Clinical outcomes of conjunctivochalasis treatment with a new ophthalmic radiofrequency device.

PURPOSE: To investigate the safety and efficacy of a new micro-controlled radiofrequency device for treatment of conjunctivochalasis (Cch). METHODS: Data of 127 patients (230 eyes) who underwent ophthalmic radiofrequency treatment for Cch from January 2020 to June 2023 were analyzed retrospectively. Cch coagulation was performed with a radiofrequency electrode tip (OcuRF®, Ilooda, Korea) and a high-frequency radio-wave electric unit (0.6 ~ 0.8 watts, 2 MHz, Acutron™, Ilooda, Korea). Pre- and postoperative Cch grading, slit-lamp photography, tear film break-up time (TBUT), and bulbar conjunctival hyperemia using Keratograph 5 M (Oculus, Wetzlar, Germany) were evaluated. Cch grade 0 or 1 after surgery was regarded as 'success'. Complications, recurrence, and additional treatment rates were analyzed. RESULTS: In 227 (98.7%) eyes, the radiofrequency treatment led to marked improvement of Cch, with 224 (97.4%) eyes achieving grade 0 or 1 at 2 months postoperatively. Eight eyes (3.5%) received additional treatment. TBUT improved from 3.17 ± 0.82 s to 5.28 ± 1.10 s after surgery (P < 0.001). The total bulbar conjunctival hyperemia value showed an improvement from 1.7 ± 0.6 to 1.4 ± 0.6 postoperatively (P < 0.05). No serious complications were observed. CONCLUSION: The novel ophthalmic radiofrequency device led to a marked improvement of Cch with no serious adverse events during the entire follow-up period. Our results suggest that the radiofrequency device presents a safe and efficacious treatment option for Cch.

Incidence and mechanism of conjunctival chemosis during phacoemulsification.

BACKGROUND: This study aims to determine the incidence of conjunctival chemosis during phacoemulsification and to explore the possible mechanism and treatments. METHODS: Patients with or without chemosis during phacoemulsification by the same operator within two years were included. The initial site of chemosis, the chemosis originated time, and the degree of chemosis at the end of the surgery were recorded. The differences in phacoemulsification energy/time and irrigation volume were compared between two groups. Meanwhile, the distance between the clear corneal incision (CCI) and the end of the Bowman's layer, surgically induced astigmatism (SIA), intraocular pressure and central corneal thickness were also compared between the two groups. RESULTS: The incidence of intraoperative chemosis was 9.17% (66/720). The distance between CCI and the end of the Bowman's layer in the chemosis group was significantly longer than that in the non-chemosis group postoperatively (P < 0.0001). The initial sites of chemosis were located on both sides of the external opening of the CCI. The average time from the phacoemulsification probe introducing into the anterior chamber to the originating of chemosis was 30.23 ± 49.23s. CONCLUSION: Intraoperative chemosis is related to the distance from CCI to the end of Bowmen's layer. The residual conjunctiva around the incision wraps the phacoemulsification probe sleeve to form a passage, the leakage fluid enters the subconjunctiva through this passage, forming chemosis. Conjunctival incision on both sides of CCI can effectively prevent the development of chemosis.

A case report of pituitary neuroendocrine tumor manifesting as severe conjunctival chemosis.

BACKGROUND: Conjunctival chemosis (CC) is an extremely rare symptom of pituitary neuroendocrine tumor (PitNET). We report an extremely rare case of PitNET manifesting as severe CC. CASE PRESENTATION: A 48-year-old male was admitted to our hospital with severe CC, proptosis, and ptosis of the right eye. Magnetic resonance imaging demonstrated the tumor mass invading the cavernous sinus (CS) with cystic lesion. The patient underwent emergent endoscopic transsphenoidal surgery, and the pathological diagnosis was PitNET. CC of the right eye remarkably improved after the surgery. Glucocorticoid therapy was performed for right oculomotor nerve palsy, which rapidly improved. The postoperative course was uneventful and the patient was discharged from our hospital without hormone replacement. CONCLUSIONS: CC caused by CS invasion of PitNET can be cured by early surgical treatment. Therefore, PitNET is important to consider in the differential diagnosis of CC.

