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Radiologie (Heidelberg, Germany) Mar 2024Carotid cavernous fistulas (CCFs) are rare but clinically significant vascular anomalies characterized by abnormal connections between the cavernous sinus and arteries.... (Review)
Review
BACKGROUND
Carotid cavernous fistulas (CCFs) are rare but clinically significant vascular anomalies characterized by abnormal connections between the cavernous sinus and arteries. This overview presents a comprehensive analysis of anatomy, classification, clinical presentation, diagnosis, imaging, and therapy of CCFs. The cavernous sinus, a central venous structure in the brain, is of critical importance for understanding CCFs due to its proximity to key structures such as the internal carotid artery and cranial nerves.
CLASSIFICATION
CCFs are classified into direct and dural types, with direct fistulas typically being high-flow and dural fistulas being low-flow. The symptomatology varies greatly and can range from noises in the head, diplopia, red eye, tearing, to blurred vision and headaches. The diagnostic assessment requires a combination of detailed medical history, neurological and ophthalmological examination, and the use of imaging techniques.
METHODS
In imaging, computed tomography (CT) and magnetic resonance imaging (MRI) are crucial for depicting the anatomical structures and blood vessels, while digital subtraction angiography (DSA) is considered the gold standard for accurate representation of the fistula. The treatment of CCFs is complex and depends on the type of fistula, location, and clinical condition of the patient.
CONCLUSION
This overview emphasizes the importance of precise diagnosis and individualized therapy to achieve optimal results and avoid complications. Ongoing developments in medical imaging and treatment techniques will continuously improve the treatment outcomes of patients with CCFs.
Topics: Humans; Carotid-Cavernous Sinus Fistula; Cavernous Sinus; Magnetic Resonance Imaging; Vision Disorders; Diplopia
PubMed: 38351202
DOI: 10.1007/s00117-024-01269-1 -
Current Opinion in Ophthalmology Nov 2023Teprotumumab, an inhibitor of the insulin-like growth factor 1 receptor (IGF-1R), was approved by the US Food and Drug Administration in January 2020 for the treatment... (Review)
Review
PURPOSE OF REVIEW
Teprotumumab, an inhibitor of the insulin-like growth factor 1 receptor (IGF-1R), was approved by the US Food and Drug Administration in January 2020 for the treatment of thyroid eye disease (TED). The clinical trials leading to its approval enrolled patients with recent disease onset and significant inflammatory symptoms and signs. Subsequent real-world teprotumumab use in patients with longer duration of disease also may be effective, and there have been several publications reporting on experience in these patient groups.
RECENT FINDINGS
TED results in disfiguring changes such as ocular proptosis and affects visual function by altering extraocular muscle function, leading to diplopia. Compressive optic neuropathy also may occur, and disease manifestations may persist for years. Teprotumumab treatment in cases of TED in which prior interventions (medical or surgical) had failed, or in treatment-naïve patients whose disease had been stable for years, has been reported to improve both clinical signs and symptoms (proptosis, diplopia) and to reduce the pathologic orbital changes as assessed by orbital imaging.
SUMMARY
Teprotumumab may be an appropriate treatment for TED regardless of disease duration and irrespective of the presence or absence of markers of active inflammation within the orbit.
Topics: Humans; Graves Ophthalmopathy; Diplopia; Orbit; Exophthalmos
PubMed: 37610428
DOI: 10.1097/ICU.0000000000000997 -
Thyroid : Official Journal of the... Oct 2023Corticosteroid therapy is often employed in thyroid eye disease (TED), but its efficacy is variable. Teprotumumab and tocilizumab have been considered as effective...
