[Ocular myasthenia gravis]. / Glaznaya forma miastenii gravis.

Myasthenia gravis is an autoimmune disease characterized by muscle weakness and pathological fatigue due to autoaggressive phenomena with the formation of antibodies directed against various structures of the neuromuscular synapse. In most patients, the disease begins with the involvement of extraocular muscles, presenting with symptoms such as intermittent ptosis of the upper eyelid and/or binocular diplopia. In 15% of cases, clinical manifestations are limited to impairment of the levator palpebrae superioris and extraocular muscles, characteristic of the ocular form of myasthenia gravis. Specialists often encounter challenges in diagnosing this form, as serological and electrophysiological studies may be uninformative, necessitating diagnosis based on patient history and clinical picture. This literature review outlines the key aspects of the pathogenesis, clinical manifestations, methods of diagnosis and treatment of ocular myasthenia gravis.

One- vs Two- vertical muscle surgery in the management of unilateral superior oblique muscle palsy with hypertropia over 20 PD.

Purpose: To compare the surgical outcomes of One- versus Two-vertical muscle surgery in patients with unilateral superior oblique muscle palsy (SOP) with primary position hypertropia (HT) over 20 PD. Patients in Group 1 underwent inferior oblique anterior transposition plus resection (IOATR), while patients in Group 2 underwent inferior oblique anterior transposition (IOAT) along with contralateral inferior rectus (IR) recession. Methods: Medical data of all SOP patients treated by either procedure from 2000 to 2023 in our strabismus center were recruited. We compared surgical outcomes between Group 1 and Group 2 by analyzing HT correction, rate of under-correction, and over-correction. Results: The study included 33 patients in Group 1 and 23 in Group 2. Both groups were similar in age, sex, etiology, affected side, diplopia, and head tilt. Group 2 achieved higher HT correction in all measured gazes. Group 1 had a higher risk of under-correction (18.18% in Group 1 vs 8.69% in Group 2) while Group 2 had a higher rate of over-correction (21.73% vs 0% in Group 1). Conclusion: In patients with severe unilateral SOP, Two-vertical muscle surgery achieved higher amounts of HT correction in all gazes despite a significantly higher risk of over-correction.

Slanted and Standard Lateral Rectus Recession Procedures for Convergence Insufficiency-Type Intermittent Exotropia in Children: A Retrospective Cohort Study.

Purpose: To evaluate the efficacy and safety of slanted bilateral lateral rectus recession (BLR-rec) for treating convergence insufficiency-type intermittent exotropia (CI-IXT) in children. Methods: In this retrospective cohort study, 26 patients aged <16 years with CI-IXT who underwent BLR-rec between August 2016 and July 2021 with six months of follow-up data post-surgery were classified into slanted BLR-rec group (n = 14) and standard BLR-rec group (n = 12; equal-length recession of the upper and lower horns of the LR muscle). Surgical outcomes were compared between the groups. Results: In the slanted and standard groups, the preoperative distance exodeviation was 27.9 ± 5.5 and 30.8 ± 10.0 prism diopter (PD) (p = 0.63), near exodeviation was 41.1 ± 5.6 and 42.9 ± 9.2 PD (p = 0.75), and difference between near and distance deviation (N-D deviation difference) was 13.2 ± 3.2 and 12.1 ± 3.3 PD (p = 0.30), respectively. The ratio of postoperative and preoperative N-D deviation difference was compared between the slanted BLR and standard BLR groups at six months postoperatively. The results revealed that the ratio for slanted-BLR was 0.44 ± 0.19, and for standard-BLR was 0.84 ± 0.24. In the standard group, stereoacuity remained unchanged post-surgery compared to that pre-surgery, while the slanted group showed significant improvement (p < 0.05, Wilcoxon signed-rank test). Conclusion: Compared with the standard BLR-rec procedure, the slanted BLR-rec procedure reduced N-D deviation differences in CI-IXT, positively impacting gross stereopsis.

