Levator palpebralis superioris histopathologic findings in congenital ptosis and surgical outcome after its resection: Is there any association?

PURPOSE: To evaluate the histopathologic findings of Levator palpebralis superioris (LPS) muscle biopsy after LPS resection for treatment of congenital ptosis and its possible relation with surgical outcomes.Please confirm if the author names are presented accurately and in the correct sequence (given name, middle name/initial, family name). Author 4 Given name: [Seyed Mohsen] Last name [Rafizadeh]. Author 6 Given name: [Seyed Ali] Last name [Sonbolestan].Also, kindly confirm the details in the metadata are correct.The author names and the sequence are correct. METHODS: Congenital ptosis patients were enrolled in this retrospective study. All of them underwent full ophthalmologic examination included of Margin-reflex distance 1 (MRD-1) and LPS function measurement preoperatively. The patients were followed for 3 months for the postoperative period and after that the measurements were repeated. Histologic parameters including percentages of fat, striated and smooth muscle, and fibrous tissue. The histopathologic findings and their possible correlation with the measurements are analyzed. RESULTS: Sixty-seven patients with unilateral congenital ptosis were enrolled. 45 patients (67.2%) were males. The mean age of patients was 16.10 ± 11.18 years. The patients' MRD-1 was improved significantly from 0.82 ± 1.26 mm to 3.85 ± 1.25 mm after LPS resection (P = 0.000). The success rate was 80.3%. There were no correlations between MRD change and histopathologic tissue percentages but significant correlation was found between success of surgery and fibrous tissue percentage of resected sample (P = 0.033). CONCLUSIONS: The histopathology of the LPS may be useful in prediction of surgical outcome after LPS resection in congenital ptosis patients. The percentage of fibrous tissue play an important role.

Blepharoptosis and corneal epithelial thickness alterations, is there any relation?

BACKGROUND: To compare the epithelial thickness map of ptotic eyes of blepharoptosis patients with contralateral non- ptotic eyes. METHODS: Unilateral blepharoptosis patients were enrolled consecutively. Patients were underwent full ophthalmologic examination and their demographic data such as age and gender and specific ptosis findings e.g. the cause and duration, MRD-1, and levator palpebralis superioris function were registered. Anterior segment imaging for epithelial thickness measurements was done using the Avanti RTVue-XR platform. The corneal epithelial thickness maps of ptotic and non-ptotic eyes were compared. RESULTS: 44 patients with unilateral blepharoptosis were included in the study. 27 (61.4%) of them were female and 17 (38.6%) cases were male. The mean of the patients' ages was 24.40 ± 15.16 years. Ptotic eyes had significantly thinner superior (p = 0.000), superior-temporal (p = 0.000) and superior-nasal (p = 0.005) sectors of the cornea and slightly thicker corneal epithelium (CE) in the inferior-nasal sector. The correlation of difference of superior-inferior CE was evaluated with different parameters including patient's age (p = 0.457), type of blepharoptosis (p = 0.786), duration of blepharoptosis (p = 0.477) and MRD1 (p = 0.248), but no correlation was found. CONCLUSIONS: This study revealed that lid position in blepharoptosis may have effects on the corneal epithelial thickness map. Because of the lower position of upper eyelid, a thinning effect on superior corneal sectors may happen.

Effect of orbital decompression surgery on the choroidal profile in patients with thyroid eye disease.

