Exploring the Etiologies of Acquired Unilateral Proptosis: A Literature Review with Case Presentations

Informing Patient Surveillance for the Growing Number of Survivors of Lung Cancer

Introduction : Carotid cavernous fistula (CCF) is caused by an abnormal connection between the carotid artery and the cavernous sinus, which can lead to eye-related problems. It is crucial to recognize its signs, as they can resemble other conditions.
 Case Illustration : First patient is a male 3-month-old baby, with unilateral right eye proptosis that was observed soon after birth and history of hypothyroid. Proptosis is more prominent while the baby is crying. Orbital ultrasonography revealed ophthalmica vein dilatation with S configuration, complex flow, and velocity of 10 cm/s. Second case is a 50-year-old male also with unilateral proptosis and orbital bruit with history of head trauma 2 months prior to admission. Patient came with complain of sudden blurry vision for 1 week. The results of the magnetic resonance venogram revealed tortuos dilated superior ophthalmica (SOV), cavernous sinus bulging, and dilated superior petrosal, sigmoid, transverse sinus, and internal jugular vein.
 Discussion : CCFs can occur at any age, but are more common between 40-60. Risk factors like head trauma, hypertension, connective tissue disorders increase the likelihood of developing CCF. They are often misdiagnosed as other conditions such as thyroid ophthalmopathy or conjunctivitis. Ocular doppler ultrasound can detect SOV dilation and arterial flow, while magnetic resonance imaging can show superior ophthalmic vein engorgement, muscle hypertrophy, and cavernous sinus dilatation. Treatment for symptomatic CCFs involves endovascular obliteration using balloons, embolic agents, or metallic coils, while asymptomatic and mildly symptomatic patients can be treated conservatively.
 Conclusion : Awareness of CCF symptoms, varied workup, prompt treatment is important to prevent severe complications.

A 62-year-old Caucasian female presented with spontaneous right-sided eye pain, chemosis and ophthalmoloplegia. A right carotid-cavernous sinus fistula was discovered and successfully treated with transvenous introduction of Guglielmi detachable coils. In addition to complete fistula occlusion, follow-up angiography 6 weeks post-treatment demonstrated the presence of a patent persistent trigeminal artery previously hidden by the caliber of the carotid-cavernous sinus fistula. The spontaneous nature of the presentation and the proximity of the persistent trigeminal artery to the site of coil embolization suggest spontaneous rupture of a persistent trigeminal artery aneurysm as cause of the fistula. Current literature on this finding is discussed here, demonstrating that an aneurysm of the persistent trigeminal artery leading to carotid-cavernous sinus fistula formation is rare. Furthermore, the present case is the first report of its kind in world ophthalmology literature.

Psychologic Adjustment to Irreversible Vision Loss in Adults: A Systematic Review

Background: Platinum-based combination chemotherapy, including cisplatin and carboplatin, are important cytotoxic anti-cancer agents that are widely used to treat various solid tumors. Carboplatin has a similar effect on survival in small cell lung cancer, but generally has a milder toxicity profile when compared with cisplatin. Both may cause moderate or severe neurotoxicity, but ocular neurotoxicity from carboplatin is rarely reported. Case presentation: A 79-year-old man underwent intravenous polychemotherapy (atezolizumab, etoposide, and carboplatin) for small cell lung cancer. One week after the second cycle of chemotherapy, he reported bilateral visual loss as hand motion in both eyes. Dilated fundus examination showed retinal arterial narrowing without hemorrhage, and diffuse choroidal and retinal thinning was observed in an optical coherence tomography scan. Fluorescein angiography revealed significantly delayed circulation without evidence of obstructive lesions. 30-Flicker electroretinogram testing showed a complete absence of cone response in both eyes. The patient's visual acuity aggravated to no light perception in both eyes, even after the cessation of chemotherapy. Conclusions: Carboplatin combination chemotherapy administered at therapeutic doses can result in irreversible visual loss, a side effect that is not widely acknowledged. When using carboplatin, physicians should be aware of its potential ocular toxicity.

Choroidal melanoma is the most common primary malignant intraocular tumor that rarely is left undiagnosed. We report a case of a lately detected choroidal melanoma associated with exudative retinal detachment and severe visual loss in the affected eye. The patient was referred to our clinic with visual acuity in the right eye 6/6 while in the left eye was limited to light perception. Ophthalmic examination showed a large pigmented choroidal tumoral mass occupying most of the vitreous cavity and associated exudative retinal detachment. Ultrasonography confirmed a dome-shaped tumor with internal homogeneity, while anterior choroid, ciliary body and angle were found with no invasion. The fellow eye was examined thoroughly and no lesions were detected. Further clinical investigation for metastasis was negative. Negligence of the symptoms, gradual evolution of the tumor and late referral resulted in irreversible loss of vision. The patient had no other systemic pathology and was advised for enucleation in the left eye and follow-up. Choroidal melanoma is a serious malignancy that could threaten the patient’s life and requires early detection and treatment. In large lesions enucleation is the treatment of choice. J Med Cases. 2015;6(1):10-13 doi: http://dx.doi.org/10.14740/jmc2004w

Classifications in Brief: The Spinal Instability Neoplastic Score.

