Ophthalmic histiocytic lesions: a baseline demographic and clinicopathological study of 28 cases from two eye centers.

PURPOSE: Ophthalmic histiocytic lesions comprise a heterogeneous rare group of disorders that are characterized by an abnormal proliferation of histiocytes and may affect all age groups of both sexes. The aim of this study was to highlight the basic demographic, clinical, and histopathological characteristics of this rare group of diseases in ophthalmic practice, which has not been previously studied in this area. Only individual cases have been previously reported. METHODS: This was a retrospective study of all biopsied ocular and periocular histiocytic lesions from two centers, King Khaled Eye Specialist Hospital (KKESH) and King Abdulaziz University Hospital (KAUH) in Riyadh, Saudi Arabia, from January 1993 to December 2018. The histopathological diagnosis was confirmed, and the cases were re-classified by reviewing all histopathological slides. The corresponding demographic and clinical data were analyzed. A relevant literature review was also carried out for comparison of our collected analyzed data to published data and to draw our own conclusions. RESULTS: A total of 34 ocular/periocular histiocytic lesions in 28 patients who were mostly Saudis (92.9%) were included. The male-to-female ratio was 4:3. The median age at presentation was 6.4 years (range: 2.8-35 years). Twenty-two patients had unilateral involvement, and six patients had bilateral lesions. In patients with Langerhans cell histiocytosis (LCH; L group), the most common presenting findings were eyelid swelling (75%), periocular tenderness (37.5%), proptosis/globe displacement (37.5%) eyelid erythema (25%), and orbital pain (12.5%). In patients with Rosai Dorfman disease (RDD; R group), proptosis/globe displacement occurred in all patients and 80% had decreased vision. Patients in the C group (Cutaneous non-LCH histiocytoses) had variable clinical features because of the different locations of the histiocytic lesions, with the majority involving the eyelids (66.7%). Diagnosis was accurately reached clinically in 38.8%, 33.7%, and 46.7% of patients in the L, C, and R groups, respectively. Overall, the clinical diagnosis was in concordance with the histopathologic diagnosis in 14 out of 34 lesions (41.2%). CONCLUSIONS: Histiocytic disease is more likely to be overlooked clinically owing to its rarity. In the C group, juvenile xanthogranuloma (JXG) was the most commonly encountered histiocytic lesion and had a tendency to present at a later age with extremely rare intraocular involvement in contrast to previously published reports. The median age at presentation was higher in group R. All patients in group L had strictly unilateral disease, while RDD (group R) was most commonly bilateral. Future research on genetic aspects, management, and prognosis is necessary.

Involvement of Multiple Trigeminal Nerve Branches in IgG4-Related Orbital Disease.

PURPOSE: To describe the occurrence of multiple trigeminal nerves (TGNs) enlargement in patients with orbital IgG4-related disease. METHODS: Retrospective review of MRI findings and medical records of 6 patients (10 orbits) with orbital IgG4-related disease and enlargement of more than 1 TGN. Orbital biopsies were performed in all cases revealing the typical lymphoplasmacytic infiltrate with significant plasma cell positivity for IgG4 (IgG4+/IgG ratio ≥ 40%). Three experienced neuroradiologists reviewed the MRI sequences using a digital imaging viewer system (Horos, https://horosproject.org/). RESULTS: Bilateral involvement of at least 2 TGNs divisions was detected in all 6 patients. Enlargement of both V1 and V2 nerves was diagnosed in 5 patients, and in 3 cases, all TGN divisions were involved. V2 nerves were the most affected. In this division, all 12 infraorbital nerves were enlarged, followed by lesser palatines (10/83.3%), superior alveolar (10/83.3%), and zygomatic (6/50%). V1 and V3 nerves were less affected albeit 9 (75%) frontal branches (V1), and 50% of the inferior alveolar (V3) nerves were also enlarged. CONCLUSIONS: Widespread involvement of the TGN is an important feature of IgG4-related disease.

