An Update on Dragged-Fovea Diplopia Syndrome.

Dragged-fovea diplopia syndrome (DFDS) is a type of binocular double vision caused by a displacement of the fovea in one or both eyes due to retinal disorders including epiretinal membranes or other maculopathies. DFDS induces diplopia through a mismatch between peripheral motor fusion and central (foveal) fusion. It can be diagnosed by utilizing the Lights on - Lights off test. While there is no cure, there are treatments for DFDS including monocular occlusion or blurring (tape, lenses, IOL), Bangerter filter, and Fresnel prisms. While this syndrome has been identified in the literature by multiple names including central-peripheral Rivalry (CPR)-type diplopia, macular diplopia, and foveal displacement syndrome, this article works to summarize the current known characteristics, diagnostic tests, and treatment for this syndrome.

Internuclear ophthalmoplegia following radial artery cardiac catheterization approach: An unusual presentation.

Internuclear ophthalmoplegia (INO) may happen following percutaneous coronary intervention and angiography. However, no reports of INO during radial artery angioplasty were reported yet. We report a rare case in a 47-year-old man presenting with diplopia after radial artery angioplasty. Although the symptoms were resolved after 60 days, diagnosing this obstacle is necessary to reduce the patient and physician's anxiety.

High-flow carotid cavernous fistula and 'white eye': A rare and easily misdiagnosed presentation - A case report and literature review.

The three classic symptoms of carotid cavernous fistula (CCF) are pulsating exophthalmos, bruit and conjunctival chemosis. Here, we present a clinical case of isolated abducens nerve palsy due to a high-flow CCF in an 84-year-old woman, without the typical congestive orbito-ocular features. It was a diagnostic challenge because, for patients older than 50 years with cardiovascular risk factors, ischaemic mononeuropathy is the most frequent aetiology. This case illustrates the least common type of CCF that can be easily misdiagnosed. Physicians should consider fistula as a possible diagnosis in a patient with isolated abducens nerve palsy even without the classic triad.

Recurrent Headache With Diplopia: A Common Presentation of an Uncommon Entity.

Recurrent painful ophthalmoplegic neuropathy (RPON) is a rare headache syndrome, the diagnosis of which can be daunting to those who are not familiar with it. It presents characteristically with recurrent ocular motor weakness and ipsilateral head pain without an underlying etiology and often has unique imaging findings. Even after the successful diagnosis of this entity, there are no published management guidelines. Here, we present the case of a 31-year-old man whom we diagnosed with RPON following two episodes of unilateral headache with ophthalmoplegia over a three-month period and treated successfully with high-dose steroids on both occasions. We highlight the lack of prior migraine history and seeming antecedent viral infection as potential supporting evidence that this condition has a unique pathophysiology different from migraine. We also highlight his dramatic and reproducible response to steroids as additional evidence that steroids are good acute treatment options for this condition. Finally, as our patient lacked the expected cranial nerve imaging abnormalities on head MRI, we suggest that cranial nerve thickening and/or enhancement on MR imaging is not a sine qua non for this diagnosis, contrary to the opinion of some experts.

Afectación iatrogénica de la motilidad ocular por fármacos antitumorales inhibidores de los puntos de control / Iatrogenic impairment of ocular motility by immune checkpoint inhibitors

Se encontraron 4 revisiones sistemáticas que incluían este tipo de iatrogenia ocular, así como numerosos reportes de casos aislados. Los efectos adversos reportados comprenden: paresias oculomotoras, neuropatía óptica, atrofia óptica, síndromes miasteniformes, pseudo-orbitopatía tiroidea, síndrome del ápex orbitario e hipofisitis. La mayoría se manejaron sin interrupción o con interrupción parcial del tratamiento oncológico. Se requirieron tratamientos sistémicos agresivos para el manejo adecuado de la iatrogenia ocular.Es imprescindible que el oftalmólogo se familiarice con los nuevos tratamientos oncológicos ICI, capaces de provocar iatrogenia sobre la motilidad ocular grave e incapacitante para el paciente. La comunicación de efectos adversos con los tratamientos empleados puede ayudar al manejo más adecuado de estos pacientes. La investigación debe ir orientada al diagnóstico diferencial complejo y a optimizar las decisiones sobre los tratamientos oncológicos. (AU)

