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.

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.

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.

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.

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.

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.

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.

Adenoid cystic carcinoma of the orbit with bilateral cavernous sinus extension: A case report.

INTRODUCTION AND IMPORTANCE: Adenoid cystic carcinoma (ACC) is an uncommon malignant epithelial tumor of the salivary and lacrimal glands. Orbital ACC is rare, originating commonly from the lacrimal gland, with only a few cases reported without lacrimal gland involvement. Deep orbital ACC may be associated with extension into skull base structures, and further intracranial invasion. CASE PRESENTATION: We report a 47-year-old gentleman who presented with insidious onset of bilateral proptosis, left ophthalmoplegia, and loss of vision. Imaging revealed left orbital infiltrative mass with intracranial invasion and bilateral cavernous sinus extension. The lacrimal gland was not involved clinically nor radiologically. Histopathology showed ACC with classical cribriform pattern. There was no evidence of primary source of tumor or metastasis. DISCUSSION: ACC of the orbit commonly originates from the lacrimal gland. Only a few cases of orbital ACC without lacrimal gland involvement were found in English literature. Workup for our patient did not reveal a primary source of tumor. Tumor may have risen from ectopic orbital lacrimal gland tissue, extension from non-orbital sites, or through perineural or hematogenous spread. Only one case of bilateral cavernous sinus extension has been previously reported. Treatment for advanced orbital ACC is exenteration in most cases. However, due to the advanced nature of disease in our patient, palliative radiotherapy was the treatment of choice. CONCLUSION: Orbital ACC of non-lacrimal origin is rare and is associated with high morbidity and mortality. Early recognition and treatment are key for preventing organ and life-threatening complications such as advanced intracranial spread.

Clinicopathological Correlations in Enucleated Globes of Late-Stage Coats Disease with a Review of the Literature.

BACKGROUND: Coats disease may cause diagnostic dilemma because of its variable clinical presentation that can be suspicious of retinoblastoma. Late sequelae of the disease are blinding with possible enucleation. We demonstrate the main histopathological findings of Coats enucleated eyes with literature review. METHODS: This was a retrospective study of all enucleated globes diagnosed as Coats disease over 30 years and were reviewed by two pathologists. The corresponding demographic data, clinical presentation, pre-operative clinical impression, and indication for enucleation were collected. Descriptive analysis of our own series data was performed. Our findings were then correlated to published data that were collected from 1983 to 2021 from the PUBMED database in English-written language. Shields classification was used as an inclusion criterion for the published reports to be analyzed. RESULTS: We had seven enucleated globes with Coats disease. Mean age at presentation was 3.2 years (range 3 months to 9 years). Male predominance was observed in 6 and all cases were unilateral. Strabismus was the most common initial presentation (57%, n = 4), followed by leukocoria (43%, n = 3). Indication for enucleation was mostly suspected retinoblastoma (57%, n = 4). Four eyes were classified as stage 4, and 2 had advanced stage 5 changes. Histopathologically, subretinal fluid with lipid-laden macrophages was seen in all cases, the anterior chamber was shallow in 5/7 with angle neovascularization in 2/7. Telangiectatic vessels were clearly observed in 4/7. CONCLUSION: Coats disease is a potentially visually disabling disease that is mostly unilateral in 95%, has male predominance of 81%, and wide age range with a mean of 17 years. In Saudi Arabia, the disease seems to present at younger age, tends to be more advanced, and may be indistinguishable from retinoblastoma at the time of diagnosis. Shields staging of Coats is highly recommended to be followed clinically to unify the pathways for treatment and to correlate the concluded outcomes.

Conjunctival myxoma masquerading as conjunctival lymphoma: A case report.

