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BACKGROUND: As for rhabdomyosarcoma (RMS) of other anatomic regions, the evaluation of traditional clinicopathological parameters does not allow the unequivocal outcome prediction of the single cases of orbital RMS. We investigated the role of DNA ploidy and immunohistochemical expression of p53, bcl-2, MDR-1 and Ki67 (MIB1) in the prognostic evaluation of orbital rhabdomyosarcomas. MATERIALS AND METHODS: The study population consisted of 11 selected cases. Serial sections of each tumor, stained with Feulgen's technique, were analyzed for the DNA content, using the QUANTIMET 500c Leica analyzer, QWINVO200A software. The results were compared with the immunohistochemical expression of p53 (wild plus mutated, W&M and mutated), bcl2, MDR-1 and Ki67 (MIB1), and with follow-up data. RESULTS: The statistical analysis of results showed that the cases of tetraploid and/or multiploid RMS, overexpressing p53 (W&M and mutated) and MDR-1, were characterized by an overall worse prognosis. On the contrary, the tumors with a favourable clinical course showed hyperexpression of MIB1 and absence of mutated p53 expression. Significantly higher MIB1 expression was found in the relapse-free group of tumors, with respect to the RMS with relapse (both in primary tumors and relative relapses, p0.05). CONCLUSION: The evaluation of DNA ploidy, p53, MIB1 and MDR-1 expression could be used for subtyping of orbital RMS into two prognostically different subcategories, respectively RMS responder to the therapy, with favourable clinical outcome, and RMS with a worse prognosis, requiring more aggressive therapeutic protocols.

Summary. T(1;14) (p22;q32) involving BCL10 and IGH genes is a rare but recurrent chromosomal aberration in MALT-type lymphoma. It is rarely described in ocular adnexa B cell lymphomas, although nuclear BCL10 shuttling seems to be critical for disease progression in this district. We have evaluated the translocations MALT lymphoma-related in a series of 45 ocular adnexa cases, focusing in particular on their relation with BCL10 expression and its cellular topographic distribution. A prognostic tissue microarray (TMA) with ocular adnexa MALT lymphomas was designed. A study of BCL10 expression and its topographic distribution was performed through immunohistochemistry. In addition the assessment of t(14;18) (q32;q21), t(1;14) (p22;q32) and t(11;18) (q21;q21) was determined by Fluorescent In Situ Hybridization (FISH). Our series revealed t(14;18) (q32;q21) in 6/43 cases (14,3%). t(1;14) (p22;q32), never described in ocular adnexa MALT lymphomas, was observed in 3/31 (9,7%), two of which exhibited the gain of 3’ upstream BCL10 gene signal (4%), whereas no case showed t(11;18) (q21;q21). Moreover, BCL10 expression was observed in 18/45 cases. In particular its nuclear expression was revealed in 12/45 cases, cytoplasmic expression in 5/45 and both cytoplasmic and nuclear expression in 1/45. Statistical analysis demonstrated that while BCL10 cytoplasmic expression is significantly related to the presence of the investigated chromosomal aberrations, in particular with t(14;18) (q32;q21), BCL10 nuclear shuttling does not show any correlation with these translocations. Our data support that BCL10 nuclear distribution is neither related to BCL10 rearrangement nor to other known translocations.

Objectives To evaluate the outcomes of strabismus surgery in patients with thyroid eye disease (TED) and to assess the influence of preceding treatments such as steroid administration, orbital decompression and radiotherapy. Methods Retrospective study on a cohort of patients with TED undergoing strabismus surgery. Data collection included clinical features, smoking status, preceding steroid therapy, orbital radiation therapy and orbital decompression. Qualitative evaluation, assessing diplopia and quality of life and quantitative evaluation, assessing the eye misalignment were analysed 1 year after the last squint surgical procedure. Results Seventy-nine patients were identified. Ninety-five surgical procedures were performed on 92 eyes. A significant overall improvement of ocular motility was registered ( p Conclusion Strabismus surgery in TED patients resulted in a high success rate in reducing diplopia and improving ocular motility. The success rate was not negatively associated with previous therapies. Indeed, resolution of diplopia required more squint procedures in patients who have previously undergone to orbital decompression.

