Superior Rectus Research Articles

Yokoyama procedure for cyclic strabismus with axial high myopia:a case report

BackgroundIt is a very rare form of ocular motility characterized by alternating strabismus and orthotropia. We report a patient with a 48-h cycle of esohypotropia associated with axial high myopia that resolved by Yokoyama procedure.Case presentationA 43-year-old female patient who underwent left medial rectus muscle recession and lateral rectus muscle resection elsewhere due to highly myopic strabismus 2 years ago. The patient experienced a recurrence of left esohypotropia 12 months after undergoing surgery, exhibiting a 48-hour cycle. The cycle is one full day of esohypotropia and one day of orthotropia. The patient exhibited a case of high myopia in the left eye, characterized by a diopter measurement of -24.00DS and an eye axis measurement of 28.43 mm. Orbital CT showed supertemporal dislocation of the posterior portion of the elongated globe out from the muscle cone. Based on these observations, we performed Yokoyama procedure by uniting the muscle bellies of the superior rectus(SR) and lateral rectus (LR) muscles to restoring the dislocated globe back into the muscle cone.ConclusionsWhen cyclic strabismus is combined with axial high myopia, the Yokoyama procedure was effective and cycles are successfully terminated without overcorrection on no squint days. The purpose of this procedure is to put the dislocated globe back into its muscle cone by uniting the muscle bellies of the superior rectus and lateral rectus.

The Immunoarchitecture of Human Extraocular Muscles.

The purpose of this study was to describe the immunoarchitecture of normal extraocular muscles (EOMs) in terms of presence, distribution, and organization of various immune cells. We performed unilateral orbital exenterations in six fresh human cadavers from elderly patients, followed by dissection of the medial, lateral, superior and inferior rectus, superior and inferior oblique, and superior palpebral levator muscle in their entirety. We further cross sectioned each EOM in an anterior, central, and posterior third. After immunohistochemical staining for CD3, CD8, CD20, CD138, CD68, and podoplanin, quantitative analysis was performed. We found all EOMs (rectus, oblique, and levator muscles) to harbor both T- and B-lymphocytes, with a B-lymphocyte dominance and an absence of plasma cells. The highest prevalence of immune cells was seen in the muscle bellies, with, on average, 488 ± 63 CD3+ T-lymphocytes and 44 ± 110 CD20+ B-lymphocytes per mm2, and significant differences from the anterior (T-lymphocytes) and posterior (T- and B-lymphocytes) thirds. T- and B-lymphocytes were primarily organized in hotspots in the vicinity of blood vessels. In addition, a small resident population of macrophages scattered throughout the specimens was detected. No lymphatic vessels were found in any of the EOMs. These findings can serve as a reference dataset in the assessment of EOM biopsies in the diagnostic process of inflammatory orbital and systemic disorders. Moreover, from a regenerative perspective, our results highlight the importance of taking into account the presence of a resident immune cell population when studying the host immune response on transplanted tissues or engineered constructs.

Abstract 265: A Curious Case Of Acute Binocular Diplopia, Ischemic Stroke Of The Interstitial Nucleus Of Cajal

