Characteristics and Factors Related to Eyelid Basal Cell Carcinoma in Saudi Arabia.

PURPOSE: Data on basal cell carcinoma (BCC) from the Middle East are deficient. We present the features and management outcomes for BCC over the last 36 years in Saudi Arabia. SUBJECTS AND METHODS: This retrospective chart review included BCC patients diagnosed and treated at Saudi Arabia between 1980 and 2016. Data were collected on patient demographics, clinical and histopathological characteristics of the lesions, management, and follow-up. RESULTS: One hundred and twenty-six patients with BCC were included in this study. The incidence of BCC in Saudi Arabia is 0.8 cases a year. The median age of the patients was 71 years. BCC affected 58.9% of males. The lower lid was the most common site of occurrence (52.7%). Clinically, BCC was most commonly recognized as a mixed lesion (41.1%) and 50.4% were histologically nodular. Risk factors for poor prognosis included tumor localization in the medial aspect of the lid, tumor size > 5 mm, histological subtype being ulcerative or morphea forms, affected margins, and recurrent lesions. CONCLUSION: BCC is a rare condition in Saudi Arabia. The clinical features and histopathology of BCC in Saudi Arabia are similar to the patterns observed in other regions of the world. Early detection and timely management mitigates the extensive destructive ocular/orbital damage due to BCC and results in better patient outcomes.

Histopathologic and immunohistochemical features of capsular tissue around failed Ahmed glaucoma valves.

Impervious encapsulation around Ahmed glaucoma valve (AGV) results in surgical failure raising intraocular pressure (IOP). Dysregulation of extracellular matrix (ECM) molecules and cellular factors might contribute to increased hydraulic resistance to aqueous drainage. Therefore, we examined these molecules in failed AGV capsular tissue. Immunostaining for ECM molecules (collagen I, collagen III, decorin, lumican, chondroitin sulfate, aggrecan and keratan sulfate) and cellular factors (αSMA and TGFß) was performed on excised capsules from failed AGVs and control tenon's tissue. Staining intensity of ECM molecules was assessed using Image J. Cellular factors were assessed based on positive cell counts. Histopathologically two distinct layers were visible in capsules. The inner layer (proximal to the AGV) showed significant decrease in most ECM molecules compared to outer layer. Furthermore, collagen III (p = 0.004), decorin (p = 0.02), lumican (p = 0.01) and chondroitin sulfate (p = 0.02) was significantly less in inner layer compared to tenon's tissue. Outer layer labelling however was similar to control tenon's for most ECM molecules. Significantly increased cellular expression of αSMA (p = 0.02) and TGFß (p = 0.008) was detected within capsular tissue compared to controls. Our results suggest profibrotic activity indicated by increased αSMA and TGFß expression and decreased expression of proteoglycan (decorin and lumican) and glycosaminoglycans (chondroitin sulfate). Additionally, we observed decreased collagen III which might reflect increased myofibroblast contractility when coupled with increased TGFß and αSMA expression. Together these events lead to tissue dysfunction potentially resulting in hydraulic resistance that may affect aqueous flow through the capsular wall.

Dacryocystocele After Successful External Dacryocystorhinostomy: A Variant of the Lacrimal Sump Syndrome.

A 25-year-old man presented with a mass on the right medial canthus after a successful dacryo cys torhinostomy performed 3 years ago. On examination, there was a firm, nondepressible mass inferior to the medial canthal ligament and a linear vertical scar on the lateral wall of the nose. Syringing through the right lower punctum indicated his lacrimal system was patent. Computed tomography and magnetic resonance imaging disclosed a large cystic lesion in the lacrimal sac fossa. No bone erosion was detected. The mass was approached through a tear trough incision and completely resected. The lacrimal anastomosis that was highly placed remained patent after surgery. Histopathology of the specimen revealed a cystic lesion compatible with lacrimal origin (dacryocystocele).

Lymphatic and Blood Vessel Density in Human Conjunctiva After Glaucoma Filtration Surgery.