This study aimed to investigate the effect of orbital wall decompression surgery and reduction of proptosis on the choroidal vascularity index (CVI) and subfoveal choroidal thickness (SFCT) in patients with thyroid eye disease (TED). Fifty-one eyes from 38 patients with controlled TED and proptosis were enrolled in this study. The majority of the patients (50.9%) had a clinical activity score (CAS) of zero, and none had a CAS greater than 2. The patients underwent a complete baseline ophthalmologic examination, and their choroidal profile alterations were monitored using enhanced depth imaging optical coherence tomography (EDI-OCT) before and during the three months after surgery. Changes in SFCT, luminance area (LA), total choroidal area (TCA), and the choroidal vascularity index (CVI) were measured as the ratio of LA to TCA in EDI-OCT images. The participants had an average age of 46.47 years, and 22 were female (57.9%). The SFCT of the patients exhibited a significant reduction over the follow-up period, decreasing from 388 ± 103 to 355 ± 95 µm in the first month (p < 0.001) and further decreasing to 342 ± 109 µm by the third month compared to baseline (p < 0.001). The CVI exhibited a drop from 0.685 ± 0.037 at baseline to 0.682 ± 0.035 and 0.675 ± 0.030 at 1 and 3 months post-surgery, respectively. However, these changes were not statistically significant, indicating comparable decreases in both LA and TCA. There was a significant correlation between improved proptosis and reduction in SFCT (p < 0.001) but not with CVI (p = 0.171). In conclusion, during the three months of follow-up following orbital wall decompression, CVI did not change, while SFCT reduced significantly. Additionally, SFCT was significantly correlated with proptosis reduction, whereas CVI was not.

Superficial ocular vascular changes after orbital decompression in patients with thyroid ophthalmopathy measured by anterior segment OCT angiography; an observational study.

Thyroid eye disease (TED) is a common ophthalmologic manifestation of thyroid dysfunction. Despite various imaging techniques available, there hasn't been a widely adopted method for assessing the anterior segment vasculature in TED patients. Our study aimed to evaluate alterations in ocular surface circulation following orbital decompression surgery in TED patients and investigate factors influencing these changes. Using anterior segment optical coherence tomography-angiography (AS-OCTA), we measured ocular surface vascularity features, including vessel density (VD), vessel diameter index (VDI), and vessel length density (VLD), both before and after decompression surgery, alongside standard ophthalmic examinations. Our AS-OCTA analysis revealed a significant decrease in most of the temporal vasculature measurements six weeks post-surgery (p < 0.05). However, differences in the nasal region were not statistically significant. These findings indicate notable changes in ocular surface circulation following orbital decompression in TED patients, which may have implications for intraocular pressure (IOP) control and ocular surface symptoms management. AS-OCTA holds promise as a tool for evaluating the effectiveness of decompression surgery and assessing the need for further interventions.

Orbital histiocytosis; From A to Z.

PURPOSE: Histiocytosis is one of the most challenging diseases in medical practice. Because of the broad spectrum of clinical manifestations, systemic involvements, unknown etiology, and complex management, different types of histiocytosis are still a big question mark for us. Orbital histiocytosis is characterized by the abnormal proliferation of histiocytes in orbital tissues. It could affect the orbit, eyelid, conjunctiva, and uveal tract. Orbital histiocytosis can cause limited eye movement, proptosis, decreased visual acuity, and epiphora. In this study, we review the novel findings regarding the pathophysiology, diagnosis, and treatment of different types of histiocytosis, focusing on their orbital manifestations. METHOD: This review was performed based on a search of the PubMed, Scopus, and Embase databases or relevant published papers regarding orbital histiocytosis on October 9th, 2023. No time restriction was proposed, and articles were excluded if they were not referenced in English. RESULTS: 391 articles were screened, most of them being case reports. The pathophysiology of histiocytosis is still unclear. However, different mutations are found to be prevalent in most of the patients. The diagnostic path can be different based on various factors such as age, lesion site, type of histiocytosis, and the stage of the disease. Some modalities, such as corticosteroids and surgery, are used widely for treatment. On the other hand, based on some specific etiological factors for each type, alternative treatments have been proposed. CONCLUSION: Significant progress has been made in the detection of somatic molecular changes. Many case studies describe various disease patterns influencing the biological perspectives on different types of histiocytosis. It is necessary to continue investigating and clustering data from a broad range of patients with histiocytosis in children and adults to define the best ways to diagnose and treat these patients.

Orbital intramuscular hydatid cyst causing compressive optic neuropathy: a case report and literature review.

