthalmic complications. Sinonasal tumours were the most common cause of orbital complications, of which carcinoma of maxilla was the commonest , followed by sinonasal lymphoma .Nasal obstruction was the most common ENT complaint and proptosis was the commonest orbital sign.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Conclusions: &lt;/strong&gt;A firmer knowledge of orbital anatomy with respect to sinonasal region is required for any surgeon dealing with this important area, for a safer and more secure management in this complex region. Surgery remains the main stay of treatment for sinonasal diseases with orbital complications. Rapid diagnosis and treatment are of utmost importance in preserving vision and life in these patients.&lt;/p&gt;

Study of Profile of Mucormycosis During the Second Wave of COVID-19 in a Tertiary Care Hospital.

Introduction and aimMucormycosis is a lethal opportunistic infection caused by filamentous fungi of the family Mucoraceae (black fungus). There has been a sudden increase in the incidence of these cases during the second wave of the COVID-19 pandemic due to the immunocompromised state caused by the disease and its treatment. Early diagnosis and appropriate medical management are essential to reduce disease morbidity and mortality. Through this study, we aim to study the clinical features, risk factors, laboratory investigations, and radiological findings of patients with mucormycosis as well as evaluate the clinical outcomes in each case.Methods and materialsThis was a prospective study that included only confirmed mucormycosis cases admitted in Tata Main Hospital (TMH) from April 2021 to July 2021. A case of mucormycosis was defined as the one in which clinical and radiological features were consistent with mucormycosis and fungus was demonstrated in the tissue by potassium hydroxide (KOH) mount/culture/histopathological examination (HPE). Data relating to epidemiology, risk factors, clinico-radiological features, and outcomes were analyzed and expressed as a percentage of total cases.ResultsOf the total 15 cases, three patients (33.3%) had active COVID-19 infection, eight (53.3%) were in the post-COVID-19 state, two (13.4%) had COVID-19 like illness and two (13.4%) patients did not have COVID-19 in the recent past. There was male predominance with the male to female ratio being 2.75:1. The commonest associated co-morbid condition was diabetes mellitus (13 patients, 86.7%). Amongst the myriad manifestations, periorbital swelling was the commonest symptom (11 patients, 73.3%). Among neurological manifestations, involvement of cranial nerves was found in nine (60%) patients with the third cranial nerve being the most commonly affected nerve (eight patients, 53.3%). Cavernous sinus thrombosis (CST) was found in one (6.7%) patient. Diagnostic nasal endoscopy (DNE) revealed eschar at various sites in 13 patients (86.7%). Central retinal artery occlusion (CRAO) was found bilaterally in one patient (6.7%) while two patients (13.3%) had CRAO on the left. Radiologically, the most commonly involved sinuses were maxillary and ethmoidal (eight patients, 53.3%). Bilateral sinus involvement was more common (46.7%) than unilateral sinus involvement. The average length of stay (LOS) was 17.5±7.8 days. The overall mortality was 40%. Five (33.3%) patients developed secondary bacterial infections. All patients received medical therapy with intravenous amphotericin B. In addition, seven (46.7%) patients underwent functional endoscopic sinus surgery (FESS) with debridement of which, five (71.4%) patients survived and made a good recovery. One patient (6.7%) with pulmonary mucormycosis underwent lobectomy.ConclusionNew-onset headache, black nasal discharge, periorbital swelling, retro-orbital pain, visual diminution, restriction of eye movements should prompt an immediate search for mucormycosis especially in the background of history of diabetes mellitus in patient with recent or current COVID-19 disease. Radio-imaging with computerized tomography and magnetic resonance imaging are complementary to clinical evaluation in assessing the disease extent and diagnosis of complications. Prompt diagnosis is essential due to the angio-invasive nature of the mucor and requires aggressive anti-fungal therapy and debridement of the devitalized tissue.

