Acute acquired comitant esotropia associated with Lhermitte-Duclos disease: a case report.

BACKGROUND: Acute acquired comitant esotropia caused by prolonged near work, such as the use of digital devices, has been frequently reported in recent years. However, intracranial examination is necessary even for patients with nonparalytic comitant esotropia. Lhermitte-Duclos disease is a rare tumor that grows in layers in the cerebellum. Among those with this disease, cases of esotropia have been reported due to abduction limitation of the eye, but there have been no reports of comitant esotropia. Here, we report the case of a young woman with acute acquired comitant esotropia who was found to have Lhermitte-Duclos disease. CASE PRESENTATION: A 16-year-old Japanese female patient, whose ethnicity was Asian, was referred to our hospital for acute acquired comitant esotropia. Fundus examination revealed papilledema in both eyes, and magnetic resonance imaging of the head revealed a cerebellar tumor in the right cerebellum with obstructive hydrocephalus. She underwent partial tumor resection, and a histopathological diagnosis of Lhermitte-Duclos disease was obtained. However, comitant esotropia status remained unchanged, and she underwent strabismus surgery. Finally, diplopia disappeared completely. CONCLUSION: Neurological and intracranial imaging examinations are essential when acute acquired comitant esotropia is observed. Acute acquired comitant esotropia by Lhermitte-Duclos disease did not improve with partial tumor resection and required strabismus surgery, but good surgical results were obtained.

Advances in the diagnosis and treatment of acute acquired comitant esotropia.

Acute acquired comitant esotropia (AACE) is mainly characterized by sudden onset, accompanied by diplopia, without extraocular muscles paralysis or ocular motility disorders. In recent years, the incidence of AACE has been increasing, researchers have found that this phenomenon may be related to the widespread use of electronic devices and the increase in the number of people working from home during the COVID-19 pandemic. However, its neural mechanisms have not been fully elucidated. This article primarily reviews the latest developments in the diagnosis and treatment of AACE from the perspectives of etiology and treatment methods, aiming to provide direction for future in-depth exploration of the pathogenesis and treatment approaches of this disease.

Magnetic resonance imaging findings of high myopic strabismus with sagging eye-like symptoms, heavy eye syndrome, and non-highly myopic eyes with sagging eye syndrome.

INTRODUCTION: Progressive esotropia accompanied by restricted abduction and supraduction due to high myopia is known as esotropia fixus with high myopia or heavy eye syndrome (HES). Some conditions, such as sagging eye syndrome (SES), show esotropia for distance or cyclovertical strabismus with no abduction limitations despite highly myopic eyes. We evaluated the magnetic resonance imaging (MRI) findings and clinical features of HES, high myopia with SES-like symptoms (highly myopic SES), and SES. METHODS: We reviewed all patients diagnosed with HES, highly myopic SES, and SES who underwent MRI of the orbits and brain over 6 years. To quantitatively assess the orbital anatomy, we compared the conditions of the superior rectus muscle (SR), lateral rectus muscle (LR), and inferior rectus muscle (IR) using orbital MRI among the three groups. RESULTS: Among the 14 patients (27 eyes) with high myopia, 5 (9 eyes) had HES, and 9 (18 eyes) had highly myopic SES. Eleven patients (22 eyes) with SES were also compared with these 14 patients. The mean axial length was 29.6 ± 1.0 mm in participants with HES, 29.0 ± 1.5 mm in those with HES-SES, and 23.7 ± 0.9 mm in those with SES. The average distance esotropia was 48.0 ± 19.9Δprism, 4.6 ± 1.5Δprism, and 6.1 ± 4.6Δprism for participants with HES, highly myopic SES and SES, respectively. The average distance hypertropia was 5.3 ± 5.9Δprism in participants with highly myopic SES and 4.8 ± 2.7Δprism in those with SES. The mean vertical angle of the LR was 32.6 ± 10.8°, 18.1 ± 5.4°, and 14.6 ± 6.8°; the mean tilting angle of the LR was 31.6 ± 9.2°, 15.9 ± 6.0°, and 13.8 ± 5.9°; and the mean displacement angle between the LR and SR was 152.3 ± 16.7°, 125.0 ± 7.1°, and 112.5 ± 7.5° for participants with HES, highly myopic SES and SES, respectively. The LR-SR displacement angle in HES-SES was significantly larger than in SES (p < .001) but the vertical and tilting angles were not. Also, the IR shift showed no significant difference with HES-SES and HES (5.8 ± 1.4 mm and 5.3 ± 1.2 mm) but not with SES (4.0 ± 0.8 mm) (p < .0001). DISCUSSION: SES-like symptoms can develop in highly myopic eyes; however, MRI showed that the state of the LR muscle in highly myopic SES deviated almost similarly to that in SES; however, the eyeball was more dislocated than in SES. This may be useful in deciding the appropriate operative procedure.