Purpose: To assess risks and benefits associated with surgical excision of orbital cavernous venous malformation and analyze factors influencing the outcome. Methods: Design: cohort study. Participants: 164 consecutive patients undergoing surgical removal of orbital cavernous venous malformation. Intervention: lateral or anterior orbitotomy. Outcome measures: visual acuity, visual field, pupillary function, ocular motility, and fundus examination. Influence of the mass volume and tumor location on the outcome was evaluated. The threshold value and risk group for the volume were evaluated using the receiver operating characteristic analysis; 8 orbital different quadrants, extraconal/intraconal position, and orbital apex extension were considered as variables to evaluate the location. Results: Postoperatively overall visual acuity (p = 0.0084), visual field (p < 0.0001), diplopia (p < 0.0001), proptosis (p < 0.0001), ocular motility (p = 0.02), ptosis (p = 0.033), choroidal fold (p < 0.0001), disk edema (p = 0.0004) were significantly improved. The commonest location was the lateral quadrant (28%). Choroidal folds were associated with tumor greater than 1100 mm3(p = 0.001). Visual loss occurred in 2 patients (1.2%) and tonic pupil in 5 (3%,) Visual deterioration was associated with the apical extension (p = 0.001). Pupil efferent defect was associated with lateral quadrant location (p = 0.011) and apical extension (p = 0.05). Conclusions: Surgical removal of orbital cavernous venous malformation is a useful treatment modality, particularly for large size tumors. Removal of cavernous venous malformations located at the lateral aspect of the orbit carries an increased risk of permanent tonic pupil. Postoperative blindness is rarely seen and is often associated with the apical extension. Tailoring the surgical approach, depending on the relative position to the optic nerve, may have an important bearing on outcome.

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.

BACKGROUND: Solitary fibrous tumors (SFT), formerly called hemangiopericytoma, are rare tumors derived from mesenchymal cells originally described in the pleura, but these tumors may affect extraserosal tissues including the lacrimal gland and orbit. OBJECTIVE: Conduct a multi-centered clinical, radiological and histopathological analysis of 17 orbital SFT cases. DESIGN: A retrospective case series. SETTING: Three eye centers in two countries. PATIENTS AND METHODS: The data collected from the charts of 17 adult patients presenting with tissue diagnosis of orbital hemangiopericytoma or SFT from January 2003 to December 2018 included demographics, clinical imaging and histopathological information including immunohistochemical (IHC) characteristics. MAIN OUTCOME MEASURES: The demographic characteristics, clinical presentation, and histopathological patterns or variants of SFT were analyzed. SAMPLE SIZE: 17 adult patients. RESULTS: Mean age was 45 years (range 23-80 years). Male to female ratio was 3:1. The right eye was affected in 12 (70.5%) patients. Commonest presentation was proptosis in 13/17 (76% of patients). Other symptoms were impaired motility (29%) and ptosis (11%). Lesions mostly affected the medial orbit (35%), then orbital apex in 11%. The histopathological classic pattern-less variant was the commonest. One case with aggressive behavior, multiple recurrences and atypical features was encountered. Immunohistochemical (IHC) markers used included CD34 expression in all cases, Bcl-2 expression in 10/11, CD99 in 9/9 and Vimentin in 4/4. STAT6 was used in 2 cases. CONCLUSIONS: SFTs are rare tumors affecting the orbit in both genders equally in their mid-forties, but showed male predominance in our analysis with a predominant classic histopathological pattern. Tissue diagnosis is essential and requires IHC studies for confirmation. LIMITATIONS: Sample size is relatively small owing to the rarity of this tumor in the orbit. CONFLICT OF INTEREST: None.

Purpose Programmed cell death protein 1 (PD-1) and DNA mismatch repair (MMR) deficiency play an important role in tumour progression and response to treatment. Both markers have been studied in some ocular tumours but little is known about these markers in orbital tumours. This pilot study reports on PD-L1 expression and MMR mutations using next generation sequencing (NGS) in specific orbital tumours. Methods We reviewed surgical specimens from patients with rhabdomyosarcoma, adenoid cystic carcinoma (ACC), pleomorphic adenoma (PA) and biopsy negative tissue from orbital tumours used as a control. immunohistochemistry (IHC) was performed on Formalin fixed paraffin embedded tissue using a PD-L1 antibody. DNA was extracted for targeted gene panel NGS of the MMR genes PMS2, MLH1, MSH6 and MSH2. Results The study included 17 orbital specimens. Scattered membrane PD-L1 staining was noted in 3/6 rhabdomyosarcoma specimens without an accompanying lymphocytic infiltrate. PD-L1 immunostaining was absent in 3/3 ACC, and 5/6 PA specimens. PD-L1 immunostaining was not detected in 2/2 control specimens. 4/17 samples shared the same pathogenic mutation in the MLH1 gene, including 3/6 rhabdomyosarcoma and 1/3 ACC samples. 1/6 PA samples had a mutation in MSH6. Conclusions Our study demonstrated scattered, non-quantifiable or absent PD-L1 staining in a limited sample of orbital tumours suggesting that PD-1/PD-L1 inhibitor therapy may not be useful in treatment of malignant orbital tumours (rhabdomyosarcoma and ACC) when refractory to conventional therapy. Our pilot study suggest that PD-L1/MMR axis might not play a major role in the pathogenesis of primary orbital tumour.

