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.

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.

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.

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.

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.

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.

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.

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.

A novel technique for small-incision levator resection with a double mattress suture for ptosis correction.

PURPOSE: To introduce a novel technique for small-incision levator resection in ptosis surgery and evaluate its efficacy in a pilot study among patients affected by congenital or aponeurotic ptosis. METHODS: We prospectively enrolled congenital and aponeurotic ptosis patients if their levator function was not poor (≥5 mm) from June 2021 through October 2022. Surgical technique involved a 1-cm lid crease incision, minimal dissection, and creating a loop passing through the tarsus and levator aponeurosis. Success was defined as postoperative MRD-1 ≥3 mm and inter-eyelid MRD-1 difference ≤1 mm. Eyelid contour quality was scored excellent, good, fair, and poor according to its curvature and symmetry. RESULTS: Sixty-seven eyes (35 congenital and 32 aponeurotic) were included in the study. Mean age was 34±19 years (range, 5-79 years). Mean preoperative levator function and levator resection amount were 9.53 mm and 8.39 mm in the congenital group and 12.34 mm and 4.15 mm in the aponeurotic group, respectively. Mean pre- and postoperative MRD-1 was 1.61 mm and 3.27 mm respectively (P<0.001). The overall success rate was 82.1% (95% C.I: 71.7-89.8%); the result was failure in 12 cases, of which 11 had under-correction. Preoperative MRD-1 was correlated with a success rate (P=0.017). CONCLUSION: The described technique shows non-inferior results to the previously described surgical methods and also it shows very good lid contour outcome and minimal lag. The findings suggest that the double mattress single suture technique can be used in both congenital and aponeurotic ptosis.

Corneal biophysical changes after upper eyelid blepharoplasty and ptosis surgery: a review.

Upper eyelid surgeries, such as blepharoplasty and ptosis correction, are commonly performed procedures worldwide. This review examines the effects of these surgeries on ocular properties and visual function. A search of the PubMed and Google Scholar databases was conducted to identify relevant articles published after 2000. The results demonstrate that the ocular and adnexal organs function as a unified visual system, with changes in one component affecting the functions of others. Eyelid surgery can alter ocular properties and functions by modifying retinal lighting and ocular optics. These alterations can affect intraocular pressure estimation, corneal curvature, corneal epithelial thickness, refractive power of the cornea, and intraocular lens calculation. Additionally, eyelid surgery can exacerbate dry eye symptoms and impact contrast sensitivity, which is a significant factor in visual quality. Therefore, understanding these interactions is crucial before performing eyelid surgery and during follow-up. This review summarizes recent literature on the effects of upper eyelid surgery on corneal properties and visual function, emphasizing the importance of considering these factors when planning or undergoing such procedures.

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.

Anaplastic Large Cell Lymphoma With Intraocular or Ocular Adnexal Involvement: A Case Report and Systematic Review.

PURPOSE: To review the existing literature on patients with anaplastic large cell lymphoma (ALCL) affecting the globe and/or ocular adnexa, and to present a report documenting the clinical course of a patient with ALCL that involved their globe and ocular adnexa. METHODS: PubMed, Scopus, and Google scholar were systematically searched for all cases of ALCL involving intraocular or adnexal ocular structures from inception to May 2023. Moreover, a new reported case added to the cases found in searches. RESULTS: The review identified 1680 studies, with 8 meeting inclusion criteria. A total of 9 patients were included with a mean age of 29.7 years (median: 30.0, range: 1.3-48). Primary ALCL was present in 5/9 (55.6%) patients. The most common ophthalmic manifestations included periorbital swelling (5/8), chemosis (5.8), and decreased vision (5/7). Misdiagnoses were initially made in 3 patients, and the lag time to correct diagnosis from 3 weeks to 3 months. CD30 expression was positive in all cases, and 6/9 patients were positive for anaplastic lymphoma kinase, resulting in 6/9 patients being diagnosed with anaplastic lymphoma kinase-positive ALCL. In terms of management modalities, chemotherapy was administered in 8/9 patients, while radiation therapy was utilized in 4/9 patients, and 2 underwent autologous stem cell transplantation. Five (55.6%) patients succumbed to ALCL while 4 (44.4%) were alive and disease-free at the last follow-up. The median times from the initial presentation of ALCL to death, ophthalmic presentation to death, and diagnosis to death were 4.12 months (range: 1.1-168.0), 2.62 months (range: 1.1-144), and 4.00 months (range: 0.10-168), respectively. The median follow-up duration was 21.0 months (range: 1.1-168.0). CONCLUSIONS: ALCL involving the globe and ocular adnexa is a rare and highly malignant tumor that can mimic benign clinical conditions. Early biopsy and aggressive treatment with chemotherapy regimens such as CHOP and radiation therapy may be useful.

Unexpected recurrence and rapid progression of lacrimal gland adenoid cystic carcinoma during pregnancy: a case report.