A patient with congenitally restrictive strabismus and contralateral secondary muscle enlargement.

AIM: To report the case of a congenital restrictive strabismus with a contralateral enlargement of extraocular muscles. CASE: The clinical presentation, findings, and postoperative outcomes of a 4 years old boy referred to evaluation for presenting a left eye deviation since birth are detailed. CONCLUSION: A unilateral congenital restrictive strabismus (congenital hypotropia and esotropia) can be the cause of contralateral secondary enlarged extraocular muscle and It must be included in the differential diagnosis.

Modified partial Jensen procedure to treat a hypertropic Knobby eye syndrome in a high myopic monocular patient.

We present a case of severe esotropia and hypertropia in a monocular high myope with an inferolateral staphyloma. Surgery was undertaken to secure the inferior rectus to the lateral rectus via a modified partial Jensen technique, along with medial rectus recession successfully improving head and eye positions and ductions.

Acute bilateral hypotropia and esotropia complex as first manifestation of multiple sclerosis: a case report.

A 21-year-old Japanese woman presented with sudden eye movement disorders. An ophthalmic examination revealed bilateral hypotropia and esotropia complex. Brain magnetic resonance imaging revealed abnormal signals in the posterior and medial part of the lower pontine tegmentum (including periventricular and subcortical white matter) that were suggestive of demyelination. A cerebrospinal fluid test was positive for oligoclonal bands. She was subsequently diagnosed with multiple sclerosis and was administered intravenous methylprednisolone and oral dimethyl fumarate, with complete recovery from hypotropia and esotropia after two months. Bilateral hypotropia and esotropia are important clinical signs for the accurate diagnosis of multiple sclerosis.

14†Surgical treatment of heavy eye syndrome by modified loop myopexy.

Heavy eye syndrome or convergent strabismus fixus is an acquired strabismus typically seen in eyes with high myopia. We present a case, discuss the aetiology and management, and include a short video illustrating the surgical procedure undertaken.A 47-year-old highly myopic woman with h/o bilateral cataract surgery and B/L scleral buckling for retinal detachments, had left esotropia and hypotropia measuring more than 40 prism dioptres base-out and 12 prism dioptres base up. MRI orbits showed bilateral asymmetrical medial deviation of ocular bulbs, more on left side. Also, there was degeneration of lateral rectus-superior rectus band with displacement of lateral rectus downwards.She underwent Botox to bi-medial recti after which she could demonstrate potential for binocular single vision. A left un-augmented loop Myopexy procedure and recession of the left medial rectus was thereafter performed under general anaesthesia. After surgery, her eyes were binocularly aligned for near with minimal esotropia for distance.This case suggests that patients with significant esotropia combined with high myopia should be suspected to have heavy eye syndrome. Orbital imaging should be undertaken to demonstrate the anatomical abnormality and muscle paths to confirm a definite diagnosis. Modified Loop Myopexy was found to be effective in this case of heavy eye syndrome.

20†Simultaneous development of acute acquired concomitant esotropia in two siblings during the COVID-19 pandemic: a case report.

A 5-year-old boy (sibling one) and his 11-year-old sister (sibling two) were presented to the hospital eye service in early 2021, having both developed acute-onset large angle esotropia within three months of each other. Neither had any significant past medical, ophthalmic, or family history. The siblings lived in the same household, and both experienced lifestyle changes as a result of the UK lockdown in response to COVID-19.Sibling one had a moderate right esotropia, initially maintained straight by corneal light reflex. He measured 45/50 prism dioptres (Δ) base out at near and 45Δ base out at distance. Sibling two had esophoria which broke down into a right esotropia immediately on dissociation. The esotropia measured 30Δ base out at near and 20Δ base out at distance. At four month follow up, both siblings demonstrated a constant large angle esotropia (sibling one: 54Δ base out at near and 45Δ base out at distance, sibling two: 45Δ base out at near and 40/45Δ base out at distance).Each sibling was treated with right medial rectus recession (5.5 mm) and right lateral rectus resection (7 mm), and at a three-month follow-up, both were minimally esophoric with restored binocularity.The unusual and abrupt changes in lifestyle imposed by the COVID-19 pandemic highlight the likelihood of an environmental aetiology for some forms of esotropia and raise the possibility that extended screen time may be a contributory factor.

Esotropia-hypertropia complex in a patient with myopic strabismus fixus.