Although most commonly benign, neurofibromas (NFs) can have devastating functional and cosmetic effects in addition to the possibility of malignant transformation. Orbitofacial NFs, in particular, may cause progressive, disfiguring tumors of the lid, brow, temple, face, and orbit, and clinical evidence suggests that they may have increased local aggressiveness compared to NFs developing at other sites. The purpose of this study was to identify biological differences between orbitofacial NFs and those occurring at other anatomic sites. We performed RNA‐sequencing in orbitofacial (n = 10) and non‐orbitofacial (n = 9) NFs. Differential gene expression analysis demonstrated that a variety of gene sets including genes involved in cell proliferation, interferon, and immune‐related pathways were enriched in orbitofacial NF. Comparisons with publicly available databases of various Schwann cell tumors and malignant peripheral nerve sheath tumor (MPNST) revealed a significant overlap of differentially expressed genes between orbitofacial versus non‐orbitofacial NF and plexiform NF versus MPNST. In summary, we identified gene expression differences between orbitofacial NF and NFs occurring at other locations. Further investigation may be warranted, given that orbitofacial NF are notoriously difficult to treat and associated with disproportionate morbidity., Global gene expression differences between orbitofacial neurofibromas and neurofibromas occurring at other anatomic locations were deteted through RNA sequencing analysis. Differences in pathways involved in cell cycle and inflammation were specifically detected through gene enrichment analyses, supporting prior clinical observations suggesting that orbitofacial neurofibromas are biologically distinct compared to neurofibromas developing in other anatomical sites.

Purpose: To compare the number of eye surgical procedures performed in Italy during the first month of lockdown with those performed in the same period in 2019. Methods: Review of the surgical procedures performed from 10 March to 9 April 2019 and from 10 March to 9 April 2020 (the first month of lockdown because of the COVID-19 outbreak) at six academic institutional centers of Italy. A distinction was made between urgent procedures: any trauma repairment, trabeculectomy/drainage implant for glaucoma, any operation for rhegmatogenous retinal detachment (RRD) repair, pars plana vitrectomy (PPV) for vitreous hemorrhage (VH), macular hole, or retained lens fragments; elective procedures: corneal transplant, phacoemulsification for cataract extraction, silicone oil removal, and PPV for epiretinal membrane; and intravitreal injections (either anti-vascular endothelial growth factor [VEGF] or dexamethasone) to treat exudative maculopathies. The main outcome measure was the rate of reduction in urgent and elective surgeries performed. Results: Overall, 3624 and 844 surgical procedures were performed from 10 March to 9 April 2019 and from 10 March to 10 April 2020, respectively (−76.7% in 2020 compared to 2019, p Conclusion: A significant reduction in the rate of urgent and elective surgeries and intravitreal injections was recorded during the first month of lockdown compared to the same period in 2019. With this analysis, the authors hope to provide some preliminary insights about the consequences of lockdown for the eyecare system in Italy.

Purpose: Several studies have reported conflicting results on ocular manifestations and transmission of coronavirus disease 2019 (COVID-19) whose causative virus, SARS-CoV-2, belongs to the coronavirus family, the seventh recognized as a human pathogen and the third causing a severe clinical syndrome. COVID-19 primarily affects the lungs, similar to the other human coronaviruses. Comparing the relation between the animal-to-human transmitted coronaviruses (SARS-CoV-1, SARS-Cov-2, MERS-CoV, CoV-229E, NL63, OC43, HKU1) and the eye may contribute to determining their actual eye-tissue tropism and risk of ocular transmission. Methods: Literature review was conducted via Pubmed.gov, Google Scholar and medRixv using the following keywords: COVID-19, SARS-CoV-2, SARS-CoV-1, MERS-CoV, CoV-229E, NL63, OC43, HKU1, conjunctivitis, tear swab, ocular expression, ocular symptoms and human angiotensin converting enzyme-2 expression. Studies with lack in methodology were excluded. Results: Sixteen observational studies were selected. The range for detection of viral RNA in tears was 0–8% for SARS-CoV-1 and 0–5.3% for SARS-CoV-2, while no reports were found for other coronaviruses. Ocular manifestations have been reported for NL63 and SARS-CoV-2. Ocular symptoms in the form of conjunctivitis/conjunctival congestion predominantly were detected in 65 (3.17%) out of 2048 reported patients with COVID-19 (range of 0.8–32%). Eye symptoms were not reported for the other coronaviruses. Conclusions: Data aggregation for coronaviruses shows a relatively low eye-tissue tropism. Conjunctival congestion is an uncommon manifestation of COVID-19 similar to all human coronaviruses’ infections. In a low percentage of patients, the virus can be excreted in ocular fluids at different stages of the infection, regardless of positive SARS-Cov-2 throat swab. Albeit high viral loads in ocular tissue seem to have relatively low prevalence, the eye should be regarded as a potential source of infection dissemination for COVID-19.