Corticosteroid therapy is often employed in thyroid eye disease (TED), but its efficacy is variable. Teprotumumab and tocilizumab have been considered as effective alternatives. This study aims to evaluate their clinical outcomes and safety in patients with steroid-resistant TED. A retrospective case-control study was conducted between 2018 and 2022 within a national multicenter health system. Thirty-seven patients with moderate to severe steroid-resistant TED treated with teprotumumab or tocilizumab (cases) were compared with steroid-naïve patients treated with similar therapy (controls). Due to lack of steroid-naïve patients treated with tocilizumab, a control subgroup for tocilizumab was not included in the analysis. Demographic and clinical characteristics were described. Proptosis, diplopia, clinical activity score (CAS), and disease severity (European Group on Graves' orbitopathy classification) were evaluated at weeks 0, 12, 24, and 52 after therapy initiation. Thirty-one patients received teprotumumab (13 cases and 18 controls) and 6 received tocilizumab (cases). The mean age was 57 years (standard deviation ±14.3), median duration of TED was 11.5 months (interquartile range [IQR]: 7.2-17.7), and median excess proptosis was 4 mm (IQR: 2-8) above the upper limit of normal for sex and race. At week 24, in the teprotumumab cases, 81% had proptosis response (reduction of ≥2 mm), 45.5% resolution of diplopia, 85.7% disease inactivation (CAS <3), and 58.3% reverted to mild disease severity. There were comparable results in teprotumumab controls, with no significant differences between subgroups. In the tocilizumab cases, 50% had a proptosis response, 16.7% resolution of diplopia, 100% disease inactivation, and 75% returned to mild disease. In the teprotumumab cases, there was a trend toward worsening proptosis and diplopia between weeks 24 and 52. In the same time frame, the tocilizumab cases had a trend toward worsening diplopia, disease activity, and severity. In the teprotumumab subgroup, 46.2% experienced otic changes and 23.1% hyperglycemia. In the tocilizumab subgroup, there were no reported adverse events. Teprotumumab and tocilizumab improved inflammation in patients with moderate to severe TED who had failed previous steroid therapy. Additionally, the teprotumumab cases demonstrated similar improvement in proptosis and diplopia to the teprotumumab controls. Further evaluation, particularly regarding the long-term response and side effect profile, of these medications in steroid-resistant TED is needed.
PubMed: 37515425
DOI: 10.1089/thy.2023.0167 -
Current Hypertension Reports Apr 2024This review summarizes key findings relating to the association between preeclampsia and retinal disorders. (Review)
Review
PURPOSE OF REVIEW
This review summarizes key findings relating to the association between preeclampsia and retinal disorders.
RECENT FINDINGS
Preeclampsia is a major cause of maternal morbidity. Pregnant women with preeclampsia frequently describe having visual disturbances. Retinal changes can be identified on fundoscopy in most patients with preeclampsia. While retinal pathology secondary to preeclampsia usually resolves postpartum, there is growing evidence that women with preeclampsia have a higher long-term risk of developing retinal disorders after pregnancy. Pregnant women often experience visual changes. While these symptoms may be benign, careful attention should be paid to exclude retinal disorders secondary to preeclampsia. Pregnant women complaining of new-onset or worsening blurry vision, scotomata, diplopia, or photopsia require rapid and thorough evaluation to rule out hypertensive disorders. Management of preeclampsia, including administration of magnesium sulfate and delivery of the fetus, can reverse retinal pathologies in most cases.