Cancer therapy relies on new antitumoral drugs called immune checkpoint inhibitors (ICI), which produce long-lasting anti-tumor responses and lengthen survival, but cause autoimmune-type toxicity. The clinical characteristics induced by ICI are not well characterized to date and careful collection of clinical data is required to accurately define its safety profile.We conducted a literature search in the main clinical search engines to identify pharmacological ocular iatrogenic events of ICIs related to ocular motility. Four systematic reviews were found that included this type of ocular iatrogenesis as well as numerous isolated case reports. Reported adverse effects include: oculomotor paresis, optic neuropathy, optic atrophy, myastheniform syndromes, thyroid pseudo-orbitopathy, orbital apex syndrome, and hypophysitis. Most were managed without interruption or with partial interruption of cancer treatment. Aggressive systemic treatments were required for adequate management of ocular iatrogenic events.It is essential that the ophthalmologist become familiar with the new ICI oncological treatments, capable of causing severe and disabling motilidad ocular iatrogenesis for the patient. The communication of adverse effects and the report of the treatments used can help the most appropriate management of these patients. Research should be oriented towards complex differential diagnosis and to optimize decisions on cancer treatments. (AU)

Unusual and Rare Causes of Monocular Elevation Deficit.

INTRODUCTION: To study the rare and unusual causes of monocular elevation deficit. METHODS: Five patients presenting to us with diplopia and elevation deficit were thoroughly examined and were found to have monocular elevation deficit due to rare causes. OBSERVATIONS: All five were found to have different underlying etiologies - iatrogenic, sphenoid wing meningioma, cysticercosis, sarcoidosis and mid brain infarct, and were managed appropriately. DISCUSSION: Monocular Elevation Deficit can occur due to a variety of causes. Having a high index of suspicion for the more serious etiologies is of utmost importance. Thorough clinical examination and imaging help clinch the diagnosis.

Diplopia in Movement Disorders: A Systematic Review of the Literature.

INTRODUCTION: Although the reported frequency of diplopia is between 10 to 40% of patients with Parkinson's disease (PD) and other movement disorders, it remains one of the most undiagnosed non-motor symptoms. Furthermore, it has a major impact on the quality of life of these patients. The aim of this study is to systematically review the literature regarding the frequency, causes, and implications of diplopia in movement disorders. METHODOLOGY: An electronic search was conducted in March and June 2023 using the PubMed database in order to identify appropriate studies. Studies that were written in English, that represented observational, analytical studies, and case reports, and that provided information regarding diplopia in movement disorders were included in the systematic review. RESULTS: A total of 686 articles were identified out of which 43 met the inclusion criteria. The studies included in the systematic review ranged from descriptive studies (case reports and case series) to analytical-observational studies (cross-sectional studies, prospective and retrospective cohort studies, and case-control studies). In Parkinson's disease, the incidence of diplopia ranged from 10 to 38%. In these patients, diplopia was linked to the presence of visual hallucinations and cognitive decline but also to convergence insufficiency and the presence of motor fluctuations. Cases of diplopia secondary to deep brain stimulation were also reported. Diplopia was associated with longer disease duration and worse motor and non-motor scores. Diplopia was also reported in other movement disorders such as multiple system atrophy (frequency as high as 18%) and progressive supranuclear palsy (frequency as high as 39%) and was associated with increased mortality and shorter duration in life span. CONCLUSIONS: Diplopia occurs in up to 38% of patients with movement disorders and has a negative impact on their health-related quality of life. Treating physicians should actively ask about diplopia and other ophthalmological symptoms, as many patients do not spontaneously report them. The pathophysiology of diplopia is complex, and it involves heterogeneous peripheral and central mechanisms. The management of these patients should involve a multidisciplinary team of health professionals in order to provide appropriate, tailored management.

Recurrent Tolosa-Hunt syndrome: a case report.

Introduction and importance: Tolosa-Hunt syndrome is a rare condition with unknown aetiology that manifests clinically as unilateral orbital pain and ophthalmoplegia. It is a diagnosis of exclusion that resolves spontaneously but can recur and respond dramatically to systemic steroids. Case presentation: The authors herein report a case of a 38-year-old male who presented with horizontal diplopia, limited outward movement of the right eye, and blurry vision for two days which was managed with oral Prednisolone. The patient visited 3 months later with progressive ptosis and vertical diplopia with periorbital pain over the right eye. It was eventually diagnosed via magnetic imaging resonance studies and successfully treated for Tolosa-Hunt syndrome with IV methylprednisolone followed by oral prednisolone. Clinical discussion: Hence, the typical clinical presentation of the case with significant response to steroids, exclusion of other conditions from investigation and imaging, and subsequent recurrence of similar symptoms were crucial for making the diagnosis of Tolosa-Hunt syndrome. Conclusion: Tolosa-Hunt syndrome is a syndrome of painful ophthalmoplegia which responds well to steroid therapy but has a tendency to recur. Hence, patients must be adequately informed about the reoccurrence and kept under follow-up.

