Ocular Manifestations Associated with Cerebral Vein Sinus Thrombosis Developing after SARS-CoV-2 Vaccination

Purpose@#We report a case of neuroretinitis combined with external ophthalmoplegia in a patient who developed a cerebral venous sinus thrombosis after administration of a SARS-CoV-2 vaccine.Case summary: A 26-year-old woman who was on oral contraceptives was diagnosed with a cerebral venous sinus thrombosis 1 month after the first injection of a SARS-CoV-2 vaccine (BNT162b2, Pfizer-BioNTech). The levels of factors 8 and 9 were elevated on the blood test. The corrected visual acuities were 0.3 in both eyes. A complete limitation of abduction and esotropia were evident in the left eye. Both eyes exhibited optic disc swelling and hemorrhage and retinal nerve fiber layer swelling. Subretinal fluid was apparent in the right eye. Three weeks later, the optic disc swellings and hemorrhages had worsened and both eyes evidenced macular stars. After 10 months, the corrected visual acuities improved to 0.9 in both eyes. Ocular motor function and the esotropia also improved. However, the overall contraction of the visual field did not. @*Conclusions@#SARS-CoV-2 vaccination can trigger cerebral venous sinus thrombosis, neuroretinitis, and external ophthalmoplegia. In patients with risk factors for such thrombosis, the possibility of ophthalmic complications should be considered after administration of a SARS-CoV-2 vaccine.

Atypical Ischemic Cranial Nerve Palsy in a Diabetic Patient

Purpose@#We report an atypical case of ischemic oculomotor palsy occurring sequentially in both eyes and then improving, followed by a new abducens palsy in a diabetic patient with poor blood sugar control.Case summary: A 51-year-old woman presented with diplopia, dizziness, and pain in the right eye and was diagnosed with oculomotor palsy in the right eye. Magnetic resonance imaging of her brain was normal but glycated hemoglobin concentration was 13.4%. After 2 months, the third nerve palsy in the right eye had recovered completely but a new third nerve palsy occurred in the left eye. 1 month later, the symptoms had improved, and the patient did not return to our clinic until 18 months later, when she returned with new diplopia. This time, she was diagnosed with a sixth nerve palsy in the left eye and was observed while controlling her blood sugar. She recovered after 4 months. @*Conclusions@#Generally, cranial nerve palsies occur unilaterally in situations that can cause ischemia, such as diabetes, high blood pressure, and hyperlipidemia. Rarely, they occur bilaterally, or two or more cranial nerve palsies occur simultaneously. However, it is very rare that cranial nerve palsy occurs sequentially in both eyes under the same systemic blood sugar control. Diabetic patients with poor blood sugar control require long-term monitoring, considering the possibility of symptoms developing in both eyes with a time lag or other forms of ischemic cranial nerve palsy.

Non-Arteritic Ischemic Optic Neuropathy Following COVID-19 Vaccination in Korea: A Case Series

Background@#To report the clinical manifestations of non-arteritic anterior ischemic optic neuropathy (NAION) cases after coronavirus disease 2019 (COVID-19) vaccination in Korea. @*Methods@#This multicenter retrospective study included patients diagnosed with NAION within 42 days of COVID-19 vaccination. We collected data on vaccinations, demographic features, presence of vascular risk factors, ocular findings, and visual outcomes of patients with NAION. @*Results@#The study included 16 eyes of 14 patients (6 men, 8 women) with a mean age of 63.5 ± 9.1 (range, 43–77) years. The most common underlying disease was hypertension, accounting for 28.6% of patients with NAION. Seven patients (50.0%) had no vascular risk factors for NAION. The mean time from vaccination to onset was 13.8 ± 14.2 (range, 1–41) days. All 16 eyes had disc swelling at initial presentation, and 3 of them (18.8%) had peripapillary intraretinal and/or subretinal fluid with severe disc swelling. Peripapillary hemorrhage was found in 50% of the patients, and one (6.3%) patient had peripapillary cotton-wool spots. In eight fellow eyes for which we were able to review the fundus photographs, the horizontal cup/ disc ratio was less than 0.25 in four eyes (50.0%). The mean visual acuity was logMAR 0.6 ± 0.7 at the initial presentation and logMAR 0.7 ± 0.8 at the final visit. @*Conclusion@#Only 64% of patients with NAION after COVID-19 vaccination have known vascular and ocular risk factors relevant to ischemic optic neuropathy. This suggests that COVID-19 vaccination may increase the risk of NAION. However, overall clinical features and visual outcomes of the NAION patients after COVID-19 vaccination were similar to those of typical NAION.