Introduction The nuclei in the periaqueductal gray of the midbrain are critical structures that coordinate and control vertical and torsional gaze, as well as crucial components of the vestibulo‐occular reflex. These structures are so fine that they may be missed on routine imaging however, they represent distinct localizations for the neurological exam, with lesions of these structures being important clinical considerations. The Interstitial Nucleus of Cajal (inC) coordinates signals from the saccadic burst neurons in the rostral interstitial nucleus of the medial longitudinal fasiculus (riMLF), vestibular projections (utricular and semicircular canal pathways from the vestibular nuclei via the MLF), and descending pursuit fibers, the paired interstitial nuclei of Cajal act as neural integrators for vertical gaze and torsion. These nuclei lie adjacent but dorsal and caudal to the riMLF, For upgaze, fibers from the inC cross via the posterior commissure, then connect to the contralateral superior rectus and inferior oblique subnuclei. For downgaze, fibers travel across the posterior commissure, then innervate the contralateral inferior rectus subnucleus and fourth nerve nucleus. Blood supply is derived from the terminal branches of the basilar artery. Lesions of these interstitial nuclei and their tracts may produce vertical and torsional gaze‐holding deficits such as impairments in the vertical vestibulo‐occular reflex, hypertropia, counter‐rolling of the eyes, superior oblique palsy, and see‐saw and torsional nystagmus. Methods Case report and review of literature Results 45‐year‐old, right‐handed woman with history of turner syndrome, anemia, nephrolithiasis, horseshoe kidney, premature ovarian failure on hormone replacement therapy (HRT), suspected adrenal mass of undetermined significant, and degenerative disk disease presented to the emergency department with complaints of acute binocular diplopia and dizziness clarified to be sensation of the ground/objects bobbing in visual field. Neurological exam significant for left hypertropia and exotropia in primary gaze, equal in right/left gaze and right/left head tilt, as well as large amplitude medium frequency (see‐saw) nystagmus characterized by upbeat nystagmus with intorsion on the right, and downbeat nystagmus with extorsion left. Visual fields were intact to finger count with binocular diplopia and oscillopsia noted in primary gaze. CT head and CT angiogram of the head and neck were unrevealing. Initial MRI brain negative for diffusion restriction but with flair hyperintensity in periaqueductal gray prompting repeat MRI brain without contrast with thin slices through the brainstem with showed infarct in periaqueductal gray within the region of the interstitial nuclei, with stroke etiology undetermined at the time of presentation but suspicion of small vessel disease vs hypercoagulability given propensity for thrombophilia in Turner syndrome and patient being on HRT. Full dose aspirin and high intensity statin therapy was initiated given presentation out of tPA window, and hypercoagulability workup initiated prior to discharge. Conclusion Acute ischemic stroke and lesions of the interstitial nuclei in the periaqueductal gray are important, under recognized considerations for the evaluation of binocular diplopia, that may represent an opportunity for thrombolytic administration if providers had a higher index of suspicion. Hypercoagulability and Turner syndrome are also unique qualifiers, underscoring a nuanced approach to determination of stroke diagnosis and etiology in cases of isolated ocular symptomology.

Novel Instrument for Clinical Evaluations of Active Extraocular Muscle Tension

Strabismus can be caused by abnormal tension of the extraocular muscles (EOMs) attached to the eyeball in superior, inferior, lateral, medial, superior oblique, and inferior oblique positions. Evaluating the tension in each EOM is crucial for surgical planning in strabismus, which is conducted by adjusting the tension on the EOM. The purpose of this study was to develop a compact measuring device to non-invasively evaluate the active EOM tension. The proposed device employed a cotton-tipped medical swab to transfer the EOM tension connected to the force sensor as a non-invasive medium. The tilting angle of the swab and the force of active EOM tension were wirelessly transferred to a laptop computer for recording and real-time displaying of the measured values. The active EOM tensions for the four recti muscles were 101.7 ± 15.0 g (mean ± SD) for the lateral rectus; 88.0 ± 15.4 g for the medial rectus; 61.3 ± 6.8 g for the inferior rectus; and 121.3 ± 38.5 g for the superior rectus. These values were higher than the reported values of 45–60 g measured in previous studies. In the previous studies, however, the EOM was detached from the globe and attached to a strain gauge, and, thus, there were no passive elastic forces from ocular connective tissue, resulting in lower values compared with the current study. The previous methods were also complex and not suitable for clinical measurement. Thus, the proposed method, which is non-invasive and mimics the conventional force generation test with a cotton-tipped swab, could facilitate the evaluation of active EOM tension, both clinically in strabismus management and in research into understanding its pathophysiology.