Histopathological assessment of optic nerve invasion guided by radiological findings in enucleated globes with retinoblastoma.

BACKGROUND: Optic nerve (ON) invasion is an important high-risk feature, and an indicator for neoadjuvant chemotherapy and prognosis. We aim through this study to correlate the detected-ON invasion by Magnetic resonance imaging (MRI) with the corresponding confirmed histopathological level of invasion. METHODS: A retrospective study of enucleated globes with the diagnosis of retinoblastoma received in the histopathology department(s) from January 2015 to December 2016 (2 years). Slides were reviewed for ON invasion assessment, charts were reviewed for basic demographic data. All patients underwent MRI under sedation upon diagnosis and MRI findings were collected for the above correlation. RESULTS: A total of 38 patients were included: 21 males and 17 females. 29 (77.3%) had unilateral involvement, 7 (18.4%) had bilateral involvement and 2 cases had trilateral disease. The overall mean age at diagnosis was 22.63 ± 15.15 months. Histopathological examination revealed ON invasion in 28 cases (74%) distributed as follows: prelaminar (31.6%), laminar (18.4%), and post-laminar (23.7%). MRI confirmed post-laminar ON invasion in 8 cases (true positive) but failed to detect this in 1 case. Additionally, MRI detected another 8 cases of ON invasion that were false positive on histopathology (accuracy: 63.3%; sensitivity: 88.9%; specificity: 72.4%; Positive predictive value (PPV): 50%; Negative predictive value (NPV): 95.5%). CONCLUSIONS: MRI is found to be less sensitive in evaluating prelaminar and laminar ON invasion (0.0 and 42.9%) compared to post-laminar invasion (88.9%). MRI has generally better specificity in detecting ON invasion irrespective of the invasion level. In our study, obtaining deeper and/or additional histologic sections from the other surface of the tissue block in cases where a post-laminar ON invasion by MRI is found but not confirmed histopathologically in routine sections is essential to avoid missing such an important high-risk feature.

Whipple's disease scleral nodules: a novel presentation in 2 consecutive patients.

BACKGROUND: Whipple's disease (WD) is a rare, chronic, infection caused by gram-positive filamentous aerobic actinobacterium Tropheryma whipplei occurs classically in the gastrointestinal tract and shows histopathologically foamy macrophages with typical numerous PAS-positive, non-acid fast particles. Ocular WD in the form of uveitis may occur in the absence of systemic disease but has not been reported to present with scleral manifestation. We describe for the first time to the best of our knowledge 2 cases of scleral nodules with typical histopathological morphology of WD and without systemic involvement. CASE PRESENTATION: The first was a 53-year old diabetic male farmer who presented with 2 nontender right eye scleral nodules for 3 months, had a negative systemic workup, and surgical excision showed Periodic acid Schiff (PAS)-positive eosinophilic structures inside macrophages. Grocott's methenamine silver (GMS) stain and acid-fast bacilli (AFB) stain of the tissue itself were negative. The second case was a 60-year old male who presented with an asymptomatic superior scleral nodule for 4 months, which showed similar appearance and negative GMS and AFB stains. CONCLUSION: WD should be included in the differential diagnosis of scleral nodules even in the absence of systemic symptoms. Surgical excision without systemic treatment resulted in successful outcome without recurrence.

Krause's accessory lacrimal gland dacryops - case report and literature review.

The authors are describing two rare cases of dacryops involving the Krause accessory lacrimal glands and affecting the orbit. In both cases, the lesion was located in the inferior fornix, with no preceding trauma, surgery or trachomatous conjunctival scarring. The first case involved a clear orbital extension with evidence of optic nerve compression. The second case extended from the anterior orbit to the limbus. Both had surgical removal with good outcome. As Krause gland dacryops are similar to other orbital cysts, clinical, imaging and histopathologic evaluation are essential to confirm the final diagnosis.

Clinical features differentiating intratarsal keratinous cyst from chalazion.