Integrating neural and ocular attention reorienting signals in virtual reality

Objective. Reorienting is central to how humans direct attention to different stimuli in their environment. Previous studies typically employ well-controlled paradigms with limited eye and head movements to study the neural and physiological processes underlying attention reorienting. Here, we aim to better understand the relationship between gaze and attention reorienting using a naturalistic virtual reality (VR)-based target detection paradigm. Approach. Subjects were navigated through a city and instructed to count the number of targets that appeared on the street. Subjects performed the task in a fixed condition with no head movement and in a free condition where head movements were allowed. Electroencephalography (EEG), gaze and pupil data were collected. To investigate how neural and physiological reorienting signals are distributed across different gaze events, we used hierarchical discriminant component analysis (HDCA) to identify EEG and pupil-based discriminating components. Mixed-effects general linear models (GLM) were used to determine the correlation between these discriminating components and the different gaze events time. HDCA was also used to combine EEG, pupil and dwell time signals to classify reorienting events. Main results. In both EEG and pupil, dwell time contributes most significantly to the reorienting signals. However, when dwell times were orthogonalized against other gaze events, the distributions of the reorienting signals were different across the two modalities, with EEG reorienting signals leading that of the pupil reorienting signals. We also found that the hybrid classifier that integrates EEG, pupil and dwell time features detects the reorienting signals in both the fixed (AUC = 0.79) and the free (AUC = 0.77) condition. Significance. We show that the neural and ocular reorienting signals are distributed differently across gaze events when a subject is immersed in VR, but nevertheless can be captured and integrated to classify target vs. distractor objects to which the human subject orients.

Mechanisms of Congenital Myasthenia Caused by Three Mutations in the COLQ Gene.

The gene encoding collagen like tail subunit of asymmetric acetylcholinesterase (COLQ) is responsible for the transcription of three strands of collagen of acetylcholinesterase, which is attached to the endplate of neuromuscular junctions. Mutations in the COLQ gene are inherited in an autosomal-recessive manner and can lead to type V congenital myasthenia syndrome (CMS), which manifests as decreased muscle strength at birth or shortly after birth, respiratory failure, restricted eye movements, drooping of eyelids, and difficulty swallowing. Here we reported three variants within COLQ in two unrelated children with CMS. An intronic variant (c.393+1G>A) and a novel missense variant (p.Q381P) were identified as compound heterozygous in a 13-month-old boy, with the parents being carriers of each. An intragenic deletion including exons 14 and 15 was found in a homozygous state in a 12-year-old boy. We studied the relative expression of the COLQ and AChE gene in the probands' families, performed three-dimensional protein structural analysis, and analyzed the conservation of the missense mutation c.1142A>C (p.Q381P). The splicing mutation c.393+1G>A was found to affect the normal splicing of COLQ exon 5, resulting in a 27-bp deletion. The missense mutation c.1142A>C (p.Q381P) was located in a conserved position in different species. We found that homozygous deletion of COLQ exons 14–15 resulted in a 241-bp deletion, which decreased the number of amino acids and caused a frameshift translation. COLQ expression was significantly lower in the probands than in the probands' parents and siblings, while AChE expression was significantly higher. Moreover, the mutations were found to cause significant differences in the predicted three-dimensional structure of the protein. The splicing mutation c.393+1G>A, missense mutation c.1A>C (p.Q381P), and COLQ exon 14–15 deletion could cause CMS.