Effects of various extraocular muscle enlargement patterns on muscle diameter index in graves ophthalmopathy patients: a retrospective cohort study

Graves ophthalmopathy (GO) patients often undergo retrobulbar injection of glucocorticoids (GCs) as a common therapeutic approach. This study aimed to explore the impact of various patterns of extraocular muscle (EOM) enlargement on EOM changes following retrobulbar GCs injection in patients with GO. A retrospective analysis was conducted on GO patients who underwent retrobulbar GCs injections. Data pertaining to EOM diameter (EMD) and muscle diameter index (MDI) were collected from orbital computed tomography (CT) scans. The MDI change (ΔMDI) was calculated by comparing pre- and post-injection MDI values. The relationship between each pre EMD/MDI and ΔMDI was assessed using univariate and multivariate logistic regression analysis. A total of 68 patients with GO were included in this study, accounting for 118 eyes. After retrobulbar injections of GCs, 84 eyes showed a decrease in the MDI, while 34 eyes exhibited an increase in MDI. A threshold effect was observed in the relationship between medial pre EMD/MDI and ΔMDI. When the medial pre EMD/MDI was less than 0.28, a higher medial pre EMD/MDI was associated with a smaller ΔMDI (β = − 25.21, p = 0.0175). However, when the medial pre EMD/MDI was greater than 0.28, no significant association was found between pre EMD/MDI and ΔMDI. There was a negative correlation between medial + lateral pre EMD/MDI and ΔMDI (β = − 11.76, p < 0.0189). A higher medial + lateral pre EMD/MDI was associated with a greater decrease in MDI. Additionally, there was a positive correlation between superior rectus muscle-levator complex (SRLC) pre EMD/MDI and ΔMDI (β = 11.92, p = 0.040). The higher the value of SRLC pre EMD/MDI, the greater the ΔMDI. There was an association between pre EMD/MDI and changes in EOMs after retrobulbar injection of GCs in GO patients. In patients with predominantly enlarged medial rectus muscles and severe degrees of enlargement, retrobulbar injection of GCs should be assessed for its benefit; a combination of medial and lateral rectus muscle enlargement is beneficial for the shrinkage of EOMs following retrobulbar injections; the involvement of the SRLC rectus muscle may be a disadvantageous pattern of shrinkage of EOMs following retrobulbar injections.Trial registration This study is retrospectively registered. We have registered this study with the Chinese Clinical Trials Registry (www.chictr.org.cn, registration number: ChiCTR2200063429).

THU668 Euthyroid Graves’ Ophthalmopathy: Case Series

Abstract Disclosure: S.S. Alfadl: None. A.A. Asiri: None. A.A. Jammah: None. O. Alsagheir: None. Introduction: Graves’ ophthalmopathy is an autoimmune disorder that affects the eyes, characterized by swelling, proptosis, lid lag, inflammation and eyelid retraction. A significant proportion of cases are associated with thyrotoxicosis and positive serum TSH receptor antibody tests. Case Description: We present a case series of euthyroid Graves’ ophthalmopathy in patients with a negative TSH receptor antibody. The first case is of a 37-year-old lady who was recently diagnosed with idiopathic intracranial hypertension due to history of recurrent headaches and was found on CT brain to have an incidental empty sella syndrome and findings highly suggestive of thyroid associated orbitopathy. A dedicated MRI orbit showed bilateral mild proptosis with bilateral medial and inferior recti thickening, sparing the tendinous insertion. The involved extraocular muscles show mild peripheral high T2 signals and minimal enhancement on post gadolinium images. The enlarged extraocular muscle demonstrates diffusion restriction with low Apparent Diffusion Coefficient (ADC) value. Clinically, the patient did not exhibit any symptoms of hyperthyroidism or hypothyroidism. She did not notice any changes in her eyes’ appearance, nor any changes in vision or double vision. On examination, she had mild proptosis, no restriction of extraocular movement or provoked double vision. Consequently, she was referred to Ophthalmology for further management. Investigations included a pituitary panel which did not reveal any abnormalities and a thyroid panel which revealed that she was biochemically euthyroid with a negative TSH receptor antibody test. The second case is of a 24-year-old male who is medically free and initially visited the ophthalmology clinic complaining of double vision, without any additional ophthalmic complaints. He was subsequently referred to our care to rule out thyroid eye disease. Clinically, the patient had no indications of thyroid dysfunction. Upon examination, he exhibited severe right eye proptosis with dystopia and early left eye proptosis. CT orbit showed swelling of the right-sided superior rectus, medial rectus, lateral rectus and left-sided superior rectus muscles. The left-sided superior rectus muscle was abutting the optic nerve. Nevertheless, all tendons were spared. Biochemically, he was euthyroid with unremarkable laboratory results. Conclusion: These findings provide insight into the clinical presentation of euthyroid Graves’ ophthalmopathy in patients without thyrotoxicosis or detectable circulating levels of TSH receptor antibodies. Thus, suggesting that thyroid ophthalmopathy might present earlier than Graves’ disease and even earlier than the presence of TSH receptor antibodies. We conclude that a high index of suspicion and proper follow up is recommended in similar patients for the development of Graves’s disease. Presentation: Thursday, June 15, 2023