BACKGROUND: Echinococcosis, commonly known as hydatid disease, is a zoonotic infection resulting from the tapeworm Echinococcus granulosus. The occurrence of hydatid cysts in the orbital region is uncommon, representing less than 1% of all reported hydatid cases. This report details a unique case of an intramuscular hydatid cyst in the orbital region that led to compressive optic neuropathy. CASE PRESENTATION: A 22-year-old male from Kabul, Afghanistan presented with a five-month history of progressive proptosis in his left eye, associated with a gradual decrease in vision over the past three weeks. The left eye exhibited upward globe dystopia, ocular motility limitation, mild conjunctival injection, and chemosis. Diagnosis was achieved through imaging and histopathological examination. Treatment involves surgical removal of the cyst and prolonged albendazole therapy. The postoperative course showed significant improvement in the patient's condition and restoration of his vision. CONCLUSIONS: Despite its rarity, this case underscores the importance of awareness and knowledge of hydatid disease among physicians, especially those working in endemic areas. It emphasizes the importance of including hydatid disease in the differential diagnosis of orbital masses, particularly in endemic regions.

Lower Eyelid Position Changes Following Müller's Muscle-Conjunctival Resection Blepharoptosis Surgery.

BACKGROUND: Asymmetry of the lower eyelids and a lower position of the lower eyelid on the ptotic side are common issues in patients with upper lid ptosis. Understanding the relationship between upper lid ptosis correction and lower eyelid position will facilitate better treatment strategies for ptosis. OBJECTIVES: To assess the lower eyelid position before and after Müller's muscle-conjunctival resection (MMCR) in unilateral myogenic ptosis (MP) and aponeurotic ptosis (AP). METHODS: This prospective interventional before-and-after study, included adults with blepharoptosis. Measurements were taken before and 6 months after the surgery. RESULTS: A total of 47 patients included, with 29 having MP and 18 having AP. The mean ages were 31.55 ± 5.30 years and 50.11 ± 6.45 years, respectively. In MP group, there was a significant association between baseline MRD2 and the severity of ptosis (MRD1) (ß= -0.739, p=0.004, multivariate regression analysis). After MMCR, significant decreases were observed in MRD2 for both MP (-0.38 ± 0.48 mm, p<0.001) and AP (-0.39 ± 0.47 mm, p=0.003). The symmetry of MRD2 remained stable at 41.4% in the MP, while it decreased from 44.4% to 16.7% in the AP. The baseline MRD2 was the only factor associated with postoperative MRD2 in the MP. CONCLUSIONS: Patients with myogenic and aponeurotic ptosis often exhibit a lower position of the lower eyelid, which typically elevates after posterior upper lid ptosis surgery. In myogenic cases, the severity of preoperative lower eyelid position correlates with the severity of upper lid ptosis and predicts postoperative elevation of the lower lid.

Skin and ophthalmic complications of chloroquine and hydroxychloroquine in patients with rheumatoid arthritis and systemic lupus erythematous.

Immunosuppressive agents are routinely used to control autoimmunity. However, some adverse events are correlated to their clinical applications. The aim of this study was to study the clinical findings and ocular and cutaneous side effects of chloroquine (CQ) and hydroxychloroquine (HCQ), as current immunomodulators, in patients with rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE). This descriptive study was performed on 360 individuals referred to the Rheumatology clinic during 2003-2020. Demographic characteristics and other information were collected from patients with RA and SLE. Skin and ocular complications were evaluated in patients who were on treatment with CQ and HCQ. Study populations consisted of 199 subjects with RA and 161 cases with SLE. The frequencies of skin and ocular complications in all patients treated with CQ and HCQ were 32 (17.65%) and 94 (51.9%), respectively. The prevalence of skin complications in patients with RA and SLE was 20 (10.05%) and 22 (13.66%), respectively. The frequencies of ocular complications in patients with RA and SLE were, respectively, 58 (29.4%) and 36 (22.5%). Multiple logistic regression analysis revealed that ophthalmic complications of CQ and HCQ in all patients were dependent on the effects of the duration of drug uses, disease duration, and cumulative doses (p < 0.05), unlike skin complications. Disease types had no effect on ocular complications. Based on these findings, treatment with CQ and HCQ participates in some skin and ocular complications in patients with RA and SLE which are largely associated with the duration of disease and treatment.

