Chorio-Retinal Folds Associated With Orbital Cavernous Venous Malformations.

PURPOSE: To determine the incidence of clinically detectable chorio-retinal folds (CRFs) with orbital cavernous venous malformations (OCVMs) and their recovery. METHODS: Retrospective case-note and imaging review, with estimation of odds ratios in relation to the position and volume of the mass. RESULTS: Records for 402 patients were reviewed, 83 (21%) having CRFs. The mean logarithm of minimum angle of resolution acuity was similar with or without CRFs (0.37 and 0.31, respectively; p = 0.46), but induced hyperopia was commoner with CRFs (76% vs. 12%; p < 0.001), exophthalmos greater (4.52 mm vs. 2.97 mm; p < 0.001), eye movement restriction commoner (37% vs. 21%; p = 0.004), and disc swelling more prevalent (42%, vs. 17%; p < 0.001). Orbital cavernous venous malformations with CRFs were almost all intraconal (98%; odds ratio 9.96; p = 0.002), and 93% (77/83) midorbital (odds ratio 6.02; p < 0.001). The median size with CRFs was twice that of those without (3.85 ml vs. 1.92 ml; p < 0.001), and two-thirds OCVMs with folds had volumes >2.5 ml ( p < 0.001). The OCVM was excised in 76 of 83 (92%) of CRF group and 213 of 319 (67%) of those without ( p < 0.001). The postoperative acuity was improved or unchanged in 67 of 76 (88%) eyes with folds, and 184 of 213 (83%) without CRFs ( p = 0.84). The proportion recovering an acuity within 1 Snellen line (or better than) the unaffected side was 80% with CRFs and 77% in their absence ( p = 0.63). Induced hyperopia persisted in 39% of all patients, with the mean being higher with CRFs (2.22D vs. 1.02D; p = 0.017). CONCLUSIONS: CRFs occur in ~25% of mid-intraconal OCVMs. Despite OCVM excision, 39% of operated patients retain significant residual hyperopia (54% if CRFs present before surgery), and 41% of such CRFs remain clinically detectable after surgery (with variable visual impairment). Earlier surgery might, therefore, be advisable in patients with CRFs and/or induced hyperopia.

Kimura Disease of the Periorbita and Earlobe in a 15-Year-Old Male: A Case Report and Review of the Literature.

PURPOSE: The authors describe a case of Kimura disease (KD) affecting the lacrimal gland, the ipsilateral periorbita, and the contralateral earlobe. The authors also review the literature for characteristics and outcomes of KD affecting the periorbita or earlobe. METHODS: The authors review retrospectively a single chart and abstract data from the English language literature. RESULTS: A 15-year-old Samoan male presented with a left upper eyelid mass and later the contralateral earlobe and ipsilateral periorbita. Surgical resection was partially effective and normal vision and eye motility were maintained. Histopathology was consistent with KD. Thirty-seven cases of periorbital KD were identified in the literature. The average age of patients with periorbital KD was 31.9 years (standard deviation: 17.8 years), with 81.1% (30/37) of patients being male and 69.4% (25/36) Asian. Over half of periorbital KD patients had lacrimal gland involvement (51.3%; 19/37). Fifteen patients were initially treated with surgery; 3 (20%) had recurrence of the disease. Another 15 patients were initially treated with corticosteroids; 12 (80%) had recurrence of the disease. Ophthalmologic data, when reported, demonstrated that, most patients had no visual (77.8%; 14/18 patients) or motility disturbances (82.4%; 14/17 patients) but most had proptosis 71.4% (10/14). Only 4 cases of earlobe involvement in KD were identified, all in women. CONCLUSIONS: In the literature, orbital KD was rare but often affected the lacrimal gland and caused proptosis, commonly in young adult Asian males. Vision and extraocular motility were usually normal. Earlobe involvement was very rare. This unique case of KD that affected the orbit and contralateral earlobe corroborates the prior literature that KD is prone to recurrence but may not adversely affect vision or extraocular motility.

Giant Lacrimal Gland Cyst in a Pediatric Patient: Case Report and Review of the Literature.

Lacrimal gland cysts are rare clinical entities in the pediatric population. Herein is described a 6-year-old male patient presenting with progressive left upper lid ptosis, found to have a large ipsilateral superotemporal orbital mass. Diagnosis of a giant lacrimal gland cyst was confirmed excisional biopsy. Despite the resolution of mechanical blepharoptosis, the patient maintained visual acuity limitation due to suspected deprivation amblyopia. The pathophysiology and clinical manifestations of lacrimal gland cysts in the pediatric population are reviewed to emphasize the importance of expedited identification and management in this patient cohort.