Heavy eye syndrome as a cause of recurrent strabismus

This is the case of a 69-year-old female with strabismus. She had a corrective surgery; however, 20 years later, progressive esotropia and hypotropia occurred. Magnetic resonance imaging (MRI) of the orbits showed increased dislocation angles of the bilateral lateral recti (LR) and superior recti (SR). Both LR-SR bands were not clearly appreciated. Other findings are staphyloma and irregular borders of the medial recti. MRI findings are suggestive of heavy eye syndrome.

A Novel Technique Using Paretic Superior Rectus as a Globe Suspender for Monocular Elevation Deficiency.

Surgical innovations in strabismus provide opportunities to improve visual function, eye alignment, and cosmesis in rare pediatric ophthalmological conditions. Monocular elevation deficiency is a rare and multifactorial disease in which the affected eye is equally limited in terms of elevation during adduction and abduction. We aimed to present a novel procedure for the treatment of acquired monocular elevation deficiency using the paretic superior rectus muscle as a globe suspender to resolve hypotropia. We report the case of an eight-year-old girl with left eyelid ptosis and hypotropia two months after draining a left orbital abscess. Left inferior rectus muscle recession was performed at five years, with residual left hypotropia. Ophthalmological examination revealed a best-corrected visual acuity of 20/20 OD and 20/100 OS. Severe left eyelid ptosis and poor levator function were also observed. Extraocular motility showed left hypotropia of 40 prism diopters with the left superior rectus muscle under action (-4) in the adduction and abduction positions. A force duction test negative for restrictions on the inferior rectus muscle was performed intraoperatively. To reduce the risks of the Knapp procedure, the left superior rectus muscle was split into medial and temporal halves. Double-armed sutures were secured in half, and the halves were detached from the sclera. The medial and temporal halves were reattached anteriorly to the medial and lateral rectus insertions, respectively. Eight weeks after surgery, the patient had nine prism diopters of hypotropia in the primary gaze. Ten weeks after surgery, there was no change in visual acuity. In the cover test, the patient exhibited residual left hypotropia of nine prism diopters with a restriction (-4) of elevation in adduction and abduction. The parents were pleased with the satisfactory cosmetic outcomes, and postoperative clinical photographs of the patient showed improved hypotropia and persistent minimal elevation of the left eye during adduction and abduction. Superior rectus muscle splitting and vertical transposition to the medial and lateral rectus could be safer and simpler alternatives to the Knapp procedure and may offer a lower risk of anterior segment ischemia. Further studies are required to confirm these findings.

Eye Muscle MRI in Myasthenia Gravis and Other Neuromuscular Disorders.