The differential diagnosis for proptosis and periorbital swelling is broad and includes infectious, malignant, vascular, and rheumatologic etiologies. In this study, we report a case of carotid-cavernous fistula as the cause of acute-onset unilateral proptosis and periorbital swelling of the right eye in a 44-year-old female patient whose symptoms were initially attributed to possible immunoglobulin G4-related disease (IgG4-RD). The patient initially received antibiotics for presumed cellulitis and steroid treatment for a possible autoimmune cause, however; her autoimmune work-up was negative. Radiologic imaging later confirmed that she had a direct spontaneous carotid-cavernous fistula. She experienced significant improvement in her symptoms and vision after embolization treatment. Due to the risk that a carotid-cavernous fistula will progress quickly and cause neurological damage, this is a key diagnosis that should not be missed in patients with acute-onset periorbital and visual symptoms. Rheumatologists should include this condition in the differential for any patient who presents with periorbital swelling and vision disturbances.

Objectives: To report a rare pediatric case of spontaneous high-flow direct carotid–cavernous fistula (CCF) presenting with rapid, vision-threatening orbital congestion, and to highlight the diagnostic pathway and management barriers in a low-resource setting. Case Presentation: An 12-year-old boy presented with 3–4 weeks of progressive left-eye proptosis, orbital pain, and visual decline, culminating in no light perception. Examination showed marked left-sided proptosis, afferent pupillary defect, and elevated intraocular pressure (24–25 mmHg; right eye 12 mmHg). Funduscopy revealed dilated, tortuous retinal veins with venous pulsations, without cherry-red spot or disc edema. Orbital B-scan demonstrated a well-defined hypoechoic retrobulbar structure suggestive of vascular dilation. Brain-orbit MRI identified multiple T2 flow voids, a markedly dilated superior ophthalmic vein draining into the cavernous sinus and appearing connected to the left internal carotid artery, associated enlarged extraocular muscles, and retrobulbar edema—findings consistent with a high-flow direct CCF. Urgent digital subtraction angiography and endovascular embolization were recommended to prevent intracranial venous hypertension and irreversible optic neuropathy. Due to the absence of interventional neuroradiology services and endovascular equipment locally, the patient was referred abroad for definitive management, with close ophthalmic monitoring advised pending transfer. Conclusion: CCF, uncommon in children, should be considered in pediatric proptosis with visual decline—even without trauma. Imaging enables early recognition; however, timely access to endovascular therapy determines visual and neurological outcomes. This case underscores gaps in neurovascular care within low-resource settings and supports investment in endovascular capacity, clinician training, and referral networks to mitigate preventable vision loss in presentations.

Steroid-induced glaucoma (SIG) is the most common adverse steroid-related effect on the eyes. SIG patients can suffer from trabecular meshwork (TM) dysfunction, intraocular pressure (IOP) elevation, and irreversible vision loss. Previous studies have mainly focused on the role of extracellular matrix turnover in TM dysfunction; however, whether the cellular effects of TM cells are involved in the pathogenesis of SIG remains unclear. Here, we found that the induction of cellular senescence was associated with TM dysfunction, causing SIG in cultured cells and mouse models. Especially, we established the transcriptome landscape in the TM tissue of SIG mice via microarray screening and identified ANRIL as the most differentially expressed long non-coding RNA, with a 5.4-fold change. The expression level of ANRIL was closely related to ocular manifestations (IOP elevation, cup/disc ratio, and retinal nerve fiber layer thickness). Furthermore, p15, the molecular target of ANRIL, was significantly upregulated in SIG and was correlated with ocular manifestations in an opposite direction to ANRIL. The reciprocal regulation between ANRIL and p15 was validated using luciferase reporter assay. Through depletion in cultured cells and a mouse model, ANRIL/p15 signaling was confirmed in cellular senescence via cyclin-dependent kinase activity and, subsequently, by phosphorylation of the retinoblastoma protein. ANRIL depletion imitated the SIG phenotype, most importantly IOP elevation. ANRIL depletion-induced IOP elevation in mice can be effectively suppressed by p15 depletion. Analyses of the single-cell atlas and transcriptome dynamics of human TM tissue showed that ANRIL/p15 expression is spatially enriched in human TM cells and is correlated with TM dysfunction. Moreover, ANRIL is colocalized with a GWAS risk variant (rs944800) of glaucoma, suggesting its potential role underlying genetic susceptibility of glaucoma. Together, our findings suggested that steroid treatment promoted cellular senescence, which caused TM dysfunction, IOP elevation, and irreversible vision loss. Molecular therapy targeting the ANRIL/p15 signal exerted a protective effect against steroid treatment and shed new light on glaucoma management.

