Lacrimal sac ciliated HPV16 positive, adenosquamous carcinoma-A case report of a unique histological variant at this site and a review of the literature.

A 47-year-old female developed a reddish swelling of the right medial canthus over 3 months. On examination, a red, firm mass, involving the right medial canthal and extending into the inferior fornix was present and the globe was displaced upwards and inwards. A staging MRI scan confirmed a lacrimal sac lesion with anterior orbit extension. After an equivocal biopsy, the patient underwent debulking surgery. Histology showed a lacrimal sac invasive adenosquamous carcinoma, comprising poorly differentiated squamous carcinoma and invasive adenocarcinoma areas arranged in a tubulo-glandular pattern. The adenocarcinoma harboured numerous cilia. p16 showed block positivity of both components and micro-dissected tissue from both areas showed the presence of HPV16 DNA by PCR. This is the first description of ciliated adenosquamous carcinoma of the lacrimal sac and this finding is placed into the context of what is known about ciliated head and neck adenosquamous carcinomas and the role of high-risk HPV.

Genetic Profiling of Primary Orbital Melanoma: An Analysis of 6 Cases with Clinicopathologic Correlation.

PURPOSE: To analyze the genetic profile of 6 cases of primary orbital melanoma with clinicopathologic correlation. DESIGN: Retrospective noninterventional study to analyze the genetic profile of 6 cases of primary orbital melanoma and to correlate the genetic findings with prognosis and clinicopathologic features. Inclusion criteria were patients with primary orbital melanoma with no evidence of primary eyelid skin, conjunctival, uveal, or remote melanoma at extraocular sites. PARTICIPANTS: The study involved 6 primary orbital melanomas from 6 patients. Four patients were exenterated and 2 had incisional biopsies performed. METHODS: Clinical notes and radiologic records were assessed to ascertain clinical tumor behavior. Sections were stained with hematoxylin-eosin and exposed to immunohistochemistry for S100, MelA, HMB45, Sox10, and BAP1. Melanoma DNA was exposed to array comparative genomic hybridization to assess gross chromosomal copy number changes. Point mutation assessment and Sanger sequencing were performed for GNAQ, GNA11, BRAF, NRAS, pTERT, SF3B1, and EIF1AX. MAIN OUTCOME MEASURES: These were the presence of gross chromosomal copy number changes and the presence of mutations in GNAQ, GNA11, BRAF, NRAS, pTERT, SF3B1, and EIF1AX; the presence of metastases and time period between diagnosis and death from melanoma; and correlation between the tumor genetic profile and the clinical behavior of the tumor. RESULTS: One of the 6 cases was clinically associated with oculodermal melanocytosis. Of the 6 patients, 3 died of melanoma metastases and 1 of unrelated causes; 2 remain alive at last review. Three of the 6 cases were histologically associated with a benign precursor lesion. All melanomas expressed S100, MelA, HMB45, and Sox10. One patient showed loss of BAP1 nuclear staining. The most frequent chromosomal gains across the 6 cases, in order of frequency, were 6p, 8q, 17q, 6q, and 20p. The most frequently lost regions were 1p, 9p, 16q, and 17p. One patient showed monsomy 3 and gain of 8q (and showed the BAP1 loss). Mutations were found in GNAQ (1 case), GNA11 (1 case), SF3B1 (2 cases), NRAS (2 cases), and pTERT (2 cases). CONCLUSIONS: The data point to 2 genetic groups for primary orbital conjunctiva melanoma-like and a uveal melanoma-like group. A larger study would help confirm this suggestion.

Excision and delayed reconstruction with paraffin section histopathological analysis for periocular sebaceous carcinoma.

PURPOSE: To evaluate the use of excision and delayed reconstruction with rapid paraffin section analysis in patients with sebaceous carcinoma (SC) of the periocular region. METHODS: A retrospective study of patients with SC. Patients were identified from a contemporaneously maintained database and medical notes reviewed. Data were collected on known risk factors. Standard management started with conjunctival mapping biopsies. The tumor was excised with a 3-mm clinical margin and sent in formalin for histopathological analysis. The patient went home with dressings and returned 3 days later. Further excision or reconstruction was performed as indicated. Follow-up data were collected. RESULTS: Seventeen patients had excision and delayed reconstruction with paraffin section control. Ten had clear margins after 1 excision, and 7 were clear after 2 excisions. Reconstructive technique varied according to the defect. Three patients developed further tumor. One of these had a local recurrence treated with further excision and reconstruction. One developed a multicentric tumor with regional metastasis, and the third patient developed distant metastasis. Two patients died from SC. Average follow up was 5 years (2-9 years). CONCLUSIONS: Excision and delayed reconstruction using paraffin section histopathological analysis are in widespread use for the management of basal cell carcinomas in the periocular region. While some authors advocate the use of Mohs' micrographic surgery in patients with SC, this technique has been questioned due to the possible misinterpretation of subtle intraepithelial pagetoid spread with frozen section analysis. To preserve the function of the eyelid and ease of reconstruction, it is important to try and preserve as much healthy tissue as possible while effecting a successful excision. Excision and delayed reconstruction offer an excellent option for the management of this rare and highly malignant tumor.