Cushing's syndrome of the orbit: congestive orbitopathy and optic neuropathy associated with steroids.

A 56-year-old female with a history of chronic systemic steroid use for asthma control displayed orbital congestion, exophthalmos, a mild abduction deficit, and optic neuropathy. Laboratory workup was unrevealing. Neuroimaging showed increased orbital fat compartments, though the orbital fat was unremarkable on biopsy. The patient was diagnosed with iatrogenic Cushing's syndrome of the orbit and underwent orbital decompression. Early published literature declared this orbitopathy benign. However, newer cases describe more pathologic changes, suggesting the disease is diagnosed later and/or treatment is delayed.

Severe Chemosis: Initial Misdiagnosis of an Emergent Complication of Acute Rhinosinusitis.

BACKGROUND: Orbital findings such as cellulitis, ophthalmoplegia, and proptosis are possible complications of sinusitis. Acute sinusitis can also cause a conjunctival cyst-like lesion induced by severe chemosis; however, it is an uncommon complication. CASE REPORT: We report the case of a 53-year-old man who presented to our facility's emergency department. The patient complained of orbital discomfort resulting from an inability to close both eyes, and orbital discharge, accompanied by general weakness. On physical examination, cyst-like-appearing lesions with erythema of the eyelids were identified bilaterally and initially misdiagnosed as inflammatory lesions of the orbital area. However, orbital pathology was excluded by the ophthalmologist, and it was found that the patient had bilateral pansinusitis on the imaging studies. It was suspected that the sinusitis was responsible for the ocular symptoms. The patient was treated with emergent endoscopic sinus surgery, and the symptoms were promptly relieved. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Although it is well known that orbital complications of sinusitis can occur, a conjunctival cyst-like-appearing lesion induced by severe chemosis is uncommon and is difficult to diagnose at first sight. Emergent surgical drainage is needed to prevent permanent orbital damage and other sinusitis-related complications. Therefore, recognizing the unique clinical presentation of a conjunctival cyst-like lesion resulting from severe chemosis is the key to appropriate diagnosis and management.

Ophthalmic manifestations of COVID-19; a less-appreciated yet significant challenge.

AIM: The current world has changed in all shapes since the emergence of the novel coronavirus (nCoV-2) also known as COVID-19. Among the extra-pulmonary manifestations of nCoV-2, ophthalmic symptoms have less been systematically studied. The so far existing body of evidence indicates that nCoV-2 has the potential to affect both anterior and posterior chambers of the eye. Albeit, the exact mechanisms which underlie ophthalmic manifestations of nCoV-2 are yet to be elucidated. METHODS: The present brief review is an attempt to put together and highlight the significant yet limited number of studies which have spotlighted ophthalmic issues in nCoV-2 patients using a systematic literature search strategy. RESULTS: All case series or reports (including both published and preprint articles) which described ocular manifestations of patients with COVID-19 and/or documented testing of SARS-COV-2 in ocular secretions via various sampling or detection methods were sought to be included. CONCLUSION: The ophthalmic presentations in SARS-COV-2 are often found to be salient. Raising awareness in this respect may help defining evidencebased protective measures in today's practice of ophthalmology and allied disciplines.

Temporary suture tarsorrhaphy at the time of orbital ball implantation.

PURPOSE: Conjunctival chemosis is a common, usually benign, complication of ocular and oculoplastic surgery. After enucleation, evisceration, or secondary ball implant, however, conjunctival edema can cause conformer extrusion, forniceal eversion, and shortening which may complicate the future fitting of a prosthesis. This longitudinal chart review aimed to estimate the efficacy of a temporary suture tarsorrhaphy to minimize postoperative edema and improve conformer retention. METHODS: We retrospectively reviewed the charts of 415 consecutive patients undergoing enucleation, evisceration, and secondary ball implantation between 1990 and 2016. Three-hundred twenty-eight patients had complete data which was analyzed for age, gender, surgical details, complications, and follow-up. RESULTS: We identified 328 eyes that underwent enucleation (48%), evisceration (3%), or secondary implantation (49%) during the study period. Eighty-nine patients had a suture tarsorrhaphy at the time of surgery, and 239 did not. There was a statistically significant incidence of postoperative conjunctival edema in the no tarsorrhaphy group. There was also a significantly increased rate of conformer loss associated with conjunctival edema. Need for further surgery was not uncommon with 64% and 43% of patients requiring a secondary procedure in the no tarsorrhaphy and tarsorrhaphy groups respectively. CONCLUSIONS: Temporary suture tarsorrhaphy is a simple and effective procedure that reduces conjunctival edema and increases conformer stability when performed at the time of enucleation, evisceration, or secondary ball implantation.