Ferrous ion accumulation and lethal oxidative stress mediate irreversible retinal pigment epithelial (RPE) cell ferroptosis and subsequent photoreceptor degeneration, a potential key pathogenic factor in the onset of dry age-related macular degeneration (dAMD), causing irreversible vision loss in the global elderly population. However, currently, no effective interventional treatment strategy exists in clinical practice. Herein, lesion site-targeted melanin-like nanoparticles, named ConA-MelNPs, are designed as a novel ferroptosis inhibitor for retinal degenerative diseases. ConA-MelNPs possessed chelating iron ion characteristics, alleviating severe mitochondrial damage caused by oxidative stress and protecting RPE cells from ferroptosis induced by sodium iodate (NaIO3). In a preclinical dAMD mouse model, a single intravitreal injection of ConA-MelNPs yielded significant responses in electroretinograms and visually-driven optomotor responses in visually impaired mice, resisting the challenge posed by secondary NaIO3-induced injuries, with the long-term sustainability of its therapeutic effect. Mechanistically, ConA-MelNPs achieve a therapeutic effect by interrupting the detrimental cascade involving "RPE cell ferroptosis, lethal oxidative stress, and microglial proinflammatory activation," affording the restoration of retinal homeostasis. The synthesized ConA-MelNPs demonstrated good biosafety, with no detected ophthalmic or systemic side effects. Collectively, ConA-MelNPs are proposed as a promising therapeutic option for atrophic retinal diseases such as dAMD.

There is limited published evidence on the accuracy of temporal artery US for detecting GCA. Ultrasound seems to be moderately sensitive when the specificity is good, but data were heterogeneous across studies and either did not use the same halo thickness threshold or did not report it. We can draw no conclusions from accuracy studies on whether US can replace TAB for diagnosing GCA given the very low certainty of the evidence. Future research could consider using the 2016 revision of the ACR criteria as a reference standard, which will limit incorporation bias of TAB into the reference standard.

O31 Giant cell arteritis - a rare ocular presentation

Commentary: Postrecurrence survival in patients with lung cancer after curative surgery warrants systematic investigation to optimize management strategies

This book is written by a group of Neuroradiologists from an Indian university and published by Jaypee Brothers Medical Publishers (P) Ltd see (Figure 1). The first chapter discusses the physical principles of MRI and the 2nd chapter illustrates brain anatomy. From the 3rd to 11th chapter, all chapters discuss different malformations and disorders that affect the brain. Chapters 12th and 13th are glossary and acronyms respectively. The only colored pictures are in the plate and the rest of the book illustrations has no colors. Each chapter is written by a Neuroradiologists of the 14 authors of the book (3 editors and 11 contributors). The used method in 3rd to 11th chapter is a presentation of a case as an example of the brain malformation or disorder on an MRI scan. The book uses Radioland’s style in presenting the cases. Each case will have a case presentation and history, MRI scan pictures, MRI findings, comments and explanations, opinions, and clinical discussion. This book is (101), so it is for beginners and it does not cover all MRI neuroradiology topics since it has 101 cases only. The book has too many English language mistakes and a significant lack of proofreading. Some of the MRI images have poor image quality. The book is printed on high-quality papers. In some chapters of the book, there are some CT scans to show the difference between MRI and CT in the ability to show some of the abnormalities. The medical illustration is not high-quality illustration; for example; the Percheron artery was illustrated in the book poorly. Nobody should expect to see professional illustrations like Netter’s style in illustrations in this book. In the book, they rarely use arrows to indicate the location of the pathology. The book does not mention some of the famous neuroradiology signs. In the subdural hematoma case, they present a case of acute on chronic hematoma as subdural hematoma case which would be better to show acute in one case, chronic in one case, and acute on chronic on a third case because this book is for beginners. Some anatomical illustrations are out of the chapter which discuss the anatomy of the brain like the illustration of the cerebral venous system on page 137, figure 28. From page 139 to 144, the book presents three anatomical variations in the brain’s vasculatures (carotid cavernous fistula, persistent occipital sinus, and fetal posterior cerebellar artery) which would be better to be in a separate chapter about anatomical variations. Sometimes, the book will mention a famous neuroradiology sign without providing any MRI image as a demonstration. As well, the book will describe some findings which is invisible on MRI images and the authors will write “not seen in these images”. If the finding is not demonstrated for the readers, the authors should not mention it in the findings. Instead, they can include this undemonstrated finding in the comments and explanation section of the case. This book will mention too many things (radiological signs, findings, associated conditions, etc.) without showing figures for what they are writing about like in the astrocytoma case they mention astrocytoma’s types without differentiating them on figures. Plus, the cases are not mentioned in an organized fashion. For example, the book will speak about a tumor that affects a specific region (let’s call it region number 1) then they will write about another tumor in a different region (let’s call it region number 2) then they jump back to the first region and write about another tumor that affects region number 1. The book needs to be organized to write; for example; the tumors in cerebellopontine angle together which will help the reader to collect and compare all the information about the tumors that affect this region then move to another region. The book will provide a low image quality with too many artifacts; for example; the diffusion image in the hypoglycemia case. The artifacts chapter discusses only three common types of artifacts which are the Gibbs phenomenon, zipper artifact, and susceptibility artifact. The chapter can be improved by organizing the artifacts like; patient-related, signal processing related, and machinery (hardware) related artifacts. The artifacts’ chapter is placed in between clinical cases which should be removed to the beginning or at the end of the book. The last chapter collected different cases under miscellaneous.