MRI of extra-ocular muscles (EOM) in patients with myasthenia gravis (MG) could aid in diagnosis and provide insights in therapy-resistant ophthalmoplegia. We used quantitative MRI to study the EOM in MG, healthy and disease controls, including Graves' ophthalmopathy (GO), oculopharyngeal muscular dystrophy (OPMD) and chronic progressive external ophthalmoplegia (CPEO). Twenty recently diagnosed MG (59±19yrs), nineteen chronic MG (51±16yrs), fourteen seronegative MG (57±9yrs) and sixteen healthy controls (54±13yrs) were included. Six CPEO (49±14yrs), OPMD (62±10yrs) and GO patients (44±12yrs) served as disease controls. We quantified muscle fat fraction (FF), T2water and volume. Eye ductions and gaze deviations were assessed by synoptophore and Hess-charting. Chronic, but not recent onset, MG patients showed volume increases (e.g. superior rectus and levator palpebrae [SR+LPS] 985±155 mm3 compared to 884±269 mm3 for healthy controls, p < 0.05). As expected, in CPEO volume was decreased (e.g. SR+LPS 602±193 mm3, p < 0.0001), and in GO volume was increased (e.g. SR+LPS 1419±457 mm3, p < 0.0001). FF was increased in chronic MG (e.g. medial rectus increased 0.017, p < 0.05). In CPEO and OPMD the FF was more severely increased. The severity of ophthalmoplegia did not correlate with EOM volume in MG, but did in CPEO and OPMD. No differences in T2water were found. We observed small increases in EOM volume and FF in chronic MG compared to healthy controls. Surprisingly, we found no atrophy in MG, even in patients with long-term ophthalmoplegia. This implies that even long-term ophthalmoplegia in MG does not lead to secondary structural myopathic changes precluding functional recovery.

Quantitative assessment of extraocular muscles in Graves' ophthalmopathy using T1 mapping.

To evaluate the performance of T1 mapping in the characterization of extraocular muscles (EOMs) of Graves' ophthalmopathy (GO) patients and investigate its feasibility in assessing the response to glucocorticoid therapy in active GO patients. A total of 133 participants (78 active GO, 23 inactive GO, 18 Graves' disease (GD) patients, and 14 healthy volunteers) were consecutively enrolled from July 2018 to December 2020. Native T1 (nT1) and postcontrast T1 (cT1) values of EOMs were measured and compared. The variations in T1 mapping metrics of EOMs were compared pre/post glucocorticoid treatment in 23 follow-up active GO patients. Logistic regression analysis and receiver operating characteristic (ROC) curve analysis were performed. The nT1 of EOMs in GO patients was higher than that in GD patients and healthy volunteers. The nT1 of superior rectus (SR) in active GO was higher than that in inactive GO patients, and it could be used as a potential marker of GO activity (OR: 1.003; 95% CI: 1.001, 1.004), with a diagnostic sensitivity of 86.3% and specificity of 43.7%. Meanwhile, the cT1 of SR, inferior rectus (IR), and medial rectus (MR) in inactive GO patients were higher than those in active GO patients. The nT1 of EOMs achieved sufficient diagnostic performance in evaluating the response to glucocorticoid therapy for follow-up active GO patients (AUC, 0.797; sensitivity, 71.9%; specificity, 85.7%). T1 mapping could quantitatively assess the activity of GO and the response to glucocorticoid therapy in active GO patients and may even potentially reflect the fibrosis of EOMs. T1 values can reflect the pathological status of the extraocular muscle. T1 mapping could help to quantitatively assess the clinical activity of GO and the response to glucocorticoid therapy in active GO patients. • Graves' ophthalmopathy patients had greater nT1 of extraocular muscles than Graves' disease patients and healthy volunteers, and nT1 of the superior rectus could be a potential marker of Graves' ophthalmopathy activity. • The cT1 of extraocular muscles in inactive Graves' ophthalmopathy patients was higher than that in active Graves' ophthalmopathy patients, and it might be associated with muscle fibrosis. • nT1 of extraocular muscles could offer sufficient diagnostic performance in evaluating the response to glucocorticoid therapy for follow-up active Graves' ophthalmopathy patients.

Clinical features of Helveston syndrome and discussion of individualized surgical design.