P37 Significance of a swollen eye in a child with Down syndrome

Case report - IntroductionDue to dysregulation of their immune system, children with Down syndrome are at increased risk of a number of conditions, including malignancy, infection and autoimmune disorders. This case highlights the importance of the MDT team working in order to fully explore possible diagnoses and reach the correct conclusion. We present a case of a relatively rare eye disorder, initially treated as peri-orbital cellulitis, that required a collaborative team approach to reach the final diagnosis.Case report - Case descriptionA 6-year-old-girl with Down syndrome was referred to the oncology and tertiary ophthalmology teams by her local paediatric team with a history of recurrent right-eye peri-orbital swelling. The impression had been of recurrent peri-orbital cellulitis which was treated with and responsive to antibiotics. However, the most recent presentation was unresponsive to antibiotics and had associated orbital symptoms of proptosis and restricted eye movement. An MRI-head showed a protruding right eye with an enhancing mass centred in the right-superior temporal-extraconal-orbit. The mass was infiltrating the extra-ocular muscles and there was low bone-marrow signal compared to the left-side. The MRI findings were discussed in the oncology MDT-meeting. Appearances were felt to be more in keeping with malignancy, rather than infection. An ophthalmology review confirmed no evidence of optic-nerve compression, so she was discharged on antibiotics with a plan made to admit for biopsy and bone marrow aspirate under general anaesthetic. Histology of the lesion was reported to show a mixed inflammatory-mass, angiocentric with no elements of neoplasia. Oncology-specific stains were negative. BMA was normal. The differential at this point was orbital-pseudo-tumour; if infection and systemic-auto-immune conditions could be excluded. Histochemical-staining of the biopsy excluded fungi, bacteria or mycobacteria. The granulomatous-nature of the infiltrate and the conspicuous presence of eosinophils were consistent with an autoimmune-condition. The little girl was discharged from the oncology team into the care of the ophthalmology and paediatric rheumatology teams for further work-up and management. She was commenced on prednisolone.On rheumatology-review, focussed-history and examination were unremarkable in terms of an underlying systemic-inflammatory condition. A full-blood-work-up was requested. Her pANCA and MPO were mildly raised, as were her inflammatory markers. In view of these results, a CT chest/abdomen angiogram was arranged which was normal. A diagnosis of orbital-pseudotumour was made. There was clinical improvement on prednisolone, so a tapering plan was provided.Case report - DiscussionFirst described by Birch-Hirschfield in 1905, orbital pseudo-tumour, also known as idiopathic orbital inflammatory syndrome (IOIS) is a benign, non-infective, inflammatory condition of the orbit without identifiable local or systemic causes. Post-treatment recurrence occurs in 37% of cases.In this case, as steroids were weaned, there was recurrence of eye symptoms with proptosis and redness of the eye. Repeat bloods were performed, and an MRI-head requested. The repeat ANCA-MPO was negative. Whilst awaiting her MRI, a further clinical review was carried out jointly by paediatric rheumatology and ophthalmology. At this appointment, there was notable proptosis which appeared to be getting worse. The impression was that there was recurrence of the inflammatory swelling, and that the sensible option would be to restart treatment with oral prednisolone. At this point, addition of a steroid-sparing DMARD was discussed. Methotrexate was chosen and commenced. The little girl continued to attend for joint rheumatology and ophthalmology reviews. She continued to have proptosis in the right eye but had excellent ocular motility and good visual-acuity. She was noted to develop a worsening in the lid swelling and possible proptosis with an intercurrent illness, perhaps suggesting that the underlying inflammatory process was still active. The plan was to continue methotrexate for 2 years and perform another orbital MRI before potentially reducing methotrexate. However, in this time the methotrexate dose was reduced due to deranged LFTs. She continued to have “flare ups” with increasing proptosis despite treatment with methotrexate. A repeat MRI after 2 years of treatment showed reduction in the size of the right supra-lateral orbital mass compared to the first scan. However, there was uniform enhancement following gadolinium injection with mild proptosis of the right-globe. The appearances were in keeping with an active granulomatous lesion. The current plan is to try and re-optimise the dose of methotrexate. If not tolerated, an alternative DMARD will need to be considered.Case report - Key learning pointsThe diagnosis of orbital pseudo-tumour is one of exclusion with evaluation directed to exclude neoplasms, infections, and systemic inflammatory disorders. It can manifest as a bilateral condition in 13% and with constitutional symptoms in 40%.Points for discussionDifferential of orbital pseudo-tumours in the paediatric populationPrognosis of pseudo-tumoursSuggested treatment pathway -- Is control to allow normal eye movement enough or should we be treating more aggressively?