Endoscopic-assisted Orbitotomy for Obliteration of Orbital Arteriovenous Fistula Refractory to Endovascular Techniques.

Orbital arteriovenous fistula is a rare acquired disorder. The coincidence of arteriovenous fistula with lymphaticovenous malformation is even rarer. The optimal treatment, thus, is controversial. Surgical approaches vary widely, with associated pros and cons. The purpose of this case report is to describe an orbital arteriovenous fistula in a 25-year-old man with congenital fronto-orbital lymphaticovenous malformation, refractory to endovascular techniques, which was later successfully ablated by a direct endoscopic-assisted orbital approach.

The Isabel Differential Diagnosis Generator for Orbital Diagnosis.

PURPOSE: The Isabel differential diagnosis generator is one of the most widely known electronic diagnosis decision support tools. The authors prospectively evaluated the utility of Isabel for orbital disease differential diagnosis. METHODS: The terms "proptosis," "lid retraction," "orbit inflammation," "orbit tumour," "orbit tumor, infiltrative" and "orbital tumor, well-circumscribed" were separately input into Isabel and the results were tabulated. Then the clinical details (patient age, gender, signs, symptoms, and imaging findings) of 25 orbital cases from a textbook of orbital surgery were entered into Isabel. The top 10 differential diagnoses generated by Isabel were compared with the correct diagnosis. RESULTS: Isabel identified hyperthyroidism and Graves ophthalmopathy as the leading causes of lid retraction, but many common causes of proptosis and orbital tumors were not correctly elucidated. Of the textbook cases, Isabel correctly identified 4/25 (16%) of orbital cases as one of its top 10 differential diagnoses, and the median rank of the correct diagnosis was 6/10. Thirty-two percent of the output diagnoses were unlikely to cause orbital disease. CONCLUSION: Isabel is currently of limited value in the mainstream orbital differential diagnosis. The incorporation of anatomic localizations and imaging findings may help increase the accuracy of orbital diagnosis.

Coloboma Accompanying Microphthalmos With Orbital Cyst in a Mother and Child.

Microphtalmos with orbital cyst is a rare congenital abnormality of the eye and orbit that is caused by incomplete closure of the embryonic fissure. The cysts project through in a coloboma of the affected eye. It may be sporadic or genetic. Herein, the authors present a 32-year-old mother with unilateral and her 4-month-old daughter with bilateral microphtalmos and accompanying orbital cyst.

Erdheim-Chester disease and vemurafenib: a review of ophthalmic presentations and clinical outcomes.

PURPOSE: To provide a comprehensive review of ocular and orbital manifestations of Erdheim-Chester Disease (ECD) and compare clinical outcomes with vemurafenib (INN) to historical treatments (HT). Primary outcomes are ophthalmic findings on presentation, changes in visual acuity, and mortality rate. Secondary outcomes include the progression of ocular findings, systemic involvements, and treatment modalities. METHODS: All published literature from January 1983 to March 2021 was searched for ophthalmic manifestations of ECD. Clinical outcomes following HT were collected and compared with INN. RESULTS: Forty-seven patients with ECD and ophthalmic presentations were identified. The mean age was 49.6 years (SD = 15.0). Proptosis (65.6%) and extraocular muscle restrictions (42.5%) were the most common presenting signs. Of 41 (87.2%) patients with orbital masses on radiologic examination, 90.2% were bilateral, and 53.7% were located in the intraconal space. Ophthalmic examination was significant for xanthelasma (27.2%), optic disc edema (34.0%), and subretinal changes (21.3%). Common treatments were systemic steroids (76.6%), interferon-α (17.0%), and cyclophosphamide (14.9%). INN was less commonly used (12.8%). The mean change in logMAR visual acuity declined with HT (29.9%) but improved with INN (79.1%) (p > 0.05). The proportion of eyes with complete vision loss increased after HT (p < 0.05). The overall mortality rate was 27.7% and notably higher in the HT group (29.3%) when compared to the INN group (16.7%) (p > 0.05). CONCLUSION: ECD presents with many ophthalmic manifestations. Although the intraocular treatments remain controversial, INN should be highly considered in treating orbital ECD patients with BRAF-V600E mutations to prevent and reverse vision loss.