An 11-year multicentric surgical experience on pediatric orbital floor trapdoor fracture: A World Oral Maxillofacial Trauma (WORMAT) project.

INTRODUCTION: Trapdoor fractures of the orbital floor occur almost exclusively in the paediatric population. Despite being widely discussed in the literature, their management remains controversial. The objective of this retrospective study was to analyse the surgical experiences on paediatric trapdoor fractures in the maxillofacial centres participating in the WORMAT project. MATERIALS AND METHODS: 14 centres collected data for patients aged ≤16 years operated between January 2011 and December 2022. The demographic, cause and type of fracture, timing from injury to surgery, surgical approach, type of floor repair and outcomes were recorded. Diplopia, surgical wound infection, hardware loosening and dysesthesia in the infraorbital nerve area were recorded at follow-up. RESULTS: 43 patients were included: 25 children (0-12 y) and 18 adolescents (13-16 y) (mean age, 11.1 years). Surgical treatment was performed within 24 h in 51 % of the patients, within 24-72 h in 33 %, and beyond 72 h in the remaining. The orbital floor was repaired with a resorbable implant/membrane in 63 % of the patients, open reduction without an implant in 30 %, a titanium mesh implant in 3 adolescent patients. At follow-up (mean 16.3 months), 14 patients had residual diplopia in the upper fields, only two of these resolved within 6 months. DISCUSSION: A tendency toward an increased incidence of postoperative diplopia with longer intervals between trauma and surgery was observed. This study showed different choices regarding the material placed on the floor, with a preference for open reduction without implants in children, compared to the use of resorbable implants or membranes in adolescents.

Isolated abducens nerve palsy following upper respiratory infection in a patient with ipsilateral chronic sphenoid sinusitis.

The abducens nerve, which is vulnerable because of its complex anatomy at the skull base, is seldom affected by acute or severe sphenoid sinusitis. Notably, abducens nerve palsy following asymptomatic chronic rhinosinusitis (CRS) in a healthy young individual after a mild upper respiratory infection (URI) remains undocumented in the literature. Herein, we report a case of acute unilateral abducens neuropathy in a healthy 35-year-old woman with CRS in the ipsilateral sphenoid sinus, following a mild URI 2 weeks earlier. She presented with sudden-onset diplopia, was afebrile, and had normal serum inflammatory biomarkers. Comprehensive ophthalmological and neurological exams revealed no abnormalities except limited lateral gaze in the left eye. Imaging revealed mucosal swelling on the hyperpneumatized left sphenoid sinus, which thinned the clivus and positioned the inflamed mucosa close to the Dorello's canal, likely facilitating the spread of inflammation to the ipsilateral abducens nerve. Urgent endoscopic sinus surgery combined with systemic corticosteroids and antibiotics led to complete resolution by postoperative day 10. The present case demonstrates acute abducens nerve neuropathy from URI-induced exacerbation of sphenoid sinus CRS with specific anatomical predispositions.

Three-dimensional computer navigation in the reconstruction of complex unilateral orbital fractures: evaluation and review of applications.

BACKGROUND: The eyes are the central aesthetic unit of the face. Maxillofacial trauma can alter facial proportions and affect visual function with varying degrees of severity. Conventional approaches to reconstruction have numerous limitations, making the process challenging. The primary objective of this study was to evaluate the application of three-dimensional (3D) navigation in complex unilateral orbital reconstruction. METHODS: A prospective cohort study was conducted over 19 months (January 2020 to July 2021), with consecutive enrollment of 12 patients who met the inclusion criteria. Each patient was followed for a minimum period of 6 months. The principal investigator carried out a comparative analysis of several factors, including fracture morphology, orbital volume, globe projection, diplopia, facial morphic changes, lid retraction, and infraorbital nerve hypoesthesia. RESULTS: Nine patients had impure orbital fractures, while the remainder had pure fractures. The median orbital volume on the normal side (30.12 cm3; interquartile range [IQR], 28.45-30.64) was comparable to that of the reconstructed orbit (29.67 cm3; IQR, 27.92-31.52). Diplopia improved significantly (T(10) = 2.667, p = 0.02), although there was no statistically significant improvement in globe projection. Gross symmetry of facial landmarks was achieved, with comparable facial width-to-height ratio and palpebral fissure lengths. Two patients reported infraorbital hypoesthesia at presentation, which persisted at the 6-month follow-up. Additionally, five patients developed lower lid retraction (1-2 mm), and one experienced implant impingement at the infraorbital border. CONCLUSION: Our study provides level II evidence supporting the use of 3D navigation to improve surgical outcomes in complex orbital reconstruction.