Adenoid cystic carcinoma (ACC) is the most common malignant neoplasm involving the lacrimal glands, with high rates of recurrence and metastasis. During the pregnancy, reports of recurrence of ACC of the salivary glands and trachea have previously been published, but no lacrimal gland ACC recurrence has been reported. We present a 35-year-old woman with lacrimal gland ACC who was initially treated by surgical resection and adjunctive radiotherapy, but her cancer recurred during pregnancy, with rapid progression to cavernous sinuses and brain. Estrogen and progesterone receptors have been detected on lacrimal glands and ACCs of salivary glands. Thus, hormonal changes during pregnancy might contribute to the recurrence of ACC. However, the inherent invasive and recurrent nature of ACC could also account for the regrowth in this patient and further molecular studies can provide more accurate explanations.

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.

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.

COVID-19 associated rhino-orbito-cerebral mucormycosis, risk factors and outcome predictors; a multicentric study.

BACKGROUND: Since the onset of the Covid-19 pandemic, an increase in mucormycosis cases has been observed in many countries, including Iran. However, the role of covid-19 and associated risk factors have not been thoroughly investigated. OBJECTIVE: This study is designed to identify epidemiologic characteristics, risk factors, and outcome predictors of Covid-19-Associated Rhino-Orbito-Cerebral Mucormycosis (C-ROCM). METHODS: Data of pathology proven Covid Associated ROCM cases were retrospectively obtained from 7 tertiary care centers throughout Iran from February 20, 2021, to July 22, 2021. Univariate and multivariate analyses were performed using binary logistic regression to assess the effects of various factors on the outcome. RESULTS: A total of 132 patients with C-ROCM were included in the study. The mean age of patients was 61.6 ± 13.9 (60.6% male). In 12 patients (9.1%), both eyes were involved. Diabetes was the most common comorbidity (94.7%). The mortality rate was 9.1%, higher in males (12.5%) than females (3.8%). Severe vision impairment was seen in 58 patients (43.9%). Main factors that had a negative impact on the outcome in the univariate analysis include older age (P < 0.001), higher steroid dosage (P < 0.001), higher HbA1c level (P < 0.001), Covid-19 severity (P < 0.001), and brain involvement (P < 0.001). However, in the multivariate analysis, the effects of age (P = 0.062), steroid dosage (P = 0.226), and Covid-19 intensity (P = 0.084) decreased, and the difference was no longer statistically significant. CRAO was a predictor of mortality in the univariate analysis (P = 0.008, OR = 4.50), but in the multivariate analysis, this effect decreased and was no longer significant (P = 0.125). CONCLUSION: The risk of C-ROCM and its complications may increase in patients with more severe Covid-19, steroid over-prescription, ICU admission due to Covid-19, and poor glycemic control during and after Covid-19 treatment.

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.

Buccal mucosal membrane graft for correction of cicatricial lower eyelid retraction.

BACKGROUND: To evaluate the outcomes of a surgical technique using buccal mucosal membrane graft for correction of cicatricial lower eyelid retraction. METHODS: Twelve patients with unilateral cicatricial lower eyelid retraction were enrolled in the study. All patients underwent a four-step surgical technique consisted of release of scars, midface lift, transfer of buccal mucosal membrane to posterior lamella as spacer graft, and canthal tightening. All patients were followed for at least 12 months. RESULTS: Mean preoperative Margin-to-Reflex-Distance 2 (MRD2) was 7.73 ± 1.10 mm, compared to mean postoperative MRD2 of 5.04 ± 0.49 mm (P < 0.0001). The mean improvement in retraction was 2.69 mm. Postoperative scleral show was present in only one case and no major complications were observed. CONCLUSION: The four-step procedure (scar release, midface lift, buccal mucosal graft and canthal tightening) was an effective procedure to correct cicatricial lower eyelid retractions with acceptable outcomes and a low morbidity rate.

Phakomatous Choristoma of the Eyelid and Orbit: A Case Report.

Phakomatous choristoma was first described as a distinct pathologic entity by Zimmerman in 1971. Report of only 26 cases of this tumor so far is an indicator of the rarity of phakomatous choristoma. We present a 4-month-old infant with an orbital mass beneath the right lower eyelid. Surgical excision was undertaken and the histopathologic findings of a dense fibrocollagenous stroma containing small to medium size islands and glandular-like structures surrounded by thick basement membrane and filled by amorphous eosinophilic material confirmed the diagnosis. Immunohistochemical study showed positive staining for S-100 and vimentin and negative staining for cytokeratins, glial fibrillary acidic protein, smooth muscle actin, synaptophysin, CD34, melan-A, and epithelial membrane antigen markers. This is the first patient with phakomatous choristoma presented from our country and the 27th reported case worldwide. Phakomatous choristoma is a rare, benign congenital tumor of lenticular anlage, almost always presenting in the medial lower eyelid and anterior orbit. Surgical excision is curative and allows precise diagnosis due to the unique histopathologic and immunostaining characteristics of this choristoma.