The clinico-pathologic profile of primary and recurrent orbital/periorbital plexiform neurofibromas (OPPN).

To evaluate and compare the clinical and histopathological profile of primary and recurrent orbital-periorbital plexiform neurofibromas (OPPN) in patients with neurofibromatosis type 1. We retrospectively evaluated 43 primary or recurrent neurofibroma (NF) specimens from 26 patients (2002 to 2018) at the King Khaled Eye Specialist Hospital, Saudi Arabia. Demographics, clinical presentation, and surgical intervention data were collected. Histopathological specimens were studied with hematoxylin-eosin, Alcian blue, and immunohistochemical markers; S-100, CD44, CD117, smooth muscle actin (SMA), neurofilament, and Ki-67. Of the 43 NFs specimens, 20 were primary and 23 recurrent tumors. For primary NF, the ratio of plexiform to the diffuse type was 13:7, however in recurrent tumors was 3:8 after the first recurrence, and 1:5 after multiple recurrences. Of the 17 patients with primary tumors that had paired recurrent tumors, 12/17 (70.6%) primary NFs were plexiform and 5/17 (29.4%) were diffuse. However, when tumors recurred, 13/17 tumors (76.5%) were diffuse and only 4/17 tumors (23.5%) had a plexiform pattern. The odds of a tumor having a diffuse pattern in recurrent NF was significantly higher than the plexiform pattern [OR = 7.8 (95% confidence interval 1.69:36.1) P = 0.008]. Primary plexiform NFs underwent an excision at a significantly younger age than the diffuse type. Recurrent NFs had significantly higher CD44, CD117, and neurofilament labeling (P = 0.02, P = 0.01 and P<0.001 respectively) but had significantly decreased Alcian blue, and S-100 labeling (P = 0.03, and P = 0.02 respectively) compared to primary tumors. SMA and Ki-67 proliferation index were not different between primary and recurrent NFs (P = 0.86, and P = 0.3 respectively). There appears to be a high risk for primary plexiform NFs to develop a diffuse histologic pattern when they recur. Immunohistochemical staining suggests a role of mast cells (CD117) and expression of infiltration makers (CD44) in the transformation of plexiform tumors to the diffuse phenotype.

The association of cosmetic-related lacrimal sac black deposits with primary-acquired nasolacrimal duct obstruction and dry eye disease.

PURPOSE: To determine the incidence of cosmetic-related lacrimal sac black deposits (LSBDs) in primary-acquired nasolacrimal duct obstruction (PANDO) biopsies and the role of LSBD in the pathogenesis of PANDO, in addition to their association with dry eye disease (DED). METHODS: A clinicopathological study included all patients who underwent surgical management of PANDO. We excluded patients in whom lacrimal sac biopsy was not taken during the surgery. Lacrimal sac tissues were evaluated for the presence of LSBD and related inflammation, with correlation to the demographics, clinical presentation, and pre-operative clinical assessment of dry eye. P <0.05 was considered statistically significant. RESULTS: Of the 177 PANDO specimens, black deposit aggregates were noted in the sac stroma of 61 lacrimal sac specimens (34.5%; 95% confidence interval: 27.5-47.5). LSBDs were significantly more common in females (P < 0.001). The age, residence, past ailments, and laterality were not associated with LSBD. Dry eye was more common with LSBD (P = 0.004). Other presenting symptoms were not significantly associated with LSBD. The stromal black deposits in biopsies were mostly extracellular or in macrophages. The LSBD in only 10 specimens demonstrated birefringence. Energy dispersive spectroscopy determined that carbon and sulfur were the main elements in the black aggregates. CONCLUSION: Cosmetic-related LSBD is unlikely to play a role in the pathogenesis of PANDO. However, they were significantly associated with DED.

Eyelid sebaceous gland carcinoma: An assessment of the T classification of the American Joint Committee of Cancer TNM staging system 8th versus 7th edition.