AIMS: The aim of this study is to assess the value of ultrasound in the differential diagnosis of orbital lesions. METHODS: One hundred and thirty-eight patients with clinical features of an orbital mass were examined by orbital ultrasound prior to undergoing surgery, from January 2000 to January 2017. All patients underwent excisional or incisional orbital biopsy. The results of orbital ultrasonography were compared with the final histological diagnosis. RESULTS: Orbital lesions were localized by ultrasonography in 133/138 cases (96.4%); the false-negative rate of orbital echography was 3.6% (5/138). The nature of the orbital lesions was correctly determined by ultrasonography in 54.3% of the cases (75/138) preoperatively (true positives). In 58/138 (42%) patients, there was no correspondence between the ultrasound diagnosis and the histological diagnosis (false positives). The sensitivity of orbital ultrasonography for the detection of an orbital mass was 93.75% (CI 87.87–99.63%), while the specificity yielded no meaningful result (CI 0.00–60.24%). Moreover, the specificity of orbital ultrasonography to identify a malignant tumor falls within a CI of 0–62.72%. CONCLUSIONS: Orbital ultrasonography is a rapid and noninvasive test that is highly sensitive in displaying an orbital mass; however, the specificity in the differential diagnosis of orbital lesions is not meaningful, particularly if malignancy is suspected. The assessment of orbital diseases requires multiple diagnostic approaches to balance the strengths and weaknesses of each method.

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.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.

Purpose: Two new cases of primary cutaneous CD30+ anaplastic large-cell lymphoma (cALCL) of the eyelid are reported; these are analysed alongside existing cases to identify challenges relating to the diagnosis and management of such rare lesions. Material and Methods: A review of existing literature on the PubMed database is conducted using the keywords: ‘eyelid lymphoid proliferations’, ‘lymphoma of the eyelid’, and ‘primary cutaneous CD30+, ALK-anaplastic large-cell lymphoma of the eyelid’. Two new cases of cALCL are reported. Cases where patients present solely with a nodular periocular lesion are analysed for recurrence and survival rate. Results: Two new patients with a painless ulcerated nodule on the upper eyelid receive a confirmed diagnosis of cALCL after undergoing an excisional biopsy. The first, elderly patient has spontaneous remission; the second patient, with a concomitant chronic infection of hepatitis C virus (HCV), presents a more diffuse disease at the onset and requires radiotherapy. Together with 13 patients a primary cALCL identified from 11 previous studies, this constitutes a cohort of 15 patients. Of these, 10 present with an exclusively nodular lesion of the eyelid and four experience disease recurrence; no deaths from cALCL are reported. Conclusion: Differential diagnosis between primary cALCL and lymphomatoid papulosis is essential and requires careful consideration of clinical and pathologic features. Radiologic staging examination is crucial in order to exclude systemic ALCL, particularly for patients with comorbidity. Though cALCL has the pathological features of a malignant lesion, the prognosis seems favourable for patients; a relatively high percentage even experience spontaneous resolution.

PURPOSE: Intravenous antibiotic prophylaxis is used for many clean-contaminated surgeries or clean surgeries with an implant, but its value for clean orbital surgery has not been determined. This study investigated infection risks and adverse effects related to antibiotics in patients undergoing orbital surgery. METHODS: A prospective, nonrandomized comparative case series of all patients undergoing orbital surgery with participating surgeons between October 1, 2013, and March 1, 2015. Types of surgery, antibiotic regimens, corticosteroid use, antibiotic side effects, and surgical site infections (SSIs) were entered into an electronic database and subsequently analyzed. Cases in which patients received postoperative oral antibiotics were analyzed separately. RESULTS: Of 1,250 consecutive orbital surgeries, 1,225 met inclusion criteria. A total of 1208 patients were included in the primary analysis: 603 received no antibiotic prophylaxis (group A), and 605 received a single dose of intravenous antibiotic (group B). Five patients (0.42%) developed an SSI, 3 in group A and 2 in group B. The difference in SSI rates was not statistically significant between the 2 groups (p = 0.66). Antibiotic prophylaxis, alloplastic implants, paranasal sinus entry, and corticosteroid use were not associated with differences in SSI rates. All SSIs resolved on a single course of oral antibiotics; an implant was removed in 1 case. There were no complications associated with a single dose of intravenous prophylaxis. However, 12% of 17 patients (group C) who received 1 week of oral postoperative prophylactic antibiotics developed antibiotic-related complications (diarrhea, renal injury), yielding a number needed to harm of 8.5. CONCLUSIONS: In this large series, antibiotic prophylaxis does not appear to have reduced the already low incidence of SSI following orbital surgery. Given the detriments of systemic antibiotics, the rarity of infections related to orbital surgery, and the efficacy of treating such infections should they occur, patients undergoing orbital surgery should be educated to the early symptoms of postoperative infection and followed closely, but do not routinely require perioperative antibiotics.