PURPOSE: To investigate the lymphatic vascular microvessel density (LVD) and the blood vascular microvessel density (MVD) and their distribution in excised leaking blebs after mitomycin C trabeculectomy and normal conjunctiva specimens. MATERIALS AND METHODS: LVD and MVD in normal human conjunctiva (n=8) and excised blebs in the hypocellular stroma and peribleb tissue (conjunctiva adjacent to hypocellular bleb tissue) (n=8) were evaluated by immunohistochemistry using antibodies raised against Lymphatic Vessel Endothelial Receptor 1 (D2-40, lymphatic endothelium) and CD34 (vascular endothelium). LVD and MVD counts were performed by light microscopy in 5 fields at ×20 magnification by 3 observers. Differences were determined using Mann-Whitney U test (P<0.05 was considered significant). RESULTS: The leaking blebs showed typical epithelial-stromal domes with areas of acellular stroma covered by attenuated epithelium and surrounded by normal conjunctival epithelium and a dense scar-like matrix replacing the substantia propria. The LVD and MVD were significantly reduced to nil in the hypocellular conjunctival stroma of the excised blebs compared with normal conjunctiva (21.42 vs. 1.16, P<0.002 and 24.28 vs. 1, P<0.008, respectively). The LVD and MVD was also reduced (2- to 2.5-fold) in the peribleb stroma when compared with normal conjunctiva specimens. CONCLUSIONS: In this study we show reduced LCD and MVD in the hypocellular and peribleb stroma. These results may suggest a role of these vessels in an altered immune response in leaking blebs leading to an increased risk for blebitis.

IgG4 immunostaining and its implications in orbital inflammatory disease.

OBJECTIVE: IgG4-related disease is an emerging clinical entity which frequently involves tissue within the orbit. In order to appreciate the implications of IgG4 immunostaining, we analyzed gene expression and the prevalence of IgG4- immunostaining among subjects with orbital inflammatory diseases. METHODS: We organized an international consortium to collect orbital biopsies from 108 subjects including 22 with no known orbital disease, 42 with nonspecific orbital inflammatory disease (NSOI), 26 with thyroid eye disease (TED), 12 with sarcoidosis, and 6 with granulomatosis with polyangiitis (GPA). Lacrimal gland and orbital adipose tissue biopsies were immunostained for IgG4 or IgG secreting plasma cells. RNA transcripts were quantified by Affymetrix arrays. RESULTS: None of the healthy controls or subjects with TED had substantial IgG4 staining. Among the 63 others, the prevalence of significant IgG4-immunostaining ranged from 11 to 39% depending on the definition for significant. IgG4 staining was detectable in the majority of tissues from subjects with GPA and less commonly in tissue from subjects with sarcoidosis or NSOI. The detection of IgG4+ cells correlated with inflammation in the lacrimal gland based on histology. IgG4 staining tissue expressed an increase in transcripts associated with inflammation, especially B cell-related genes. Functional annotation analysis confirmed this. CONCLUSION: IgG4+ plasma cells are common in orbital tissue from patients with sarcoidosis, GPA, or NSOI. Even using the low threshold of 10 IgG4+ cells/high powered field, IgG4 staining correlates with increased inflammation in the lacrimal gland based on histology and gene expression.

Ocular malignant tumors. Review of the Tumor Registry at a tertiary eye hospital in central Saudi Arabia.

OBJECTIVE: To present the epidemiologic profile and magnitude of ocular malignant tumors (MT) representative of the Saudi population from the Tumor Registry (TR) at King Khaled Eye Specialist Hospital (KKESH). METHODS: This study evaluated the demographic information, clinical features including tumor laterality, ocular tissue of origin, and diagnosis of patients from the TR registry between 1983 and 2012 at KKESH, Riyadh, Kingdom of Saudi Arabia. The incidence of MT among Saudi adults (>/= 15 years old), and children (<15 years old) was estimated. RESULTS: The TR recorded 4,146 neoplasms (2,509 [60.5%] benign tumors, and 1,637 [39.5%] MT). The incidence of MT in children was 3.6 per million/year (M/Y), and 2.4/M/Y for adults. Retinoblastoma (Rb) (n=763, 91%) was the most common ocular malignancy in children. In adults, the most common MT was squamous cell carcinoma (SCC) (n=363, 45.8%), basal cell carcinoma (BCC) (n=186, 23%), uvealmelanoma (n=94, 11.9%), sebaceous gland carcinoma (n=54, 6.8%), lymphomas (orbital, adnexal) (n=46, 5.8%), and others (n=53, 6.8%). The Rb (7.7/M/Y in <5 years old Saudi children) was less frequent than that reported in some Gulf countries, but higher than that reported from the West. The SCC was less frequent in countries with comparable sun exposure than in other continents, but the incidence remained unchanged over 3 decades. There was a significant increase in BCC between 1983-1992 and 2003-2012. CONCLUSION: The rates of all cancers remained stable over 3 decades except BCC, which showed a significant rise.

Achondroplasia associated with bilateral keratoconus.

