A Case of Congenital Idiopathic Enlargement of Extraocular Muscles.

The purpose of this case report is to report a case of congenital idiopathic enlargement of extraocular muscles. A four-month-old girl showed limitation of adduction and supraduction in the right eye. A computerized axial tomography (CAT) scan revealed hypertrophy of the lateral rectus muscle and inferior rectus muscle of the right eye. Thyroid hormone and antibody levels were normal. No inflammatory findings on magnetic resonance imaging (MRI). A traction test under general anesthesia revealed a strong limitation of supraduction and a mild limitation of adduction. Therefore, the inferior rectus muscle was recessed 4.5 mm at the age of six months. A partial biopsy of the inferior rectus showed no inflammatory cell infiltration. After the first surgery, the patient's limitation of supraduction improved, but the limitation of adduction persisted. So, a 5 mm recession of the right lateral rectus muscle was added at one year and one month. However, the hypertropia of the sound eye became stronger after treatment of amblyopia. Because of the strong limitation of supraduction, tenotomy of the inferior rectus was performed at the age of six years. Postoperatively, no impairment of infraduction occurred, and the limitation of supraduction was mildly improved. Since the findings on MRI were not changed through our observation period, we concluded that the patient had idiopathic external ophthalmoplegia.

Oral Health-Related Quality of Life in Rare Disorders of Congenital Facial Weakness.

Congenital facial weakness (CFW) encompasses a heterogenous set of rare disorders presenting with decreased facial movement from birth, secondary to impaired function of the facial musculature. The aim of the present study is to provide an analysis of subject-reported oral health-related quality of life (OHRQoL) in congenital facial weakness (CFW) disorders. Forty-four subjects with CFW and age- and sex- matched controls were enrolled in an Institutional Review Board (IRB)-approved study. Demographic data, medical and surgical history, comprehensive oral examination, and the Oral Health Impact Profile (OHIP-14) were obtained. Compared to unaffected controls, subjects with CFW had higher OHIP-14 scores overall (mean ± SD: 13.11 ± 8.11 vs. 4.46 ± 4.98, p < 0.0001) and within five of seven oral health domains, indicating decreased OHRQoL. Although subjects with Moebius syndrome (MBS) were noted to have higher OHIP-14 scores than those with Hereditary Congenital Facial Paresis (HCFP), there was no significant correlation in OHIP-14 score to age, sex, or specific diagnosis. An increase in OHIP-14 scores in subjects was detected in those who had undergone reanimation surgery. In conclusion, subjects with CFW had poorer OHRQoL compared to controls, and subjects with MBS had poorer OHRQoL than subjects with HCFP. This study provides better understanding of oral health care needs and quality of life in a CFW cohort and suggests that guidelines for dental treatment are required.

Extreme Tolerance of Extraocular Muscles to Diseases and Aging: Why and How?

The extraocular muscles (EOMs) possess unique characteristics that set them apart from other skeletal muscles. These muscles, responsible for eye movements, exhibit remarkable resistance to various muscular dystrophies and aging, presenting a significant contrast to the vulnerability of skeletal muscles to these conditions. In this review, we delve into the cellular and molecular underpinnings of the distinct properties of EOMs. We explore their structural complexity, highlighting differences in fiber types, innervation patterns, and developmental origins. Notably, EOM fibers express a diverse array of myosin heavy-chain isoforms, retaining embryonic forms into adulthood. Moreover, their motor innervation is characterized by a high ratio of nerve fibers to muscle fibers and the presence of unique neuromuscular junctions. These features contribute to the specialized functions of EOMs, including rapid and precise eye movements. Understanding the mechanisms behind the resilience of EOMs to disease and aging may offer insights into potential therapeutic strategies for treating muscular dystrophies and myopathies affecting other skeletal muscles.

Genetic investigation in a four-generation Chinese family with congenital fibrosis of extraocular muscles and keratoconus.

