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.

Clinicopathological study of ophthalmic cutaneous and mucocutaneous non-langerhans cell histiocytic lesions.

BACKGROUND: The "C group" of the histiocytic disorders is characterized by non-Langerhans-cell histiocytic lesions in the skin, mucosal surfaces, or both, out of which Juvenile xanthogranuloma (JXG) is the most common typically affecting the skin. The eye is the most common extra-cutaneous site of JXG., we aim at providing our clinical and histopathological experience with this group of diseases including the adult-onset xanthogranuloma (AXG). METHODS: This is a retrospective cohort study of all patients with the tissue diagnosis of ocular and periocular cutaneous and mucocutaneous non-LCH disorders who presented to us over a period of 25 years (January 1993 to December 2018). RESULTS: Twenty patients were diagnosed as "Group C" disease with an age range of 2 months-60.9 years. Eleven patients were females (55%) and nine were males (45%). The involvement was mostly unilateral in 80.9%. All cases fell into the xanthogranuloma family with 11 JXG patients, 8 AXG patients of skin and ocular surface, and one patient with solitary reticulohistiocytoma (SRH). The clinical site of involvement in JXG was primarily in the eyelid in 5 patients (45%), ocular surface lesions in 2 (18%), iris in 2 (18%), choroidal and bilateral orbital lesions in 1 patient each (9%). The group of AXG, presented equally with eyelid lesions in 4/8 and ocular surface lesions in 4/8. The non-Langerhans' histiocytic infiltrate showed supportive immunohistochemical staining properties (reactive to CD68 marker and negative to S-100 and langerin markers). CONCLUSION: Among the rare histiocytic disorders, xanthogranulomatosis is the commonest and has wide clinical manifestations. Accurate diagnosis needs to be supported by typical histopathological findings. JXG was the commonest in our study with relatively older mean age at presentation and frequent eyelid rather than iris involvement. AXG is often confused with xanthelasma when involving the eyelids with corneal limbal involvement is relatively frequent.

Ophthalmic manifestations of nasopharyngeal carcinoma (NPC): A systematic review of reported cases with and without prior history of nasopharyngeal carcinoma.

PURPOSE: We aimed to study reported cases of nasopharyngeal carcinoma presenting with ophthalmic manifestations with and without a prior diagnosis of nasopharyngeal carcinoma. METHODS: We conducted a systematic review following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). A literature search was conducted using the MEDLINE database in PubMed and Google Scholar. We included patients with a previous diagnosis of nasopharyngeal carcinoma in Group I and those without a prior diagnosis of nasopharyngeal carcinoma in Group II. Data included demographics, clinical presentation, history of nasopharyngeal carcinoma, treatment, histopathological description, World Health Organization classification, and outcome. RESULTS: Fifty-eight patients (26 in Group I and 32 in Group II) were included. The male-to-female ratio was 3:1. The mean age of the patients (53.3 ± 11.7 years and 54.8 ± 16.2 years, respectively) and gender did not differ significantly between the two groups. The most common ocular presentations were diplopia and proptosis in the first group (each in 34.6%), whereas visual disturbance was most common in the second group (46.9%). Treatment options and World Health Organization grading were comparable. The outcome in 38 patients (after a comparable follow-up period) was significantly better in group II (p=0.003). There was no statistically significant difference in the outcome of 23 patients in correlation with World Health Organization grades II versus III irrespective of group (p=0.094). CONCLUSIONS: The demographics of patients with nasopharyngeal carcinoma presenting with ophthalmic manifestations were similar between the two study groups, with a wide age range and male predominance. Patients presenting initially to ophthalmologists with no history of nasopharyngeal carcinoma have a more favorable outcome. World Health Organization grading may have less value as a prognostic indicator.

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.

Ophthalmic manifestations of nasopharyngeal carcinoma (NPC): A systematic review of reported cases with and without prior history of nasopharyngeal carcinoma

ABSTRACT Purpose: We aimed to study reported cases of nasopharyngeal carcinoma presenting with ophthalmic manifestations with and without a prior diagnosis of nasopharyngeal carcinoma. Methods: We conducted a systematic review following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). A literature search was conducted using the MEDLINE database in PubMed and Google Scholar. We included patients with a previous diagnosis of nasopharyngeal carcinoma in Group I and those without a prior diagnosis of nasopharyngeal carcinoma in Group II. Data included demographics, clinical presentation, history of nasopharyngeal carcinoma, treatment, histopathological description, World Health Organization classification, and outcome. Results: Fifty-eight patients (26 in Group I and 32 in Group II) were included. The male-to-female ratio was 3:1. The mean age of the patients (53.3 ± 11.7 years and 54.8 ± 16.2 years, respectively) and gender did not differ significantly between the two groups. The most common ocular presentations were diplopia and proptosis in the first group (each in 34.6%), whereas visual disturbance was most common in the second group (46.9%). Treatment options and World Health Organization grading were comparable. The outcome in 38 patients (after a comparable follow-up period) was significantly better in group II (p=0.003). There was no statistically significant difference in the outcome of 23 patients in correlation with World Health Organization grades II versus III irrespective of group (p=0.094). Conclusions: The demographics of patients with nasopharyngeal carcinoma presenting with ophthalmic manifestations were similar between the two study groups, with a wide age range and male predominance. Patients presenting initially to ophthalmologists with no history of nasopharyngeal carcinoma have a more favorable outcome. World Health Organization grading may have less value as a prognostic indicator.