A rare case of inferior rectus dermoid cyst causing proptosis and optic neuropathy.

Purpose: To report a rare case of orbital dermoid cyst, involving the inferior rectus muscle, in a 26-year-old female presenting with proptosis and blurred vision in her right eye. Observations: The unique features of this case are discussed, including the location and contents of the cyst, the surgical approach, and the postoperative outcome. Conclusion and importance: The importance of considering dermoid cysts in the differential diagnosis of an orbital mass involving extraocular muscles and the need for a thorough evaluation to diagnose and manage these rare lesions.

Addressing Binocular Diplopia in a Diabetic Patient: Efficacy of Corticosteroids.

This report outlines an unusual instance involving a 65-year-old man who has a medical history of beta thalassemia minor, diabetes mellitus complicated by retinopathy, and iron deficiency anemia. He presented with binocular diplopia, a condition that frequently presents treatment difficulties. Despite inconclusive magnetic resonance imaging (MRI) findings, the patient's symptoms improved significantly with prednisone treatment. The etiology likely includes underlying retinopathy and cranial nerve palsy associated with diabetic complications. This case highlights utilizing corticosteroids, a largely unexplored treatment option, to optimize outcomes in managing binocular diplopia potentially linked to diabetic etiologies. It is notable that this patient was treated in a clinic for uninsured patients, emphasizing his low socioeconomic background, which adds further layers of complexity to his medical care. The complexities of managing diplopia in diabetes, compounded by limited treatment efficacy and financial constraints hindering complete diagnostic evaluation, underscore the intricate challenges of addressing diverse binocular diplopia presentations.

Internuclear Ophthalmoplegia Following COVID-19 with Abnormal Findings on Neuroimaging; A Case Report.

COVID-19 had been reported to be associated with neuro-ophthalmic complications. However, there is a shortage of evidence regarding internuclear ophthalmoplegia (INO) presentation after COVID-19 infection with related brain magnetic resonance imaging (MRI). Herein, we report a case of INO in a 69-year-old woman with presentation of diplopia following COVID-19 infection, along with her neuroimaging. We concluded that it is important to recognize the possibility of INO in patients with diplopia after COVID-19 and to consider brain MRI as an informative tool in these patients.

The Challenge of Strabismus Surgery for Patients with Hemianopic Visual Field Defects Associated with Ocular Deviations.

Homonymous and heteronymous hemianopias associated with strabismus are a therapeutic challenge because surgery may result in diplopia, decrease the visual field (VF), and/or lead to recurrence of the deviation. We present four cases: two homonymous hemianopias and two heteronymous hemianopias. Of the four patients, three had exotropia and one had esotropia. The origin of hemianopias was neoplastic in two cases and traumatic in two cases. Strabismus surgery was performed in three cases, but only one case had a good result; recurrence of the deviation and diplopia occurred in the other two cases. In conclusion, exotropia may develop as a compensatory mechanism in a patient with congenital or early-onset homonymous hemianopia and realigning the eyes could reduce the binocular VF and cause diplopia. In heteronymous hemianopias, there is little risk of the surgery causing diplopia, but the strabismus is unstable because of the absence of binocular vision and vergence reflexes if VF loss includes the macula. Further, strabismus, in association with hemianopia, may be caused by coexistent cranial nerve and/or gaze palsy. The prognosis, objective, and results of the strabismus operation should be clearly discussed with the patients or their guardians before it is performed if hemianopias are present.