PURPOSE: To assess the prognostic values of the T classification of the 8th edition of the American Joint Committee of Cancer staging system and compare it to the 7th edition. METHODS: Multicenter retrospective study of patients with eyelid sebaceous gland carcinoma. The primary outcome measure was the differences between outcomes when tumors were staged with either 7th or 8th edition. The measures evaluated included presenting features, management, histopathology, metastasis, recurrence, and mortality. RESULTS: Of the 60 patients (median age 73 years), 31 (51.7%) were females. A change in T staging occurred in 39 patients (65%) when the 8th edition was applied. Advanced categories (T3/T4) were significantly associated with nodal metastasis (p = 0.037) using the 8th edition criteria but not with the 7th edition (p = 0.066). The 8th edition T categorization significantly correlated with eye survival (p = 0.022) while the 7th edition did not (p = 0.058). Applying the 8th edition, category T4 at presentation was associated with a higher risk of nodal metastasis (p = 0.037) but not associated with local recurrence, distant metastasis, or tumor-related death (p = 0.281, p = 0.737, p = 0.319, respectively). T3/T4 category tumors were significantly associated with poor tumor differentiation (p = 0.001), and papillary histologic pattern (p = 0.024) but not with pagetoid spread (p = 0.056). CONCLUSION: The application of the 8th edition AJCC staging system for eyelid SGC may accurately predict nodal metastasis. Local recurrence and distant metastasis were not significantly associated with T classification, using either edition. Poor tumor differentiation and papillary pattern were associated with T3/T4 categories suggesting that pathological features may assist in determining prognosis.

The HYLAN M Study: Efficacy of 0.15% High Molecular Weight Hyaluronan Fluid in the Treatment of Severe Dry Eye Disease in a Multicenter Randomized Trial.

The aim of the HYLAN M study was to investigate if symptoms and/or signs of patients suffering from severe dry eye disease (DED) can be improved by substituting individually optimized artificial tear therapy by high molecular weight hyaluronan (HMWHA) eye drops. In this international, multicenter study, patients with symptoms of at least ocular surface disease index (OSDI) 33 and corneal fluorescein staining (CFS) of at least Oxford grade 3 were included. A total of 84 per-protocol patients were randomized in two study arms. The control group continued to use their individual optimum artificial tears over the study period of eight weeks; in the verum group, the artificial tears were substituted by eye drops containing 0.15% HMWHA. At the week 8 visit, the average OSDI of the verum group had improved by 13.5 as compared to the control group (p = 0.001). The best corrected visual acuity (BCVA) had improved by 0.04 logMAR (p = 0.033). CFS, tear film break-up time (TBUT), Schirmer I, lid wiper epitheliopathy (LWE), mucocutaneous junction (Yamaguchi score), and tear osmolarity were not significantly different between the verum and control groups (p > 0.050). We conclude that for most patients with severe DED, 0.15% HMWHA eye drops provide excellent improvement of symptoms without impairment of dry eye signs.

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.

Management outcomes of canalicular laceration in children.

PURPOSE: To report the epidemiological data, clinical profile, management, and outcomes of canalicular lacerations in the pediatric age group in a tertiary eye care hospital in Saudi Arabia. METHODS: This retrospective study evaluated pediatric patients who underwent canalicular laceration repair in the last 15 years at King Khalid Eye Specialist Hospital (KKESH), Saudi Arabia. Demographics, causes of injury, type of trauma, surgical approach, and outcomes were analyzed. The success of repair was defined as the absence of epiphora after canaliculus repair with negative dye disappearance test (DDT). Success within subgroups was compared. P < 0.05 was considered statistically significant. RESULTS: The study sample was comprised of 43 patients, with a median age of 6.35 years (range, 1.77-17.96 years). Most of the patients were males (69.8%). Sharp objects were the most common cause of canalicular laceration (46.5%), being 9 (20.9 %) caused by a metallic clothing hanger. Lower canaliculus was involved in 65.1%, upper canaliculus in 32.6%, and both canaliculi in 2.3% of patients. Canaliculus repair was performed with a bicanalicular stent in 58.1 % and monocanalicular stent in 41.9 % of patients.The success rate and risk of complications using bicanalicular or monocanalicular stent did not differ (P = 0.065). Functional success was achieved in 87.5% of patients. CONCLUSION: Canalicular laceration is common in male children, mainly affecting the lower canaliculus. There was no difference in success rate between monocanalicular and bicanalicular stent. As canalicular laceration could be related to social determinants, the main causes should be highlighted in community health education initiatives.

Adult presentation of retinoblastoma in Saudi patient.

