A meta-analysis of sutureless scleral-fixated intraocular lens versus retropupillary iris claw intraocular lens for the management of aphakia.

This study compared the visual outcomes and complications between sutureless scleral-fixated intraocular lens and iris claw intraocular lens implantation in aphakia without adequate capsule and/or zonule support. Studies comparing the clinical outcomes of scleral-fixated intraocular lens and iris claw intraocular lens implantation published until April 2022 were retrieved from the PubMed, EMBASE, Cochrane Library, and Google Scholar databases. The outcomes included postoperative final visual acuity, surgical time, surgery-induced astigmatism, and complications. The weighted mean difference and odds ratio were calculated. Two randomized controlled trials and five cohort studies, including 244 and 290 eyes in the scleral-fixated intraocular lens group and iris claw group, respectively, were included. Scleral-fixated intraocular lens implantation results in a better postoperative final corrected distance visual acuity compared with iris claw intraocular lens implantation; however, it is more time-consuming. Scleral-fixated intraocular lens implantation seems to have lesser incidences of surgery-induced astigmatism. Furthermore, both procedures have a similar complication rate. Therefore, based on current best evidence, these two procedures should be considered according to patient's conditions.

A Rare Case of Orbital Castleman Disease with Overlapping IgG4-Related Disease.

Multicentric Castleman disease (MCD) is a systemic lymphoproliferative disorder that can lead to mass lesions in various body parts, including the lungs, kidneys, and extranodal sites. Meanwhile, orbital Castleman disease is extremely rare. Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized fibroinflammatory disorder and is characterized by the formation of tumor-like lesions with lymphoplasmacytic infiltrates, which are enriched in IgG4-positive plasma cells and may present with a characteristic storiform pattern of fibrosis to variable degrees. In this study, we report a case of a 67-year-old Taiwanese man with a 7-year history of bilateral eyelid swelling and proptosis. Orbital magnetic resonance imaging revealed soft tissue lesions in the bilateral intraconal region, demonstrating strong enhancement in the lacrimal glands, and extension into the bilateral infraorbital foramen, suggesting an orbital lymphoproliferative disease. The histopathological results of the intraorbital tumor excision were suggestive of a plasma-cell-predominant mixed-cell variant of MCD. However, the patient also showed definitive signs of IgG4-RD, including lacrimal gland enlargement and histopathological results of plasmacytosis, fibrosis, and germinal centers, with an increased ratio of IgG4 cells and elevated serum IgG4 levels. This case suggests a potential interacting pathway between these two disease entities that needs further studies.

Corneal topographic changes in patients with thyroid eye disease: A retrospective cross-sectional study.

PURPOSE: This study aimed to evaluate corneal topographic findings in patients with thyroid eye disease (TED) using a Galilei camera and compare their corneal topographic parameters with those of control patients. METHODS: This retrospective cross-sectional study included 52 eyes of 26 patients with TED (study group) and 40 eyes of 20 controls treated at a tertiary medical center in Taiwan between January and December 2021. All participants underwent basic ophthalmological examinations, corneal topography examination using a Galilei dual Scheimpflug camera, thyroid function examination, and orbital computed tomography. The corneal topographic parameters and cross-sectional area of the extraocular muscles were compared between the TED and control groups. RESULTS: Several corneal topographic parameters, including the Inferior-Superior Index, Keratoconus Prediction Index, Surface Asymmetry Index, Surface Regularity Index, simulated keratometry astigmatism, and anterior instantaneous astigmatism axis, differed significantly between the groups (P ≤ 0.05). The anterior instantaneous astigmatism axis was more oblique in the TED group than in the control group. A scatter plot revealed a more scattered distribution in the anterior instantaneous astigmatism axis in the TED group than in the control group. However, the corneal topographic parameters did not differ significantly among the different thyroid dysfunction groups. CONCLUSIONS: The corneas of patients with TED are suspicious and keratoconus-like, and TED can increase anterior corneal astigmatism in the oblique axis, albeit with large variations in the extent of change. These TED-related corneal changes may aid in the early diagnosis of TED, especially "silent" TED.