INTRODUCTION AND IMPORTANCE: Ocular myxomas are very rare and can involve the orbit, eyelids, and conjunctiva. Conjunctival myxoma can be misdiagnosed as amelanotic nevus, conjunctival cyst, or ocular surface squamous neoplasia, among others. They can appear as an isolated lesion or can be associated with systemic manifestations as part of the Carney complex or Zollinger-Ellison syndrome. CASE PRESENTATION: We describe a 64-year-old healthy male who presented with a right eye painless peri-limbal salmon-colored patch lesion in the infero-temporal bulbar conjunctiva over a period of 2 years. There was no of ocular trauma or surgery and no effect on vision. The mass was not tender, raised, and mobile with fine intrinsic vascularity. Excisional biopsy with the presumed diagnosis of lymphoma revealed a typical sub-conjunctival myxoma. DISCUSSION: The recognition of ocular myxoma necessitates systemic evaluation to rule out possible associated cardiac myxoma in Carney complex, thus can prevent life-threatening events. The excised mass in our patient showed an area of pseudo-elastotic degeneration, which has further complicated the clinical appearance of the lesion, however, the color, and consistency of the mass were highly suspicious of lymphoma. The diagnosis of myxoma by histopathology was helpful especially in presence of atypical appearance such as in our case. CONCLUSION: The histopathological characteristics of conjunctival myxoma can aid in the diagnosis. The lesion in our case was associated with focal severe pseudo-elastotic degeneration and prominent salmon-patch appearing area thus was initially misdiagnosed clinically as a conjunctival lymphoma.

Clinical features and histopathological analysis of corneal myxoma.

To describe the presentation, histopathological characteristics, and management outcomes for corneal myxoma. This one-armed cohort study evaluated histologically confirmed consecutive cases of corneal myxoma. Data were evaluated on demographics, clinical presentation, management, histopathological and immunohistochemical features, and outcomes; visual acuity and corneal clarity. The study sample was comprised of 10 eyes (10 patients). The median age at presentation was 10.5 years. Five eyes had high intraocular pressure, four eyes had decreased distance visual acuity and one eye became discolored. Surgical management included penetrating keratoplasty (8 eyes), phototherapeutic keratectomy (1 eye), and evisceration because of a blind painful eye (1 eye). Postoperative best-corrected distance vision ranged from 20/20 to 20/60 (1 eye), < 20/60 to 20/200 (2 eyes), < 20/200 to 20/400 (1 eye), < 20/200 to light perception (4 eyes) and no light perception (1 eye). The histopathology of these lesions showed typical subepithelial proliferating spindle-shaped cells of mesenchymal origin within a myxoid stroma rich in glycosaminoglycan. The median duration of follow-up was 5 years. Recurrence was observed in an eye that underwent local excision. Corneal myxoma is a rare lesion that is presumably isolated, secondary, and reactive in nature. Surgically management yields reasonably favorable outcomes.

Femtosecond laser assisted superficial lamellar keratectomy as a successful treatment of corneal opacity in a patient with Thiel Behnke corneal dystrophy.

INTRODUCTION AND IMPORTANCE: Thiel Behnke corneal dystrophy (TBCD) and Reis Buckler corneal dystrophy (RBCD) are Bowman's layer dystrophies with overlapping clinical features causing diagnostic confusion. However, each entity has typical histopathological features. We describe in this case the successful use of Femtosecond laser (FSL) in the treatment of TBCD-related corneal opacity. CASE PRESENTATION: We present a 54-year-old male with bilateral superficial corneal opacities consistent with TBCD based on clinical appearance, anterior segment optical coherence tomography (AS-OCT), and In vivo confocal microscopy. Management options were discussed with the patient before proceeding with Femtosecond Laser Assisted Superficial Lamellar Keratectomy (FSLASLK). The histopathological findings of the excised left anterior lamellar corneal flap were typical of TBCD and the patient had a satisfactory outcome. CLINICAL DISCUSSION: TBCD typically affects Bowman's layer centrally with progressive opacities involving the deeper layer of the corneal stroma and the periphery with advancing age. Histopathology typically shows subepithelial fibrosis with interrupted basement membrane and totally replaced Bowman's layer by uneven fibrous tissue forming the characteristic saw tooth pattern. The treatment of such cases is challenging with variable success and recurrence rates. Our case was managed successfully using FSL. CONCLUSION: TBCD, even though a rare type of dystrophy, should be suspected based on the appearance of the corneal opacities clinically. It can be diagnosed by typical AS-OCT findings supported by histopathological confirmation and can be successfully treated by FSASLK.

Recurrent Oncocytoma of the Lacrimal Sac.