[COVID-19 associated sino-orbital mucormycosis]. / COVID-19-assotsiirovannyi sinoorbital'nyi mukormikoz.

OBJECTIVE: To analyze the dynamics of symptoms in patients with COVID-19 associated sino-orbital mucormycosis. MATERIAL AND METHODS: We describe a series of 13 patients with COVID-19 associated sino-orbital mucormycosis aged 43 to 80 years diagnosed from August to October 2021. All of the patients had a severe disease and required noninvasive ventilation or intubation and administration of dexamethasone. 12 out of 13 patients (92.3%) suffered from diabetes mellitus. Symptoms of fungal infections of the nose and paranasal sinuses appeared in the interval from 7 to 25 days of hospital stay, most often in the second week (from 8 to 12 days). According to clinical and CT features the patients were divided into three groups, combining similar phenotypes of the disease. Group 1 - 1 patient with sinonasal mucormycosis, destruction of the alveolar ridge and the hard palate. Group 2 - 12 patients with sino-orbital mucormycosis. We noted, that in cases of bilateral sinus lesions orbital complications were unilateral in all patients, on the side of more severe lesion. Group 2 was divided into 2 subgroups: subgroup 2a included 2 patients with the superior orbital fissure syndrome: ptosis, proptosis, ophthalmoplegia, periorbital pain, pain or hypoesthesia of half face; subgroup 2b included 10 patients with the orbital apex syndrome, who, in addition to the above symptoms, had loss of vision and conjunctival chemosis. Group 3 - rhino-sino-cerebral mucormycosis. 2 patients from subgroup 2b were transferred to this group due to the intracranial spread of the process and focal neurological symptoms. CONCLUSION: Clinical forms of mucormycosis reflect successive stages of invasive spread of fungi.

Rhino-orbital mucormycosis in a patient with no susceptibility following P.vivax malaria infection-a case report.

BACKGROUND: Mucormycosis is a potentially lethal, angioinvasive fungal infection caused by the Mucoracea family comprising Mucor, Rhizopus, and Absidia species. It is commonly associated with uncontrolled diabetes mellitus, the use of corticosteroids, immunosuppressive drugs, and Covid-19 infection. The occurrence of mucormycosis in an immunocompetent patient is rare. Also, only a few case reports have been published where patients developed mucormycosis with associated malarial infection. CASE PRESENTATION: A young female presented with a 3-weeks history of painful swelling and outward protrusion of the right eye with complete loss of vision. She had a history of P.vivax malaria two weeks before her ocular symptoms. On ocular examination, there was proptosis and total ophthalmoplegia with loss of corneal sensations in the right eye. Hematological examination revealed normocytic normochromic anemia and thrombocytopenia. MRI was suggestive of right-sided pansinusitis and orbital cellulitis with right superior ophthalmic vein thrombosis and bulky cavernous sinus. Nasal biopsy was negative for fungal culture. An emergency surgical debridement of all the sinuses was done with right orbital exenteration. Histopathology confirmed the diagnosis of mucormycosis and the patient improved post-operatively on systemic antifungals. CONCLUSION: Such an association of mucormycosis with malaria infection is rarely reported in the literature and is hypothesized to be a result of immunosuppression caused by malaria. Also, emphasis is laid upon having a high index of suspicion for fungal infection in the setting of pansinusitis even if the risk factors are absent. We hereby report a case of rhino-orbital mucormycosis following P.vivax malaria in a 20-year-old female with anemia and thrombocytopenia.

Imaging features of rhinocerebral mucormycosis: from onset to vascular complications.

Rhinocerebral mucormycosis (RCM) may result in severe intracranial ischemic and hemorrhagic lesions. Both computed tomography (CT) and magnetic resonance imaging (MRI) play an essential role in the diagnosis of RCM, but whereas CT is better for assessing bone erosion, MRI is superior in evaluating soft tissue, intraorbital extension, and in assessing intracranial and vascular invasion. Specific CT and MRI techniques, such as CT angiography or enhanced MR angiography, and more advanced MRI sequences such as gadolinium-3D Black Blood imaging, contribute to the assessment of the extension of vascular invasion.In this pictorial review, we describe specific CT and MRI signs of RCM, mainly focusing on its life-threatening complications due to vascular involvement.