Preliminary data on a novel smart glasses system for measuring the angle of deviation in strabismus.

BACKGROUND/OBJECTIVES: To propose a novel smart glasses device for recording eye movement and compare its results to the prism alternate cover test (PACT). SUBJECTS/METHODS: This method comparison study enrolled patients with strabismic conditions, who first underwent conventional strabismus evaluations (PACT in the primary position), followed by the smart glasses NeuroSpeed system (NSS) recording protocols. The video recordings were analysed using specialized software, to calculate the horizontal deviation from the primary position. The results were compared with those of the PACT using Passing‒Bablok regression and Bland‒Altman analysis. RESULTS: This study included 70 individuals aged 4 to 80 years, of which 38 were men and 32 were women. The overall analysis of horizontal deviations using the Passing‒Bablok regression revealed a correlation coefficient (r) of 0.969, with a systemic bias of 0.00, a proportional bias of 0.809, and a perpendicular residual standard deviation of 4.134. CONCLUSIONS: The predictive values of eye movement examinations recorded by the NSS were comparable to those of the PACT. Thus, this new system can provide additional information for ophthalmologists to aid in the diagnosis and measurement of strabismus.

Optic neuropathy caused by orbital Kimura disease: A rare case report.

RATIONALE: Kimura disease (KD) is a rare, chronic inflammatory disease characterized by painless subcutaneous nodules predominantly located in the head and neck regions. Orbital KD, which intrudes into the intraconal space and results in compressive optic neuropathy, is rare and has not been previously reported. PATIENT CONCERNS: A 68-year-old man presented with blurred vision and progressive proptosis in the left eye that had been present for 2 years. DIAGNOSIS: Magnetic resonance imaging of the brain revealed soft tissue lesions with contrast enhancement and restricted diffusion involving the bilateral eyelids, orbits, and intraconal region; those on the left side were more prominent than those on the right side. The lesion encased the left optic nerve. Laboratory test results revealed elevated serum immunoglobulin E level and peripheral eosinophilia. An orbital mass biopsy demonstrated hyperplastic lymphoid follicles with germinal centers in the subcutaneous area and abundant mononuclear and binuclear eosinophils infiltrating the interfollicular area. A pathological diagnosis of KD was made based on the blood test results. INTERVENTIONS: Orbital decompression and debulking surgery of the orbital tumor in the left eye were performed to treat the compressive optic neuropathy. OUTCOMES: After systemic oral steroid and immunosuppressive agent therapies, the patient's visual acuity in the left eye improved, and the KD activity was stable. CONCLUSIONS: We present a rare case of orbital KD-associated optic neuropathy, wherein early diagnosis and treatment preserved the patient's vision. This complication should be considered in patients with a history of compressive optic neuropathy during the differential diagnosis.

Classification-Based Approaches to Myopia Control in a Taiwanese Cohort.

Purpose: Myopia is a disorder of growing prevalence in school-aged children worldwide, especially in Asia. Although low-dose atropine is recognized as an effective treatment to slow myopia progression, different studies have reported varying efficacies of treatment, and the optimal concentration of low-dose atropine remains an open question. Methods: A two-stage approach was conducted in this study. First, an observational study was conducted to plot the axial length growth curve for Taiwanese children. Second, an interventional 2-year study was performed in which different concentrations of low-dose atropine were applied based upon the risk-level status from the first stage. Results: A total of 4,091 subjects, consisting of 2,105 boys (51.5%) and 1,986 girls (48.5%), were enrolled in the first stage to plot the axial growth curve for Taiwanese children aged between 3 and 16 years. The percentage of children with myopia increased from 2.3% in 4-year-olds to 88.0% in 16-year-olds. At the second stage, a total of 886 subjects [307 (34.65%) at low risk, 358 (40.41%) at moderate risk and 221 (24.94%) at high risk] were enrolled to receive low-dose atropine based upon the risk level (0.02, 0.03, and 0.05%, respectively). With this approach, the mean annual myopia progression was -0.33, -0.57, and -0.82 D in the low-risk, moderate-risk and high-risk groups, respectively. Applying annual myopic progression < -1.0 D as a criterion for responder, the responder rates were 95.77, 83.52, and 70.59% in the low-risk, moderate-risk, and high-risk groups, respectively. Conclusions: We proposed a classification-based approach involving different concentrations of low-dose atropine based upon an individual's risk-level status. With this approach, myopic progression can be effectively controlled in patients without exposure to atropine side effects due to exposure to a higher dose than actually needed.