Prediction of substantial closed-globe injuries in orbital wall fractures.

PURPOSE: To determine risk factors for substantial closed-globe injuries in orbital fractures (SCGI) and to develop the best multivariate model for the prediction of SCGI. METHODS: A retrospective study was performed on patients diagnosed with orbital fractures at Farabi Hospital between 2016 and 2022. Patients with a comprehensive ophthalmologic examination and orbital CT scan were included. Predictive signs or imaging findings for SCGI were identified by logistic regression (LR) analysis. Support vector machine (SVM), random forest regression (RFR), and extreme gradient boosting (XGBoost) were also trained using a fivefold cross-validation method. RESULTS: A total of 415 eyes from 403 patients were included. Factors associated with an increased risk of SCGI were reduced uncorrected visual acuity (UCVA), increased difference between UCVA of the traumatic eye from the contralateral eye, older age, male sex, grade of periorbital soft tissue trauma, trauma in the occupational setting, conjunctival hemorrhage, extraocular movement restriction, number of fractured walls, presence of medial wall fracture, size of fracture, intraorbital emphysema and retrobulbar hemorrhage. The area under the curve of the receiver operating characteristic for LR, SVM, RFR, and XGBoost for the prediction of SCGI was 57.2%, 68.8%, 63.7%, and 73.1%, respectively. CONCLUSIONS: Clinical and radiographic findings could be utilized to efficiently predict SCGI. XGBoost outperforms the logistic regression model in the prediction of SCGI and could be incorporated into clinical practice.

Quantitative analysis of the eyelid curvature in patients with blepharoptosis.

BACKGROUND: The aim of this study was to evaluate the ability of two novel eyelid curvature measurements to distinguish between normal eyes and different severities of blepharoptosis. METHODS: A comparative cross-sectional analysis of upper eyelid curvature was performed for different severities of patients with unilateral blepharoptosis (congenital and aponeurotic) and normal controls. Mean upper lid contour index (ULCI) and area circularity index (ACI) were calculated for each group by dividing the intercanthal distance by upper eyelid margin length (ULCI) and dividing the interpalpebral area by the area of a circle enclosing the eye (ACI). The ratio of each index for the study and fellow normal eye of each patient was also calculated and compared between groups. RESULTS: A total of 106 eyes including 30 eyes in the control group and 25, 27, and 24 eyes in the mild, moderate, and severe ptosis groups were enrolled in the study. ULCI and ACI showed a statistically significant difference between the groups (p < 0.001, p < 0.001). The inter-eye ratio (ULCI-ratio and ACI-ratio) of indices was also significantly different between groups (p = 0.002, p < 0.001). Pairwise comparisons revealed that ACI and ACI-ratio were significantly different between all pairs of study groups. CONCLUSION: The results of our study showed that ACI based on area measurements may distinguish blepharoptosis patients from normal controls and from each other. Including the data from the fellow normal eyes in the form of ratio indices may improve the differentiating power. These results can be useful in designing the optimal eyelid curvature measurements.

Uveal melanoma in the Iranian population: two decades of patient management in a tertiary eye center.