Painful Diplopia as an Initial Presentation of VEXAS Syndrome.

This is a case report describing an unusual presentation of acute painful diplopia that led to the diagnosis of VEXAS syndrome. VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) syndrome is an adult-onset monogenic auto-inflammatory disease due to somatic UBA1 gene mutation in haematopoietic progenitor cells. Our patient was a 67-year-old diabetic male who presented with painful eye movements associated with diplopia, left periorbital pain and swelling. Imaging revealed an inflammatory process involving multiple intra- and extra-orbital structures. The patient improved initially with a short course of intravenous steroids. However, two months later he re-presented with right facial swelling. Bone marrow biopsy demonstrated UBA1 gene mutation supporting the diagnosis of VEXAS syndrome. This case highlights a unique ocular presentation of VEXAS.

A Case of Neurosyphilis Presenting with Isolated Abducens Nerve Palsy.

We herein report a case of neurosyphilis that presented with isolated bilateral abducens nerve palsy. A 39-year-old man was referred to our department with diplopia. He had a history of homosexual relationships and showed only bilateral abducens nerve palsy upon a neurological examination. Positive syphilis tests in the serum and cerebrospinal fluid and a contrasting effect on the abducens nerve on magnetic resonance imaging (MRI) confirmed the diagnosis of active neurosyphilis. When a patient manifests isolated abducens nerve palsy, neurosyphilis can be a differential diagnosis, although rare, and contrast-enhanced MRI may help diagnose the disease.

Neurologic Deficits Following Oral Misuse of the Nasal Decongestant Propylhexedrine.

INTRODUCTION: Propylhexedrine is an over-the-counter nasal decongestant used recreationally for amphetamine-like effects. Prior reports have associated intravenous misuse with brainstem dysfunction and diplopia. This is a case of propylhexedrine ingestion resulting in neurological impairment with associated MRI findings. CASE REPORT: A 21-year-old man presented with neurologic symptoms after ingesting propylhexedrine extracted from a 250 mg nasal inhaler into a lemon juice solution following recommendations from an online forum. He also followed recommendations to simultaneously ingest 1 g L-arginine to theoretically mitigate the risk of vasoconstriction. He developed flushing and euphoria before falling asleep. He awoke 12 h later with dizziness, intractable vomiting, diplopia, ataxia, dysmetria, and spontaneous horizontal nystagmus. Magnetic resonance imaging (MRI) revealed T2 enhancement concerning for vasogenic edema of the right posterior limbs of the internal capsule extending into the midbrain, cerebellar peduncles, and cerebellar white matter. Workup for primary vascular or autoimmune etiologies was unrevealing. Expanded drug testing was positive for mitragynine and THC. Blood analyzed via liquid chromatography quadrupole time-of-flight mass spectrometry was positive for propylhexedrine. On hospital day 6, the patient was discharged to physical rehabilitation with unresolved dysmetria and ataxia. DISCUSSION: Recreational oral misuse of propylhexedrine may be associated with neurologic injury. MRI findings in this case demonstrated vasogenic edema suggesting vasospasm as a possible etiology. Serum testing confirmed the presence of propylhexedrine. Although testing was also positive for mitragynine and THC, these have not been associated with similar neurologic deficits or MRI findings.

Surgical treatment of unilateral acquired superior oblique muscle palsy in adults by inferior oblique belly transposition: a retrospective analysis.