Oncocytoma of the lacrimal sac is an extremely rare tumor. In this report, we present the case of an 82-year-old woman who presented with swelling in the region of the lacrimal sac. Systemic examination and ophthalmic examination of both eyes were unremarkable. Computed tomography of the brain and orbits revealed a mass lesion involving the right lacrimal sac with expansion of the related nasolacrimal duct. Neither bone destruction nor tissue invasion was observed. Right external dacryocystectomy and debulking of the tumor were performed. Histopathological examination of the surgical specimen showed oncocytic cells arranged in an adenomatous fashion, and a diagnosis of benign oncocytoma was made. Three years later, the same patient presented with a similar complaint that was pathologically proven to be a recurrent benign oncocytoma of the lacrimal sac.

Demographics and histopathological characteristics of enucleated microphthalmic globes.

Microphthalmia is a rare ocular anomaly with a poorly understood etiology that is most likely related to heritable and/or environmental factors. Many papers have been published pertaining to the clinical manifestations and management of this condition; however, few reports have reported detailed histopathological findings, which are the focus of this study, in addition to highlighting the basic demographics in these cases. This was a retrospective, observational study of all consecutive enucleated microphthalmic globes (with or without cysts) at 2 tertiary eye hospitals in Riyadh, Saudi Arabia. Globes were classified into 2 groups: severe microphthalmos (axial length or mean diameter less than 10 mm in infancy or 12 mm after age 1 year) and mild microphthalmos based on larger measurements. Clinical and demographic data collected included sex, age at enucleation, eye involvement, nationality/region, consanguinity, family history of eye anomaly, pregnancy, systemic disease, or syndromes. For histopathological data, a descriptive analysis was mostly performed. For correlations of some of our qualitative data, Fisher's exact test was used. Eleven cases (6 mild and 5 severe microphthalmos) were initially identified with a female to male ratio of 4:7. Ten patients were Saudis, 7 of whom were from the central region. Consanguinity was found in 36% (4/11), and 3 of them had other ocular or systemic abnormalities (duodenal atresia, microcephaly, kidney agenesis, cryptophthalmos, and dysmorphic facial features). Histopathological data were available for 10 cases, half of which showed a coloboma and/or anterior segment anomaly. There was no significant correlation among gender, severity of microphthalmos or the presence of coloboma, although severe microphthalmic globes had a higher median of abnormal intraocular structures (9-interquartile range = 2 compared to 6-interquartile range = 1 in the mild group). Aphakia was found in half of the globes with associated anterior segment dysgenesis. We have concluded that microphthalmos is a visually disabling congenital anomaly that can be isolated or associated with other periocular or systemic anomalies, possibly in relation to consanguinity in our cases. Congenital aphakia was found in half of these cases and was mostly associated with absent Descemet's membrane and agenesis of anterior chamber angle structures, supporting previously suggested embryological concepts. These findings necessitate further wider genetic testing and proper premarital counseling in Saudi Arabia.

Ectopic ocular tissue in testicular teratoma: A case report and review of the literature.

INTRODUCTION AND IMPORTANCE: Teratoma is a common neoplasm in prepubertal and post-pubertal periods. It consists of various types of tissues arising from different germinal layers, endoderm, mesoderm, and ectoderm. Ectopic ocular tissue is a rare phenomenon, with only few reported cases in other locations. CASE PRESENTATION: This is a 10-month-old boy who presented with a painless scrotal mass. Following orchidectomy, the excised mass confirmed the presence of uveal and retinal tissues originating in a benign testicular teratoma by histopathological examination. DISCUSSION: Choroidal and retinal tissue are the most frequently encountered ectopic ocular tissue, while the least observed tissue is the lens. Most of the reported cases of ectopic ocular tissue present in ovarian teratomas. The only 2 previously reported cases of ocular-like tissue in testicular teratoma lack well-defined medullary epithelium, uveal, and retinal tissue as in our case. CONCLUSION: To our knowledge, developing ocular tissue in a testicular teratoma is extremely rare. Herein we report a unique case with mature defined ocular tissue within a testicular teratoma in an infant, which should not be overlooked.

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.

Periocular intravascular papillary endothelial hyperplasia: A retrospective study.