Isolated bilateral orbital and paranasal Rosai-Dorfman disease affecting two brothers: a case report and a systematic literature review.

A systematic literature review revealed 88 Rosai-Dorfman Disease (RDD) cases affecting the orbit. We present a review of the literature on orbital RDD in addition to two brothers with isolated bilateral extra-nodal orbital RDD cases associated with G-6PD deficiency. The disease manifested as asymmetric extensive orbital and paranasal sinus infiltration, with orbital bone destruction. Orbital mass debulking and steroids improved the condition in both patients. This is the most extensive case series of orbital RDD reported, including clinical manifestation, imaging, pathologic results, management, and outcome. Vigilant follow-up is essential to monitor for potential malignant transformation, systemic manifestations, potential vision loss, or life-threatening recurrences.

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.

Morphologically aggressive appearance of a human papilloma virus (HPV)-related conjunctival papilloma that was clinically suspicious for malignancy - A case report.

INTRODUCTION AND IMPORTANCE: Conjunctival squamous papilloma is a benign tumor that might be linked to Human papillomavirus (HPV) infection. The most common appearance is a pedunculated fleshy mass. We are reporting a unique of HPV-related conjunctival papilloma that was excised with the suspicious of malignancy. CASE PRESENTATION: A 35-year-old Saudi male who has been investigated for a suspicious pancreatic lesion and presented with a concurrent right eye conjunctival yellowish cystic mass. The mass was excised for diagnostic purpose to rule out malignancy in view of the history, the clinical appearance of the lesion, and the adjacent conjunctival severe congestion. The histopathological examination concluded a viral-related conjunctival papilloma. There was no evidence of recurrence of the lesion 2 years postoperatively. CLINICAL DISCUSSION: The common clinical appearance of a benign conjunctival papilloma is usually described as fleshy pedunculated mass with irregular grayish red surface. Such a lesion might be related to several etiologies, one of which is HPV. The mass in our case was highly vascularized and cystic with yellowish areas, which raised the suspicion of a malignant lesion. The histopathological findings, however, confirmed the benign nature of the lesion and supported the viral etiology by HPV. CONCLUSION: HPV-related conjunctival papilloma may have various morphological appearances. Histopathological examination is the key for final diagnosis of such types of lesions.

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.

Histopathological changes of lymphatic malformation after bleomycin injection / Alterações histopatológicas da malformação linfática após injeção de bleomicina

ABSTRACT Lymphatic malformation is a rare orbital tumor that used to be treated surgically, with high complication rates, or recently with intralesional bleomycin injection. We report for the first time the histopathological changes of eyelid lymphatic malformation after water-soluble intralesional bleomycin injection in a 20-year-old woman who had unsuccessful orbital surgical debulking during childhood. The changes confirmed the assumption of fibrosis induced by intralesional bleomycin injection. The minimal bleeding during surgical intervention made it much easier than the usual lymphatic malformation bloody procedure, without postoperative recurrences and with favorable aesthetic outcomes.

RESUMO A malformação linfática é um tumor orbital raro que costumava ser tratado cirurgicamente, com alta taxa de complicações. Mais recentemente, passou a ser tratado com uma injeção intralesional de bleomicina. Este é o primeiro relato sobre as alterações histopatológucas da malformação linfática palpebral após uma injeção intralesional de bleomicina hidrossolúvel em uma mulher de 20 anos de idade que sofreu uma cirurgia malsucedida de debulking orbital durante a infância, confirmando a suposição de fibrose induzida por injeções intralesionais de bleomicina. O sangramento mínimo durante a intervenção cirúrgica tornou esta muito mais fácil que o procedimento sangrento habitual, sem recidivas pós-operatórias e com desfechos estéticos favoráveis.

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.

Histopathological changes of lymphatic malformation after bleomycin injection.

Lymphatic malformation is a rare orbital tumor that used to be treated surgically, with high complication rates, or recently with intralesional bleomycin injection. We report for the first time the histopathological changes of eyelid lymphatic malformation after water-soluble intralesional bleomycin injection in a 20-year-old woman who had unsuccessful orbital surgical debulking during childhood. The changes confirmed the assumption of fibrosis induced by intralesional bleomycin injection. The minimal bleeding during surgical intervention made it much easier than the usual lymphatic malformation bloody procedure, without postoperative recurrences and with favorable aesthetic outcomes.

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.