Early Intervention in Orbital Floor Fractures: Postoperative Ocular Motility and Diplopia Outcomes.

Purpose: Orbital floor fractures commonly occur during orbital trauma. Currently, the indications for orbital fracture repair and the appropriate duration between trauma and surgical intervention remain controversial. Methods: Eyes diagnosed with orbital floor fractures that underwent reconstruction surgery were retrospectively reviewed. Demographic data were analyzed. Patients were classified based on the timing of the surgical intervention after injury. Ocular limitation and diplopia were evaluated preoperatively and postoperatively at one week, one month, and three months. Results: Two hundred seventy eyes of 270 patients (174 males and 96 females, mean age: 40.9 ± 16.3 years) were identified. The mean duration from injury to surgical intervention was 18.0 ± 21.2 days (range: 0−117 days). In the subgroup analysis, compared to delayed treatment, the early repair of floor fractures, i.e., within 7 days, was associated with significant motility and diplopia resolution at one week (p = 0.001, p < 0.001), one month (p < 0.001, p < 0.001), and three months (p < 0.001, p < 0.001). Sex and the duration from injury to repair were significantly associated with postoperative ocular motility (p = 0.001; p = 0.024) and diplopia (p < 0.001; p = 0.008) at three months. Multivariate analysis revealed that preoperative limitation and diplopia were correlated with postoperative limitation (p = 0.007) and diplopia (p = 0.001), respectively. Conclusions: The duration between orbital floor fracture and surgical treatment was associated with postoperative limitation and diplopia. Our results suggest that earlier intervention in symptomatic patients with orbital trauma may improve postoperative visual function.

Improvement of Ocular Surface Disease by Lateral Tarsoconjunctival Flap in Thyroid-Associated Orbitopathy Patients with Lid Retraction.

There is a high incidence of ocular surface disease (OSD) in thyroid-associated orbitopathy (TAO) patients as a result of incomplete eyelid closure and chronic inflammatory eyelid status. This study was performed to evaluate the efficacy of a lateral tarsoconjunctival flap (LTF) in improving OSD in TAO patients from the perspective of correcting eyelid closures. As a study design, TAO patients were enrolled in this study to evaluate OSD perioperatively before they were scheduled for LTF surgery. Additional lid surgery was also recorded. The outcome was evaluated with perioperative OSD measurements and tear inflammatory mediators at baseline and one month and three months postoperatively. As a result, 42 patients (5 male, 37 female) underwent LTF surgery, and 13 patients received medial pretarsal support with collagen grafts. Eleven patients underwent blepharotomy, and 6 patients received Botox injections for upper lid retraction. The mean age of the participants was 46.4 years old, and the mean follow-up period was 10.6 months. Their clinical activity score (CAS) at the time of the operation was 2.1. Dry eye parameters, such as ocular surface disease index, tear breakup time, Schirmer's I test score, and meibomian gland dropout were all significantly improved 3 months postoperatively. Tear osmolarity and inflammatory mediators in tear fluid, such as IL-6, IL-8, IL-18 and MCP-1, were also significantly improved after the procedures. After the surgery, all complications were mild and temporary. As a conclusion, LTF could provide TAO patients with both lid retraction correction and improvement of ocular surface disorders. Dry eye parameters significantly improved 3 months postoperatively. This method can serve as an alternative treatment option for lid correction in TAO patients.