A red eye induced by a spontaneous carotid cavernous fistula.

A 73-year-old woman initially presented to our ophthalmology department with complaint of a red eye with gradual proptosis and mild restricted movement of the right eye who was misdiagnosed with conjunctivitis during 2 months. One week later she returned to the emergency department with a painful, swollen right eye and a high intraocular pressure of 41 mm Hg and limitation of her extraocular muscles in nearly all fields of gaze. As her IOP was high, she was diagnosed as having secondary glaucoma and was prescribed timolol 0.5% (twice a day) and Latanoprost eye drop at night in the right eye. During follow-up, her IOP remained in high level despite maximum medications. Computed tomography scan of the orbit showed an enlarged superior ophthalmic vein (SOV). In view of her persistent elevated IOP despite maximum medications and dilated conjunctival veins, she was advised digital subtraction angiography (DSA) and imaging revealed a carotid cavernous fistula (CCF). The purpose of this article is to describe ophthalmological complaints of CCFs to help ophthalmologist in understanding the clinical features of spontaneous CCF and avoid misdiagnosis.

Characteristics of orbital lymphoma: a clinicopathological study of 26 cases.

OBJECTIVE: Evaluation of the medical data of patients with orbital and adnexal lymphoma. DESIGN: Cohort study of all cases diagnosed with orbital or adnexal lymphoma at Meir Medical Center between 1993 and 2007. PARTICIPANTS: Twenty-six patients, with intraorbital or subconjunctival masses with orbital involvement, were examined and followed up between 1 and 8 years. MATERIALS AND METHODS: Examined data included: clinical presentation, age, gender, imaging, tumor location, surgical management, and pathological diagnosis. RESULTS: Presenting signs and symptoms included proptosis, eyelid lesions, tearing, chemosis, decreased visual acuity, ptosis, pain, squint, and optic nerve compression. In five cases, lymphoma was misdiagnosed on neuroimaging. Bone changes were seen in four patients. All cases were B cell lymphomas; with the majority (22 cases) of small B cell type; consisting of primary extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue and two cases of small cell lymphoma. One small cell lymphomas was of follicular type on a background of CLL, and the other was CLL/SLL type. Fourteen cases were primary orbital disease, and 12 cases were systemic disease. Macroscopic appearance of lymphoma at open biopsy was characteristic in most cases. Flow cytometry phenotyping gave rapid reliable diagnosis of the disease. CONCLUSIONS: Epiphora or chemosis in the presence of an orbital mass should alert the ophthalmologist to suspect lymphoma. Lymphoma may be easily misinterpreted on neuroimaging for other diseases. Bone changes seen on CT are more common than is generally perceived. Macroscopic appearance at open biopsy was characteristic.

[Aging prevention, new surgical techniques and future options for facial rejuvenation]. / Prévention du vieillissement, techniques chirurgicales récentes et voies d'avenir de la chirurgie du rajeunissement facial.

Prevention of aging is mainly obtained through appropriate health practices, modulated by the genetic causes of aging. Causes of facial structural aging include gravity, volume loss, contraction of the mimic muscles and obviously biological aging of tissues. Among the very numerous new surgical technique of facial rejuvenation, we could point out: for the frontal region, obviously we are focus on the endoscopic and non-endoscopic frontal lifts. But also, we should note the transcutaneous Brow Shaping which with a well defined, step by step technique is indeed a simple way to improve a possible difficult situation; concerning the mid-face lift, as the main risk is the secondary eyelid malposition, a high Smas or a temporo-malar lift can be performed. It ensures a good temporal effect but there is nearly no improvement at the mid-pupilla level. A mid-face lift is nevertheless mandatory to achieve an efficient skin removal on the mid-pupilla line. This is performed most of the time with a complete orbicularis oculi muscle opening, and only with a small lateral opening in case of the concentric malar lift, which minimize the risk of eyelid malposition. Based on a new description of the lymphatic draining of the lower lid, a new treatment of the chemosis is proposed with a corticoid injection at infero-lateral part of the malar area, in the preperiosteal plane. For the oval, the DAO section and the suspension of the middle premasseter space beyond the anterior border of the masseter where the Smas overlies the buccal fat pad, stay efficients. Regarding the neck, the suspension of the posterior border of the platysma to the Loré's fascia, the digastric corset with or without a platysma corset are advanced and valuable techniques. Lately, a new option was described using a lateral skin-platysma flap to minimize platysma bands and even more recently a neck lift with fixation of the anterior platysma to the deep cervical fascia and suspension of the lateral platysma flap.