PURPOSE: To present the differentiating clinical findings between intratarsal keratinous cyst (IKC) and chalazion. METHOD: A retrospective review of medical records of all patients who presented between 2010 and 2018 at King Abdulaziz University Hospital with proven histopathological diagnosis of IKC was done. Complete ophthalmologic evaluation at presentation, surgical procedures performed, complications, histopathological findings, response to treatment and follow-up were recorded. RESULTS: Twelve patients were found to have IKC. All patients presented with an eyelid mass with no signs of local inflammation. All lesions were fixed to the tarsus with freely mobile overlying skin, which was found to be slightly pale compared to the surrounding skin in six patients. On palpation, IKC had well-defined boarders. Isolation with clear surgical plane for cyst excision was achieved in nine patients as they were superficially involving the tarsus. When IKC involved the deep part of the tarsus, bluish/ whitish nodules were seen upon eyelid eversion. Six patients were misdiagnosed and surgically treated as a chalazion elsewhere prior to presentation to us with recurrence. CONCLUSION: Differentiating IKC from chalazion can be challenging. Careful clinical evaluation helps reaching the right diagnosis and providing the correct treatment, which involves complete excision of IKC to prevent recurrence.

High-risk features in primary versus secondary enucleated globes with advanced retinoblastoma: a retrospective histopathological study.

PURPOSE: The management of bilateral advanced retinoblastoma (RB) cases is challenging with attempts to use neoadjuvant therapy salvaging of one of the globes. Our aim in this study was to demonstrate the effect of this primary therapy on the histopathological features and risk factors in secondary enucleated compared to primarily enucleated globes with groups D and E RB. METHODS: We retrospectively reviewed all enucleated globes with advanced RB received in the pathology laboratories over a period of 5 years. Patients were divided into two groups: one with primary enucleations and another with at least one secondary enucleated globe, and their demographic and clinical data were analyzed. The enucleated globes in the two groups were analyzed to compare the general histopathological features including tumor seeding, size, differentiation, growth pattern, mitotic figures, and focality. More importantly, high-risk features: choroidal invasion, optic nerve (ON) invasion, iris/anterior chamber invasion, ciliary body invasion, and scleral and extra-scleral extension, as well as the pathological classification of the tumor (pT) according to the American Joint Committee on Cancer 7th edition were also compared between the two groups. RESULTS: We had a total of 106 enucleated globes (78 primary and 28 secondary enucleations) from 99 patients with advanced RB (73 patients with primarily and 26 with secondarily enucleated globes). Demographic and clinical profiles of patients were similar in both, but the mean interval from presentation to enucleation was significantly longer in the secondary enucleations (P = 0.015). Rare/occasional mitotic figures were observed in secondary enucleations using multivariate analysis (P = 0.003). Primarily enucleated globes had higher risk of tumor seeding (P = 0.020), post-laminar/surgical margin ON invasion (P = 0.001), and massive choroidal invasion (P = 0.028). Half of the secondary enucleated globes had tumors confined to the globes without invasion (pT1) and statistically significant lower tumor classifications (pT1 or pT2a) compared to primary enucleations (P =0.001). However, 18% of the secondarily enucleated globes in 3 patients had unfavorable outcome with RB-related mortality after a period of 1-4 years. CONCLUSIONS: Secondary enucleated globes with advanced RB show favorable histopathological findings mainly less mitosis. These eyes have significantly lower chance for harboring choroidal and ON invasion, thus mostly classified as pT1 or pT2a when compared to primarily enucleated globes. The decision for secondary enucleation was observed to be significantly delayed (8.0 months ± 9.8). Prompt decision for needed enucleation based on the response to primary treatment and careful histopathological examination of enucleated globes are essential to prevent disease-related mortality.

Recurrent Orbital Inflammation Mimicking Orbital Cellulitis Associated With Orbitopalpebral Venous Lymphatic Malformation.

An 8-year-old boy initially presented with an orbitopalpebral mass diagnosed clinically and radiologically as a low-flow diffuse venous lymphatic malformation involving the left upper eyelid and orbit. Over 8 months of follow up, he had 2 acute episodes of severe orbital inflammation that warranted hospitalization and treatment with intravenous antibiotic, steroids and surgical debulking. After a third surgical excision, the lesion remained clinically stable.