Here, we have reported the genetic and clinical characteristics of four generations of a family patient from China with congenital fibrosis of extraocular muscles 1 (CFEOM1) and keratoconus (KC). The history of diseases, clinical observations, and blood samples of all family members were collected. A total of 100 healthy participants were recruited as normal controls. The whole exome sequencing of the genomic DNA and polymerase chain reaction were performed on samples obtained from the controls and their family members to verify the gene variants. The functional analyses of the variants were performed by using different software. Two single nucleotide polymorphisms were detected in the proband and other patients in his families, including a heterozygous missense variation, g.39726207C > T (c.2860C > T, p.R954W, rs121912585), in the third highly conserved coiled-coil domain of KIF21A, and a heterozygous missense variant, g.30664732A > C (c.136A > C, p.S46R, rs200111443) in TGFBR2. The variant p.R954W in KIF21A was predicted to be pathogenic using software, whereas p.S46R in TGFBR2 was predicted to be of uncertain significance (VUS). Thus, KC might have occurred in the proband and his daughter because of a combination of genetic mutations and involuntary eye rubbing induced by CFEOM1. This is the first case of concomitant KC in a family having CFEOM1. Thus, the study provides new information about patients with KC having CFEOM1. Furthermore, the study suggests that attention should be paid to the early detection and diagnosis of KC in patients with CFEOM1.

MRI evaluation of cranial nerve abnormalities and extraocular muscle fibrosis in duane retraction syndrome and congenital extraocular muscle fibrosis.

PURPOSE: To investigate the alterations in extraocular muscles (EOMs) by magnetic resonance imaging (MRI) among patients diagnosed with Duane retraction yndrome (DRS) and congenital fibrosis of the extraocular muscles (CFEOM), who present with various cranial nerve anomalies in an attempt to enhance the clinical diagnostic process. METHODS: A case-control study was conducted to evaluate 27 patients with DRS and 14 patients with CFEOM. All patients underwent MRI scans of the brainstem and orbital examination. Neurodevelopmental assessments were conducted through MRI, and maximum cross-sectional area and volumes of EOMs were obtained. Three types of models were constructed using machine learning decision tree algorithms based on EOMs to predict disease diagnosis, cranial nerve abnormalities, and clinical subtypes. RESULTS: Patients with bilateral CN VI abnormalities had smaller volumes of LR, MR, and IR muscles compared to those with unilateral involvement (P < 0.05). Similarly, patients with CFEOM and unilateral third cranial nerve abnormalities had a smaller maximum cross-section of the affected eye's SR compared to the contralateral eye (P < 0.05). In patients with both CN III and CN VI abnormalities, the volume of SR was smaller than in patients with CN III abnormalities alone (P < 0.05). The prediction model using EOMs volume showed a diagnostic precision of 82.5% for clinical cases and 60.1% for predicting cranial nerve abnormalities. Nonetheless, the precision for identifying clinical subtypes was relatively modest, at only 41.7%. CONCLUSION: The distinctive volumetric alterations in EOMs among individuals exhibiting distinct cranial nerve anomalies associated with DRS or CFEOM provide valuable diagnostic insights into to Congenital Cranial Neurodevelopmental Disorders (CCDDs). MRI analysis of EOMs should thus be regarded as a crucial diagnostic modality.

Distribution and appearance of myosin, dystrophin, and collagen IV in strabismus-affected extraocular muscle tissue compared with control tissue.