Persistent Conjunctival Chemosis after Lower Lid Blepharoplasty: A Comparison of Different Surgical Techniques.

Background: Conjunctival chemosis, a complication of lower blepharoplasty, can cause persistent discomfort and functional disturbances with worsening in the postoperative period following surgery. Methods: A review of the records of the lower blepharoplasty procedures carried out at the Humanitas Research Hospital, Rozzano, Milan, Italy was performed. Patients were categorized into two groups depending on the procedure performed: (1) transconjunctival blepharoplasty with the removal of the fatty lodges with canthopexy and (2) transcutaneous blepharoplasty with the removal of the fatty lodges with lateral canthoplasty. Each group was further divided into two more groups based on the surgical method used, that is either (a) cold blade and disposable cautery or (b) radiofrequency cut and coagulation and colorado tip (respectively 1a, 1b, 2a and 2b). All patients underwent a postoperative follow-up up to 24 months, which included an evaluation of cosmetic appearance, eyelid scarring and the severity of chemosis. The aim of the study was to investigate which of the surgical procedures causes a lower incidence of persistent type 3 conjunctival chemosis. Results: A total of 1047 patients who underwent lower lid blepharoplasty were included in the study. A total of 512 patients underwent transcutaneous blepharoplasty and 535 underwent the transconjunctival procedure. Among the first group of patients, 266 belong to group 1a and 246 to group 1b. In the second group, 264 were categorized as group 2a and 271 as group 2b. The incidence of type 3 chemosis in the transcutaneous blepharoplasty procedure with lateral canthoplasty was statistically significantly higher than in the transconjunctival approach, considering both the cold blade and the radiofrequency (p = 0.012, 0.010, 0.006, 0.004, respectively). Conclusions: A higher incidence of persistent type 3 conjunctival chemosis is associated with lateral canthus surgery and with the use of radiofrequency.

Snip conjunctivoplasty associated with methylene blue staining for treatment of postoperative conjunctival chemosis.

Conjunctival chemosis usually undergoes spontaneous resolution; sometimes, it requires treatment. We present the case of a 43 years-old female patient who developed bilateral conjunctival chemosis following upper and lower blepharoplasty. Two months after the operation, patient underwent bilateral snip conjunctivoplasty with methylene blue demarcation of the chemotic conjunctiva.

Resolving conjunctival prolapse following severe blepharoptosis correction: A simple sutureless direct excision technique and systematic review.

BACKGROUND: Conjunctival prolapse (CP) is an uncommon but challenging condition following maximal levator resection (MLR) and other extensive periorbital procedures. MLR extending beyond the Whitnall's ligament is frequently performed to address severe blepharoptosis with poor levator function (LF). Patients with CP may encounter symptoms such as ocular discomfort, tearing, vision impairment, persistent conjunctival chemosis, lagophthalmos, or exposure keratopathy. Typically, surgical intervention becomes necessary if conservative measures prove to be ineffective; nevertheless, there is no consensus regarding the optimal treatment approach. OBJECTIVES: This study aimed to propose a simple sutureless direct excision method and explore the surgical advancements in CP correction through a systematic review. METHODS: Patients with recurrent CP after MLR who underwent sutureless direct excision of the conjunctiva using loupe magnifiers at a tertiary hospital were included in this study. The clinical evolution and surgical results were recorded. PubMed, MEDLINE, EMBASE, and Web of Science databases were queried following The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) protocol. RESULTS: The comprehensive analysis of 1858 articles identified 88 patients from 24 studies, highlighting that blepharoptosis surgery is predominantly associated with CP (36.6%). Surgically treated CP showed a higher resolution rate compared to those managed conservatively (54.8% vs. 45.2%; p = 0.034). No relapse was observed in patients treated with sutureless direct excision of CP in long-term follow-up. CONCLUSION: We proposed a simple sutureless direct excision technique that offers a straightforward and efficient approach in treating CP, which is particularly suitable for cases requiring excision lengths >16 mm during MLR. Furthermore, stitch removal can be obviated after surgery.