Blue Rubber Bleb Nevus Syndrome Manifesting as an Isolated Congenital Orbital Mass in a Neonate.

A male neonate presented with an isolated congenital right orbital vascular malformation without other mucocutaneous lesions or signs/symptoms of systemic disease. The orbital mass was progressive, causing amblyogenic ptosis by 6 months of age. Over 11 years, the patient underwent 4 orbital mass resections, 3 embolizations, and even a craniotomy with mass resection for an intraorbital meningoencephalocele secondary to orbital bony erosion. A diagnosis of blue rubber bleb nevus syndrome was made at age 7 when the patient developed a tender vascular lesion on his foot and was found to have other mucocutaneous lesions of the extremities and gastrointestinal tract. This is the first pediatric case of such an aggressive orbital vascular malformation from blue rubber bleb nevus syndrome causing neonatal amblyogenic ptosis and intraorbital meningoencephalocele in childhood. It is the second report of a patient presenting with an isolated orbital vascular malformation without other manifestations of blue rubber bleb nevus syndrome, leading to his delayed diagnosis.

Orbital Cyst with Ependymal Differentiation Associated with Microphthalmia.

BackgroundOrbital cysts associated with microphthalmia are colobomatous lesions that typically present unilaterally and posterior to the globe. Case Report: A male infant had an orbital cyst associated with microphthalmia located anterior to the globe composed of a neuroglial wall, ependymal-like epithelial lining, with synaptophysin-positive cells resembling the retinal neuronal layer. Conclusion: This orbital cyst may represent a malformation of the eye rather than an encephalocele.

Increased incidence of rhino-orbital mucormycosis in an educational therapeutic hospital during the COVID-19 pandemic in western Iran: An observational study.

BACKGROUND: COVID-19 patients, especially the patients requiring hospitalisation, have a high risk of several complications such as opportunistic bacterial and fungal infections. Mucormycosis is a rare and opportunistic fungal infection that mainly affects diabetic and immunocompromised patients. An increase has been observed in the number of rhino-orbital mucormycosis in patients with COVID-19 admitted to Imam Khomeini Hospital, Kermanshah, Iran, since October 2020. This is a report of the frequency, risk factors, clinical manifestations, treatment and prognosis of COVID-19 associated with mucormycosis infection. METHODS: The medical records of COVID-19 patients with rhino-orbital mucormycosis who were diagnosed in an educational therapeutic hospital in Kermanshah, west of Iran were surveyed. Several parameters were analysed including demographic, clinical, therapeutic and laboratory characteristics. RESULTS: Twelve patients with COVID-19-associated rhino-orbital mucormycosis were identified from 12 October to 18 November 2020. All cases reported as proven mucormycosis had a history of hospitalisation due to COVID-19. Comorbidities mainly included diabetes mellitus (83.33%) and hypertension (58.33%). Seventy-five per cent of patients received corticosteroids for COVID- 19 treatment. The sites of involvement were rhino-sino-orbital (83%) and rhino-sino (17%). Amphotericin B/liposomal amphotericin B alone or in combination with surgical debridement or orbital exenteration was used as the first-line therapy. The overall mortality rate was 66.7% (8/12). CONCLUSIONS: We found a high incidence of mucormycosis among COVID-19 patients. Diabetes mellitus and corticosteroid use were the dominant predisposing factor of mucormycosis. Mucormycosis is a life-threatening and opportunistic infection; therefore, physicians should know the signs and symptoms of the disease so that a timely diagnosis and therapy can be performed.

Retinal and Optic Nerve Deformations Due to Orbital Versus Intracranial Venous Hypertension.