Orbital pseudotumor can be a localized form of granulomatosis with polyangiitis as revealed by gene expression profiling.

Biopsies and ANCA testing for limited forms of granulomatosis with polyangiitis (GPA) are frequently non-diagnostic. We characterized gene expression in GPA and other causes of orbital inflammation. We tested the hypothesis that a sub-set of patients with non-specific orbital inflammation (NSOI, also known as pseudotumor) mimics a limited form of GPA. Formalin-fixed, paraffin-embedded orbital biopsies were obtained from controls (n=20) and patients with GPA (n=6), NSOI (n=25), sarcoidosis (n=7), or thyroid eye disease (TED) (n=20) and were divided into discovery and validation sets. Transcripts in the tissues were quantified using Affymetrix U133 Plus 2.0 microarrays. Distinct gene expression profiles for controls and subjects with GPA, TED, or sarcoidosis were evident by principal coordinate analyses. Compared with healthy controls, 285 probe sets had elevated signals in subjects with GPA and 1472 were decreased (>1.5-fold difference, false discovery rate adjusted p<0.05). The immunoglobulin family of genes had the most dramatic increase in expression. Although gene expression in GPA could be readily distinguished from gene expression in TED, sarcoidosis, or controls, a comparison of gene expression in GPA versus NSOI found no statistically significant differences. Thus, forms of orbital inflammation can be distinguished based on gene expression. NSOI/pseudotumor is heterogeneous but often may be an unrecognized, localized form of GPA.

Collagen Fibrils and Proteoglycans of Macular Dystrophy Cornea: Ultrastructure and 3D Transmission Electron Tomography.

We report the ultrastructure and 3D transmission electron tomography of collagen fibrils (CFs), proteoglycans (PGs), and microfibrils within the CF of corneas of patients with macular corneal dystrophy (MCD). Three normal corneas and three MCD corneas from three Saudi patients (aged 25, 31, and 49 years, respectively) were used for this study. The corneas were processed for light and electron microscopy studies. 3D images were composed from a set of 120 ultrastructural images using the program "Composer" and visualized using the program "Visuliser Kai". 3D image analysis of MCD cornea showed a clear organization of PGs around the CF at very high magnification and degeneration of the microfibrils within the CF. Within the MCD cornea, the PG area in the anterior stroma was significantly larger than in the middle and posterior stroma. The PG area in the MCD cornea was significantly larger compared with the PG area in the normal cornea. The CF diameter and inter-fibrillar spacing of the MCD cornea were significantly smaller compared with those of the normal cornea. Ultrastructural 3D imaging showed that the production of unsulfated keratin sulfate (KS) may lead to the degeneration of micro-CFs within the CFs. The effect of the unsulfated KS was higher in the anterior stroma compared with the posterior stroma.

Peri-ocular proliferative apocrine hidrocystoma (cystadenoma): A clinicopathological case series.

INTRODUCTION: Apocrine cystadenomas are rare, benign tumors that might arise in the periocular region from glands of Moll. They characteristically demonstrate proliferative features on histopathological examination, which differentiate them from simple hidrocystomas. PRESENTATION OF CASES: We retrospectively identified 4 consecutive cases of apocrine cystadenomas in male patients with a mean age of 48.5 years (range 20-62). One of the cases was a recurrent lesion. The Preoperative clinical diagnosis was mostly hidrocystoma. All patients agreed on complete surgical excision of their cystic lesions. Histopathological review of the excised cysts confirmed the diagnosis of apocrine cystadenoma based on the presence of proliferative features with no atypia or infiltrative behavior. Cases are summarized in Table 1 and the histopathological appearance is demonstrated in the included figure. DISCUSSION: Apocrine cystadenoma is rare. It occurs in the areas of skin with hair follicles, such as the axilla, neck, and trunk, and may be mistaken for other skin lesions, such as nevi or syringomas. In the eyelid region, they are commonly missed and frequently diagnosed as simple hidrocystoma due to the presence of bluish hue such as in our series. Recurrence is rare but was found in one of our patients at initial presentation. Our series included unique locations of this lesion in the medial canthus in one patient and near the eyelid tarsus in another. CONCLUSION: Ophthalmologist should be aware of the rare occurrence of this lesion in the periocular region. Further studies to explain the etiology of such proliferative nature in apocrine cysts would be interesting.