To describe an unusual case of retinoblastoma in adult Saudi. A 21-year-old Saudi male developed acute unilateral visual loss due to a large intraocular lesion in his left eye. Within five days of presentation, the tumor enlarged rapidly causing angle closure glaucoma and orbital cellulitis like picture. The patient underwent enucleation and the histopathology specimen confirmed the presence of an undifferentiated type tumor cell that was consistent with retinoblastoma with choroidal and optic nerve invasion. Retinoblastoma should be considered in the differential diagnosis of an intraocular mass in adults. To the best of our knowledge, this is the first reported case of an adult presentation of retinoblastoma in Saudi patient.

Clinical and histological features and outcomes of upper eyelid colobomas in the Saudi population.

PURPOSE: To study the clinical features, histopathology, and management of congenital upper eyelid coloboma (CEC) in the Saudi population. METHODS: A retrospective review of health records evaluated the demographics, histopathology, and surgical outcomes of patients with CEC. RESULTS: Thirty-nine eyelids of 27 patients were included in this study. CEC was bilateral in 12 (44.4%) patients, isolated in 17 (62.9%), and as part of a syndrome in 10 (37.1%) patients. CEC was commonly located in the medial upper lid (22 lids, 56.4%) and mostly involved the full thickness of the lid (27 lids, 69.2%). Corneal adhesion (18 eyes, 46.1%) and poorly formed eyebrows (21 eyebrows, 53.8%) were the most common ocular/adnexa associations. Histopathology was similar in all cases and the main features were scarred dermis, atrophic orbicularis oculi, and atrophic or absent tarsus. Visual acuity at the final follow-up was 20/50 or better in 13 (33.3%) eyes. Complete lid closure without lagophthalmos after one or more surgical procedures was achieved in 11 (40.7%) cases. CONCLUSIONS: CEC features in Saudi patients are similar to those described in the literature. Dermal scarring and defective orbicularis muscles are common. Achieving cosmetic and functional success after management remains challenging.

Isolated superior oblique muscle extranodal marginal zone B cell lymphoma: case report.

We describe a rare case of isolated extraocular muscle ocular adnexal lymphoma of a middle-aged female who presented with redness in the left eye associated with progressive proptosis over one year. Magnetic resonance imaging of the orbit indicated isolated enlargement of the left superior oblique (SO) muscle with an apparent diffusion coefficient (ADC) of (0.77 ±â€¯0.11 × 10-3 mm2/s). Histopathology with immunohistochemical staining of the incisional biopsy from the SO muscle belly confirmed the diagnosis of extranodal marginal zone B cell lymphoma.

Biomaterials and Tissue Engineering Strategies for Conjunctival Reconstruction and Dry Eye Treatment.

The ocular surface is a component of the anterior segment of the eye and is covered by the tear film. Together, they protect the vital external components of the eye from the environment. Injuries, surgical trauma, and autoimmune diseases can damage this system, and in severe cases, tissue engineering strategies are necessary to ensure proper wound healing and recovery. Dry eye is another major concern and a complicated disease affecting the ocular surface. More effective and innovative therapies are required for better outcomes in treating dry eye. This review focuses on the regenerative medicine of the conjunctiva, which is an essential part of the ocular surface system. Features and advances of different types of biomolecular materials, and autologous and allogeneic tissue grafts are summarized and compared. Specifically, vitrigel, a collagen membrane and novel material for use on the ocular surface, offers significant advantages over other biomaterials. This review also discusses a breakthrough microfluidic technology, "organ-on-a-chip" and its potential application in investigating new therapies for dry eye.

Clinical features and bacteriology of lacrimal canaliculitis in patients presenting to a tertiary eye care center in the Middle East.