Study of safety and efficacy of sub-Tenon and subconjunctival anesthesia in manual small-incision cataract surgery for hard-grade cataracts.

PURPOSE: The purpose was to study the safety and efficacy of sub-Tenon and subconjunctival anesthesia in manual small-incision cataract surgery (MSICS) in patients with hard-grade cataracts. STUDY DESIGN: The design of the study was a prospective, observational, and randomized control study. MATERIALS AND METHODS: A total of 196 eyes, including 98 eyes in the subconjunctival anesthesia group (group A) and 98 eyes in the sub-Tenon anesthesia group (Group B), undergoing MSICS were enrolled in the study. A single surgeon performed all the surgeries. Intraoperative and postoperative pain scores, patient comfort, surgeon's satisfaction, and intraoperative complications were examined. RESULTS: The mean age of patients in Group A was 66.64 ± 9.95 years and that of patients in Group B was 64.52 ± 9.46. No statistically significant difference was noted in the intraoperative (P = 0.54) and postoperative pain (P = 0.66) scores between the two groups. There was no pain (0 score) in 30% of patients in Group A and 35% of patients in Group B intraoperatively. The average surgical time (P = 0.66) and surgeon's comfort (P = 0.34) were not statistically significant. The mean corneal haze was 0.054 ± 0.12 in group A and 0.065 ± 0.22 in group B (P = 0.45). Two patients in group A required supplemental anesthesia. There were no surgical complications that could compromise visual outcomes. No patients in either group showed alterations in vital parameters or required intravenous sedation. CONCLUSION: Both techniques of anesthesia are safe and effective for performing MSICS in hard-grade cataracts. However, it is prudent to choose a technique according to the surgeon's requirements.

Management of postblepharoplasty chemosis.

Clinically significant chemosis occasionally complicates lower eyelid blepharoplasty. In this report, the etiologic components of postblepharoplasty chemosis are discussed. The time course and duration of chemosis vary according to the underlying cause. Early, late, and prolonged chemosis are managed with different strategies. Diagnostic and therapeutic algorithms for chemosis assessment and management are presented.

Clinical efficacy of endovascular treatment approach in patients with carotid cavernous fistula: A systematic review and meta-analysis.

Background and objectives: Carotid-cavernous fistulas (CCFs) represent a group of rare, abnormal arteriovenous communications between the carotid arterial system and the cavernous sinuses (CS). CCFs often produce ophthalmologic symptoms related to increased CS pressures and retrograde venous drainage of the eye. Although endovascular occlusion remains the preferred treatment for symptomatic or high-risk CCFs, most of the data for these lesions is limited to small, single-center series. As such, we performed a systematic review and meta-analysis evaluating endovascular occlusions of CCFs to determine any differences in clinical outcomes based on presentation, fistula type, and treatment paradigm. Method: A retrospective review of all studies discussing the endovascular treatment of CCFs published through March 2023 was conducted using PubMed, Scopus, Web of Science, and Embase databases. A total of 36 studies were included in the meta-analysis. Data from the selected articles were extracted and analyzed using Stata software version 14. Results: 1494 patients were included. 55.08% were female and the mean age of the cohort was 48.10 years. A total number of 1516 fistulas underwent endovascular treatment, 48.05% of which were direct and 51.95% of which were indirect. 87.17% of CCFs were secondary to a known trauma while 10.18% were spontaneous. The most common presenting symptoms were 89% exophthalmos (95% CI: 78.0-100.0; I2 = 75.7%), 84% chemosis (95% CI: 79.0-88.0; I2 = 91.6%), 79% proptosis (95% CI: 72.0-86.0; I2 = 91.8%), 75.0% bruits (95% CI: 67.0-82.0; I2 = 90.7%), 56% diplopia (95% CI: 42.0-71.0; I2 = 92.3%), 49% cranial nerve palsy (95% CI: 32.0-66.0; I2 = 95.1%), 39% visual decline (95% CI: 32.0-45.0; I2 = 71.4%), 32% tinnitus (95% CI: 6.0-58.0; I2 = 96.7%), 29% elevated intraocular pain (95% CI: 22.0-36.0; I2 = 0.0%), 31% orbital or pre-orbital pain (95% CI: 14.0-48.0; I2 = 89.9%) and 24% headache (95% CI: 13.0-34.0; I2 = 74.98%). Coils, balloons, and stents were the three most used embolization methods respectively. Immediate complete occlusion of the fistula was seen in 68% of cases and complete remission was seen in 82%. Recurrence of CCF occurred in only 35% of the patients. Cranial nerve paralysis after treatment was observed in 7% of the cases. Conclusions: Exophthalmos, Chemosis, proptosis, bruits, cranial nerve palsy, diplopia, orbital and periorbital pain, tinnitus, elevated intraocular pressure, visual decline and headache are the most common clinical manifestations of CCFs. The majority of endovascular treatments involved coiling, balloons and onyx and a high percentage of CCF patients experienced complete remission with the improvement of their clinical symptoms.