PURPOSE: Evaluate and analyze the efficacy of inferior oblique belly transposition (IOBT) in treating adult patients with diplopia and small-angle hypertropia caused by mild to moderate inferior oblique overaction (IOOA) secondary to acquired superior oblique palsy (SOP). METHODS: Nine adult patients with diplopia and small-angle hypertropia associated with mild to moderate IOOA secondary to unilateral acquired SOP were included in the current retrospective study. All patients received the IOBT procedure between February 2019 and May 2023 at The Second People's Hospital of Jinan and were followed up for more than 6 months after the surgery. During the procedure, the belly of the inferior oblique muscle was fixed to the sclera at 5 mm posterior to the temporal insertion of the inferior rectus muscle. The following indicators were reviewed pre- and post-surgery: the vertical deviation (VD) in the primary position and in the Bielschowsky test, the fovea disc angle (FDA) of the affected eye, changes in IOOA, and diplopia. RESULTS: After IOBT, the VD in the primary position decreased from 7.22△ ± 1.72△ (range 4△-10△) to 1.22△ ± 1.30△ (range 0△-3△). The VD in the Bielschowsky test decreased from 13.00△ ± 1.80△ to 3.22△ ± 1.09△. The FDA decreased from 10.02° ± 3.34° to 6.26° ± 1.91°. The grade of IOOA was reduced from 2.00 (1.00, 2.00) to 0.00 (0.00, 1.00). All changes were statistically significant (P < 0.001 or P = 0.006). Diplopia was resolved completely for all patients. CONCLUSIONS: IOBT can effectively treat adults with diplopia and small-angle hypertropia caused by mild to moderate IOOA secondary to acquired SOP.

Superior oblique paresis after endoscopic brow lift: A case report.

Key Clinical Message: Although a forehead lift is generally a safe surgery, it has well-known complications. Iatrogenic SO paresis is one of the rare complications following forehead lifting procedures which almost resolves spontaneously. Abstract: This report aims to introduce a woman with superior oblique (SO) muscle paresis following the brow and forehead lift procedure. A 30-year-old woman with a history of brow and forehead lift surgery was referred to the ophthalmic emergency department complaining of vertical diplopia. A right eye hypertropia was obvious at the left gaze. A Park's three-step test showed right eye superior oblique paresis. Other ophthalmic examinations including slit-lamp examination, tonometry, and dilated fundoscopy were unremarkable for both eyes. After a 3-month follow-up period, she had no diplopia. No sign of SO paresis was apparent in her ocular motility examinations. Iatrogenic SO paresis is one of the rare complications following forehead lifting procedures which almost resolves spontaneously.

Surgical management of chronic sixth cranial nerve palsy: case report and literature review.

Background: Esotropia resulting from sixth cranial nerve palsy can substantially impact an individual's visual acuity and overall quality of life. If the condition does not resolve in 6-10 months, surgical intervention may be necessary. Various muscle surgeries may be considered, with vertical rectus muscle transposition emerging as the primary option for treatment of complete palsy. However, this technique carries the risk of anterior segment ischemia and post-surgery deviations. Herein, we present a successful treatment of chronic complete sixth nerve palsy using a modified Nishida procedure, without splitting or tenotomy, and an adjunct botulinum toxin A (BTA) injection in the ipsilateral medial rectus muscle. Case Presentation: A 59-year-old woman with a history of traumatic sixth nerve palsy had previously undergone horizontal muscle strabismus surgeries. Following multiple left medial rectus recessions, lateral rectus resection, and BTA injections, esotropia persisted. The worsening of her condition led to emotional distress and impaired social interaction. Initial examination revealed marked esotropia and limited left eye abduction. Magnetic resonance imaging (SIGNA MR750w, GE Healthcare, Waukesha, WI, USA) of the left eye revealed a contracted medial rectus muscle and substantial atrophy of the left lateral rectus muscle. A modified Nishida procedure was performed with an injection of 3 units of BTA into the ipsilateral medial rectus muscle, resulting in improved ocular alignment and stable findings after nine postoperative months. Furthermore, we supported our successful outcome with a summary of similar reported cases of sixth nerve palsy managed using the modified Nishida procedure with or without adjunctive procedures. Conclusions: Following the modified Nishida procedure, the patient experienced a reduction in diplopia, improved ocular alignment and stability, and an increased binocular diplopia-free field. This case underscores the importance of an individualized approach to complex strabismus cases and highlights the modified Nishida procedure as a valuable tool in such circumstances. In the future, strabismus management will focus on refining personalized treatment and exploring innovative techniques for complex cases. Our success in using a combination of Nishida procedure and BTA injection should be further investigated in large-scale studies.