PURPOSE: To study the clinical features and bacteriology of canaliculitis in patients presenting to King Khaled Eye Specialist Hospital (KKESH), a major tertiary eye care center in the Middle East and compare the results to previous studies from other countries. METHODS: In this retrospective study, a chart review was performed of 131 patients (135 eyes) diagnosed with lacrimal canaliculitis who underwent treatment between January 1983 and December 2012 at KKESH. Data were evaluated on demographics, presenting signs and symptoms, diagnostic studies, causative organisms, treatment rendered including medical or surgical interventions and rate of recurrence. RESULTS: There were 47 males and 84 females with a mean age of 64 years. The average duration of symptoms was 81.38 weeks. The most common presenting symptom was eye discharge (68.7%). The lower canaliculus was most commonly involved (49.6%) and 27 (20.6%) patients had upper and lower canaliculi involved. The left eye was most commonly involved in 71 patients (54.2%). Microbiological studies were available for 101 (77.1%) patients. Streptococcus species (48.2%) were the most commonly cultured organisms. Concretions were noted in 45 (34.4%) patients. Canaliculotomy was performed in 33 (25.2%) patients. Topical Penicillin G was the most commonly used antibiotic (65.7%). Seventeen (13%) patients had a recurrence of canaliculitis. CONCLUSION: Canaliculitis is frequently overlooked and misdiagnosed as conjunctivitis. Persistence or recurrence may complicate the condition. New organisms are emerging as the most common causative agents. Canaliculotomy with removal of all concretions is still considered the gold standard of treatment to eliminate the infection and improve patient symptoms.

Ocular adnexal lymphoproliferative disorders in an ophthalmic referral center in Saudi Arabia.

PURPOSE: To study the pattern of ocular adnexal lymphoproliferative disorders (OALD) in an ophthalmic referral center in Saudi Arabia and to review their, histopathological characteristics with clinical correlation. METHODS: Retrospective chart review of 40 cases of patients who underwent incisional biopsy with the suspected diagnosis of periocular and/or adnexal lymphoid lesions over the period: 2000-2012 at the King Khaled Eye Specialist Hospital (KKESH), Riyadh, Saudi Arabia. The routine histopathologic slides are reviewed by a single pathologist to identify cases of Benign Reactive Lymphoid Hyperplasia (RLH), Atypical Lymphoid Hyperplasia and probable lymphoma. The identification of the specific types of lymphoma is performed at a tertiary general hospital: King Faisal Specialist Hospital and Research Centre (KFSH&RC). RESULTS: Forty patients are included with an age range of 11-91 years and a median of 36 years. The males constitute 70% and females 30% of the cases. The right eye and/or orbit are involved in 48%. The left eye is involved in 45% while a bilateral disease is found in 7.5%. The median duration of symptoms is 5 months. The site distribution is conjunctiva (42.5%), orbit (25%), lacrimal gland (12.5%), eyelid (10%), lacrimal sac (7.5%) and caruncle (2.5%). One case is excluded after histopathologic diagnosis of malignant melanoma. Diagnosis in the remaining 39 cases includes: RLH in 14 cases (35%), atypical lymphoid hyperplasia in three cases (9%), and lymphoma in 22 cases (56%). Classification of the lymphoma group is: extranodal marginal zone lymphoma (EMZL) in 9/22 cases (41%), diffuse large B cell lymphoma (DLBCL) in 4/22 cases (18%), Castelman's disease in 3/22 cases (14%), Burkitt's lymphoma in 2/22 cases (9%), follicular lymphoma and T cell-rich B cell lymphoma: one case each (4.5%).Two cases remain unclassified. CONCLUSION: We have a wide age range which is comparable to other studies. Our results show male predominance and the commonest site of involvement is conjunctival, however if RLH cases are excluded, the commonest site for lymphoma is orbit/lacrimal gland in 45% followed by conjunctival in 23%. The commonest type of lymphoma is: EMZL in 41% followed by DLBCL in 18% then other types of lymphoma including follicular lymphoma.

Unilateral optic neuritis, scleritis and exudative retinal detachment due to recurrent orbital pseudotumor.

Idiopathic orbital inflammation, also known as orbital pseudotumor, describes a spectrum of idiopathic, non-neoplastic, non-infectious, space-occupying orbital lesions without identifiable local or systemic cause. The condition occurs mainly in young adults who may present with acute pain, proptosis, chemosis and limitations of extraocular movements. Decreased vision due to optic nerve infiltration and macular edema as a result of scleritis is less common sequel of orbital pseudotumor. Herein, we present a case of unilateral orbital pseudotumor in a young male who presented with chief complaints of painful decreased vision which was attributed to optic neuritis and macular edema as a result of scleritis. Imaging studies were helpful in the establishment of the correct diagnosis and patient's complaints improved with administration of systemic corticosteroids.