Reasonable personalized surgical design can achieve good treatment results for Helveston syndrome in one surgery, reducing the psychological and economic burden on patients. This article aims to explore the clinical characteristics of Helveston syndrome and the clinical effectiveness and feasibility of individualized surgical design. In this retrospective case series study, 28 patients who underwent strabismus correction for Helveston syndrome at the Affiliated Hospital of Yunnan University from June 2018 to December 2020 with complete follow-up data were enrolled. Preoperatively, all patients received standard assessment of vision, intraocular pressure, slit lamp, fundus and refractive status, excluding other eye diseases, as well as detailed special examination of strabismus. These patients were divided into two groups according to the surgical modality: the horizontal muscle surgery alone group and the horizontal muscle surgery combined with superior oblique muscle surgery (combined surgery) group. We used SPSS software for data analysis and compared the postoperative eye position, eye movement, success rate, and reoperation rate between these two groups. Clinical measurement data were compared and analyzed with Fisher's exact test for count data, the t-test for normally distributed measurement data, and the Mann-Whitney U test for non-normally distributed measurement data. P<0.05 was considered statistically significant. This study included a total of 28 patients with Helveston syndrome, including 20 males and 8 females. The average age at the time of surgery is 12.04 ± 8.67 years (range, 4-43 years). The postoperative A-pattern degree was significantly greater in the group undergoing horizontal muscle surgery alone [6.23±1.31 prism diopters (PD); range, 0-10 PD] than in the group undergoing combined surgery (0.53±0.32 PD; range, 0-4 PD; P=0.002). Superior oblique muscle overactivity was significantly reduced in the combined surgery group (0.20±0.11+; range, 0-1+) compared to the horizontal muscle surgery alone group (1.31±0.26+; range, 0-2+; P=0.002). However, there was no significant difference in success rate or reoperation rate between the two groups. Additionally, after combining the recession of the superior rectus muscle with the horizontal muscle, the number of A-pattern degrees was greatly reduced. Helveston syndrome can be improved using a personalized surgical design according to the degree of external strabismus A-pattern, superior oblique muscle overaction, and dissociated vertical deviation (DVD) degree, which improves the success rate of single surgery.

High Myopia and Thickness of Extraocular and Masticatory Muscles-7T MRI, Preliminary Study.

(1) Background: Myopia is one of the most common refractive errors in the world. The aim of this study was to evaluate the transverse dimensions of selected masticatory muscles (temporalis muscle and masseter muscle) versus the transverse dimensions of selected extraocular muscles (superior rectus, inferior rectus, medial rectus and lateral rectus) in emmetropic and high myopic subjects. (2) Methods: Twenty-seven individuals were included in the analysis, resulting in 24 eyeballs of patients with high myopia and 30 eyeballs of emmetropic subjects. A 7 Tesla resonance was used to analyze the described muscles. (3) Results: Statistical analysis showed differences in all analyzed extraocular muscles and all analyzed masticatory muscles between emmetropic subjects and high myopic subjects. In the high myopic subject group, statistical analysis showed four correlations. The three negative correlations were between the lateral rectus muscle and an axial length eyeball, refractive error and an axial length eyeball, and the inferior rectus muscle and visual acuity. The positive correlation was between the lateral rectus muscle and the medial rectus muscle. (4) Conclusions: The high myopic subjects are characterized by a larger cross-sectional area of extraocular muscles and masticatory muscles compared to the emmetropic subjects. Correlations were observed between the thickness of the extraocular muscles and the masticatory muscles. The lateral rectus muscle was related to the length of the eyeball. The phenomenon requires further study.

A Rare Case of Vascular Malformation in the Orbit

Summary We present the case of an 87-year-old female with a one-week history of redness, swelling, and pain in the left eye, accompanied by headache and neck pain. Computed tomography (CT) scan demonstrated extreme enlargement of the supraorbital artery, engaging the superior rectus muscle of the left eye. We could not find similar articles in well-recognized scientific networks. Conservative treatment was conducted, with no improvement. The patient refused surgery.

Rapidly Resolving Orbital Pseudotumor in Systemic Lupus Erythematosus

Rapidly Resolving Orbital Pseudotumor in Systemic Lupus Erythematosus

Extraocular Muscle Enlargement in Acromegaly.