BACKGROUND: To assess the characteristics and outcomes of uveal melanoma management at a tertiary center in the Middle East. METHODS: A study on 164 patients with uveal melanoma was conducted by reviewing the available medical records, ultrasound, and pathology report results. Age at diagnosis, tumor location and size, treatment mode, visual outcome, metastasis, mortality, and survival were studied. RESULTS: The mean age of patients was 52.0 ± 15.0 years, and 52.5% were male. Choroidal melanoma was the most common uveal melanoma, followed by the ciliary body and iris melanoma. The mean thickness of tumors was 8.29 ± 3.29. The majority of patients (n = 111, 67.9%) were managed by brachytherapy with ruthenium-106 plaques. Enucleation was performed primarily in 46 (28%) patients and secondarily in nine (5.5%) patients. The sexual disparity was detected as the proximity of uveal melanoma to the fovea in males. For a 61-month mean follow-up period, mortality occurred in eight of our cases, six of which were due to metastasis. The most common site for distance metastasis was the liver (5/6), followed by the lung (1/6). The five-year and eight-year overall survival (OS) rate was 0.947%± 0.019. The 5-year survival rate reached zero in metastatic patients. OS was not statistically different depending on the age, tumor diameters, the primary treatment received, or the histopathologic findings (p > 0.50 for all). CONCLUSION: In this study, individuals diagnosed with UM exhibited an OS rate of around 94% at the five-year mark, which remained consistent up to eight years. Notably, the presence of distance metastasis emerged as the sole statistically significant factor influencing overall survival.

Orbital primary intraosseous hemangioma in a three-month old infant: A case report.

Purpose: To report a 3-month-old boy with rapid progressive orbital intraosseous hemangioma which was treated with excisional biopsy and orbital rim reconstruction. Observation: A 3-month-old boy was referred with the aggressive growth of a mass on the right orbital region. The mass was noted to develop over 4 weeks. On presentation this firm nontender orbital mass measuring 5 × 5cm mimicked a more ominous malignancy. The spiral computed tomography scan showed a destructive mass with protrusion superiorly and inferiorly toward the orbital cavity and anterior cranial fossa. The patient underwent gross tumor resection and reconstruction of the orbital rim. Histology findings revealed an intraosseous hemangioma. There was no evidence of recurrence after 1-year follow-up. Conclusion and Importance: Due to rapid progression, the patient's age, and lesion size, this case is unique. There were additional challenges regarding complete resection, intra-cranial extension, and significant blood loss in an infant. Therefore, in the face of rapidly progressing orbital tumors in infants, despite the very low prevalence of intraosseous hemangioma, this diagnosis should be considered.

Surgical Correction of Cicatricial Lower Eyelid Retraction: A Systematic Review.

BACKGROUND: Cicatricial lower eyelid retraction is a challenging condition. It involves scarring of the lower eyelid, which causes it to retract and expose the sclera. This can lead to complications such as dry eye syndrome and corneal melting. It can be caused by trauma, burns, or previous eyelid surgery. Detailed assessment and understanding of eyelid anatomy and retraction are critical for successful surgical planning. Dynamic and static examinations of the eyelid including measurements of the lower eyelid margin reflex distance (MRD2) and scleral show are also essential to determine the appropriate treatment approach. METHODS: A systematic review was conducted using Medline, Scopus, and Cochrane databases with keywords related to cicatricial lower eyelid retraction. The publication language was limited to English after 2000. A total of 29 articles were included for data extraction and analysis. RESULTS: The main surgical techniques include tarsoconjunctival grafts, spacers, midface lift, and lateral canthal tendon suspension, although no single procedure has been universally recognized as the gold standard. New innovations such as synthetic grafts and xenografts are being explored for their potential in eyelid reconstruction. Severe cases, defined as those with inferior scleral show greater than 2 mm, may require a combination of reconstruction methods. CONCLUSIONS: Correcting cicatricial lower eyelid retraction is a major challenge in oculoplastic reconstruction. The surgical approach should be individualized, considering the pathologies and etiologies of lid retraction. In-depth knowledge and careful surgical planning are essential for best outcomes. There is no gold standard technique, and postoperative outcomes, complications, and management vary depending on the surgical approach used.

Pediatric orbital subperiosteal abscess outbreak in Iran: characteristics and causes.