Retinal Nerve Fiber Layer-to-Disc Ratio Distinguishing Glaucoma from Nonarteritic Anterior Ischemic Optic Neuropathy

Purpose@#To evaluate the diagnostic value of retinal nerve fiber layer-to-disc ratio (RDR) compared to established parameters including retinal nerve fiber layer thickness (RNFLT), Bruch membrane opening-minimum rim width (BMO-MRW), and Bruch membrane opening-minimum rim area (BMO-MRA) for differentiating between open angle glaucoma (OAG) and nonarteritic anterior ischemic optic neuropathy (NAION). @*Methods@#This retrospective study included 23 optic disc size-matched normal control eyes and 23 OAG and NAION eyes matched according to global RNFLT. The RDR, RNFLT, BMO-MRW, and BMO-MRA were analyzed; the diagnostic capabilities of these parameters were compared using the area under the receiver operating characteristic curve (AUC). @*Results@#The global BMO-MRW and total BMO-MRA were lower in the OAG group than in the other two groups (all p < 0.001). RDR was greatest in the OAG group, followed by the healthy and NAION groups (p < 0.001). Between the OAG and NAION groups, the AUCs for temporal inferior BMO-MRW, total BMO-MRA, and RDR were 0.987, 0.996, and 1.000, respectively; these were greater than the temporal inferior RNFLT (0.811; p = 0.005, p = 0.006, and p = 0.006, respectively). @*Conclusions@#RDR could be one of the useful parameters for differential diagnosis in OAG and NAION.

Orbital Apex Syndrome and Central Retinal Vein Occlusion after Blow-out Fracture Repair

Purpose@#To report a case of orbital apex syndrome and central retinal vein occlusion after blow-out fracture repair.Case summary: A 22-year-old man who underwent emergency re-operation in a department of plastic surgery due to pain, decreased visual acuity, and ophthalmoplegia in all direction after orbital blow-out fracture repair was referred to ophthalmologist on postoperative day 2 and there was no improvement in symptoms. He showed severe complications, including optic neuropathy and ophthalmoplegia in all direction, central retinal vein occlusion, and outer retinal disruption caused by orbital apex hemorrhage. Although we were concerned that it was too late, we started high dose steroid intravenous pulse treatment and the visual acuity, ophthalmoplegia, and retinal findings were improved. However, the optic atrophy and visual field defect did not. @*Conclusions@#After a complicated blow-out fracture repair in other department, if a patient shows severely decreased visual acuity or ocular movement limitation, a thorough and immediate ophthalmologic examination is recommended. During ophthalmologic examination, meticulous examination of the entire eye, including the retina, is required, in addition to general orbital complications. A relatively good prognosis can be expected through accurate cause analysis and appropriate treatment for the confirmed abnormal findings.

Transient Total Occlusive Attack of the Ophthalmic Artery with Asymptomatic Patent Foramen Ovale

Purpose@#To report a case of transient total occlusive attack of the ophthalmic artery with asymptomatic patent foramen ovale.Case summary: A 31‐year‐old female presented with worsening of intermittent visual loss in her right eye from the previous day. The visual acuity in the right eye was reduced to 0.04 at her first visual examination. In the doctor’s room, her visual acuity in the right eye improved to 1.0 at first; however, after several minutes, it decreased to no light perception. A relative afferent pupillary defect was observed. Following that, fluorescein angiography was performed, and visual acuity improved and worsened repeatedly; corresponding intermittent perfusion and occlusion of the ophthalmic artery were observed during the examination. Further evaluation was performed to determine the cause of intermittent occlusion of the ophthalmic artery; patent foramen ovale was diagnosed. Transient ophthalmic artery occlusion was presumed to have occurred owing to embolism by the patent foramen ovale. Aspirin was used as a prophylaxis. Since then, there have been no recurring symptoms of visual impairment. @*Conclusions@#Patients with ophthalmic diseases such as ophthalmic artery occlusion, retinal artery occlusion, and amaurosis fugax, especially young people, require a thorough assessment to identify potential causes of embolism.