OBJECTIVE: Extraocular muscles have complex development processes. The present study aimed to analyze the presence of myosin, dystrophin, and collagen IV in the strabismus-affected extraocular muscle. METHODS: This research was an observational case-control study. Myosin, dystrophin, and collagen IV were detected by histological and immunohistochemical analyses of extraocular muscle samples from concomitant strabismus patients and controls. A semi-quantitative grading method and statistical analysis were used. RESULTS: In the strabismus-affected extraocular muscle, morphological analysis demonstrated different-sized muscle fibers. Immature muscle fibers and an increased amount of connective tissue were also noted. Strong positive correlations were identified between myosin and collagen IV and between dystrophin and collagen IV. CONCLUSIONS: The presence of newly formed muscle fibers, increased connective tissue, and variable diameters of skeletal striated muscle fibers indicate the decreased quality of extraocular muscles in strabismus cases. Reduced levels of myosin and dystrophin and a near absence of collagen IV in strabismus-affected skeletal striated muscle fibers characterized the muscular dystrophy of strabismus. Adjuvant therapy aimed at normalizing the metabolism of these muscles may be appropriate alongside concomitant strabismus treatment.

New Multi-Parameters Combination of Technetium-99m-Diethylene-Triamine-Pentaacetate Orbital Single-Photon Emission Computed Tomography/Computed Tomography for the Evaluation of Graves' Orbitopathy Activity.

PURPOSE: This study investigates the new combined parameters of 99mTc-DTPA orbital single-photon emission computed tomography/computed tomography (SPECT/CT) for the evaluation of Graves' orbitopathy (GO) activity. METHODS: A retrospective analysis was performed on 41 patients. All the patients undergone the 99mTc-DTPA orbital SPECT/CT and were categorized into active and inactive group based on the standard combined by the clinical active score (CAS), magnet resonance imaging (MRI) and/or follow-up results. Quantitative parameters of lacrimal gland (LG) including the protruding degree of lacrimal gland herniation (LGH) and uptake ratios (URs) of region of interest (ROI) drawn on lacrimal gland and occipital bone. SPECT/CT reading results were based on visual analysis. Parameters were compared between the two groups and the diagnostic value on discrimination of GO activity was also evaluated. RESULTS: All parameters of SPECT/CT for active GO groups were significantly higher than those of the inactive groups (p<.05). There were notable linear positive correlations between the assumption standard and readings as well as combination models 2 and 3 (r = .794, r = .772, r = .760, respectively). ROC analysis indicated that model 2 provided the highest diagnostic performance, exhibiting an area under the curve (AUC) of .947, a sensitivity of 92.7%, and a specificity of 88.6%. CONCLUSIONS: The combined use of SPECT/CT reading results and DTPA uptake parameters of LG offers a more objective and precise evaluation of active GO. This study further recommends 99mTc-DTPA SPECT/CT might be serving as a supplementary beneficial approach for CAS in evaluating GO activity.

Extraocular muscle stem cells exhibit distinct cellular properties associated with non-muscle molecular signatures.

Skeletal muscle stem cells (MuSCs) are recognised as functionally heterogeneous. Cranial MuSCs are reported to have greater proliferative and regenerative capacity when compared with those in the limb. A comprehensive understanding of the mechanisms underlying this functional heterogeneity is lacking. Here, we have used clonal analysis, live imaging and single cell transcriptomic analysis to identify crucial features that distinguish extraocular muscle (EOM) from limb muscle stem cell populations. A MyogeninntdTom reporter showed that the increased proliferation capacity of EOM MuSCs correlates with deferred differentiation and lower expression of the myogenic commitment gene Myod. Unexpectedly, EOM MuSCs activated in vitro expressed a large array of extracellular matrix components typical of mesenchymal non-muscle cells. Computational analysis underscored a distinct co-regulatory module, which is absent in limb MuSCs, as driver of these features. The EOM transcription factor network, with Foxc1 as key player, appears to be hardwired to EOM identity as it persists during growth, disease and in vitro after several passages. Our findings shed light on how high-performing MuSCs regulate myogenic commitment by remodelling their local environment and adopting properties not generally associated with myogenic cells.

Conformation of Horizontal Extraocular Muscle Insertions in Acute Acquired Concomitant Esotropia Induced by Excessive Digital Device Usage.