Background: Multi-parametric MRI used for preoperative assessment of orbital lesions does not routinely include DCE-MRI, since its accuracy in differential diagnosis of orbital mass is still under debate. Aim of this study is to characterize orbital lesions by multi-parametric MRI, analysing the incremental predictive value of DCE-MRI in differential diagnosis of orbital lesions. Methods: In this prospective triple-blind study, 43 consecutive patients with unilateral orbital lesion underwent conventional multimodal MRI and DCE-MRI before biopsy in a tertiary referral centre. Pre-operative MRI examination including conventional unenhanced MRI protocol, DWI with ADC maps, static CE 3D-T1 w and dynamic CE T1 w sequences, was performed within 1 week from surgery (anterior/lateral orbitotomy depending on location of the lesion, to carry out incisional/excisional biopsy). Results: Comparison between conventional T1 w/T2 w, DWI, CE 3D-T1 w and DCE-MRI groups showed a statistically significant difference in scores distribution (p

Ocular adnexal aterio-venous malformations (AVMs) are rare congenital disabling anomalies, which may enlarge causing disfiguring deformity and rarely severe hemorrhage. These lesions are generally treated by preliminary endovascular embolization to shrink the arterio-venous malformation, followed by surgical gross total resection. The authors report a case of eyelid arterio-venous malformation in a 12-year-old girl, which progressively increased in size in few months. The patient complained mild itching, blurring of the vision, and mild tenderness. Magnetic resonance imaging showed an expansive mass with multiple arterial vessels at the left superior eyelid and left forehead. The diagnosis of arterio-venous malformation was then confirmed by digital subtraction angiography. Primary surgical excision was excluded because of the high risk of intrasurgical bleeding. Embolization through superselective cannulation of the left external carotid feeder vessels was performed resulting in flow exclusion up to the 80% of the nidus. Subsequent surgical resection was not recommended due to clinical evidence of keloid-prone skin.

In response to COVID-19 pandemic the European Society of Ophthalmic Plastic and Reconstructive Surgery (ESOPRS) recommends to avoid any treatment unless urgent or emergent care, in order to reduce ...

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.

To review the clinical and histopathological findings associated with ectopic lacrimal gland tissue.A systematic review of the literature on ectopic lacrimal gland tissue was performed. Studies which met clinical and histolopathological criteria for ectopic lacrimal gland tissue written in English and published in peer-reviewed journals were included.Sixty-nine publications detailing 180 cases of ectopic lacrimal gland tissue were published between 1946 and 2018. Males were affected slightly more than females (57% vs. 43%) and 95% cases were unilateral. Patients presented at a mean age of 21 years, most commonly with a mass, although orbital lesions commonly presented with proptosis. The most common location for ectopic lacrimal gland tissue was epibulbar conjunctiva (62%), other locations included orbital (16%), eyelid (11%), intraocular (9%), lacrimal sac (2%), and nasal mucosa (0.6%) sites. Most lesions (86%) were locally resected. The most common histological types were complex choristomas (56%) and simple choristomas (38%). However, neoplastic transformation to pleomorphic adenoma (5%), adenocystic carcinoma (0.6%), and adenocarcinoma (0.6%) was reported.Ectopic lacrimal gland tissue is rare but should be considered in the differential diagnosis of masses in the epibulbar conjunctiva, eyelid, orbit, and lacrimal sac, particularly in childhood, as the diverse way it presents means that it may mimic more common choristomas. It can undergo neoplastic transformation in the same way as can the lacrimal gland and incomplete excision can result in recurrence.We review the clinical and histopathological findings associated with ectopic lacrimal gland tissue. Ectopic lacrimal gland tissue is susceptible to neoplastic transformation in the same way as is the lacrimal gland.Supplemental Digital Content is available in the text.We review the clinical and histopathological findings associated with ectopic lacrimal gland tissue. Ectopic lacrimal gland tissue is susceptible to neoplastic transformation in the same way as is the lacrimal gland. Supplemental Digital Content is available in the text.