PURPOSE: To evaluate demographics, characteristics, and management of pediatric patients with subperiosteal abscesses (SPA) secondary to orbital cellulitis and discuss the etiology of a dramatic rise in SPA. METHODS: Data were gathered by retrospective chart review of patients admitted to a tertiary referral eye hospital (Farabi Eye Hospital) diagnosed with orbital cellulitis with subperiosteal abscess from October 2022 to March 2023 (six months). Data on demographic information, clinical examination, radiographic evidence of sinusitis, orbital cellulitis, SPA, surgical and non-surgical management taken, isolated bacteria, and duration of hospital stay were gathered. RESULTS: 24 patients were admitted during these six months, with a diagnosis of orbital SPA secondary to paranasal sinusitis, confirmed by an orbital Computed Tomography (CT) scan. The age range was 11 months to 16 years. 75% of patients were male. All patients had a history of flu-like illness before developing orbital cellulitis. All patients had concurrent sinusitis, and 18 underwent initial surgical abscess drainage. The ethmoid sinus was the most involved, and most patients had a medially located SPA. Abscess volume ranged from 0.78 to 7.81 cm3 (mean: 3.52 cm3). One patient had concurrent central retinal artery occlusion due to orbital cellulitis. CONCLUSIONS: In this study, we report a dramatic increase in the incidence of SPA referred to our hospital. Larger abscess volumes and an increased number of cases that needed initial surgical drainage are also of note. An influenza outbreak in the autumn and winter, undiagnosed Corona Virus Disease 2019 (COVID-19) infection, increased antimicrobial resistance due to excessive off-label use of antibiotics during the COVID-19 pandemic, and more virulent bacterial infections are the most probable hypotheses to justify this observation.

The role of Whitnall's ligament position in the success of levator resection surgery in congenital ptosis.

PURPOSE: This study aimed to investigate the role of Whitnall's ligament position in the success of levator resection surgery in congenital ptosis. METHODS: It was an interventional case series on patients with congenital ptosis who underwent levator muscle resection in Farabi Eye Hospital (2020-2022). Patients with incomplete follow-up, a history of trauma, poor Bell's phenomenon, previous ocular and lid surgeries, poor levator function (≤ 4mm), and syndromic ptosis or systemic diseases were excluded. During the surgery, several factors, including the distance between Whitnall's ligament and the upper edge of the tarsus (W-distance), the vertical length of the tarsus (T-length), and the amount of levator muscle resection (LMR), were measured. A successful outcome was defined as the inter-eye difference of margin reflex distance-1 (MRD1) ≤ 1 and post-op MRD1 ≥ 3 OR the inter-eye difference of MRD1 ≤ 0.5 with any value of post-op MRD1 in unilateral cases and Postop-MRD1 > 3 in bilateral cases during the 3-months period. RESULTS: Thirty four eyes of 34 patients were included, and 79.4% of patients achieved successful outcomes. In univariate analysis, Preop-MRD1 and Preop-LF had meaningful negative correlations with the amount of LMR to reach the successful outcome (p < 0.05), which was only meaningful for Preop-LF in multivariable analysis (p < 0.05). Noticeably, W-distance had a significant positive correlation in univariate and multivariable linear regression (p < 0.05). CONCLUSIONS: W-distance can be considered a significant new parameter other than Preop-LF influencing the amount of levator resection needed to achieve success in levator resection surgery.

Sector area index: a novel supporting marker for blepharoptosis screening and grading.

PURPOSE: To introduce a new supporting marker for discriminating different grades of ptosis called Sector Area Index (SAI) and a semi-automated technique to calculate it. METHODS: In this cross-sectional comparative case series, a circle enclosing the intercanthal distance was automatically drawn after choosing two points as the medial and lateral canthus and manually selecting the palpebral fissure region. Finally, 15-degree apart sectors are applied to the enclosed circle. SAI was measured automatically by dividing the area of each 15-degree sector marked with the upper eyelid contour by the total area of the sector marked with the edge of the surrounding circle. SAI values and inter-eye SAI differences were compared between patients with different grades of ptosis as well as normal patients. RESULTS: In the current study, 106 eyes were recruited (30, 25, 27, and 24 in the control, mild, moderate, and severe ptosis groups, respectively). Mean values of SAI in all sectors showed a decreasing trend from normal individuals toward patients with severe ptosis. The mean difference values of SAI between study eyes and fellow eyes in all four groups of patients showed a statistically significant difference (p < 0.05). In a pairwise comparison between groups, mean values of SAI in all nasal sectors from 15° to 60° showed a statistically significant difference between all groups (p < 0.05). CONCLUSION: The mean difference of SAI between study eyes and fellow eyes, including eyelid curvature, especially in 15°-60° and 120°-165° sectors, can demonstrate differentiating performance for detecting and discriminating varying grades of ptosis.