Accidental Swallowing of Nasal Packing Gauze during Silicone Tube Intubation under Local Anesthesia

Purpose@#To report a case of accidental swallowing of nasal packing gauze during silicone tube intubation under local anesthesia.Case summary: A 58-year-old male patient underwent silicone tube intubation for partial nasolacrimal duct obstruction on the right side. In preparing for surgery, six cottonoids soaked were packed in righ nasal cavity, to facilitate anesthesia and prevent bleeding. Strings detectable by X-ray were attached at the cottonoids and cut 5 cm from the nostrils. After surgical hand scrubbing, the strings were not present. One cottonoid was found in the nasal cavity, but five cottonoids could not be detected by endonasal endoscopic exploration. The patient said he swallowed “something like sputum”. He did not notice any discomfort and the surgery was performed as scheduled. Abdominal X-ray performed immediately after surgery showed the presence of the five strings in the stomach. The strings were not seen on X-ray conducted 4 days later. The patient defecated normal stools three times and did not notice any discomfort over the course of 4 days. @*Conclusions@#Surgeons need to be aware of the possibility of accidental swallowing of nasal packing gauze during nasolacrimal duct surgery. Use of gauze detectable by X-ray is helpful to determine its location.

A Case of Adult Orbital Lymphatic Malformation in the Lacrimal Gland

PURPOSE: When there is a mass in the superior temporal orbit area, a lacrimal gland tumor should be suspected. We report a rare case of orbital lymphatic malformation that was histologically diagnosed in a patient with typical clinical features of the lacrimal gland. CASE SUMMARY: A 55-year-old female with no underlying disease and no ophthalmic history visited our clinic with a right upper eyelid edema associated with an enlarged painless eyelid mass 1 month prior to her visit. The patient stated that she discovered the mass 1 year previously.The palpebral lobe of the lacrimal gland protruded slightly with congestion of the surrounding conjunctiva. Enhanced computed tomography showed a 3 cm well-defined heterogeneous mass in the right lacrimal gland area and several well-defined round calcifications within the mass. Orbital tissue or bone involvement was not observed. The pleomorphic adenoma of the lacrimal gland was the most clinically suspicious, so complete resection of the mass was performed using lateral orbitotomy. Histopathologically, lymphangioma (lymphatic malformation) originating from the lacrimal gland was diagnosed. CONCLUSIONS: Orbital lymphatic malformation can occur in the lacrimal gland. The present case showed that differential diagnosis can reveal the presence of an adult lacrimal gland tumor.

An Unusual Case of Double Lacrimal Puncta

PURPOSE: Congenital double puncta are usually unilateral, and the accessory punctum exists on the medial side in a slit configuration that is distinct from the shape of the normal punctum. We report a case of an unusual case of double lacrimal puncta which the lateral, rather than the medial, punctum was judged to be the accessory punctum. CASE SUMMARY: A 39-year-old male patient with no underlying disease and no ophthalmologic history visited our clinic with right eye epiphora of 2 weeks duration. On slit lamp examination, double puncta were observed in the right lower eyelid and the remaining puncta were normal. On lacrimal syringing test and dacryocystography were performed and revealed incomplete obstruction with partial narrowing of the nasolacrimal duct. Silicone tube intubation was performed through the right lower medial punctum and symptoms improved postoperatively. CONCLUSIONS: The present case is an unusual case of double lacrimal puncta which has not been reported in Korea. Unlike the previous literature, the lateral, rather than the medial, punctum was judged to be the accessory punctum. Because accessory punctm can be present on the lateral side, it is necessary to distinguish between the accessory punctm and the main punctum through the accurate dacryocystography and lacrimal syringing test for the treatment of the patient.

An Unusual Case of Benign Episodic Bilateral Mydriasis

PURPOSE: Although benign episodic mydriasis has been rarely reported worldwide, most of the reports so far have occurred in unilaterally. To report an unusual case of benign episodic bilateral mydriasis. CASE SUMMARY: Nineteen-year-old woman who presented with intermittent dilation of both pupils two months ago. She had difficulty on reading and doing tasks because of her visual blur. This symptom usually lasted for 1–2 hours, occurred once every 2–3 days, most frequently during stressful situations. Headaches and dizziness accompanied the eye symptoms, and her pupils were both 5 mm in a lighted room and both 7 mm in a dark room. All tests, including brain magnetic resonance imaging, showed no abnormal findings. We diagnosed her as benign episodic bilateral mydriasis. The frequency of her symptoms decreased during the university vacation period. CONCLUSIONS: In the absence of ophthalmologic or neurologic abnormalities other than headaches in patients with transient mydriasis, benign episodic mydriasis should be considered as a differential diagnosis. Although benign episodic bilateral mydriasis has a unilateral predominance, for the first time the authors report that benign episodic mydriasis may occur in both eyes during same episode.