Characteristics of responders to atropine 0.01% as treatment in Asian myopic children.

Recently, low-concentration atropine (0.01%) has gained increased attention in controlling myopia progression with satisfying effects and minimal side effects. However, studies concerning responders to 0.01% atropine are limited. This retrospective observational cohort study aimed to determine the responder characteristics of 0.01% atropine in Asian children. One hundred forty children (aged between 3 and 15 years) receiving 0.01% atropine were analyzed for the factors influencing annual spherical equivalent changes (SE). The mean age was 9.13 (2.6) years, the mean baseline SE was - 1.56 (1.52) diopters (D), and the mean annual SE change was - 0.52 (0.49) D. A 58.63% responder rate (146/249) of myopic control was achieved with 0.01% atropine in our entire cohort under the criteria of less than 0.5 D of myopic progression annually. The subjects were stratified into 4 subgroups based on a cut-off point of baseline SE of - 1.5 D and baseline age of 9 years. The responder rate differed significantly with the highest being the youngest with the lowest myopia subgroups. Our results demonstrated that children with myopia better than - 1.5 D and younger than 9 years had the highest potential to achieve successful myopic control under 0.01% atropine therapy.

Statin Use Is Associated With a Lower Risk of Blepharitis: A Population-Based Study.

Background: Blepharitis is a common eye disorder that may be overlooked by patients and clinical practitioners. The symptoms of blepharitis often manifest as irritation, a burning sensation, grittiness, and itchiness and may decrease visual acuity if not treated promptly. Meibomian gland dysfunction (MGD), a common cause of blepharitis, is believed to be associated with increased inflammatory marker levels that may disrupt the composition of lipids produced by the sebaceous glands in the eyelids and ultimately cause tear film instability. Methods: This is a retrospective, population-based study using National Health Insurance Research Database (NHIRD) data from a 14-year period (2000-2015). Pearson chi-squared and Student's t-tests were used to assess the differences in categorical and continuous variables, respectively, between statin users and non-statin users. Univariate and multivariate Cox regression analyses were performed to calculate the hazard ratios (HRs) after adjusting for confounders. Kaplan-Meier analysis was used to assess the cumulative risk of blepharitis between the two cohorts. Results: A total of 67,014 patients who used statins were enrolled as the study cohort, and 268,056 patients who did not use statins were enrolled as the comparison cohort. The incidence of blepharitis was 3.04% with statin treatment and 3.72% without statin treatment (p < 0.001). Patients who used statins had a lower risk of developing blepharitis [adjusted hazard ratio (aHR): 0.746, p < 0.001] than those who did not. In addition, diabetes mellitus (DM), hypertension, coronary heart disease (CHD), stroke, chalazion, rosacea, Sjogren syndrome, psoriasis and atopy were found to be possible risk factors for blepharitis. Conclusion: Statin use can decrease the risk of developing blepharitis. However, further prospective studies are needed to evaluate statin treatment for various subtypes of blepharitis and to identify the associated mechanism.

A Novel Method Ensuring an Immediate Target Angle After Horizontal Strabismus Surgery in Children.