Case report: Bilateral optic nerve sheath meningocele: clinical aspects.

Optic nerve sheath meningocele is an enlargement of the sheath itself, consisting of a collection of cerebrospinal fluid along the perineural space. It should be considered primary if it is not associated with orbital-cerebral neoplasm or with cranio-orbital junction malformations. We report three cases of bilateral primary idiopathic optic nerve sheath meningocele, two of them with gradual vision loss. The first case presented a history of monocular blurred vision of the right eye and headache. It was initially treated with acetazolamide without any improvement, after which optic nerve sheath fenestration was required. The second case showed intermittent binocular diplopia with central 24-2 perimetry defects in the left eye. The third case was first presented as a subacute bilateral conjunctivitis with a suspected orbital pseudotumor. An incidental bilateral optic nerve sheath meningocele was found in the orbital imaging, being totally asymptomatic. In all the cases, orbital and cranial magnetic resonance with contrast and fat suppression was crucial in the diagnosis.

Clinical Significance of the Inferomedial Orbital Strut in Orbital Blowout Fractures: Incidence of Symptomatic Diplopia in a Fractured vs. Intact Strut.

Background/Objectives: This study aims to compare the clinical findings, particularly symptomatic diplopia, associated with an inferomedial orbital strut fracture versus intact strut and to determine the clinical significance of the inferomedial orbital strut in patients with orbital floor and medial orbital wall fractures. Methods: A 10-year retrospective observational study involving orbital blowout fracture cases was conducted in our institution. Patients with fractures of the orbital floor medial to the infraorbital groove and medial orbital wall, as seen on computed tomography (CT) scans, were included in this study. Patients with concomitant orbital rim fracture and those with old orbital fractures were excluded. Fracture of the inferomedial orbital strut was diagnosed via coronal CT images and patients were classified into those with an inferomedial orbital strut fracture and those without. Results: A total of 231 orbits from 230 patients was included in the study (fractured strut on 78 sides and intact strut on 153 sides). Approximately 2/3 of patients in both groups had the field of binocular single vision in primary position upon first examination (p = 0.717). Patients with strut fractures demonstrated only comminuted or open fractures, while those without strut fractures showed diverse fracture patterns (p < 0.001). Conclusions: Inferomedial orbital strut fracture does not automatically result in diplopia in patients with orbital blowout fractures. The integrity of the orbital periosteum plays a more essential role in hampering extraocular muscle displacement, thereby preventing symptomatic diplopia in these patients.

Medial Rectus Muscle Injuries After Functional Endoscopic Sinus Surgery: A Case Study.

Functional endoscopic sinus surgery (FESS) is the preferred method for managing obstructive sinus disorders. However, its proximity to the orbit poses a risk of orbital complications. This study presents a case of a 61-year-old female who underwent FESS for chronic rhinosinusitis with nasal polyps and subsequently experienced a serious ophthalmic complication including retrobulbar hemorrhage and medial rectus muscle hematoma, leading to adduction deficit and diplopia. The patient's condition was evaluated through clinical assessment and imaging studies, to address the extent and nature of the injury to the medial rectus muscle. Management strategies included surgical exploration and resection along with botulinum toxin injection to the lateral rectus muscle in the affected eye done six months after observation and regular ophthalmic examination to ensure the stability of the angle of deviation. This case highlights the importance of proper preoperative assessment and personalized treatment plans to manage the complications associated with FESS and optimize patient outcomes.

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.