Last Guidelines Overview of Consecutive Esotropia Management: Review Article

Background: Consecutive esotropia (ET) is persistent esodeviation for 24 weeks afterward bilateral lateral rectus recession (BLRR) for correcting exotropia (XT) with or without diplopia. Some patients may have limited eye movement; amblyopia and loss of binocularity can result. Early postoperative overcorrection has been recommended in surgical treatment of intermittent XT due to tendency towards postoperative exotropic drift. ET with small angles (within 15 PD) vanishes naturally over time, whereas bigger angles are more likely to be present at the start. Patients who have ET that has persisted for at least 24 weeks after BLRR and has been present for more than 15 postoperative days should have surgery. Objective: Hallmark the updated lines of management of consecutive esotropia. Conclusion: For the purpose of maintaining one MR muscle for a future intervention, several research have shown that ET can be performed consecutively after BLR recession by advancement of LR muscle previously recessed and MR muscle recession in the more deviating eye. Studies in recent years have sought to determine the effectiveness of the use of lateral rectus advancement in the treatment of consecutive ET.

Clinical Correlation Between Vertical Gaze Palsy and Midbrain Volume in Progressive Supranuclear Palsy.

Supranuclear vertical gaze palsies and slowed vertical saccades are characteristic clinic features of progressive supranuclear palsy (PSP). The "hummingbird sign," reflective of midbrain atrophy, is a classic radiographic sign of PSP. Correlation between eye movement abnormalities and radiographic findings in PSP has been reported previously. However, due to the use of clinical criteria not commonly employed in neuro-ophthalmic practice and neuroimaging techniques that are not widely available, it remains unclear whether correlation between midbrain structure and characteristic ocular-motor disturbances can be helpful to neuro-ophthalmologists seeking to adjudicate difficult or unusual diagnostic cases. Patients with a diagnosis of probable PSP according to Movement Disorders Society criteria were studied retrospectively. A neuroradiologist calculated brainstem volumes in enrolled participants and normal controls. Spearman correlations were used to correlate the extent of eye movement limitation as assessed by 2 neuro-ophthalmologists with brainstem volumes. Fourteen participants with PSP and 15 healthy controls with similar age and gender distribution were enrolled and evaluated retrospectively. All 14 participants with PSP had undergone MRIs. Midbrain atrophy significantly correlated with the PSP rating scale (P < 0.001). PSP patients had significantly reduced volumes in the midbrain (P -0.0026), tegmentum (0.0001), tectum (0.0001), and medulla (P = 0.0024) compared with normal controls. Notes documenting quantified ocular motor function were available in 7 of 14 participants with PSP. Midbrain atrophy significantly correlated with in the extent of upward gaze limitation (P = 0.03). The severity of upward gaze limitation correlates with the severity of midbrain atrophy in patients with PSP. Recognition of this correlation may help to adjudicate diagnostic dilemmas and guide further evaluation.