To determine whether acromegaly is associated with increased extraocular muscle (EOM) size at time of presentation. Patients with a new diagnosis of acromegaly in a single tertiary care clinic with a CT scan that adequately delineated the EOMs were included. Control subjects were age- and sex-matched patients with a new diagnosis of nonfunctioning pituitary adenoma. Retrospective chart review was performed to extract baseline clinical and laboratory parameters including growth hormone, insulin-like growth factor 1, thyroid stimulating hormone, free T3, and free T4. A single neuroradiologist analyzed all CT scans and measured the maximum diameter and cross-sectional area of the superior rectus, inferior rectus, medial rectus, and lateral rectus in both eyes of all patients. We evaluated 17 patients with acromegaly and 18 control subjects. Mean maximum diameter of the superior, inferior, medial, and lateral recti were 4.80 mm (SD = 0.81), 4.67 mm (SD = 0.54), 4.86 mm (SD = 0.77), and 4.53 mm (SD = 0.70) respectively, in the acromegaly group. In the control group, they were 3.62 mm (SD = 0.58),3.71 mm (SD = 0.46), 3.66 mm (SD = 0.32), and 3.21 mm (SD = 0.44), respectively. The maximum diameter and cross-sectional area of all 4 EOMs measured in the acromegaly group were significantly larger ( P < 0.001) compared with the control group. Patients with acromegaly present with significantly enlarged EOMs compared with control subjects with nonfunctioning pituitary adenomas.

Ocular Motility Abnormalities in Ehlers-Danlos Syndrome: An Observational Study

Purpose: To evaluate ocular motility (OM) abnormalities associated with Ehlers-Danlos Syndrome (EDS). Materials and methods: In this cross-sectional observational study, patients with EDS underwent a complete orthoptic examination. The following orthoptic tests were performed: corneal light reflex test, stereoscopic test, cover test, OM assessment, evaluation of eye pain in different gaze positions and red filter test for diplopia. Results: The corneal light reflex test at 33 cm showed an intermittent divergent deviation in 31.7% of patients and an intermittent horizontal deviation associated with a vertical deviation in 4.9% of patients. A manifest strabismus was observed in 2.4% of patients, whereas 2.4% of patients showed a microstrabismus. The corneal light reflex test at 5 m revealed microstrabismus in 9.8% and manifest strabismus in 2.4% of our patients. Moreover, intermittent exotropia was observed in 2.4% of cases. No significant alterations involving the inferior rectus and the superior oblique muscles were observed. Significant associations were observed between medial rectus muscle deficit of both eyes with pain (p = 0.020) and diplopia (p = 0.014). Furthermore, a significant association between lateral rectus muscle alteration of both eyes and pain was observed (p = 0.004). Conclusions: Our results show various OM alterations in patients with EDS, specifically superior and medial rectus muscle hypofunction. A full orthoptic evaluation in these patients is recommendable to detect OM involvement and possible ligamentous laxity changes over time through an accurate OM assessment.

Modified Nishida Procedure Combined with Lateral Rectus Disabling for Duane Retraction Syndrome

ABSTRACT Duane retraction syndrome (DRS) is a complex congenital cranial dysinnervation disorder. The choice of surgical procedure in esotropic-DRS depends upon several factors that include: the amount of esotropia in the primary position, the presence and severity of palpebral fissure narrowing, globe retraction, presence of medial rectus muscle (MR) contracture, the likelihood of improving abduction, age of the patient, and the presence of binocularity and stereopsis. In the presence of MR contracture, MR recession is performed either alone (unilaterally or bilaterally) or in conjunction with Y splitting plus recession of the lateral rectus muscle (LR) for reducing globe retraction. MR recession, with or without adjustable sutures, may be simultaneously combined with partial thickness vertical rectus muscle transposition (VRT) or with superior rectus muscle transposition (SRT). We describe a novel combination of surgical procedures in the management of esotropic-DRS in two patients. In our first patient, following an initial MR recession combined with LR disinsertion and periosteal fixation (LRDAPF), a modified Nishida procedure was performed. In our second patient following a prior simultaneous MR recession and LR Y splitting with recession, we combined periosteal fixation of the LR with a modified Nishida procedure of the vertical rectus muscles.