Purpose: Acute acquired concomitant esotropia induced by excessive digital device usage, especially smartphones (SAACE), has been increasing over the past few years. Convergence spasm induced by excessive near work has been suggested as a mechanism. Anatomical differences could also potentially contribute to SAACE onset. The present study investigated the conformation of horizontal recti between SAACE patients and normal subjects. Patients and Methods: In 15 SAACE patients (SAACE group), the distances between the limbus and insertion of the horizontal recti (LI distance) and the widths of horizontal recti on the insertion (insertion width) were measured. The control group consisted of 30 patients who underwent retinal detachment surgery. Differences in LI distances and insertion widths were compared between SAACE and control groups. Results: While there were no differences between the two groups for LI distances and insertion widths of lateral recti, there were significantly shorter LI distances for the medial recti in the SAACE group (P<0.05). Moreover, the SAACE group tended to exhibit larger insertion widths of the medial recti. Medial/lateral ratio of LI distances were significantly lower and insertion widths were significantly higher in the SAACE compared to the control group (P<0.05). Conclusion: Based on the observations of more anterior insertion and larger muscle widths, this suggests there are stronger forces of medial recti in SAACE. In addition to excessive accommodation followed by increases in medial recti tonus, the results also suggest that an anatomical imbalance between lateral and medial recti contributes to esotropia onset following excessive near work.

Magnetic resonance imaging of extraocular rectus muscles abnormalities in acute acquired concomitant esotropia.

AIM: To investigate the difference of medial rectus (MR) and lateral rectus (LR) between acute acquired concomitant esotropia (AACE) and the healthy controls (HCs) detected by magnetic resonance imaging (MRI). METHODS: A case-control study. Eighteen subjects with AACE and eighteen HCs were enrolled. MRI scanning data were conducted in target-controlled central gaze with a 3-Tesla magnetic resonance scanner. Extraocular muscles (EOMs) were scanned in contiguous image planes 2-mm thick spanning the EOM origins to the globe equator. To form posterior partial volumes (PPVs), the LR and MR cross-sections in the image planes 8, 10, 12, and 14 mm posterior to the globe were summed and multiplied by the 2-mm slice thickness. The data were classified according to the right eye, left eye, dominant eye, and non-dominant eye, and the differences in mean cross-sectional area, maximum cross-sectional area, and PPVs of the MR and LR muscle in the AACE group and HCs group were compared under the above classifications respectively. RESULTS: There were no significant differences between the two groups of demographic characteristics. The mean cross-sectional area of the LR muscle was significantly greater in the AACE group than that in the HCs group in the non-dominant eyes (P=0.028). The maximum cross-sectional area of the LR muscle both in the dominant and non-dominant eye of the AACE group was significantly greater than the HCs group (P=0.009, P=0.016). For the dominant eye, the PPVs of the LR muscle were significantly greater in the AACE than that in the HCs group (P=0.013), but not in the MR muscle (P=0.698). CONCLUSION: The size and volume of muscles dominant eyes of AACE subjects change significantly to overcome binocular diplopia. The LR muscle become larger to compensate for the enhanced convergence in the AACE.

ULTRASOUND EXAMINATION OF THE ORBIT IN PATIENTS WITH THYROIDASSOCIATED ORBITOPATHY - EXAMINATION GUIDE AND RECOMMENDATIONS FOR EVERYDAY PRACTICE. A REVIEW.

The purpose of this study is to present the possibilities and benefits of ultrasonography (US) of the orbit in the diagnosis and treatment of thyroidassociated orbitopathy (TAO). Methods: US examination of the orbit is an essential addition to clinical and laboratory examination in TAO patients. Nevertheless, it is often neglected in clinical practice or indicated with delay. Based on previously published studies and our experience with the diagnosis and treatment of TAO patients, we aim to highlight the clear benefit of US examination of the orbit and oculomotor muscles, not only for correct TAO diagnosis but also in the monitoring of the disease over time. However, knowledge of the drawbacks and limitations of this method is also essential, as we shall point out. It is always necessary to remember that US examination must be evaluated in connection with the clinical findings. A detailed recommendation for US examination of the extraocular muscles and the orbit based on our experiences with diagnosing and treating TAO patients in daily practice is also included. Conclusion: According to our experience, US examination of the orbit is an excellent and irreplaceable tool for timely TAO diagnosis and further disease monitoring. However, considerable examiner experience and detailed knowledge of the clinical and ultrasound manifestations of TAO are essential.