Effects on Periocular Tissues after Proton Beam Radiation Therapy for Intraocular Tumors

BACKGROUND: To present our experience on orbital and periorbital tissue changes after proton beam radiation therapy (PBRT) in patients with intraocular tumors, apart from treatment outcomes and disease control. METHODS: Medical records of 6 patients with intraocular tumors who had been treated with PBRT and referred to oculoplasty clinics of two medical centers (Seoul National University Hospital and Seoul Metropolitan Government-Seoul National University Boramae Medical Center) from October 2007 to September 2014 were retrospectively reviewed. The types of adverse effects associated with PBRT, their management, and progression were analyzed. In anophthalmic patients who eventually underwent enucleation after PBRT due to disease progression, orbital volume (OV) was assessed from magnetic resonance (MR) images using the Pinnacle3 program. RESULTS: Among the six patients with PBRT history, three had uveal melanoma, and three children had retinoblastoma. Two eyes were treated with PBRT only, while the other four eyes ultimately underwent enucleation. Two eyes with PBRT only suffered from radiation dermatitis and intractable epiphora due to canaliculitis or punctal obstruction. All four anophthalmic patients showed severe enophthalmic features with periorbital hollowness. OV analysis showed that the difference between both orbits was less than 0.1 cm before enucleation, but increased to more than 2 cm3 after enucleation. CONCLUSION: PBRT for intraocular tumors can induce various orbital and periorbital tissue changes. More specifically, when enucleation is performed after PBRT due to disease progression, significant enophthalmos and OV decrease can develop and can cause poor facial cosmesis as treatment sequelae.

Using 17-gauge Spinal Anesthesia Needle as an Insertion Guide for Frontalis Sling Surgery with Silicone Rod

PURPOSE: In this study we investigated the surgical outcome and effectiveness of using a 17-gauge spinal anesthesia needle for guiding insertion of the silicone rod in frontalis sling surgery for patients having severe myogenic or neurogenic ptosis with risk of exposure keratopathy. METHODS: This study included on 8 patients (11 eyes) who previously received frontalis sling surgery with a 17-gauge spinal anesthesia needle because of severe myogenic or neurogenic ptosis with risk of exposure keratopathy. We investigated the technique, surgical outcome, and clinical features following frontalis sling surgery, and evaluated the advantages of using a 17-gauge spinal anesthesia needle. RESULTS: The mean age of patients was 54 years. Third nerve palsy was the most common etiology in severe ptosis with risk of exposure keratopathy (5 of 8, 62.5%). Main advantages of the 17-gauge spinal anesthesia needle are smaller skin incision that allows for minimal damage during tissue passage and easy guide for insertion. Other advantages included sterilization, minimizing infections, sharpness without distortion because it is disposable, and economic value. The mean follow-up period was 21.4 months, and the upper lid margin of all patients was adequately high above the pupil margin. Additionally, no major complications were observed in the patients. CONCLUSIONS: Frontalis sling surgery with a silicone rod is a safe and effective method for patients with severe myogenic or neurogenic ptosis with risk of exposure keratopathy. The 17-gauge spinal anesthesia needle is useful and economical in frontalis sling surgery for guiding insertion of the silicon rod as an alternative option to the Wright needle.

Intraoperatively Observed Lacrimal Obstructive Features and Surgical Outcomes in External Dacryocystorhinostomy

PURPOSE: To analyze the features of lacrimal drainage system obstruction confirmed during external dacryocystorhinostomy surgeries and report the surgical outcomes. METHODS: We reviewed the medical records of a total of 769 cases who underwent external dacryocystorhinostomy for primary lacrimal drainage obstruction between 2005 and 2014. Data about detailed location and extent of obstruction were collected intraoperatively. The sites of obstruction were classified into nasolacrimal duct obstruction (NLDO), common canalicular obstruction (CCO), and canalicular obstruction. Lacrimal sac mucosa and lumen were grossly inspected, and the frequency of lacrimal sac changes, such as significant inflammation or fibrosis, was analyzed in cases of CCO or canalicular obstruction. The surgical success rate was also evaluated including effect of lacrimal sac status in the CCO and canalicular obstruction groups. RESULTS: Of 769 cases, primary NLDO with patent canaliculi was diagnosed intraoperatively in 432 cases (56.2%), CCO in 253 (32.9%), and canalicular obstruction in 84 (10.9%). Of 253 cases with CCO, 122 (48.2%) showed clear lacrimal sac lumen, and the other 131 (51.8%) showed significant inflammation or fibrosis of the lacrimal sac. In cases with canalicular obstruction, 35 of 84 (41.7%) showed a clear lacrimal sac, and the other 49 cases (58.3%) cases revealed mucosal changes of the lacrimal sac. The functional success rate was 87.5% for primary NLDO, 75.5% for CCO, and 72.6% for canalicular obstruction. In the CCO group, the functional success rate was lower in cases with significant lacrimal sac change (p = 0.044). CONCLUSIONS: Even in patients with CCO or canalicular obstruction, a large number of cases have lacrimal sac changes, and those changes were associated with lower functional success rate.