English

BACKGROUND Thyroid eye disease is a relatively rare condition, with an incidence of 2.9 to 16.0 cases per 100 000 population per year. Approximately 50 % of patients with Graves’ disease (GD) develop clinically apparent thyroid eye disease. It may cause severe damage to vision and orbital architecture. It is the most frequent cause of unilateral or bilateral proptosis in adults. METHODS A cross sectional study of 80 patients with GD was carried out in association with thyroid clinic of Government Medical college Thiruvananthapuram for a period of 1 year from April 2017 to March 2018. Subjects who have a prior diagnosis of Graves’ disease including those who are on antithyroid drugs were included in the study. Patients who are sick due to other systemic diseases like cardiac failure and end stage renal disease were excluded. RESULTS Eighty patients with mean age of 45.31 years were studied. Out of them, 66% were females and 34% were males. Ophthalmopathy was present in 38.8%.Majority had mild and bilateral disease (61.2 %). Only a small percentage had sight threatening disease (6.4 %).The mean age of patients with ophthalmopathy was 47.93. Major population with ophthalmopathy was females. Majority of patients with ophthalmopathy (64.5 %) retained a good visual acuity better than 6 / 9. Lid retraction was the most common manifestation among patients with Graves’ ophthalmopathy that is 74.2% followed by exophthalmos (64.5 %) and eye movement restriction and soft tissue involvement (58.1 %). Diplopia, optic nerve dysfunction were rare (3.2 %). Only 19.3 % patients had active disease according to clinical activity score. Major clinical sign of activity was redness of conjunctiva. Maximum no. of patients with active disease had a clinical activity score of 4. Smoking showed a significant association with the severity of ophthalmopathy. (p value 0.001) There was a significant association between age and activity of disease. (p value 0.021). No association was found between duration of disease with presence or severity of ophthalmopathy. There was no association between co- morbidities with presence or severity of ophthalmopathy. No association was found between hormone status and presence or severity of ophthalmopathy. CONCLUSIONS Our results indicated that the prevalence of ophthalmopathy in our population with GD evaluated at our tertiary care centre was similar to that reported in the Caucasians of European origin. Clinically active and sight threatening ophthalmopathy was uncommon. KEYWORDS Graves’ Disease, Ophthalmopathy

Clinical Characteristics, Molecular Background, and Survival of Egyptian Patients With Gaucher Disease Over a 20-Year Follow-up.

This study analyzes the general disease characteristics, impact of enzyme replacement therapy (ERT), and overall survival (OS) of 156 Egyptian patients with Gaucher disease (GD) enrolled on hormone replacement from 1998 to 2017. The mean age at diagnosis was 32.46±12.68 months. Anemia was noted at diagnosis in 50%, thrombocytopenia in 30.7%, severe splenomegaly in 58.7%, severe hepatomegaly in 11.9%, and skeletal findings were detected in 24.3% of the patients. The most prevalent GD type was type 3 (54.5%). Twenty-two of type 3 patients had no neurological manifestations at diagnosis, and 12 developed variable central nervous system manifestations during follow-up. The most common neurological features were limited eye movements, oculomotor apraxia, and squint. Of the 60 patients for whom genotypes were obtained, homozygous L444P was the most common (n=35/60, 58.3%). Treatment with ERT (imiglucerase) revealed significant improvements in blood indices, organ volumes, and growth parameters (P<0.05). Ten (11.7%) type 3 patients did not develop any neurological manifestations under ERT over 20 years. Mortality was 16%, and the 20-year OS was 73.3%. We conclude that in Egypt, type 3 is the most prevalent phenotype of GD, and homozygous L444P is the predominant GBA genotype of GD. Early age at diagnosis and treatment with ERT over 20 years revealed significant improvements in disease manifestations, with an OS of 73.3%.

Ectopic intraconal orbital meningioma – A rare case report

Background: Ectopic orbital meningiomas (OM) are a rare subset of OMs which are neither attached to the optic nerve sheath nor to the surrounding bone.Case Description: We report the case of a 65-year-old female who presented with a 1 year history of proptosis followed by visual loss and restricted right eye movements since 3 months. Radiology of the orbits was suggestive of intraorbital, intraconal, and heterogeneous contrast enhancing right eye lesion which was completely excised through supraorbital orbitotomy approach. Intraoperatively, the right optic nerve, though compressed, and displaced inferiorly, was free from the lesion. The final histopathological diagnosis was “Meningioma WHO Grade I.” At 3 months follow-up, patient’s vision in the right eye improved from perception of light positive to 6/12 and there was no evidence of recurrence.Conclusion: Rarity of ectopic OM, total surgical excision with an excellent postoperative visual outcome prompted us to report this case.