Pure Orbital Floor Blow-In Fracture: An Extremely Rare Case.

A 57-year-old man presented with left diplopia on an upward gaze and ophthalmalgia after hitting the left side of his head. CT revealed a fracture on the left side of the orbital floor without orbital rim fractures and the protrusion of a small bone fragment into the orbit. Hess charts indicated markedly limited vertical movement of the left eye. Based on these findings, the patient was diagnosed with a pure orbital floor blow-in fracture (BIF). Symptoms persisted after a two-week monitoring period; therefore, the bone fragment was removed by a transcutaneous surgical approach with the assistance of a navigation system and an endoscope. Symptoms resolved after surgery, and CT and Hess examinations six months after surgery showed a good outcome. A pure BIF is rare, particularly on the orbital floor. Only a few similar case reports have been published to date, and we herein describe the surgical procedures performed and the treatment outcome of our case.

Post-traumatic Sinus Syndrome, Proposal for a New Clinical Entity (CDR Syndrome) as Variant of the Silent Sinus Syndrome: Systematic Review and Case Series.

The diagnostic criteria for silent sinus syndrome (SSS) are still controversial, especially for the post-traumatic/surgery cases that are, nowadays, excluded from the diagnosis of SSS because lacking of spontaneously. We present a systematic review of the last 10 years and our case series of SSS associated to previous trauma/surgery, proposing a new interpretation of SSS. In this work, following the PRISMA guide lines for systematic reviews, we collected 86 articles published on PubMed, Cochrane Library and Medline Plus since 2013 to 2023 about SSS. We divided them in six groups forming the structure of the review: (1) epidemiology, (2) clinical presentation, (3) imaging, (4) etiopathogenesis, (5) sss and craniofacial trauma and (6) treatment. We reported two explicative clinical cases: two men of 34 and 37 years old, involved in motorcycle accident in 2020 and 2014, respectively, and underwent surgery. They came back in 2023 referring diplopia documented by Hess-Lancaster test. CT-scan reported two clear cases of SSS. Basing on what is reported in literature, and basing on our experience, the post-traumatic/surgery SSS are more frequent than the idiopathic ones. Our proposal is to considered them as two individual entities. We propose to adopt the name of Post-traumatic sinus syndrome, or CDR syndrome (Catalfamo-De Rinaldis), for all cases that respect four specific diagnostic criteria reported into the text.

Etiological distribution of isolated oculomotor nerve palsy: analysis of 633 patients and literature review.

BACKGROUND AND PURPOSE: The etiological distribution of oculomotor nerve palsy has varied amongst the studies. This study aimed to define the clinical features and underlying etiologies of isolated oculomotor nerve palsy by recruiting patients from all departments in a referral-based university hospital. METHODS: The medical records of 672 patients who had a confirmed diagnosis of isolated oculomotor nerve palsy at all departments of Seoul National University Bundang Hospital, Seongnam, South Korea, from 2003 to 2020 were reviewed. A proportion of the etiology of isolated oculomotor nerve palsy was also compared with that of patients pooled from the previous studies that were searched on PubMed in May 2022. RESULTS: The most common etiology was microvascular (n = 168, 26.5%), followed by vascular anomalies (n = 110, 17.4%), neoplastic (n = 86, 13.6%), inflammatory (n = 79, 12.5%), idiopathic (n = 60, 9.5%) and traumatic (n = 53, 8.4%). Neurologists were mainly involved in the management of microvascular and inflammatory oculomotor nerve palsies whilst ophthalmologists mainly participated in the care of idiopathic, neoplastic and traumatic palsies. Neurosurgeons mostly took care of oculomotor nerve palsy due to vascular anomalies. CONCLUSIONS: The proportion of etiologies of isolated oculomotor nerve palsy may differ according to the specialties involved in the management. The results of previous studies on the etiological distribution of isolated oculomotor nerve palsy should be interpreted with this consideration.

Superior Orbital Fissure Syndrome in the Setting of Relapsing Polychondritis with Concurrent Sjogren's Syndrome: A Case Report.