INTRODUCTION: Intravascular papillary endothelial hyperplasia (IPEH) is a rare proliferation of endothelial cells with uncertain etiology related to thrombus formation. Diagnosis is usually confirmed histopathologically. This condition has been previously described in the periocular region but not in the conjunctiva. METHODS: It is a retrospective case series in which we evaluated seven patients with histopathologically confirmed IPEH cases. Data regarding the demographics, clinical presentation, radiological description, histopathological features including any IHC staining, suspected underlying vascular etiology, management options, and follow up outcome were collected. RESULTS: A total of seven cases of histologically confirmed IPEH were included. Five out of seven patients were male (71.4%). The age range was between 6 and 69 years with a median age of 36 years. Three cases involved the eyelid (42.8%) and another three were found in the conjunctiva (42.8%). Pre-existing underlying vascular lesions were observed in all patients, five malformations (mostly lymphatic-venous) and two conjunctival hemorrhagic lymphangiectasis. All cases were treated with excisional biopsy with no signs of recurrence within an average of 7 months follow up. CONCLUSIONS: Periocular IPEH is a rare tumor that is likely to coexist with underlying vascular lesions and thrombus formation. We are reporting its existence in the conjunctiva for the first time. Therefore, pathologists should be aware of the histopathological spectrum of this lesion.

Dacryops and clinical diagnostic challenges.

OBJECTIVE: The study aims to describe the clinical and histopathologic features of different types of dacryops and their clinical diagnostic challenges. METHODS: This is a retrospective cohort study of all surgically excised cases of dacryops in 2 tertiary eye hospitals in Riyadh, Saudi Arabia. RESULTS: The study included 58 dacryops specimens from 55 patients with an average age of 41.2 years (range, 4-78 years). The most common location was the upper lid (60.3%), whereas the least expected location was the caruncle (6.9%). The most common site of dacryops occurrence was in the accessory lacrimal gland (55.2%), the main lacrimal gland (32.8%), and then ectopic dacryops (12%). All patients presented with lid swelling alone except for 3 patients who experienced secondary mechanical ptosis. On physical examination, conjunctival scarring existed in 4 patients (6.9%). Preoperative diagnosis of dacryops was accurate in 44.8% of the cases. Dacryops of the main lacrimal gland was accurately diagnosed clinically in all cases compared with other locations, which was statistically significant (p < 0.001). The causes of inaccurate clinical diagnoses were hidrocystoma (26.9%), inclusion cyst (11.5%), and dermoid cyst (7.7%), whereas the remaining cases were diagnosed as cysts without a specific subtype (53.9%). Recurrence of the lesion was observed in 2 cases (3.5%). No clinical or histopathologic factors were associated with a risk of recurrence. CONCLUSION: Dacryops can represent a diagnostic challenge to ophthalmologists. Familiarity with clinical presentations and findings is required to diagnose dacryops outside the main lacrimal gland.

Paraneoplastic pemphigus associated with rapidly progressing endophthalmitis caused by Morganella Morganii: A case report.

INTRODUCTION: The ocular presentation of paraneoplastic pemphigus (PNP) has rarely been reported in the literature. In this report, we describe a 61-year-old male presenting with eruptive skin lesions associated with underlying non-Hodgkin's lymphoma who had rapid progressive corneal perforation with secondary endophthalmitis in the setting of PNP. CASE DESCRIPTION: A 61-year-old male presented to the emergency department complaining of skin eruption mimicking Stevens-Johnson syndrome, which was later found to be related to PNP. Initially, the patient complained of progressive ocular surface dryness in both eyes. Meanwhile, he developed mild pain in the right eye associated with blurry vision in both eyes and was managed with lubricants and topical antibiotics. A few days later, he was found to have corneal perforation with features suggestive of left endophthalmitis with possible early panophthalmitis. Intravenous antibiotic was administered, and primary evisceration of the left globe was performed. Histopathology revealed acute necrotizing keratitis and endophthalmitis. Vitreous analysis showed numerous gram-negative bacilli and a positive culture of Morganella morganii. The patient continued to be managed with frequent lubrications and punctual plugs in the fellow eye during the follow-up period. CONCLUSION: We describe the first case of endophthalmitis developing secondary to PNP-induced corneal melting and perforation. Anticipating unusual infectious sequelae in the setting of PNP might be warranted to actively detect and successfully manage dry eye disease before devastating complications develop.