We report a rare case of bilateral keratoconus in association with achondroplasia. A 26-year-old male, with a known case of achondroplasia, complained of bilateral gradual deterioration in vision for the past few years. Slit lamp biomicroscopy showed bilateral central corneal protrusion and stromal thinning at the apex consistent with keratoconus. a trial of hard contact lens fitting failed to improve VA in the left eye (LE). Right eye (RE) improved to 20/25. The patient underwent penetrating keratoplasty (PKP) in his LE. Twenty-seven months postoperatively, uncorrected visual acuity (UCVA) was 20/30. Ophthalmologists should be aware that patients with achondroplasia who complain of poor vision should be suspected of having keratoconus once other more common conditions are ruled out.

Nummular dystrophic calcification of an Ahmed glaucoma valve in a child.

We report the case of an otherwise-healthy 4.5-year-old girl with primary congenital glaucoma who had multiple nummular calcium deposits on the dorsal plate of a previously-implanted Ahmed glaucoma valve. The child had received the implant in the right eye at 6 months of age and developed increased intraocular pressure requiring surgical excision of surrounding fibrous encapsulation. Intraoperative inspection revealed that the dorsal plate surface was covered with multiple white nummular lesions; staining and X-ray microanalysis confirmed the lesions to be calcium deposits. Calcium deposits have been documented for various ophthalmic implants, including silicone scleral buckles and intraocular lenses, but to the best of our knowledge they have not previously been described in association with a glaucoma drainage device.

Epithelial lacrimal gland tumors: pathologic classification and current understanding.

OBJECTIVE: To apply the updated epithelial salivary gland classification scheme to a large cohort of lacrimal gland tumors so as to provide an updated lacrimal gland tumor classification scheme. METHODS: A retrospective multicenter cohort study of 118 cases of epithelial neoplasia was undertaken. Main outcome measures included pathologic analysis, subtyping, and survival. RESULTS: Of 118 cases, 17 (14%) were reclassified using the proposed expanded classification scheme based on the current World Health Organization classification of salivary gland tumors. The most frequent neoplasms were pleomorphic adenoma and adenoid cystic carcinoma, of which we highlight more unusual histologic features. Three tumors were found to be unclassifiable with the updated scheme, with 2 having histologically malignant features. Deficiencies and variations in pathologic assessment were noted. Variation in the histologic findings of pleomorphic adenoma and assessment of the extent of invasion of carcinoma ex pleomorphic adenoma were highlighted. CONCLUSIONS: The use of the more histologically diverse classification of salivary gland tumors can be successfully applied to the epithelial lacrimal gland neoplasms. This expanded classification system led to reclassifying 14% of cases. Currently, there are no consistent pathologic standards for processing and evaluating these lesions.

Infantile corneal myxoma.

Myxomas, benign tumors of modified fibroblasts that produce small amounts of collagen but large amounts of glycosaminoglycans, typically occur in the left atrium of the heart or various soft tissues. Corneal myxoma is extremely rare, with only 8 previously reported cases to date, all of which were in individuals 26 years of age or older. The current report documents the clinical and histopathologic features of a 4-month-old girl with infantile corneal myxoma.

Osseous choristoma of the lateral canthus.

Ocular osseous choristoma, a rare idiopathic benign deposit of bone, is typically a static epibulbar lesion that occurs sporadically in the supertemporal quadrant of an otherwise normal eye. The subject of this report is a unique U-shaped subcutaneous osseous choristoma that did not involve the eye itself but rather conformed to the right lateral canthus in an otherwise normal 2-year-old boy.

Bilateral congenital stromal cyst of the cornea.

Stromal corneal cysts have not been documented in histological studies of congenital corneal leukoma. We describe a bilateral congenital case in which the cysts occupied posterior excavations of the cornea and the affected infant developed angle-closure glaucoma.

Infected epithelial inclusion cyst mimicking subconjunctival abscess after strabismus surgery.

Presumed subconjunctival abscess has been reported as a rare postoperative complication of strabismus surgery.(1-2) We report the case of a child who initially was diagnosed with subconjuctival abscess after strabismus surgery for whom histopathology demonstrated an infected epithelial inclusion cyst. We suggest that previous reports of presumed subconjunctival abscess after strabismus surgery also may have been caused by a similar mechanism.

Management of congenital unilateral proptosis from an orbital epithelial cyst by cyst aspiration.

A rare cause of axial proptosis, orbital epithelial cysts are presumed to be of conjunctival origin if the wall is composed of nonkeratinized squamous epithelium (especially if goblet cells are present). These cysts can occur at any age and are typically surgically resected if symptomatic. The subject of this report is the management of a congenital orbital epithelial cyst by needle aspiration in a newborn with ipsilateral esotropia and severe unilateral proptosis.