PURPOSE: Ophthalmic manifestations of varying severity are often associated with systemic autoimmune conditions. Superior orbital fissure syndrome (SOFS) is a rare cranial neuropathy affecting nerves passing through the superior orbital fissure that causes a distinctive pattern of extraocular and pupillary findings. We report the coexistence of SOFS, relapsing polychondritis (RP) and Sjögren's syndrome (SS) in a 52-year-old female who presented with a past medical history of hypothyroidism, Raynaud's syndrome, and intermittent dry mouth and a 1-week history of worsening chemosis, proptosis, diplopia, and painful ophthalmoplegia. METHODS: Following a comprehensive eye examination, the patient underwent a CT head with contrast, MRI of the orbit, lumbar puncture, and laboratory investigations. RESULTS: CT and MRI examination revealed inflammatory standing in periorbital subcutaneous soft tissues and bilateral exophthalmos with right intraconal fat stranding surrounding the intraorbital and intracanalicular segments of the nerve, respectively. Lumbar puncture and laboratory investigations revealed an elevation in inflammatory biomarkers, a negative infectious workup, and ruled in SS when considering her history alongside a positive Schirmer test. She was started on high-dose steroids, which led to significant improvement; however, treatment revealed type 2 diabetes, necessitating a faster steroid taper, during which there was a reoccurrence of scleritis and ophthalmoplegia, leading to the initiation of rituximab infusions. After completing rituximab course, she was transitioned back to steroid therapy and was successfully tapered without event. CONCLUSION: This case is notable for the rare coexistence of SOFS with RP/SS overlap syndrome and highlights the management of concurrent orbital inflammatory syndrome and autoimmune diseases.

Ocular neuromyotonia: an unusual case after radiotheraphy for nasopharyngeal carcinoma.

Ocular neuromyotonia (ONM) is an infrequent disorder characterised by recurrent episodes of binocular diplopia caused by paroxysmal contraction of one or several extraocular muscles innervated by the same cranial nerve. It can be triggered spontaneously or caused by prolonged contraction of specific eye muscle(s) and is usually related to a local intracranial radiotherapy antecedent. We report the case of a 46-year-old woman who developed intermittent episodes of binocular diplopia eight years after radiotherapy for a nasopharyngeal carcinoma. After a complete neuro-ophthalmic assessment we diagnosed the case as an abducens nerve neuromyotonia. Although it is infrequent, radiotherapy to the nasopharynx is a possible cause of ONM, due to the proximity to the base of the skull and extraocular motor nerve pathways, especially that of the VI cranial nerve, as is the case presented in this article, about a patient whose history is a nasopharyngeal carcinoma treated with local radiotherapy.

Occurrence of acute retrobulbar hemorrhage during birth.

OBJECTIVE:  Presentation of acute retrobulbar subperiostal hemorrhage (hematoma) in the course of delivery. The occurrence, possible threats and recommended methods of treatment are described. Introduction: Acute retrobulbar hemorrhage is always a serious condition. Even if not connected with other ocular trauma, it could cause permanent blindness. The reason is based on constriction of the eye, decreasing of the blood supply and thus disruption of the oxygen supply to sensitive retinal tissues. After a short time, these tissues start to deteriorate and lose their natural function. This event is often connected with exophthalmia and diplopia. The primary diagnostic procedure is to measure intraocular pressure (IOP). Even if the ideal diagnostic tools are not accessible, performing a lateral canthotomy (event. with inferior cantholysis) is recommended to relieve IOP in acute situations. Normal intraocular pressure is considered to be 8-21 mmHg. Case report: Our 29-year-old female patient was in the second stage of delivery and suddenly got retrobulbar hemorrhage, resulting in exophthalmia and diplopia. Her baby was delivered shortly after the event. The following delivery course was normal, including her perineum repair and puerperium. Our patient was fortunate because her visual acuity and IOP were normal. Therefore, we chose an observational treatment strategy. After 5 weeks, we noted successful disintegration of the hematoma and decreased exophthalmia and diplopia without other consequences. Conclusion: We described retrobulbar subperiostal bleeding in our patient in the course of delivery. We depicted possible threats that could result in blindness and described recommended methods of treatment. Even if such a situation is extremely rarely, we believe that knowledge of these guidelines could help medical professionals broaden their treatment options. This particularly occurs when a trained eye surgeon is not available.

Isolated Inferior Division Oculomotor Nerve Palsy as the First Manifestation of Human Immunodeficiency Virus and Syphilis Co-Infection: A Rare Case Report.

Human immunodeficiency virus (HIV) and syphilis are two sexually transmitted diseases that continue to pose significant public health challenges globally. HIV and syphilis can be seen together; individuals with one of these infections are at risk of developing the other. Sharing common risk factors such as sexual transmission or intravenous drug use makes their coincidence likely. Syphilis, an infection caused by a spirochaete (Treponema pallidum), is a great mimicker and can present with a wide variety of clinical manifestations. Syphilis can cause various neurological symptoms including complete oculomotor nerve palsy; however, it is not a common manifestation. Here, we report for the first time a case of persistent incomplete oculomotor nerve palsy with pupillary involvement caused by the involvement of the inferior division of the oculomotor nerve, secondary to HIV and syphilis co-infection.