Purpose: Pediatric strabismus surgery has low success rates and high reoperation rates because of difficult alignment measurements and the nature of different strabismus types. Furthermore, adjustable sutures are not easily employed in children on an OPD basis. Methods: This was a retrospective comparative case study of children less than 12 years old who underwent strabismus surgery and were followed up at least 6 months postoperatively. We proposed a novel method that combines adjustable sutures and corneal light reflexes in regular strabismus surgery to improve surgical results. Efficacy and safety were evaluated and compared with those in a regular fixed-suture group. Results: In total, 128 children (88: exotropia and 41: esotropia) in the novel method group (Group 1) and 109 (71: exotropia and 38: esotropia) in the regular fixed-suture group (Group 2) were enrolled. The primary outcome was the immediate target angle (for esotropia within 4 PD of orthotropia and exotropia within 8 PD of esotropia within the first week postoperatively); the secondary outcome was success at the 6-month visit (angle of deviation < 10 PD). Consequently, there was a significantly higher proportion of achieving the immediate target range and success rate in both exotropic and esotropic patients in Group 1 than in Group 2. A significantly lower reoperation rate was also demonstrated in Group 1. No complications were noted in either group.Conclusions: The novel method enabled a higher proportion of subjects to achieve an immediate target range and success rate and a lower chance of reoperation among both esotropic and exotropic patients.

Relationship between Using Fibrate and Open-Angle Glaucoma in Hyperlipidemic Patients: A Population-Based Cohort Study.

BACKGROUND: This study aimed to investigate the associated risk between using fibrate and open-angle glaucoma (OAG) in hyperlipidemic patients from the National Health Insurance Research Database (NHIRD). METHODS: We collected data over a 16-year period from the NHIRD, and used the Fisher's exact test and Pearson chi-square test to analyze variables. Adjusted hazard ratios (aHR) were used to examine the risk factors for disease development. We applied Kaplan-Meier analysis to compare the cumulative incidence of OAG. RESULTS: A total of 10,011 patients using fibrate were enrolled in the study cohort, and 40,044 patients not using fibrate were enrolled in the control cohort. The incidence of OAG was lower in the study cohort than in the control cohort (aHR = 0.624, p = 0.007). The overall incidence of OAG was 463.02 per 100,000 person-years in the study cohort and 573.65 per 100,000 person-years in the control cohort. We used the Kaplan-Meier method to calculate the cumulative risk of developing OAG. The results revealed that after using fibrate for over seven years, the study cohort had a greatly lower rate of developing OAG than the control cohort (log-rank test p = 0.050). CONCLUSIONS: Our studies showed that using fibrate for over seven years may lead to a lower risk of OAG in patients with hyperlipidemia. Nevertheless, further prospective studies that comprehensively investigate the relationship between using fibrate and OAG are needed.

Factors Predicting the Success of Combined Orbital Decompression and Strabismus Surgery in Thyroid-Associated Orbitopathy.

To evaluate the safety and efficacy of orbital decompression combined with strabismus surgery in thyroid-associated orbitopathy (TAO) and identify factors leading to surgical success. A retrospective comparative case series was conducted on 52 patients who were treated with combined orbital decompression and strabismus surgery. Outcome measurements included perioperative Hertel exophthalmometry and strabismus measurements. Surgical success was defined as binocular single vision (BSV) in the primary and reading positions within 5 prism diopters (PDs). As a result, the average reduction in proptosis was 3.23 mm, with a mean preoperative Hertel measurement of 22.64 mm. Forty-four patients (84.6%) achieved the success criterion and composed the success group. In addition to sex and underlying hyperthyroidism, symmetry of orbitopathy, interocular exophthalmos difference of more than 2 mm, predominant esotropia type, mixed type strabismus, baseline horizontal deviations, baseline vertical deviations, and combination with one-wall decompression surgery were significantly different between the success and failure groups. All complications were mild and temporary. Orbital decompression combined with strabismus surgery produced satisfactory outcomes in selected patients with efficacy and safety. Symmetry between the two eyes with relatively simple strabismus and proptosis ensured surgical success. With experienced surgeons, advanced techniques, and selected patients, this method can serve as an alternative treatment option to minimize the number of surgeries, medical costs and recovery period.

Successful Resection of Retrobulbar Carcinosarcoma without Recurrence: A Case Report.