Variable presentations of six conjunctival/limbal ocular surface squamous neoplasia (OSSN) cases: How good is our clinical judgment evidenced by the correlation to the histopathological findings and diagnosis?

INTRODUCTION: Ocular surface squamous neoplasia (OSSN) may have atypical or unusual presentations and may attain large sizes especially in cases of delayed presentation resulting in late diagnosis, treatment, and eventual guarded visual prognosis. We are reporting an interesting cases series of OSSN with variable clinical presentations to highlight the importance of the pre-operative clinical judgment and tissue diagnosis. PRESENTATION OF CASES: Six patients (4 females and 2 males; mean age 59 years; range 42-79 years) were included with suspicious conjunctival lesions. The maximum dimension of the lesions was 17 mm. The initial suspected pre-operative clinical diagnosis -other than OSSN- included pterygium/pinguecula (n = 2), benign squamous papilloma (n = 1), cyst versus pyogenic granuloma (n = 1), and lymphoma (n = 1). This work has been reported in line with the PROCESS criteria. DISCUSSION: The final histopathological diagnosis was unexpectedly invasive squamous cell carcinoma (SCC) in 4, one SCC in-situ, and squamous dysplasia in one. The primary treatment included MMC 0.02 % for 2 cycles for chemo-reduction in one of the cases where OSSN was suspected. Excisional biopsy was performed eventually for all lesions with application of one or more of the following modalities: MMC 0.02 %, absolute alcohol 99 %, and cryotherapy to the conjunctival margin. No tumor recurrence was noted in any of the patients after an average period of follow-up of 26 months. Even though the outcome was good, tumor-related morbidity and delay in the referral by general ophthalmologists are to be considered. CONCLUSION: Conjunctival OSSN has wide presentation clinically and can be challenging in terms of diagnosis. Histopathological evaluation is essential for a definitive diagnosis and treatment. Accurate clinical diagnosis might affect the management plan with consideration for topical therapeutic modalities, however, these cases are best managed by wide excision using the no-touch technique and double-freeze-thaw cryotherapy to the conjunctiva with consideration of topical chemotherapy.

Corneal myxoma following micro-pulse cyclophotocoagulation in a young female: A case report.

INTRODUCTION: Corneal myxoma is a rare benign tumor that can occur in the cornea where the exact cause remains unknown. However, it is thought to be a reactive process that can be caused by corneal infections, ectasia, ocular trauma, or surgery. PRESENTATION OF CASE: In this case report, we describe a 35-year-old-woman who presented with corneal myxoma after micro-pulse cyclophotocoagulation (MPCPC). The patient presented with decreased vision due to a large pedunculated white corneal mass after being treated with MPCPC as a non-surgical management of her pre-existing glaucoma. The corneal mass was localized to the sub-epithelial space and was excised successfully by a minimal invasive procedure without recurrence over a 1-year period. DISCUSSION: Corneal myxomas are unusual benign tumors typically seen in adults as whitish gelatinous lesion. Only a few individual cases and case series have been reported in the literature. The exact pathogenesis is yet to be known. The lesion is thought to be due to an inflammatory process. We are reporting a case of corneal myxoma that has occurred after MPCPC which is a non-surgical cyclodestructive procedure. The procedure has not been mentioned previously as a risk factor for corneal myxoma. Our case is the first corneal myxoma developing after MPCPC. CONCLUSION: We report a corneal myxoma noted in a young female after a non-surgical laser procedure. Though the lesion is rare, it should be considered in physicians' differential of a corneal mass especially in the presence of chronic ocular.