Multiple Cerebral Infarctions with Neurological Symptoms and Ophthalmic Artery Occlusion after Filler Injection

PURPOSE: To report a case of visual loss, side weakness and facial palsy due to ophthalmic artery occlusion with diffuse multiple cerebral infarctions after injection of hyaluronic acid. CASE SUMMARY: A 50-year-old female visited our clinic for visual loss in the left eye after filler injection in the glabella. Her best corrected visual acuity was 1.0 in the right eye and hand motion in the left eye. The intraocular pressure was 8 mm Hg in the right eye and 14 mm Hg in the left eye. In the left eye, there was abnormal pupillary light reflex and complete extra-ocular muscles palsy with blepharoptosis. A pale retina with a cherry-red-spot also appeared in the left fundus. A central retinal artery occlusion was observed on fluorescein angiography and brain magnetic resonance imaging showed multiple cerebral infarctions at the frontal, temporal, parietal and occipital lobes. Four days later, the motor weakness was aggravated and dysarthria and aphasia became worse. According to symptoms, a hemorrhagic transformation in subacute infarctions developed based on brain computed tomography. After 3 months of follow up, the visual acuity in the left eye was no light perception. However, the general conditions including ophthalmoplegia and motor weakness were improved.

Outcomes of Isolated Medial Orbital Wall Fracture Reconstructions

PURPOSE: To evaluate surgical results after the reconstruction of isolated medial orbital wall fractures. METHODS: We performed a retrospective chart review of patients with isolated medial orbital wall fractures who underwent reconstruction using the transcaruncular approach from March 2012 to October 2013. Computed tomography (CT) was performed before and after surgery. Postoperative follow-ups were conducted at 1 week, 1 month, and 3 months. Diplopia, ocular motility, postoperative complication, and exophthalmometry were recorded at each follow-up visit. Preoperative and postoperative enophthalmos was quantified using Hertel exophthalmometry and a quantitative method for the area of the circular sector under the chord (CA)/orbital area (OA) ratio at the CT scan. Patients were divided into either the incomplete or complete reduction groups based on the degree of reduction observed on postoperative CT. RESULTS: We evaluated 55 patients (42 males, 13 females) with an average age of 36 years. Five of 55 patients with preoperative enophthalmos of more than 2 mm obtained good symmetry after surgery. Diplopia at primary gaze was resolved in 9 of 9 patients and 2 patients had residual diplopia on lateral gaze. The difference of exophthalmometry and CA-to OA ratio between before and after reconstruction was not significantly changed in either the incomplete or complete groups. CONCLUSIONS: Enophthalmos and diplopia were well corrected in all subjects. If reduction was successfully performed in the anterior portion of medial orbital wall fracture, clinically significant enophthalmos might be preventable even if complete reduction was not obtained.

Clinical Usefulness of Binocular Multifocal Electroretinography in Patients with Monocular Macular Disease

PURPOSE: To evaluate the clinical usefulness of binocular multifocal electroretinography (mfERG) by comparing results with conventional monocular mfERG in patients with monocular macular disease. METHODS: mfERG testing was conducted on 32 patients with monocular macular disease and 30 normal subjects. An initial mfERG was simultaneously recorded from both eyes with two recording electrodes under binocular stimulation. A second mfERG was subsequently recorded with conventional monocular stimulation. Amplitudes and implicit times of each ring response of the binocular and monocular recordings were compared. Ring ratios of the binocular and monocular recording were also compared. RESULTS: In the macular disease group, there were no statistical differences in amplitude or implicit time for each of the five concentric rings between the monocular and binocular recordings. However, with binocular simulation, the ring ratios (ring 1 / ring 4, ring 1 / ring 5) were significantly reduced in the affected eye. In the normal control group, there were no statistical differences in any parameters between the monocular and binocular recordings. CONCLUSIONS: Binocular mfERG could be a good alternative to the conventional monocular test. In addition, given that the test needs stable fixation of the affected eye during the binocular test, the reliability of the test results could be improved, especially for patients with monocular macular disease.