Outcomes of "a new modification of Jensen technique combined with medial rectus recession" in patients with complete sixth cranial nerve palsy.

BACKGROUND: To report the outcomes of "our modified Jensen technique" for treating esotropia in cases with complete sixth cranial nerve (CN VI) palsy. METHODS: This study is a 30-year case series of isolated complete CN VI palsy who underwent combined medial rectus recession and our modified Jensen operation. We modified the original technique by anchoring the muscle-unifying sutures to the sclera near the equator using a non-absorbable suture. Major outcomes such as postoperative diplopia, face turn, horizontal and vertical eye deviations, and abduction limitation were assessed. Successful surgery was defined as orthotropia or esotropia equal to or less than 8 prism diopters (PD) and the absence of any vertical deviation. RESULTS: Fifty-three cases with a mean age of 28 years old were included in this study. The male-to-female ratio was 3 to 1. Forty-five cases (85%) presented with unilateral palsy, whereas 8 patients (15%) had bilateral palsy. Trauma was the most common etiology (85%). Diplopia or face turn, presented in 42 patients before the operation, remained in seven cases after the operation. Primary position esotropia, which was the main complaint in all patients, decreased from 49 PD to 4 PD in unilateral palsy and from 101 PD to 10 PD in bilateral palsy. The mean reduction of abduction deficit was 1.78 in unilateral and 1.75 in bilateral palsy. The success rate was 76% in unilateral and 62% in bilateral palsy. CONCLUSION: Our modified Jensen operation was effective in treating patients with complete CN VI palsy, producing no significant permanent complications.

Non-Epstein-Barr Virus Sinonasal Undifferentiated Carcinoma Presenting as Diplopia and Rhinorrhea.

Sinonasal undifferentiated carcinoma (SNUC) is an extremely rare and highly aggressive malignant neoplasm of the nasal cavity and/or paranasal sinuses. SNUC is clinicopathologically distinctive from other tumors but is difficult to study due to its low incidence. There is also very little consensus about the etiology of SNUC, including its association with Epstein-Barr virus (EBV). Treatment modalities include surgery, chemotherapy, and radiation depending on the stage and grading. Herein, we discuss a patient who presented to the emergency department with chronic rhinorrhea and various ophthalmologic symptoms such as flashes, floaters, and diplopia. The patient was later diagnosed with SNUC in the setting of negative serological testing for EBV in addition to his previously concomitant history of bladder cancer. The purpose of this case report is to contribute to the broader literature of SNUC and the specifics surrounding the diagnostic modalities utilized, management, and outcome of non-EBV sinonasal undifferentiated carcinoma in a patient with atypical symptomatology.

A case of bilateral amyloidosis localized to extraocular muscles mimicking thyroid eye disease.

BACKGROUND: Amyloidosis is a rare condition characterized by the abnormal deposition of amyloid proteins in various tissues and organs. While systemic amyloidosis has been well-documented, amyloid deposition in extraocular muscles is an exceptionally rare occurrence, with only 35 reported cases. This case report sheds light on the importance of considering amyloidosis in the differential diagnosis of patients presenting with proptosis and diplopia, which are often associated with thyroid eye disease. CASE PRESENTATION: A woman in her twenties sought medical attention due to a complaint of diplopia. Her ocular examination revealed almost normal findings except for exotropia and proptosis. Orbital magnetic resonance imaging displayed fusiform enlargement of nearly all eye muscles, a presentation typically observed in thyroid eye disease. However, despite corticosteroid therapy, her symptoms showed no improvement. Given the unusual lack of response to conventional treatment, and inhomogeneous enhancement of the muscle, an extraocular muscle biopsy was conducted. This biopsy yielded a unique finding-amyloid deposition within the muscle tissue. This discovery was particularly intriguing due to the extreme rarity of amyloidosis affecting extraocular muscles, with fewer than three dozen documented cases worldwide. CONCLUSION: This unique case underscores the critical need for a comprehensive approach to diagnosing patients with proptosis and diplopia. While these symptoms are commonly attributed to thyroid eye disease, it is essential to consider alternative diagnoses such as amyloidosis, especially when standard treatments fail to yield results. The discovery of amyloid deposition in the extraocular muscles, although exceedingly rare, emphasizes the significance of a thorough differential diagnosis. In conclusion, this case report highlights the importance of vigilance in clinical practice, encouraging ophthalmologists to explore less common diagnostic possibilities when faced with challenging cases. Further research and clinical investigation are warranted to better understand the mechanisms and potential treatments for amyloidosis affecting the extraocular muscles.