Topics: Female; Humans; Pregnancy; Pre-Eclampsia; Hypertension; Vision Disorders; Retina
PubMed: 38133842
DOI: 10.1007/s11906-023-01290-0 -
Klinische Monatsblatter Fur... May 2024Giant cell arteritis (GCA) is the most common primary vasculitis and is associated with potential bilateral blindness. Neither clinical nor laboratory evidence is simple... (Review)
Review
Giant cell arteritis (GCA) is the most common primary vasculitis and is associated with potential bilateral blindness. Neither clinical nor laboratory evidence is simple and unequivocal for this disease, which usually requires rapid and reliable diagnosis and therapy. The ophthalmologist should consider GCA with the following ocular symptoms: visual loss or visual field defects, transient visual disturbances (amaurosis fugax), diplopia, eye pain, or new onset head or jaw claudication. An immediate ophthalmological examination with slit lamp, ophthalmoscopy, and visual field, as well as color duplex ultrasound of the temporal artery should be performed. If there is sufficient clinical suspicion of GCA, corticosteroid therapy should be initiated immediately, with prompt referral to a rheumatologist/internist and, if necessary, temporal artery biopsy should be arranged. Numerous developments in modern imaging with colour duplex ultrasonography, MRI, and PET-CT have the potential to compete with the classical, well-established biopsy of a temporal artery. Early determination of ESR and CRP may support RZA diagnosis. Therapeutically, steroid-sparing immunosuppression with IL-6 blockade or methotrexate can be considered. These developments have led to a revision of both the classification criteria and the diagnostic and therapeutic recommendations of the American College of Rheumatologists and the European League against Rheumatism, which are summarised here for ophthalmology.
Topics: Giant Cell Arteritis; Humans; Diagnosis, Differential; Adrenal Cortex Hormones; Immunosuppressive Agents; Temporal Arteries; Evidence-Based Medicine; Treatment Outcome; Biopsy
PubMed: 38593832
DOI: 10.1055/a-2252-3371 -
Seminars in Ophthalmology Jul 2024Dragged-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... (Review)
Review
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.
Topics: Humans; Diplopia; Syndrome; Fovea Centralis; Vision, Binocular; Visual Acuity; Tomography, Optical Coherence; Retinal Diseases
PubMed: 38591258
DOI: 10.1080/08820538.2024.2323121 -
Practical Neurology Nov 2023Classic Raymond syndrome is a rare neurological presentation comprising ipsilateral abducens palsy, contralateral facial paresis and contralateral hemiparesis. We...
Classic Raymond syndrome is a rare neurological presentation comprising ipsilateral abducens palsy, contralateral facial paresis and contralateral hemiparesis. We present a man in his late 60s who presented with diplopia, dysarthria and right-sided limb weakness. This syndrome is one of a group of 'crossed paralyses' of the caudal pons.
Topics: Male; Humans; Brain Ischemia; Stroke; Pons; Facial Paralysis; Paresis; Ischemic Stroke
PubMed: 37524438
DOI: 10.1136/pn-2023-003782 -
Archivos de La Sociedad Espanola de... Jun 2024Trochleitis is clinically and/or radiologically evidenced inflammation of the trochlea or orbital pulley. Clinically it is characterized by pain and hypersensitivity in... (Review)
Review
Trochleitis is clinically and/or radiologically evidenced inflammation of the trochlea or orbital pulley. Clinically it is characterized by pain and hypersensitivity in the superomedial orbital angle, which is increased or triggered by direct palpation of the area and/or eye movements. During the REM (rapid eye movements) phase of sleep, patients with trochleitis suffer from nocturnal micro-awakenings that impede their rest, and pain is often associated with visual symptoms (diplopia or Brown's syndrome). The lack of common guidelines for diagnosis and treatment of this disease, its low prevalence and the lack of knowledge of the different entities associated with trochlear pain, leads to underdiagnosis or misdiagnosis. It is essential to know the characteristics of this pathology and to diagnose it correctly, differentiating it from other trochlear pain entities, in order to be able to carry out an adequate therapeutic and prognostic approach. The lack of consensus on the therapeutic protocol means that various treatments are used, in different order and often with a combination of several without a firm scientific basis. This comprehensive review of previous studies concludes that nonsteroidal anti-inflammatory drugs (NSAIDs) achieve an overall complete cure rate of 77%, although this rate decreases to 30% in case of motility restriction or diplopia. Intratrochlear corticosteroid injection achieves an overall complete cure rate of 86%, even in the worst prognosis trochleitis, being the only effective option in NSAID-refractory trochleitis and currently being questioned as the first treatment option.
PubMed: 38901607
DOI: 10.1016/j.oftale.2024.06.008