Impact of sphenoid trigone size and extraocular muscle thickness on the outcome of lateral wall orbital decompression for thyroid eye disease.

BACKGROUND: To retrospectively review lateral wall orbital decompression for thyroid eye disease (TED) and to evaluate pre-operative CT scans to analyse the variation in proptosis reduction. METHODS: Consecutive lateral wall orbital decompressions performed by a single surgeon were retrospectively reviewed. Pre-operative CT scan features and post-operative proptosis reduction were analysed. The sphenoid trigone cross-sectional areas were summed and multiplied by the slice thickness to yield bone volume. Cumulative extraocular muscle thickness was calculated by combining the maximum thickness of the four recti. "Trigone volume" and "cumulative muscle thickness" were correlated with proptosis reduction at 3 months post-surgery. RESULTS: Out of 73 consecutive lateral wall orbital decompressions, 17 orbits had prior endonasal medial wall orbital decompression. In the remaining 56 orbits, the mean pre-operative and post-operative proptosis were 24.3 ± 1.6 mm and 20.9 ± 2.3 mm respectively. The proptosis reduction ranged from 1 to 7 mm (mean of 3.5 mm ± 1.3 (p < 0.001)). Mean sphenoid trigone volume was 895 ± 434.4 mm3. The mean cumulative muscle thickness was 20.4 ± 5 mm. The correlation coefficient between muscle thickness and proptosis reduction was - 0.3 and was statistically significant (p = 0.043). The correlation coefficient between sphenoidal trigone volume and proptosis reduction was 0.2 (p = 0.068). With a multivariate analysis, the coefficient of efficient of regression for muscle thickness was - 0.007 (p = 0.42) and the coefficient of regression for trigone volume was 0.0 (p = 0.046). CONCLUSION: Proptosis reduction following lateral wall orbital decompression can be variable. Extraocular muscle thickness had a significant correlation with the outcome, with greater proptosis reduction in orbits with thin muscles. The sphenoidal trigone size had a weak correlation with decompression outcome.

Echographic characterization of extraocular muscles in pediatric patients with thyroid dysfunction.

PURPOSE: To characterize the size of extraocular muscles (EOMs) in a pediatric population with thyroid dysfunction using orbital echography. METHODS: Patients under age 18 with thyroid dysfunction who presented to an academic ophthalmology department from 2009 to 2020 and received orbital echography were included in this IRB-approved retrospective study. Data collected included age, clinical activity score (CAS), thyroid stimulating immunoglobulin (TSI), and extraocular recti muscle thickness on echography. Patients were organized into three age cohorts, after which statistical analysis compared recti measurements to previously reported normal ranges. RESULTS: Twenty patients with thyroid dysfunction were included. When comparing average recti muscle thicknesses of study patients to those of previously published normal children in similar age ranges, the levator-superior rectus complex was significantly increased in all age groups of children with thyroid dysfunction (p-values = <.004), and the levator-superior rectus complex was most frequently enlarged compared to published normal values (78% of eyes). CAS was not correlated with EOM size in the youngest group (5-10 years old, p-values >.315) but was significantly correlated in older groups (11-17 years old, p-values <.027). TSI was not correlated with EOM size in any group (p-values >.206). CONCLUSIONS: Echographic reference ranges for EOMs in children with thyroid dysfunction were established. There are increased rates of levator-superior rectus complex enlargement in children with TED compared to adults with TED, and EOM size is correlated with CAS in children older than 10 years. Though limited, these findings may serve as an additional tool for ophthalmologists to ascertain disease activity in pediatric patients with thyroid dysfunction.