Macular buckling for highly myopic traction maculopathy

Objective: To observe the efficacy of macular buckling in the treatment of highly myopic traction maculopathy. Methods: Retrospective case series study. The patients with high myopia who underwent macular buckling at the Zhongshan Ophthalmic Center of Sun Yat-sen University from June 2014 to June 2019 were enrolled, including 136 males and 212 females. The age was (56.68±11.59) years old. The outcomes measured included retinal reattachment rate, foveoschisis recovery rate, macular hole closure rate, postoperative best corrected visual acuity (BCVA), axial length (AL), and complications. The measurements were recorded preoperatively and at 1 month, 6 months, 1 year, 2 years, and 3 years postoperatively. The data was statistically analyzed using paired t test. Results: A total of 378 eyes were included, including 216 eyes with foveoschisis and macular detachment and 162 eyes with macular holes and macular detachment. Among them, 296 eyes underwent macular buckling, and the other 82 eyes underwent macular buckling combined with pars plana vitrectomy. During the follow-up period, 373 eyes (98.68%) achieved retinal reattachment; in patients with foveoschisis, 204 eyes (94.44%) were recovered; in patients with macular holes, 89 eyes (54.09%) achieved closure. All the postoperative results of BCVA were better than the preoperative value (1.459±0.841). BCVA continued to increase from postoperative month 1, remained stable at 1 year, and reached 0.908±0.606 at 3 years (t=6.896, P<0.01). All the postoperative results of AL were shorter than the preoperative value. The AL shortened by (4.423±1.740)mm at one month (t=33.144, P<0.01), increased gradually thereafter, remained stable at 1 year, and shortened by (2.101±1.643) mm at three years (t=6.392, P<0.01). The common complications included transient high intraocular pressure in 98 eyes (25.92%), epiretinal hemorrhage in 67 eyes (17.72%), and vitreous hemorrhage in 9 eyes (2.38%), which all resolved spontaneously within 1 month. In the early postoperative period, all patients had a certain degree of eye movement limitation, and 39 eyes (10.31%) had diplopia which resolved within 6 months without treatment. The strabismus surgery was arranged to treat esotropia in 6 eyes (1.58%). The macular buckle was removed from 1 eye (0.26%) because of the inability to tolerate diplopia. There were 8 eyes (2.11%) requiring a second operation to adjust the position of the buckle. The macular buckle was also removed from 4 eyes (1.05%) due to the implant rejection. Conclusion: Macular buckling can effectively shorten the AL, resolve posterior scleral staphyloma, and improve vision in the treatment of highly myopic traction maculopathy. (Chin J Ophthalmol, 2021, 57: 433-439).

Clinical improvement in indirect carotid cavernous fistulas treated endovascularly: A patient based review

Patients with indirect carotid cavernous fistulas (CCF) were reviewed to emphasize the importance of diagnosing patients even with trivial findings and to raise awareness. Eighteen patients diagnosed as CCF were included. Neuro-ophthalmological findings before and after treatment, diagnostic investigations, treatment, clinical course was noted. Twelve patients were female (67%), 6 were males (33%) and the mean age at presentation was 54 years (range: 29–70 years). Conjunctival hyperemia was present in all patients. Seventeen (94%) patients had proptosis and diplopia, nine (50%) had orbital pain and/or headache, four (22%) had blurred vision, one (5.5%) had ptosis. On examination, 17 patients (94%) had restricted eye movements, four (22%) had low visual acuity and five patients had (28%) increased intraocular pressure (IOP). One patient had been diagnosed as myasthenia gravis and two as thyroid orbitopathy and had been on treatment accordingly before CCF was diagnosed. In two patients, bilateral findings were present despite unilateral CCF on angiography. Barrow Type B fistula was found in 7 (38%), Barrow Type D in 11 (62%) patients. In three bilateral CCF was detected. All were treated by endovascular intervention. Residual deficits at the sixth month control were, eye movement deficits in seven (39%), decrease in visual acuity in one (5.5%) and elevated IOP in one (5.5%) patient. Indirect CCF patients generally present with mild symptoms and the diagnosis may be overlooked. Mild progressive ophthalmoparesis with conjunctival hyperemia must be warning. Though rare bilateral CCF can be detected as well as unilateral CCF with bilateral findings.