Follicular lymphoma (FL) is the second most commonly reported B-cell lymphoma and accounts for 6% of all orbital lymphomas. FL presentation in the pediatric population is rare. This study reported an 18-year-old male with a pink elevated lesion in the conjunctiva of the left eye and normal ocular parameters. Following a complete excisional biopsy, the lesion was oval and smooth grossly and showed dense proliferation with a high nuclear-cytoplasmic ratio microscopically. Immunohistochemical studies revealed positive CD20 and bcl-6 while bcl-2 was negative, which is a common feature for pediatric FL in contrast to bcl-2 positivity found by immunohistochemistry in FL occurring in adult patient population. No signs of recurrence were observed over 2 years of follow-up. This study adds a new case to the existing few reports. Interestingly, all reported cases were males including the patient, mean duration of onset was 5 months, and mean duration of follow-up was 18.4 months. The authors emphasize that total surgical excision with close monitoring is reasonable management of such clinical entity.Follicular lymphoma (FL) is a rare entity in pediatric patients. Six cases have been reported with conjunctival FL in the literature (age between 6 and 21 years). Interestingly, all of them were male including the patient.

Merkel cell carcinoma (MCC) is one of the rarest eyelid tumors, with high mortality rate due to lymphatic and metastatic spread. We hereby report six cases of patients with histological diag- nosis of MCC referred to our Orbit Unit between 2012 and 2018, focusing on diagnosis, treatment, and subsequent follow up. All patients underwent surgical excision and systemic work-up. Both MCC TNM and eyelid MCC TNM were used to stage lesions. MCC of the eyelid is usually misdiagnosed as benign or other malignant lesions. A prompt examination and a wide local excision are mandatory. A close follow-up of these patients is advised due to high recurrence rate and lymphatic spread

Purpose Myeloid sarcoma (MS) of the orbit is an uncommon condition in occurring in children, generally coupled to myeloproliferative neoplasms. Observations We describe two rare cases of orbital MS in young boys with aggressive local symptoms but without evidence of acute myeloid leukemia (AML), both patients underwent orbitotomy for gross-tumor resection and biopsy. At follow up, there was no evidence of recurrence nor evolution of the myeloproliferative neoplasms clinically and by radiological and laboratory work-up. We also provide a detailed description of the magnetic resonance imaging presentation, with an extensive pathological analysis correlation. Conclusions and importance A comprehensive revision of the literature on isolated orbital MS was carried out with particular emphasis on clues for differential diagnosis and treatment options, stressing the need to consider MS even in the absence of sign and symptoms of an underlying myeloproliferative disorders.

BACKGROUND AND AIMS: To evaluate the efficacy of orbital radiotherapy (OR) for the treatment of thyroid eye disease (TED). METHODS: Thirty-five consecutive patients with active TED with contraindications to steroid therapy received a course of OR. Bilateral retrobulbar irradiation was performed with a total dose of 20 Gy. 7-points clinical activity score (7-CAS), ocular motility, visual acuity (VA), exophthalmos and eyelid retraction were prospectively evaluated at 3, 6 and 12 months and compared with baseline data. RESULTS: There was a statistically significant improvement in 7-CAS at 3, 6 and 12 months post-treatment (p < 0.05). Ocular motility disturbances improved at 6 and 12 months (p < 0.05). Visual acuity remained stable; there was no significant change in exophthalmos (mean 24 mm, SD 3 mm) or eyelid retraction (marginal reflex distance mean 6 mm, SD 1.5 mm) during the follow-up period. No side effects were registered. CONCLUSIONS: This study suggests that OR might be effective in reducing 7-CAS and ocular motility disturbances. No significant improvement in proptosis or eyelid retraction should be expected from this treatment. OR might be considered a suitable alternative treatment in TED for patients who cannot tolerate steroids.

Coexistence of orbital cavernous hemangioma and other vascular malformations is unusual and few cases have been reported. We describe the clinical and radiological features of three cases of orbital cavernous hemangiomas associated with other vascular malformations, selected reviewing a series of 181 cases of cavernous hemangiomas. All patients were males (age ranging from 43 to 67 years) without vascular systemic disorders and/or a clinical syndrome. They experienced slow progressive exophthalmos. One of them developed acute pulsatile proptosis (case 2), while another experienced slow progressive diplopia (case 3). In one case vascular lesions were bilateral (case 3) and in two patients two different lesions coexisted in the same orbit (cases 1 and 2). All patients underwent surgical excision, which was partial in two cases. Two patients had cavernous hemangiomas in association with a venous malformation (a varix in case 1 and a lymphangioma in case 2), while in the other ones (case 3) cavernous hemangioma was associated with a low-flow arteriovenous malformation. No patient denied visual impairment postoperatively. Few cases of orbital cavernous hemangiomas coexisting with other vascular malformations have been reported in the literature. This entity seems to be an association of different variants of orbital vascular malformations, presenting with a wide spectrum of clinical forms and probably with the same pathogenesis.