Case report: Clinical features and management outcomes of isolated corneal intraepithelial neoplasia.

Purpose: To report clinical features and treatment outcome of three cases with isolated corneal intraepithelial neoplasia (CIN). Methods: This case series presents 3 patients with isolated CIN. Data collected included, presenting signs and symptoms including vision, anterior segment examination, medical and surgical outcomes and signs and symptoms at lost post-treatment visit. Results: Case 1 was a 45-year-old male who presented with an isolated grayish amoeboid corneal lesion which was excised with alcohol assisted epitheliectomy, he also received 6 cycles of topical mitomycin C (MMC) 0.02% and one injection of interferon alfa-2b with no recurrence during the 10-year follow-up period. Case 2 was 78-year-old male referred for a suspicious white corneal lesion which was completely excised, the patient also received 6 subconjunctival injections of interferon alpha-2b. However, the lesion recurred at 2.5-years post-treatment. Case 3 was a 63-year-old male patient who presented with an isolated corneal lesion that was excised using alcohol-assisted epitheliectomy, patient received four cycles of topical 5-fluorouracil with no recurrence at last follow-up visit at 6 months. Conclusion: Isolated corneal intraepithelial neoplasia (CIN) is a rare entity with few reported cases in the literature. In this case series, we report long and short-term management outcomes of combined surgical and medical therapy for isolated CIN.

An advanced case of pediatric ciliary body medulloepithelioma with detailed literature review.

INTRODUCTION AND IMPORTANCE: Medulloepithelioma is the second most common primary intraocular malignant tumor in children but is often diagnosed late, which worsens the prognosis. CASE PRESENTATION: We are reporting a 6-year-old boy presenting with a ciliary body (CB) teratoid malignant medulloepithelioma (TMM), which was missed at the initial presentation. We added our case to the 97 previously reported cases in our literature review that were confirmed by cytological or histopathological examination. DISCUSSION: Medulloepithelioma has a wide age range at presentation with a mean of 5.3 ± 4.1 years, and slight male predominance (M: F ratio of 1.15). Clinically, the main symptoms/ signs are reduced visual acuity, lens changes, or a pupil "mass." Almost all reported medulloepitheliomas were unilateral (98 %). Histopathologically, non-teratoid malignant medulloepithelioma (NTMM) (35.7 %) and TMM (34.5 %) were the commonest. Enucleation was the main treatment modality because of the large tumor size of 72.7 %. A combination of chemotherapy and/or radiotherapy was used in 15.1 % and brachytherapy for small lesions in 14.1 %. Orbital exenteration was needed in 3 cases because of orbital invasion. CONCLUSION: A CB medulloepithelioma tumor can be easily missed, diagnosed late, with less chance for globe salvaging. The malignant types are more common and may result in orbital invasion, requiring even more extensive surgery. Therefore, when facing a child with lens changes, a pupil "mass," or raised intraocular pressure, pediatricians and general ophthalmologists must have an earlier suspicion and be aware of this rare entity to improve outcomes and reduce loss of vision or other disease-related morbidity.

Giant cell temporal arteritis: a clinicopathological study with emphasis on unnecessary biopsy.