Development of the extraocular muscles during the fetal period.

PURPOSE: The aim of this study was to investigate the morphometric development of the extraocular muscles in the fetal period and to create a modified Tillaux spiral. METHODS: We dissected 157 fetal eyes (82 right eyes, 75 left eyes) obtained from 79 fetuses (46 boys, 33 girls) between 13 and 40 weeks of gestation. The tendon widths of the extraocular muscles and the distances of the tendon attachment sites to the limbus were measured. Tillaux's modified spiral was created. RESULTS: In addition to the rectus muscles, we added tendon widths and tendon-limbus distances of the upper (SO) and lower (IO) obliques to the modified Tillaux spiral. When tendon widths were compared between genders, no statistically significant difference was observed. When tendon widths were compared between the sides, it was determined that SO was more in the left eye, whereas other extraocular muscles were more in the right eye. There was no statistically significant difference between genders when the distances of tendon attachment sites to the limbus were compared. There was no statistically significant difference in SO and IO values between the sides. There was a statistically significant difference in the rectus muscles and this difference was found to be higher in the right eye. CONCLUSION: We think that the findings obtained will contribute to disciplines such as fetopathology, obstetrics, ophthalmology and plastic surgery and to future studies on this subject.

MYH13, a superfast myosin expressed in extraocular, laryngeal and syringeal muscles.

MYH13 is a unique type of sarcomeric myosin heavy chain (MYH) first detected in mammalian extraocular (EO) muscles and later also in vocal muscles, including laryngeal muscles of some mammals and syringeal muscles of songbirds. All these muscles are specialized in generating very fast contractions while producing relatively low force, a design appropriate for muscles acting against a much lower load than most skeletal muscles inserting into the skeleton. The definition of the physiological properties of muscle fibres containing MYH13 has been complicated by the mixed fibre type composition of EO muscles and the coexistence of different MYH types within the same fibre. A major advance in this area came from studies on isolated recombinant myosin motors and the demonstration that the affinity of actin-bound human MYH13 for ADP is much weaker than those of fast-type MYH1 (type 2X) and MYH2 (type 2A). This property is consistent with a very fast detachment of myosin from actin, a major determinant of shortening velocity. The MYH13 gene arose early during vertebrate evolution but was characterized only in mammals and birds and appears to have been lost in some teleost fish. The MYH13 gene is located at the 3' end of the mammalian fast/developmental gene cluster and in a similar position to the orthologous cluster in syntenic regions of the songbird genome. MYH13 gene regulation is controlled by a super-enhancer in the mammalian locus and deletion of the neighbouring fast MYH1 and MYH4 genes leads to abnormal MYH13 expression in mouse leg muscles.

Computed tomography and magnetic resonance imaging of the orbit in the diagnosis and treatment of thyroid-associated orbitopathy - experience from practice. A Review.

The purpose is to acquaint readers with the contribution of imaging methods (IMs) of the orbit, specifically computed tomography (CT) and magnetic resonance imaging (MRI), in the diagnosis of thyroid-associated orbitopathy (TAO). Methods: IMs of the orbit are an indispensable accessory in the clinical and laboratory examination of TAO patients. The most frequently used and probably most accessible method is an ultrasound examination of the orbit (US), which, however, has a number of limitations. Other methods are CT and MRI. Based on the published knowledge implemented in our practice and several years of experience with the diagnosis and treatment of TAO patients, we would like to point out the benefits of CT and MRI in the given indications: visualisation of the extraocular muscles, assessment of disease activity, diagnosis of dysthyroid optic neuropathy and differential diagnosis of other pathologies in the orbit. Our recommendation for an ideal MRI protocol for disease activity evaluation is also included.  Conclusion: IMs play an irreplaceable role not only in the early diagnosis of TAO, but also in the monitoring of the disease and the response to the applied treatment. When choosing a suitable IM for this diagnosis, a number of factors must always be taken into account; not only availability, cost and burden for the patient, but especially the sensitivity and specificity of the given method for the diagnosis of TAO.