Evaluation of Eye Alignment before and after Cataract Surgery

ABSTRACT Purpose: To assess changes in eye alignment before and after cataract surgery. Subjects and methods: The medical records of 786 cataract patients without eye movement limitation who underwent bilateral cataract surgery under topical anesthesia with monofocal IOL placement were retrospectively reviewed at the Eye Center of Sanno Hospital in Japan. Results: Before cataract surgery, 121 of the 786 patients (15.4%) had constant strabismus, 263 (33.5%) had intermittent strabismus, and 402 (51.1%) had phoria. Among the 121 patients with constant strabismus on the preoperative exam, 64 (52.9%) had exotropia without vertical strabismus, 7 (5.8%) had esotropia without vertical strabismus, and 6 (5.0%) had vertical strabismus without a horizontal component. After surgery, 98 of the 786 patients (12.5%) had constant strabismus, 126 (16.0%) had intermittent strabismus, 562 (71.5%) had phoria. The rate of eye alignment agreement before and after surgery was 72.3% overall. We found that 17 patients (2.2%) who did not have constant strabismus preoperatively developed constant strabismus postoperatively, and all of these patients experienced binocular diplopia. Characteristics of patients at risk for constant strabismus and diplopia postoperatively included patients with vertical deviation preoperatively (intermittent or phoria). Conclusions: Uncomplicated cataract surgery under topical anesthesia did not influence eye alignment classification in 72.3% of patients; however, patients with vertical deviation preoperatively were more likely to transition to constant strabismus after surgery.

Clinical Analysis of 2 170 Cases of Thyroid-Associated Ophthalmopathy Involving Extraocular Muscles

To explore the clinical features of thyroid-associated ophthalmopathy (TAO) with extraocular muscle involvement. The data of 2170 TAO patients who were seen at the Orbital Disease Clinic, West China Hospital, Sichuan University from September, 2009 to January, 2020 were collected retrospectively. The extraocular muscle involvement of these patients was confirmed by CT or MRI. Their general condition, medical history, clinical manifestations and imaging features were analyzed retrospectively. Among the 2170 TAO patients, 932 were male and 1238 were female. The mean (± SD) age of all the patients was (46.95±13.06) years, ranging between 6 and 85. 1684 patients (77.60%) suffered from hyperthyroidism, 13 patients (0.59%) had thyroid cancer, 80 patients (3.69%) had hypothyroidism, and 393 patients (18.11%) had normal thyroid function. Proptosis (55.25%) and diplopia (33.09%) were the main reasons for their visits to the clinic, and restricted eye movements (83.46%) was the most common sign. 122 patients with a mean age of (53.24±13.07) years did not show any eyelid sign and had only extraocular muscle involvement. The 2170 TAO patients had a total of 3799 eyes of extrocular muscle involvement, with 541 patients experiencing monocular involvement and 1629 patients, binocular involvement; 1204 eyes (31.69%) had a single extrocular muscle involved and 2595 eyes (68.31%) had multiple extrocular muscles involved. Inferior rectus was the most commonly involved muscle, followed by superior rectus, medial rectus, and lateral rectus in descending order of involvement frequency. Of the 1014 patients who underwent enhanced MRI, 71.99% were shown to be in the active phase. 69.03% of the 775 patients identified as being in inactive phase according to their clinical activity score (CAS) were shown to be in the active phase according to their MRI results. TAO patients with extraocular muscle involvement have their own specific clinical manifestations. CT and MRI can both be used to assist in the diagnosis of extraocular muscle involvement. MRI can be used to assess the pathological stage of extraocular muscles and is more sensitive than CAS.