Bilateral Large Orbital Lymphoma With Proptosis.

Diffuse large B-cell lymphoma (DLBCL) is the most common lymphoid malignancy in adults. It is an aggressive malignancy and requires a multidisciplinary approach with various modalities which include chemotherapy, radiotherapy as well as immunotherapy. A 63-year-old Malay male patient with underlying type 2 diabetes mellitus, hypertension, ischemic heart disease, and stage II chronic kidney disease presented with a one-month history of bilateral eye proptosis associated with lid swelling and red eye. He also complained of progressive right eye blurring of vision. Visual acuity was counting fingers on the right and 6/18 on the left. On examination, the relative afferent pupillary defect was negative. There was bilateral eye proptosis, conjunctival chemosis, and restricted extra-ocular movement in all gazes. There was also exposure keratopathy over the right eye, and intraocular pressure was raised. Bilateral cervical and axillary lymph nodes were palpable. A computerized tomography scan of the brain and orbit revealed bilateral orbital masses with no bony erosions. An incisional biopsy over the upper lid confirmed the diagnosis of diffuse large B-cell lymphoma with multiple myeloma-1 (MUM-1) positivity which defines the activated B-cell subtype (ABC). He was co-managed with a hematologist and was commenced on the rituximab-cyclophosphamide, doxorubicin, vincristine, prednisone (R-CHOP) chemotherapy regime. Bilateral eye proptosis, chemosis, and restriction of extra-ocular movement resolved after the completion of treatment. However, right eye vision remains poor as the patient developed central self-sealed corneal perforation with iris plugging which has healed with scarring. Diffuse large B-cell orbital lymphoma is a fast-growing and aggressive tumor, hence early diagnosis and prompt multi-disciplinary treatment are crucial for a good outcome.

Non-medial Subperiosteal Abscess in a 63-Year-Old Female: A Case Report and Literature Review.

We present a rare case of non-medial subperiosteal abscess secondary to orbital cellulitis in a 63-year-old female. The patient reported a five-day history of progressive swelling, pain, and diminished vision in the left eye. Computed tomography (CT) of the orbit revealed an extraconal soft tissue density, suggestive of an orbital collection, which when correlated clinically and radiologically, was diagnosed as orbital cellulitis secondary to sinusitis, leading to a subperiosteal abscess. Despite undergoing multiple external drainage procedures, the patient, unfortunately, experienced complete vision loss in the affected eye. While non-medial abscesses due to orbital cellulitis are infrequent, they are often associated with more severe outcomes, including vision loss and intracranial complications, compared to those in medial locations. This case underscores the importance of combined surgical approaches, including both sinus and external drainage, to prevent severe vision loss and potentially life-threatening intracranial sequelae.

Carotid-Cavernous Fistula in a Patient With Unilateral Periorbital Swelling and Proptosis Initially Attributed to Possible Immunoglobulin G4-Related (IgG4-Related) Disease.

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.