Case Report: Extraocular muscles paralysis associated with GAD65 antibody: a case series study.

Objective: To explore the clinical manifestations of glutamic acid decarboxylase 65 (GAD65) antibody-positive patients with extraocular symptoms and the possible mechanism. Method: Assays for the presence of GAD65 antibodies were performed on patients' serum and cerebral spinal fluid (CSF). The brain and ocular structures involved in eye movement were assessed via magnetic resonance imaging (MRI). Tests such as electromyography (EMG), particularly repetitive nerve stimulation (RNS), and neostigmine tests were utilized for differential diagnosis. Additionally, the interaction of GAD65 antibodies with muscle tissue was confirmed using immunofluorescence techniques. Result: Each patient exhibited symptoms akin to extraocular myasthenia gravis (MG), with two individuals reporting diplopia and two experiencing ptosis. GAD65 antibodies were detected in either the serum or CSF, which were shown to bind with monkey cerebellum slides and mouse muscle slides. Neuroimaging of the brain and extraocular muscles via MRI showed no abnormalities, and all patients tested negative for the neostigmine test, RNS via EMG, and the presence of MG antibodies. However, thyroid-related antibodies were found to be abnormal in four of the patients. Conclusion: Our results showed that GAD65 antibodies are not only associated with encephalitis, cerebellum ataxia or stiff-person syndrome caused by the decrease of GABAergic transmission but also diplopia and ptosis. Therefore, we should pay more attention to extraocular muscle paralysis patients without pathogenic antibodies directed against the components of neuromuscular junctions.

[Ocular myasthenia gravis]. / Miasteniya, glaznaya forma.

The article describes an urgent medical and social problem - the diagnosis and treatment of the ocular myasthenia gravis. Despite recent advances in the study of synaptic pathology, the diagnosis of the ocular form of myasthenia gravis remains a challenging problem. This is due to the poor information content of laboratory and electromyographic methods in the diagnosis of ocular myasthenia gravis, and the generalized myasthenia gravis manifests in 90% of cases by external ophthalmoplegia and ptosis. The article highlights the features of the diagnosis and differential diagnosis of ocular myasthenia gravis. Data on the course and features of the clinical presentation of the disease are presented. The importance of analyzing anamnestic data and clinical manifestations of the disease is emphasized. The article discusses modern approaches to the treatment of the ocular myasthenia gravis. The authors present clinical observations of 2 patients with ocular myasthenia gravis.

Pulled-in-two syndrome in strabismus surgery for congenital fibrosis of the extraocular muscles.

Purpose: The aim of this study is to present six cases of pulled-in-two syndrome (PITS) in strabismus surgeries and to discuss our prevention and management strategies. Methods: This is a retrospective study presenting cases of PITS during strabismus operations. The medical records of the subjects who underwent operation in the strabismus unit of Beyoglu Eye Training and Research Hospital were reviewed retrospectively, from January 2000 till March 2022. Detailed ophthalmological examination records and angle of deviation were noted. Results: A total of six cases (four males and two females) with a mean age of 37.2 ± 28.0 (min 9-max 71) years were included in this study. All of the cases had congenital fibrosis of extraocular muscles (CFEOM). The most commonly involved muscle was medial rectus (83%). Majority of cases (67%) were adults. In all cases, the muscle was found and reattached to the globe. No patients had diplopia after surgery. Conclusion: PITS is a rare complication that can be seen during strabismus surgeries, and CFEOM patients are in the risk group. Therefore, surgeons should be cautious and prepared in risky patients to be able to manage this complication.