Introduction: Temporal artery (TA) biopsy is commonly used for the diagnosis of giant cell arteritis (GCA). However, a positive biopsy is no longer mandatory for diagnosis. This study aims to correlate the histopathological findings of TA biopsies in suspected cases of GCA to the clinical presentation in an ophthalmic tertiary eye care center to draw useful conclusions and advocate the possible implementation of guidelines for TA biopsy. Methods: Data was collected from patients' medical records including, demographics, clinical data, and histopathological findings and diagnosis. The 2022 American College of Rheumatology/ European Alliance of Associations for Rheumatology (ACR/EULAR) criteria have been used and partially adopted as a guide to compare the variables between TA biopsy-positive and negative groups as well as the TA biopsy-positive group and the group of patients with TA biopsy showing atherosclerosis. Results: Out of the total 35 patients who underwent a TA biopsy during the period of 23 years, 22.9% of patients had histopathological findings consistent with GCA and 42.9% had TA atherosclerotic changes, while the remaining 34.3% had histologically unremarkable TA. The mean age of all patients was 66 ± 10.9 years. Slightly more than half were females (54.3%) and the remaining were males (45.7%). In the group with positive TA biopsies, the mean age was 71 ± 8.4 years with a higher female predominance (female-to-male ratio of 5:3). The mean diagnostic clinical score used in our study was higher (7.5 ± 2.33) in the GCA-positive group when compared to the other groups with statistical significance (mean of 4.85 ± 2.01 in patients with overall GCA-negative biopsies and 5.13 ± 2.10 in the group with atherosclerosis). Other three clinical variables that were found to be statistically significant in the GCA biopsy-positive group were scalp tenderness, jaw claudication, and optic nerve pallor. Discussion: The mean age (71 ± 8.4 years) and the female predominance of GCA in our group of patients with positive TA biopsy (62.5%) was like other reports. In our study 22.9% of performed TA biopsies over the period of the study were positive confirming the diagnosis of GCA on histological exam, which was similar to another report and is considered to be relatively low. The incorporation of increased clinically focused assessments and algorithms, with the aid of the ACR/EULAR criteria, may decrease the frequency of TA biopsies that carries unnecessary cost and risk of procedure-related morbidity. We highly recommend applying the age of ≥ 50 years as an initial criterion for diagnosis, followed by the consideration of the statistically significant clinical features: scalp tenderness, jaw claudication, and optic nerve pallor.

Ophthalmic histiocytic lesions (diseases of the L group): A multicenter clinicopathological study of 18 cases and review of literature.

INTRODUCTION: Langerhans cell histiocytosis (LCH) and Erdheim-Chester disease (ECD) are rare histiocytic disorders in the L (Langerhans) group diseases. They range from self-limited benign diseases to lethal disseminated forms. METHODS: This retrospective study was conducted in 3 tertiary hospitals in Saudi Arabia and Brazil. Histopathological records were searched for all patients diagnosed with ocular and periocular histiocytic disorders from January 1993 to December 2018. Histopathological slides and medical files were reviewed for data collection and simple analysis of demographics, clinical manifestations, and management. The relevant literature is reviewed. RESULTS: Twenty-two eyes of 18 patients with biopsy-proven histiocytic disorders in the L group were included. Female-to-male ratio was 1.25:1. Average age at presentation was 14 years (range, 1-54). LCH was diagnosed in 14 eyes, while eight eyes had ECD. All LCH cases were unilateral and confined to the bone as cases of eosinophilic granuloma (EG), while patients with ECD were bilateral. Commonest presentations in EG and ECD were eyelid swelling (85.7%) and periocular xanthomas (75%), respectively. Orbit was involved in 100% of EG cases, with bony erosion in 54.5%. Relevant systemic involvement was found in 100% of ECD and 21% of EG cases. Surgical intervention was needed in 16 of the 22 eyes (72.7%). All EG and 25% of patients with ECD required surgical excision. CONCLUSIONS: Histiocytic disorders are a rare group of diseases, including the L group. Relevant systemic associations require specific and selective therapy. A high clinical index and multidisciplinary collaboration are essential for the proper evaluation and management of these patients.

Successful management of a retained host Descemet's membrane after penetrating keratoplasty (PKP) - A case report.