Patients with highly myopic strabismus generally develop esotropia and hypotropia. We report the case of a 54-year-old woman with a combination of esotropia-hypertropia complex in the right eye and esotropia-hypotropia complex in the left eye, with unilateral congenital severe ptosis. The patient had inferotemporal prolapse of the globe in the right eye. Inferotemporal prolapse in this case could have been due to extraocular muscle pulley dehiscence in this area, causing the inferior oblique muscle to slip around the globe along the path of least resistance. Subsequently, the inferotemporal staphyloma would have prevented the typical esotropia and hypotropia of the globe.

Extraocular muscle enlargement of indeterminate cause in pediatric patients: case series and literature review.

PURPOSE: To specify the clinical characteristics of enlarged extraocular muscles of indeterminate cause in pediatric patients based on a case series and literature review. METHODS: The medical records of pediatric patients who presented between January 2019 and January 2022 with enlarged extraocular muscles, where the underlying cause could not be determined, were retrospectively reviewed. RESULTS: Four patients were included. The main reason for presentation was evaluation of abnormal head posture. Head tilt or turn with duction deficit was observed in all patients. The age of onset ranged from 6 months to 1 year. Two patients presented with esotropia and hypotropia; the other 2 patients, with large-angle esotropia. Orbital imaging was performed in all cases and revealed unilateral rectus muscle enlargement sparing the muscle tendon. All 4 patients were found to have an enlarged medial rectus muscle. In the 2 patients with hypotropia, the inferior rectus muscle was also involved. No underlying systemic or orbital disease was found. There were no changes in the orbit or extraocular muscles on follow-up imaging test. The intraoperative forced duction test revealed severe restriction in the direction of gaze opposite to the primary field of action of the enlarged muscles. CONCLUSIONS: Enlargement of extraocular muscles should be considered in the differential diagnosis when large-angle incomitant vertical or horizontal misalignment and abnormal head posture are observed in infancy.

An atypical case of myopic strabismus fixus.

We present an atypical case of progressive fixed hypertropia in a patient with extreme myopia. Myopic strabismus fixus is typically characterized by progressive esotropia and hypotropia, which is caused by the protrusion of the posterior eyeball from the superotemporal quadrant of the extraocular muscle cone. In this case, the patient's right eye was fixed in a superomedial position, and the left eye was fixed and deviated upward. Magnetic resonance imaging revealed protrusion of a posterior scleral staphyloma inferotemporally in each eye. This case illustrates how the pattern of strabismus fixus caused by high myopia depends on the direction of the protrusion of the posterior pole and the resulting displacement of extraocular muscle paths.

Long-term results of strabismus surgery for treatment of esotropia in patients with Möbius syndrome.

PURPOSE: To evaluate the surgical results of Möbius syndrome esotropia following bilateral medial rectus recession with and without bilateral Hummelsheim procedure. METHODS: The medical records of patients with Möbius syndrome operated on by a single surgeon from 1993 to 2022 were studied retrospectively. For each patient, age, sex, and ocular deviation before and after surgery were retrieved. Success was defined as a horizontal deviation of up to 15Δ and vertical deviation of <10Δ. RESULTS: Eleven patients were included. Average patient age at the time of surgery was 4 years. Eight patients with esodeviation up to 70Δ underwent bilateral medial rectus recession; 3 patients with esotropia >70Δ underwent recession plus bilateral Hummelsheim procedure. The mean esodeviation before surgery was 52.5Δ in the first group and 86.6Δ in the second group. The mean horizontal deviation at early follow-up was esotropia of 7.4Δ in the first group and of 15Δ in the second group. The early success rate was 87% in the first group and 67% in the second group. Long-term follow-up revealed exoshift in 43% of the patients in the first group and all 3 patients in the second group. CONCLUSIONS: In our small patient cohort of patients with Möbius syndrome, bilateral medial rectus recession corrected esodeviations of up to 70Δ. In more severe cases (>70Δ), adding bilateral Hummelsheim procedure was beneficial.

Neurological pathologies in acute acquired comitant esotropia.