Background/Aim. To evaluate the efficacy of methotrexate for the treatment of thyroid eye disease (TED). Methods. 36 consecutive patients with active TED, previously treated with corticosteroids but stopped due to the occurrence of side effects, were commenced on methotrexate therapy. Two different weekly doses were administered depending on the weight of the patient (7.5 mg or 10 mg). Clinical activity score (7-CAS), visual acuity (VA), ocular motility, exophthalmos, and eyelid position were retrospectively evaluated at 3, 6, and 12 months and compared with baseline data. Results. There was a statistically significant improvement in 7-CAS at 3, 6, and 12 months after treatment ( ). There was no significant change in visual acuity. Ocular motility disturbances improved at 6 and 12 months ( ). There was no significant change in exophthalmos (mean 24 mm, SD 3 mm) or eyelid position (marginal reflex distance mean 6 mm, SD 1.5 mm) during the follow-up period. No side effects were registered. Conclusions. Methotrexate therapy is effective in reducing CAS and ocular motility disturbances. No significant improvement in proptosis or eyelid retraction should be expected from this treatment. Eventually, it might be considered a suitable alternative treatment in TED for patients who cannot tolerate steroids.

Optic nerve sheath meningioma (ONSM) is a rare orbital tumor that generally induces a slow progressive visual loss in affected patients. Radiotherapy (RT) has currently become the first choice to treat ONSM. In this study our experience in ONSM treatment with fractionated stereotactic radiotherapy (FSRT) is reported. Five patients with diagnosis of orbital ONSM were treated between April 2007 and December 2009 at the Radiation Oncology department of our institution. All patients underwent history and physical, and ophthalmic examinations. Orbital MRI was performed before and 6 weeks after treatment; thereafter every 6 months for the first 2 years. By previous stereotactic localization of the target, RT was delivered with 28 daily fraction of 1.8 Gy by multiple non coplanar arcs dynamically conformed by a micro multileaf-collimator. At diagnosis, in all 5 patients, visual acuity limitations of different degrees were found, while exophthalmos was present in 2, diplopy in 2, orbital pain in 1, and proptosis in 1. In all patients pre-treatment MRI showed an orbital mass involving the optic nerve. After radiotherapy, previous symptoms improved in all patients. However, after RT the MRI consistently showed a stationary status compared to the MRI before RT. At a median follow up of 26 months (range 9-37) all patients had a subjective and/or objective better visual performance than before RT without any evidence of disease progression. No late side effects were recorded. Accordingly to the current literature, our experience confirms the efficacy and the safety of FSRT in patients with orbital ONSM.

Objective To assess the influence of hepatitis C virus (HCV) infection on disease appearance and outcome of ocular adnexal non-Hodgkin lymphoma (ONHL). Design Retrospective comparative study (from January 1, 1992, through December 31, 2006). Methods The medical records of 129 patients with ONHL were retrospectively reviewed. All the patients were tested serologically for the presence of HCV infection. Patients were divided into 2 groups according to the presence or absence of HCV infection. Main Outcome Measures Prevalence of HCV infection, staging to evaluate the extent of disease at the onset, and clinical outcome data on overall and disease-free survival. Results The prevalence of HCV infection among the patients with ONHL was 17.8%. Seropositivity for HCV infection was significantly associated with extraorbital lymphoma at the onset ( P = .006). High prevalence of mucosa-associated lymphoid tissue disease (79.8%) was registered. Protocol therapy included radiotherapy and chemotherapy, depending on the stage of the disease. Complete remission was achieved in 99 patients (76.7%). A total of 23.6% of patients with HCV-seronegative status and 21.7% of those with HCV-seropositive status experienced relapse of the lymphomatous disease. No significant differences in the 5-year overall survival and disease-free survival between the 2 groups were observed. Conclusions Prevalence of HCV infection in patients with ONHL is a relevant issue, accounting for 17.8% of the examined patients. Infection with HCV may influence the initial appearance of ONHL because it is associated with more widespread disease at the onset. However, the overall and disease-free survival of the infected patients are not statistically different than that of patients who are not infected.

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.

A 16-year-old girl with Parry–Romberg syndrome presented with monolateral exudative neuroretinitis and retinal telangiectasis that had been observed for 42 months. She was treated with immunosuppressive therapy with A-cyclosporine for 1 year, followed by laser treatment of telangiectasis. Her visual acuity improved from 20/200 to 20/70 in 1 year with a reduction of neuroretinal exudation. Laser treatment resulted in a further improvement of visual acuity to 20/40. Clinical findings remained unchanged during 2 years of follow-up. The association of immunosuppressive treatment and laser therapy may improve neuroretinal disease in the presence of Parry–Romberg syndrome.