INTRODUCTION AND IMPORTANCE: To report the 21st case showing the rare occurrence of retained Descemet's membrane (DM) following penetrating keratoplasty (PKP). We intend to investigate possible etiologies, expected sequelae, and outcome of neodymium-dpoed yttrium alumnium garnet (Nd: YAG) laser membranectomy. CASE PRESENTATION: Our case is a 74-year-old male who underwent PKP surgery in the right eye secondary to corneal decompensation following cataract surgery in addition to corneal thinning secondary to superficial keratectomy related to the pre-existing climatic droplet keratopathy (CDK). Postoperative assessment revealed a retro-corneal membrane within the anterior chamber, which was affecting his vision. CLINICAL DISCUSSION: Based on the post-operative course and the decreased vision as an indication for intervention, it was decided to excise the retained DM. Membranectomy with Nd: YAG laser was performed, and the patient's visual acuity measurement improved from 20/400 to 20/25. However, the endothelial cell count decreased from 1479 to 520 cells/mm2 (35 % loss) at 15 months post YAG membranectomy with clear graft. Histopathological examination confirmed the clinical suspicion of a retained DM, since it was absent in the submitted host corneal tissue in addition to the pre-existing CDK. CONCLUSION: Retention of DM following PKP is a rare but possible complication and high index of suspicion is required for proper diagnosis and management to obtain better visual outcome. Nd: YAG laser membranectomy was effective in excising the retained DM and improving vision. Endothelial cell loss following Nd: YAG laser membranectomy as a complication was observed and should be addressed during the treatment plan.

Successful removal of a congenital fibrovascular pupillary membrane in 2 cases with variable age at presentation and sparing of the crystalline lens.

INTRODUCTION AND IMPORTANCE: Congenital fibrovascular pupillary membranes (CFPMs) represent a rare poorly understood condition that has been sporadically reported in the literature. The presence of such a membrane can cause pupillary block and further complications, therefore must be properly diagnosed and managed. CASE PRESENTATION: We are presenting the successful treatment of 2 cases with CFPM. The first patient presented 2-days after birth with an absent red reflex and had a less complicated clinical course. The second presented at a later age of 5-months and was referred as a case of congenital cataract. This baby had associated pupillary block glaucoma. Each of these cases was managed surgically by membrane peeling with sparing of the lens, which was found to be clear in the second case. DISCUSSION: Even though CFPM has been rarely reported, it should be correctly identified since it can progress with the development of glaucoma and lens changes. The etiology of CFPM is not well understood but might be related to the presence of ectopic iris tissue, which was suspected as an etiology in our second case. Several techniques have been described to remove the membrane, and occasionally this might necessitate removal of the lens. We described successful removal of CFPM in 2 cases without affecting the crystalline lens. CONCLUSION: General Ophthalmologists and Pediatricians should be aware of CFPM, especially when dealing with an absent or dull red reflex in a newborn. Referral for definitive diagnosis and treatment is essential to preserve vision.

Unilateral acquired blepharoptosis due to orbital metastatic disease as an initial presentation of an overlooked breast carcinoma - A case report.

INTRODUCTION AND IMPORTANCE: Orbital metastasis from breast cancer may be infrequently noted after the management of the primary lesion. It is rare in such cases to present with unilateral mechanical blepharoptosis without ophthalmoplegia. CASE PRESENTATION: We present a case of unilateral acquired blepharoptosis of the left upper eyelid without ophthalmoplegia or exophthalmos three years after the onset of a suspicious breast mass. The patient did not disclose a history of breast cancer nor any positive family history to the ophthalmologist and anesthesiologist. The radiology images revealed an ill-defined enhanced lesion at the superior medial aspect of the left orbit. The orbital biopsy of the lesion showed poorly differentiated carcinoma as per the initial histopathology report. Vigilant history-taking enabled us to get information about a previous breast lesion and to correlate this with specific histopathological findings. DISCUSSION: Management of orbital lesions might be challenging, and the approach should include detailed history and assessment. Biopsy and radio imaging are further needed to aid in providing the proper diagnosis. The clinicopathological correlation in our case has led to the final diagnosis of orbital metastatic breast cancer. CONCLUSION: Ophthalmologists should be aware of variable ocular presentations of malignancy and adopt a team approach to obtain a carefully detailed history from patients presenting with orbital diseases and communicate adequately with the ocular pathologists who are handling the biopsy. Long-term follow-up and enhancement of patients' awareness of possible late orbital metastasis are recommended in all patients with breast masses.