Carcinosarcomas are biphasic tumors comprising carcinoma and sarcoma components that occur in many tissues but are rarely found in the orbit. A 70-year-old male presented to the ophthalmic clinic with progressive proptosis, having decreased vision in the left eye for 8 months. On examination, severe exophthalmos and lagophthalmos with limited extraocular movement were noted. Orbital computed tomography scans revealed a large, well-defined, heterogeneously enhanced mass in the left retrobulbar orbital cavity. The tumor was completely resected, and the pathological examination revealed a carcinosarcoma. The prognosis was excellent without local recurrence at 48 months postoperatively. Thus, when considering treatment for effective management of such tumors, tumor resection followed by radiotherapy or chemotherapy is highly recommended.

Delayed Onset Bilateral Papilledema in a Young Boy's Eyes after Trauma.

Cerebral venous sinus thrombosis (CVST) is a rare venous thromboembolic disease that affects young adults in their thirties, with a female predilection. Head trauma accounts for only 1-3% of cases among possible etiologies. Here, we present a particular case of trauma-related CVST with delayed-onset symptoms and signs in a young boy. A 12-year-old boy presented to the emergency department with non-specific visual symptoms 11 days after head trauma. Apart from mild-grade disc swelling in the right eye and dyschromatopsia in both eyes, no significant findings were revealed during physical examinations and a non-contrast cranial computed tomography (CT) scan. Unfortunately, the patient suffered multiple seizure attacks the following day. Trauma-related CVST, complicated by delayed-onset increased intracranial pressure, and bilateral papilledema were finally diagnosed. Physicians need increased awareness of a possible CVST diagnosis if a patient with a history of head trauma shows persistent or worsening neurological symptoms despite negative results on serial non-contrast cranial CT scans.

Modified Target Angle as a Predictor of Success in Strabismus Management after Orbital Fracture.

Surgery for strabismus secondary to orbital fracture reconstruction surgery has had low success rates and high reoperation rates due to its incomitant nature and complex underlying mechanisms. There has been no consensus as to which of the various methods for improving the surgical results are best. We proposed a modified target angle criteria that combined the regular target angle and a favorable Hess area ratio percentage (HAR%) threshold to evaluate surgical results within the first postoperative week and conducted a retrospective chart review. According to the criteria of the modified target angle at the first postoperative week, a total of 63 patients were divided into two groups: Group 1, patients who fulfilled the criteria (49 patients); and Group 2, those who did not (14 patients). Sex, type of fracture, and the use of porous polyethylene sheets and titanium mesh during reconstruction surgery were significantly different between the groups. Group 1 showed a significantly higher percentage of patients who met the criteria of HAR% > 65% at the first week and >85% (i.e., a successful outcome) at the 6-month visit (p < 0.01). Additionally, Group 1 had a higher HAR% at the first postoperative week (p < 0.01). In conclusion, the patients meeting the criteria of the modified target angle at the first postoperative week had a favorable outcome at the 6-month visit in both ocular alignment and ocular movement.

A simple marking system for accurate intraoperative monitoring and adjustment of cyclotorsion strabismus surgery.

Ocular cyclotorsion is treatable only with surgery. The surgical procedure must be tailored individually to the specific etiologies causing the horizontal and vertical strabismus and its torsional components. An adjustable surgical approach is often used for postoperative or intraoperative adjustments. However, the methods currently used have some limitations. In this study, we propose a simple intraoperative marking system for all cyclotorsion correction surgery. The proposed marking system used three sets of surface markers: external horizontal markings, ocular horizontal markings, and surgical torsion markings, drawn in sequence. We retrospectively analyzed the surgical results using this novel marking system in this single-center, single-surgeon study. Fifteen patients with cyclotorsion who underwent treatment using the proposed marking system as an intraoperative aid between August 2019 and August 2021 were included. The medical charts were thoroughly reviewed, and the pre-and postoperative subjective and objective cyclotorsion were analyzed. Among the study subjects (10 males, 5 females; age range: 6-89 years), 13 had excyclotorsion and 2 incyclotorsion. Preoperative mean net subjective cyclotorsion measured by the double Maddox rod (DMR) test was 6.0° (standard deviation: 10.8°) and mean net disc-to-fovea angle (DFA) was 20.23° (13.21°). The postoperative net DMR and DFA were 0.2° (2.1°) and 14.09° (5.97°), respectively. The mean absolute net DMR and DFA being treated were 9.8° (4.8°) and 9.76° (4.61°). Overall, the proposed intraoperative marking system is a simple and quantitative method to assess, monitor, and adjust the torsional aspect for all strabismus surgeries.