Why so low? An unusual case of myositis in a child

BackgroundSarcoidosis is characterized by non-caseating epithelioid granulomas in various tissues throughout the body, most commonly the lung. Non-caseating granulomas may be seen in skeletal muscle, though typically asymptomatic and under-recognized. While rare in children, there is a need to better characterize the disease and its management. Here we present a 12-year-old female with bilateral calf pain who was ultimately found to have sarcoid myositis.Case PresentationA 12-year-old female presented to rheumatology with significantly elevated inflammatory markers and isolated lower leg pain. MRI of the distal lower extremities demonstrated extensive bilateral myositis with active inflammation, atrophy, and to a lesser extent fasciitis. This distribution of myositis in a child garnered a broad differential requiring a systematic evaluation. Ultimately, muscle biopsy revealed non-caseating granulomatous myositis with perivascular inflammation, extensive muscle fibrosis, and fatty replacement of the muscle with a CD4+ T cell predominant, lymphohistiocytic infiltrate consistent with sarcoidosis. Review of histopathology from age 6 of an extraconal mass resected from her right superior rectus muscle further confirmed the diagnosis. She had no other clinical symptoms or findings of sarcoidosis. The patient improved significantly with methotrexate and prednisone, though flared again after self-discontinuation of medications and was subsequently lost to follow-up.ConclusionThis is the second reported case of granulomatous myositis associated with sarcoidosis in a pediatric patient, and the first to present with a chief complaint of leg pain. Increased knowledge of pediatric sarcoid myositis within the medical community will enhance recognition of the disease, improve the evaluation of lower leg myositis, and advance outcomes for this vulnerable population.

Isolated superior rectus muscle hematoma with quiet eye after blunt orbital trauma – A case report

After orbital trauma, medial rectus (MR) and inferior rectus (IR) muscles are the most frequently injured, while the oblique muscles are the least frequently injured. This can be probably because of the anatomical specifications of these muscles within the orbit. It is very unusual to find an isolated muscle injury or hematoma without any other orbital or ocular damage. We report here the case of a 7-year-old boy who presented with an isolated superior rectus muscle (SRM) hematoma after a blunt orbital trauma.

The preliminary discussion about the infraduction following conjoint fascial sheath suspension

BackgroundWe discovered severe ptosis patients developed infraduction after conjoint fascial sheath (CFS) suspension, a complication that is associated with this technique. In this article, the causes and outcomes of the infraduction after CFS suspension were systematically analyzed. MethodsSeventy-four patients with severe ptosis who underwent CFS suspension between February 2020 and June 2021 were reviewed. The palpebral fissure height (PFH), the eyeball position, the infraduction and recovery distance, the eyeball position difference, and the cosmetic results were recorded and analyzed. ResultsBefore surgery, the mean PFH, eyeball position, and cosmetic results were 6.33 ± 0.72 mm, 4.68 ± 0.80 mm, and 2.03 ± 0.96, respectively. The eyeball position difference was 0.70 ± 0.41 mm for unilateral ptosis patients and 0.58 ± 0.26 mm for bilateral ptosis patients. Immediately after surgery, the mean PFH, eyeball position, and cosmetic results were 12.73 ± 1.22 mm (P < 0.0001), 3.01 ± 1.19 mm (P < 0.0001), and 5.80 ± 1.53 (P < 0.0001), respectively. The mean infraduction distance was 1.67 ± 1.04 mm. The mean eyeball position difference was 1.67 ± 1.18 mm (P < 0.0001) for unilateral ptosis patients and 0.83 ± 0.52 mm for bilateral ptosis patients. At the last follow-up, the mean PFH, eyeball position, and cosmetic result were 10.46 ± 1.14 mm (P < 0.0001), 4.19 ± 0.74 mm (P < 0.0001), and 8.24 ± 1.36 (P < 0.0001), respectively. The mean infraduction distance was 0.49 ± 0.48 mm, and the mean recovery distance was 1.18 ± 0.90 mm. The mean eyeball position difference was 0.71 ± 0.61 mm for unilateral ptosis patients and 0.49 ± 0.47 mm for bilateral ptosis patients. ConclusionThe impact on the superior rectus may lead to infraduction after CFS surgery. Patients developed infraduction immediately after surgery and recovered spontaneously. Level of evidenceIV