A Case of Lymphomatoid Papulosis of the Eyelid

PURPOSE: Lymphomatoid papulosis (LyP) is one of the primary cutaneous CD30-positive lymphoproliferative disorders. LyP of the eyelid has rarely been reported. Herein, a case of typical LyP of the medial canthal area is reported. In addition, a literature review was performed. CASE SUMMARY: A 40-year-old female presented with a skin mass in the medial canthal area of the left eye that developed 2 months earlier. Initially, a focal skin lesion developed, and even with conservative treatment at a local clinic, progressed to a mass lesion having a central ulceration and adjacent edema. After 6 weeks, the adjacent edema had gradually decreased. On ophthalmic examination, the left medial canthal lesion was a 6 x 6 mm sized elevated mass with a central crater covered by crust. The clinical impression was keratoacanthoma. The lesion was widely excised and reconstructed by a full-thickness skin graft after an incisional biopsy. Histopathologic findings showed dermal infiltration of various inflammatory cells with atypical lymphocytes showing positivity to the CD30 antigen, and LyP was diagnosed. Systemic evaluation showed no evidence of systemic lymphoma and the patient has remained free of recurrence or systemic disease after a 1-year follow-up.

Congenital Ocular Anomaly in an Infant with Trisomy 14 Mosaicism

Trisomy 14 mosaicism is a rare chromosomal abnormality with distinct and recognizable clinical features. We report a patient with presumed retinal dystrophy having diffuse retinal pigment epithelial abnormalities, which has not been previously reported in association with trisomy 14. This case expands the clinical spectrum of this rare entity.

Surgical Outcomes for Lens Fragments Dropped into the Vitreous Cavity during Cataract Surgery

PURPOSE: To assess the clinical features and outcomes of patients referred for management of dropped lens fragments during cataract surgery. METHODS: The medical records of 22 eyes from 22 patients who were referred to our hospital to undergo pars plana vitrectomy (PPV) for management of dropped lens fragments after phacoemulsification surgery at private clinics were reviewed. Data including patient demographics, preoperative and postoperative visual acuity, factors associated with dropped lens fragment, and postoperative complications were recorded. The factors were compared between 2 patient groups: postoperative Snellen acuity of 0.5 or better and acuity less than 0.5. The statistical significances of differences in factors between the 2 groups were calculated. RESULTS: The mean interval between cataract surgery and PPV was 2.6 +/- 3.7 days. At the final examination, the mean postoperative acuity was 0.57 +/- 0.20, and 16 eyes (72.7%) had a visual outcome of 0.5 or better. After excluding 7 eyes with other pre-existing ocular co-morbidities, 14 eyes (93.0%) achieved a final visual acuity of 0.5 or better. Multivariate analysis showed that the predictor for visual outcomes of 0.5 or better was absence of preoperative eye disease (p = 0.007). Complications after PPV included 2 (9.0%) cases of cystoid macular edema and 1 (4.5%) case of retinal detachment. CONCLUSIONS: Prompt referral and surgical management within 1 week for dropped lens fragments during cataract surgery may achieve a better visual outcome in cases with no pre-existing eye disease.

Amblyogenic Risk Factors in Primary Family Members of Patients with Exotropia

PURPOSE: To investigate the prevalence of amblyogenic risk factors in the primary family members (parents, siblings) of patients with exotropia. METHODS: The authors of the present study examined primary family members including parents and siblings of 58 exotropia patients in our clinic. Best corrected visual acuity, refractive error, and cover-uncover test were performed. A total of 49 normal children's family members (control group) were examined in a similar manner. The prevalence of significant ocular findings were determined and compared to the control group. RESULTS: In 58 eligible families (153 subjects except proband), 65.4% had significant ocular findings: In parents, anisometopia, astigmatism, hyperopia, and strabismus were found at a rate of 18.1%, 31.1%, 2.5%, and 11.2%, respectively. In siblings, anisometropia, astigmatism, hyperopia, and strabismus were found at a rate of 24.3%, 24.3%, 5.4%, and 18.9%, respectively. Compared to the control group, in parents of exotropia patients, anisometropia, astigmastim, and strabismus were observed at a higher rate. In siblings, anisometropia, and strabismus were observed at a higher rate. CONCLUSIONS: Primary family members of exotropia patients have a high prevalence of amblyogenic risk factors. The present study offers rationale for providing comprehensive eye exams for parents and children with a family history of exotropia.