Rare Orbital Metastasis Originating from Ampullary Adenocarcinoma.

BACKGROUND: Orbital metastasis from ampullary carcinoma is rare, with no previously reported cases. CASE PRESENTATION: We report the case of a 60-year-old man who complained of a right-sided headache, blurred vision, progressive proptosis, ptosis, and right eye pain for 3 months. His past medical history included an ampullary adenocarcinoma stage IIIA treated via the Whipple procedure and adjuvant chemoradiotherapy 1 year ago. However, he was lost to follow-up. Computed tomography of the orbit showed a soft tissue lesion in the right orbital fossa measuring 3.3 × 2 × 2 cm. An orbital mass biopsy demonstrated an intestinal-type adenocarcinoma that tested positive for cytokeratins 7 and 20 and CDX2 on immunohistochemical staining. The pathologic diagnosis was metastatic adenocarcinoma from the ampulla of Vater. Despite oncological treatment, the patient's illness progressed. He received palliative treatment and died 1 month later. CONCLUSIONS: We presented a rare case of orbital metastasis from ampullary adenocarcinoma. This should be considered in the differential diagnosis of patients with a history of ampullary adenocarcinoma who present with symptoms referring to the relevant locations.

"Pulsating proptosis and heavy eye syndrome precipitated by neurofibromatosis type 1: A case report".

RATIONALE: Neurofibromatosis type 1 (NF1) is a hereditary disease characterized by café-au-lait spots, peripheral neurofibromas, Lisch nodules, optic nerve glioma, and sphenoid wing dysplasia. Pulsating proptosis is associated with a sphenoid bony defect. Heavy eye syndrome is characterized by acquired esohypotropia in patients with high myopia. This study aimed to describe the presentation of pulsating proptosis and heavy eye syndrome precipitated by NF1 and its management. PATIENT CONCERNS: A 41-year-old woman presented with progressive pulsating proptosis and hypodeviation of the right eye over the past 2 years. The axial length of the right eye was 36.81 mm. The right eye presented with esohypotropia and hypoglobus. The ocular motility examination showed limitations in all directions, especially in supraduction. Brain computed tomography revealed sphenoid wing dysplasia of the right orbit. The meningocele protruded through the orbital defect, lifting the globe. Brain magnetic resonance imaging demonstrated superior rectus muscle (SR) medial displacement and lateral rectus muscle inferior displacement. Physical examination revealed café-au-lait macules and neurofibromas on the trunk. DIAGNOSIS: NF1 with pulsating proptosis and heavy eye syndrome. INTERVENTIONS: The patient declined neurosurgery due to risk and economic reasons. To manage her main concern regarding cosmetics, we performed orbital floor decompression, SR resection with advancement, maximal hang-back recession of the inferior rectus muscle, and a partial Jensen's procedure. OUTCOMES: Proptosis was reduced. The eye position became more symmetrical. The range of eye movements was also increased. LESSONS: This case describes a rare synchronous presentation of pulsating proptosis and heavy eye syndrome precipitated by NF1. Adult-onset presentation implied a progressive process in NF1. The case also showed a different etiology from that of typical heavy eye syndrome. It reminds ophthalmologists that orbital imaging should be performed in high myopia patients with strabismus to evaluate the extraocular muscle pathway. Furthermore, the case demonstrated a management that avoided the risk and expensive cost of neurosurgery, which has not been reported.