BACKGROUND/AIMS: Localized orbital non-Hodgkin's lymphoma is a rare form of extranodal lymphoma. The aim of this study was to evaluate the outcome of 48 patients presenting with stage I-E non-Hodgkin's lymphoma (NHL). METHODS: Out of 118 charts of patients with lymphoproliferative lesions, those of 48 patients with stage I-E orbital lymphoma seen over a 22-year period from 1977 through 1999 were reviewed. RESULTS: Twenty-five patients were male and 23 were female. Their mean age was 58.7 (12-85) years. The mass was localized inside the orbit in 37 cases and in the lacrimal gland region in 11 cases; bilateral involvement was observed in two cases. The duration of the symptoms at the time of presentation ranged from 10 days to 10 years with a mean duration of 18 months. The 24 cases with low-grade lymphoma are all alive without disease, whatever therapeutic approach was performed (biopsy, radiotherapy, chemotherapy). In intermediate and high-grade NHL the rate of recurrence after chemotherapy has been 50% and 33%, respectively, compared to 75% and 50% after biopsy alone and 75% after radiotherapy (not administered in high-grade forms). CONCLUSION: The therapeutic approach to localized orbital non-Hodgkin's lymphoma must take the histologic grading into consideration. The 24 cases with low-grade lymphoma had a good prognosis. The 17 cases with the intermediate-grade form and the 7 cases with the high-grade form had a better prognosis when treated with chemotherapy.

Purpose To describe ophthalmic complications after nasal and sinus surgery. Methods Four cases with orbital complications were retrospectively selected from among more than 2000 cases of orbital pathologies. Results Motility disturbances due to extraocular muscle injury occurred in two patients after intranasal ethmoidectomy and in one patient after a Caldwell-Luc procedure. In the fourth case an orbital apex syndrome was noted after intranasal ethmoidectomies. Conclusions Ophthalmic complications may occur after nasal and sinus surgery, even using an endoscopic procedure. Successful handling of these complications could be reached by on their early recognition and treatment.

PURPOSE: To illustrate that orbital venous-lymphatic malformations (lymphangiomas) may rarely simulate cavernous hemangiomas. METHODS: Retrospective case review. RESULTS: Five patients were identified from a series of 85 patients with venous-lymphatic malformations. The age range was 21 to 69 years, and all cases presented with a history of slowly progressive or long-standing proptosis. Computerized tomography revealed relatively homogeneous intraconal masses that were well defined anteriorly. Two of the cases had expansion of the orbit, and one had focal calcification. The three who had magnetic resonance imaging showed heterogeneous contrast enhancement. The preoperative diagnosis in every case was cavernous hemangioma, and intraoperatively the lesions resembled cavernous hemangiomas. However, posterior dissection was difficult in all patients because of dense adhesions and, in one case, led to a central retinal artery occlusion. The histology was characteristic of orbital venous-lymphatic malformations in all five cases. CONCLUSIONS: Deep orbital venous-lymphatic malformations presenting in adulthood may be rarely confused with cavernous hemangiomas. In doubtful cases, significant intralesional heterogeneity, best seen on magnetic resonance imaging, and focal calcification may help distinguish the two entities. This differentiation is important, because dissection of venous-lymphatic malformations is fraught with more complications than surgical excision of a cavernous hemangioma.

A total of 145 patients with orbital dermoid cysts examined at the Orbital Clinic, Institute of Ophthalmology, School of Medicine, University of Naples ''Federico II'' over a period of 16 years were reviewed. The orbital cysts were classified as exophytic and endophytic, according to their site of attachment in relation to the orbital rims. This classification can explain the different natural history of these lesions. The exophytic cysts growing externally are discovered in childhood, whereas the endophytic ones are discovered later in life when they produce bone damage, with or without invasion of the adjacent structures.

PURPOSE: To present the proportion of patients with periocular basal cell carcinoma (BCC) who underwent orbital exenteration and to evaluate the significance of the following risk factors: initial tumor site, pathologic features, and initial treatment. DESIGN: Retrospective, comparative, interventional case series. METHODS: Charts of all patients with BCC referred to Orbital Unit of the University of Naples "Federico II" between 1984 and 2003 were reviewed. Charts were reviewed for patient demographics, previous treatments, tumor site, clinical presentation, duration of symptoms, and histologic subtype. The main outcomes were recurrence rate, tumor-related deaths, orbital infiltration, and rate of exenteration. RESULTS: Data (including follow-up) were available for 506 patients. Twenty-eight patients (5.5%) underwent orbital exenteration. For 8 patients (28.5%), orbital exenteration was the first procedure performed. In the exenterated group, the most common tumor site was the medial cantus, whereas in the overall group, it was the lower eyelid (P = .001). The proportion of patients initially treated without margin control was significantly higher in patients undergoing exenteration (P = .0001). Pathologic examination revealed a higher incidence of infiltrative subtype in the exenterated group (P = .00019). CONCLUSIONS: The need for exenteration for BCC may be significantly higher when the lesion involves a medial canthal location, initial management does not include margin-controlled excision, or pathologic analysis reveals an infiltrative subtype. Margin-controlled excision for periocular BCC and close follow-up after excision for medial canthal BCC may be indicated.