Assessment of retinal and choroidal vessel density and nerve fibre layer thickness changes after orbitotomy in patients with severe non-active thyroid orbitopathy: a prospective study.

PURPOSE: To evaluate the optical coherence tomography angiogram changes in non-active severe thyroid-related ophthalmopathy patients after cosmetic bone decompression. METHODS: Eighteen patients (25 eyes) with severe not active not compressive (NANC) TED who were candidates for decompression surgery for cosmetic reasons were included in this study, and a 3 × 3 mm macular scan was used to measure vessel density and RNFL thickness. Whole macular vessel density in its superficial, deep and choriocapillaris layers was evaluated. The following data were extracted for each of layers: superior and inferior hemispheres, fovea, parafoveal vessel density, its superior and inferior hemispheres, and temporal, superior, nasal and inferior quadrant. RESULTS: The mean RPC increased postoperatively, which was statistically significant in small vessels of peripapillary area (p-value = 0.045). The mean RNFL thickness decreased after surgery and it was statistically significant in the peripapillary (p-value = 0.032) and Inferior-Hemifield area (p-value = 0.036). The choriocapillaris changes were significant in Superior-Hemifield (p-value = 0.031) and Fovea (p-value = 0.03). CONCLUSION: Thyroid-associated orbitopathy patients have a tendency to decrease vascular density and correlated with disease activity more than stage of orbitopathy. There was not a strong and even discrepant result in linkage of RNFL thickness and other optic nerve function tests and TED patient status and it is needed to do studies with more epidemiologic power and same methodology of study to be more comparable.

Orbital histiocytosis and fibrohistiocytosis: the clinicopathological characteristics of 117 patients, over a decade of experience.

PURPOSE: To describe the clinicopathological features of a large cohort of patients with orbital histiocytoses and fibrohistiocytosis, such as Langerhans cell histiocytosis (LCH) and non-LCH disorders, and correlate patients' clinical characteristics with their pathological diagnosis. METHODS: In this retrospective study, medical records of patients presenting to Farabi Eye Hospital, a tertiary eye care center in Tehran, Iran, from 2010 until 2022, were reviewed. Patients' demographics, chief complaint, location and laterality of the tumor, best-corrected visual acuity, presence of bone erosion on imaging, and their pathological diagnosis were retrieved. Excisional biopsy was performed and evaluated through light microscopy and immunohistochemistry study for their respective markers, including CD1a, CD68, CD207, and S100. RESULTS: A total of 117 patients with 11 pathological subtypes of histiocytoses and fibrohistiocyosis were identified, with 56.4% male and 43.6% female patients. The mean age at presentation was 23.4 years (range 1.5 months-73 years). Swelling and palpable mass were the most common chief complaints. LCH was the most common pathology (32.5%), followed by juvenile xanthogranuloma (26.5%) and adult xanthogranuloma (21.4%). Age, lesion location, and bone erosion had a statistically significant difference among the various diagnosed subtypes. CONCLUSIONS: Histiocytoses and fibrohistiocytosis are diverse and rare disorders potentially involving multiple organ systems. Ophthalmic manifestations of these diseases are even more uncommon. We reviewed their orbital presentation along with their respective histopathological findings. Our results also suggested that an orbital CT scan can be of diagnostic value to discriminate LCH from other histiocytic pathologies.