INTRODUCTION: Abnormal forces around the optic nerve head (ONH) due to orbital diseases, intracranial hypertension (IH), glaucoma, and space travel, are associated with alterations of the ONH shape. Elevated cerebral and ophthalmic venous pressure can contribute to stress and strain on the ONH and peripapillary retina. We hypothesize that IH and elevated ophthalmic venous pressure without IH cause different ONH and retinal changes. METHODS: We compared MRI and spectral domain optical coherence tomography (SDOCT) findings in patients with cavernous sinus arteriovenous shunts (CSAVSs), where orbital venous pressure is known to be elevated, with patients with intracranial dural venous sinus thrombosis and secondary IH. We also compared the results to those obtained in the Idiopathic IH (IIH) Treatment Trial. RESULTS: Among 18 patients with dural venous sinus thrombosis, the MRI/magnetic resonance venography displayed partial empty sella (61%) and optic nerve sheath distension (67%). None exhibited ophthalmic vein dilation or signs of orbital congestion. SDOCT of these eyes and IIH eyes showed a similar frequency of abnormal thickening of the mean retinal nerve fiber layer, anterior displacement of the basement membrane opening, peripapillary wrinkles, retinal folds (RF), and choroidal folds (CF). Among 21 patients with CSAVSs, MRI showed ipsilateral dilated superior ophthalmic vein (76%) and orbital congestion (52%) without distension of the optic nerve sheath or globe distortion. SDOCT showed CF (19%), one with overlying RF, and no ONH deformations. CONCLUSIONS: SDOCT findings for dural venous sinus thrombosis are similar to those seen with IIH but distinct from changes due to local ophthalmic venous hypertension. These data support the concept that IH even if due to a vascular cause and local orbital venous hypertension cause different stresses and strains on the ONH.

Mucormycosis in head and neck area - the emerging health problem in COVID-19 pandemic. The perspective of a dental practitioner.

Mucormycosis is an invasive fungal disease caused by saprophytic molds and is characterized by a fulminant course and high mortality. Reported increase of disease cases and more frequent mucormycosis superinfections in COVID-19 patients are of a serious concern. Head and neck area is the most typical location of mucormycosis and often the first symptoms are eminent in oral cavity, therefore a dentist may be the first healthcare practitioner to recognize signs of this dangerous and potentially fatal disease. Urgent diagnosis and implementation of appropriate treatment are essential for the patient's survival. The dentist's participation in postoperative care is necessary and due to the destructive nature of radical surgical treatment, prosthetic rehabilitation is required to improve the patient's function and quality of life. Furthermore the vigilance of dentists will also allow early recognition of frequent recurrences of this insidious infection.

Urgent Subconjunctival Needle Decompression for Orbital Compartment Emphysema Caused by Compressed Air Injury.

Orbital compartment syndrome is a critical ophthalmic emergency that needs urgent diagnosis and treatment to prevent permanent vision loss caused by optic nerve compression or retinal ischemia. In this article, we present a child with orbital compartment syndrome caused by orbital emphysema as a result of a rare type of ocular trauma and introduce a simple technique to decompress the pressure. The patient was a 4-year-old boy who experienced a compressed air blast to his left eye. He presented to the emergency department with a frozen globe, tight orbit, and chemosis, without any evidence of globe rupture, conjunctival laceration, and orbital bone fracture. Computed tomographic scan demonstrated extensive subcutaneous, intraorbital, and intracranial emphysema. The pressure was immediately relieved under intravenous sedation by inserting a 27-gauge needle into the chemotic subconjunctival space at the lower lid fornix, followed by gentle manipulation of the globe to help the air escape through the needle. Compressed air injury is a rare type of orbital trauma, and this patient constituted the youngest case ever reported in the English literature, to our knowledge. Air decompression through the conjunctiva as described in this article is a useful technique that can be applied by emergency medicine specialists with special caution for patients with orbital compartment syndrome and orbital emphysema caused by compressed air injury.

Rhino-orbital mucormycosis due to Apophysomyces ossiformis in a patient with diabetes mellitus: a case report.

BACKGROUND: The most common aetiological agents of mucormycosis are Rhizopus, Mucor, Apophysomyces and Lichtheimia. Apophysomyces is comparatively rare, as it has been reported in less than 3% of mucormycosis cases. The genus Apophysomyces includes six species, and only A. elegans, A. mexicanus, A. variabilis and A. ossiformis have been reported to cause infections in both immunocompetent and immunocompromised patients. CASE PRESENTATION: We present a case of a 46-year-old male patient with bilateral blepharoedema, corneal opacity in the left eye and poorly controlled diabetes mellitus. The patient was subjected to total maxillectomy, exenteration of the left orbit and treatment with liposomal amphotericin B. Direct mycological analysis with KOH 10% revealed hyaline, coenocytic, long and wide hyphae. Apophysomyces ossiformis was identified from maxillary biopsy using 18S-ITS1-5.8S-ITS2-28S rRNA gene amplification and sequencing. The patient requested to be transferred to another hospital to continue treatment, where he died on the ninth day after admittance. CONCLUSION: To the best of our knowledge, this is the first case of rhino-orbital mucormycosis due to A. ossiformis with a fatal outcome. This case reveals the need to identify the fungus causing mucormycosis with molecular methods to identify adequate treatment therapies for patients with this infection.