BACKGROUND: Acute acquired comitant esotropia (AACE) is an uncommon subtype of esotropia characterized by sudden and usually late onset of a relatively large angle of comitant esotropia with diplopia in older children and adults. METHODS: A literature survey regarding neurological pathologies in AACE was conducted using databases (PubMed, MEDLINE, EMBASE, BioMed Central, the Cochrane Library, and Web of Science) in order to collect data for a narrative review of published reports and available literature. RESULTS: The results of the literature survey were analyzed to provide an overview of the current knowledge of neurological pathologies in AACE. The results revealed that AACE with unclear etiologies can occur in many cases in both children and adults. Functional etiological factors for AACE were found to be due to many reasons, such as functional accommodative spasm, the excessive near work use of mobile phones/smartphones, and other digital screens. In addition, AACE was found to be associated with neurological disorders, such as astrocytoma of the corpus callosum, medulloblastoma, tumors of the brain stem or cerebellum, Arnold-Chiari malformation, cerebellar astrocytoma, Chiari 1 malformation, idiopathic intracranial hypertension, pontine glioma, cerebellar ataxia, thalamic lesions, myasthenia gravis, certain types of seizures, and hydrocephalus. CONCLUSIONS: Previously reported cases of AACE with unknown etiologies have been reported in both children and adults. However, AACE can be associated with neurological disorders that require neuroimaging probes. The author recommends that clinicians should perform comprehensive neurological assessments to rule out neurological pathologies in AACE, especially in the presence of nystagmus or abnormal ocular and neurological indications (e.g., headache, cerebellar imbalance, weakness, nystagmus, papilloedema, clumsiness, and poor motor coordination).

Epidemiology, clinical features, and surgical outcomes of acute acquired concomitant esotropia associated with myopia.

PURPOSE: To analyze epidemiology, clinical features, and surgical outcomes of type III acute acquired concomitant esotropia (Bielschowsky esotropia (BE)). METHODS: The medical charts of patients diagnosed with acquired concomitant esotropia between 2013 and 2021 were reviewed. Assessed data were age, gender, age at diplopia onset, age at the diagnosis, refraction, visual acuity, neuroimaging, diplopia onset, angle of deviation, stereopsis, surgical procedure, amount of surgery, and relapse of diplopia after surgery. Moreover, we investigated the correlation between the use of electronic devices and the onset of diplopia. RESULTS: One hundred seventeen patients (mean age 35.07 ± 15.81 years) were included in the study. The mean delay to the diagnosis was 3.29 ± 3.62 years. Myopia range was 0 to 17 diopters spherical equivalent. 66,3% spent more than 4 hours a day using laptops, tablets, or smartphones at the onset of diplopia, and 90,6% presented a subacute onset. None showed neurologic signs or symptoms. Patients who underwent surgery were ninety-three, with a rate of surgical success of 93.6%, and a relapse rate of 17.2%. A negative correlation resulted between pre-operative deviation and age at diagnosis (ρ = -0.261; p<0.05), whereas factors associated with surgical failure were older age at diplopia onset (p = 0.042) and longer delay between onset and diagnosis (p = 0.002). CONCLUSION: We registered an outstanding increase in prevalence of BE, which could be related to the exponential increase in the use of electronic devices for professional, educational, and recreational purposes. A prompt diagnosis and an augmented dose of surgery allows good motor and sensory results.

Inferior rectus transposition as a secondary surgery in patients with acquired abducens nerve palsy.

We report the outcomes in 2 patients with acquired abducens nerve palsy with residual esotropia following superior rectus transposition and medial rectus recession who subsequently underwent inferior rectus transposition as a second procedure. Both patients showed improved abduction and reduced esotropia, with no induced cyclotorsion or vertical deviation. Inferior rectus transposition as a secondary procedure in these 2 patients with abducens nerve palsy appeared to augment the effect of prior superior rectus transposition and medial rectus recession.

SEVERE NEAR REFLEX SPASM IN A HEALTHY TEENAGER. A CASE REPORT.

INTRODUCTION: Spasm of the near reflex usually includes accommodative spasm, esophoria/tropia, and different degrees of miosis. Patients usually refer to distance blurred and fluctuating vision, ocular discomfort, and headaches. The diagnosis is established with refraction with and without cycloplegia; most of the cases have a functional etiology. However, some cases require neurological conditions to be ruled out; cycloplegics have an important diagnostic and therapeutic role. PURPOSE: To describe a case of bilateral severe accommodative spasm in a healthy 14-year-old teenager. CASE PRESENTATION: A 14-year-old boy with progressive diminished visual acuity attended for YSP consultation. The diagnosis of bilateral spasm of the near reflex was made, based on a gap refraction of 9.75 D between retinoscopy with and without cycloplegia and esophoria with normal keratometry and axial length. The spasm was eliminated with 2 drops of cycloplegic in each eye separated by 15 days; no clear etiology was found other than the start of school. CONCLUSION: Clinicians should be aware of pseudomyopia, especially in children with acute changes in visual acuity, who are usually exposed to myopigenic environmental factors that induce overstimulation of the parasympathetic third cranial nerve's innervation.