Orbital Apex Syndrome Causing Blindness Secondary to a Vertical Root Fracture of a Tooth.

The authors report a case of a 35-year-old man who presented with left maxillary toothache associated with left sided facial pain and a rapidly progressive loss of vision in the left eye. Clinical and radiological assessments revealed it to be an odontogenic orbital apex syndrome secondary to a vertical root fracture of a tooth. The infection was treated and controlled, postoperative follow-up showed no recurrent inflammation and the patient recovered well. However, the vision was lost permanently. Odontogenic orbital apex syndrome is a rare, aggressive disease. Once the infection spreads, it progresses rapidly, becoming a dangerous condition. To achieve better prognosis and improve survival rates, dental practitioners and oral surgeons should be aware of this distinct presentation and should have a high index of suspicion for the complication of tooth-related problems however minor they may seem.

Subtotal Exenteration of the Orbit for Benign Orbital Disease.

PURPOSE: Total exenteration of the orbit with removal of the eye globe and surrounding tissues is most frequently indicated for malignant tumors. The indications for exenteration of the orbit for benign orbital lesion are rare. Not adequately treated infection of the orbit by systemic antibiotics can lead to destructive changes of soft tissues in the region of the orbit and partial exenteration with eyelid sparing technique is necessary. DESIGN: Retrospective case series. METHODS: Data of all patients between 2010 and 2018 who underwent exenteration of the orbit for periocular lesions infiltrating the region of the orbit were reviewed for patient demographics, previous treatment options, tumor localization and histopathologic type. RESULTS: In group of 14 patients with periocular lesions total orbital exenteration underwent 12 patients (86%), in 1 patient biorbital exenteration was performed and in one patient orbital exenteration with eyelid sparing technique was performed. For 2 patients (14%) orbital exenteration was the first surgical procedure performed. In the group of total exenteration in 12 cases histopathologically basal cell carcinoma from eyelids was confirmed, in one case squamous cell epibulbar carcinoma was confirmed and in 1 case subtotal exenteration with eyelid sparing technique was performed-the authors reported the case.Case report of patient with long inflammation of the lacrimal pathway leading to orbital inflammation with eye globe destruction and partial exenteration with eyelids sparing technique was indicated. A 75-year-old man presented in 2014 with blepharoconjunctivitis and lacrimal sac inflammation of the left side. Treated in outpatient tract with local antibiotics, the drainage lacrimal system was transient. Few months later developed chronic blefaroconjunctivitis in cultivation result Citrobacter koseri positive was found. Patient was treated only with local therapy at outpatient tract again. In 2017 sent to hospital with painful eye-globe, visual acuity was no light perception. Computed tomography and magnetic resonance presented soft tissue mass extending along the medial orbit region in the m.rect. medialis and m. obliquus sup. and partly also m. rect. inf. space as a lesion of size 23 × 30 mm with a slight postcontrast homogeneous saturation and this lesion tightly fitted to the eyeball. Exenteration with lid sparing technique was performed. In 2019 after healing process patient got an individual epithesis. CONCLUSIONS: Basal cell carcinoma is the most frequent indication of orbital exenteration. Rarely is indicated subtotal exenteration with eyelid sparing technique for non-cancer reason as it was in our 1 case.

Nontraumatic Subperiosteal Orbital Hematoma in a Patient With Behcet Disease.

Orbital subperiosteal hematomas are commonly caused by facial trauma. Nontraumatic subperiosteal orbital hematomas (NTSOHs) are rare but may occur in patients with underlying medical diseases. A 71-year-old woman presented to the emergency department with a 5-day history of left periorbital pain and proptosis. She was known to have Behcet disease and presented with no recent trauma. Clinical features and computed tomography findings aided in establishing the diagnosis of an NTSOH. The hematoma was evacuated via sub brow incision. After surgery, the patient's clinical symptoms resolved and did not recur during the 12-month follow-up period. On the basis of the findings of this case, the authors conclude that patients diagnosed with diseases such as bleeding disorders or autoimmune vasculitis should consider visual symptoms as a sign of an NTSOH. Patients who diagnosed with disease experience visual disturbance, periorbital pain, or periorbital swelling should undergo early surgical treatment for spontaneous NTSOH to avoid permanent visual loss.