EyeCee One preloaded intraocular lens: are patients with glaucoma more at risk?

OBJECTIVE: The Medicine and Healthcare products Regulatory Agency reported links of raised intraocular pressure (IOP) with recently implanted EyeCee One intraocular lens (IOL). This work investigates if glaucomatous eyes were more susceptible to these postoperative IOP rises and if they required more intensive management. METHODS: Retrospective observational study of all phacoemulsification surgery with implanted EyeCee One IOL, performed between 1 October 2022 and 26 January 2023 inclusive. ANALYSIS: A significant IOP elevation was defined as an IOP rise of 10 mm Hg or more from preoperative to maximal postoperative IOP reading. The management of all patients who had a significant IOP elevation was reviewed. Glaucoma/ocular hypertension cases were identified and analysed against non-glaucomatous eyes and statistical analysis performed. RESULTS: 112 glaucoma and 671 non-glaucoma cases identified; 19.6% of the glaucoma cohort had a significant postoperative IOP rise compared with 8.9% of patients without glaucoma (OR 2.49 (95% CI 1.45 to 4.20) p=0.0014). In the glaucoma cohort, 12.5% had an increase in the number of topical IOP-lowering agents (mean increase 1.65±1.58), 6.3% required systemic treatment and 2.7% surgical intervention. In the non-glaucoma group, 3.3% required topical treatment (mean number of agents 0.88±1.34), 0.8% required systemic treatment and 0.2% surgical intervention. CONCLUSION: This study shows that during the time frame in question, patients with glaucoma or ocular hypertension who had an EyeCee One IOL were almost two and a half times more likely to have a postoperative rise of 10 mm Hg or more in IOP following routine cataract surgery, requiring more aggressive management.

Ocular Trauma During the COVID-19 Lockdown.

Purpose: To identify the impact the COVID-19 lockdown had on the presentation and management of sight-threatening ocular trauma.Methods: A retrospective cohort analysis of all patients who presented to the Ophthalmology department of Royal Hallamshire Hospital Sheffield with serious ocular trauma during the COVID-19 lockdown period was performed. Data on mechanism of injury and date of injury, presentation, and surgical repair were collected. This process was repeated for the same dates in the previous 5 years for comparison.Results: During the COVID-19 lockdown period, we saw 10 cases of serious ocular trauma (4 globe ruptures, 4 full-thickness lid lacerations, and 2 intra-ocular foreign bodies). This is 3.33 times the average number of cases over the previous 5 years. The delay between injury and presentation rose to 1.1 days compared to 0.33 days pre-COVID; however, the time taken between presentation to surgery was only slightly affected (12 hours in 2020 compared to 11.38 hours pre-COVID).Conclusion: During the COVID-19 lockdown, the number of serious ocular trauma cases was more than three times the average of the previous 5 years. This increase is partially due to more DIY injuries as people stayed at home, but also surprisingly an increase in falls. There did appear to be a longer delay between injury and presentation, suggesting that patients were reluctant to come into hospital during the pandemic. Surgery was performed within 12 hours on average for both groups, reassuringly indicating that sight-saving surgery was not delayed despite extraordinary circumstances.

To implant or not to implant: emergency orbital eviscerations with primary orbital implants.

BACKGROUND/OBJECTIVES: To evaluate the outcomes of orbital evisceration with primary implant placement in acutely infected/inflamed eyes, using implant exposure/extrusion as a surrogate of success. To contextualise this with previously published literature. SUBJECTS/METHODS: A retrospective case series of all patients with acutely infected/inflamed eyes undergoing urgent orbital evisceration with primary implants, at a British tertiary centre between January 2006 and August 2018. A systematic literature review of orbital eviscerations with primary implant placement in acute endophthalmitis/infection and recent trauma. RESULTS: Twenty-six eyes were eviscerated in the context of acute infection/inflammation. Twenty-four eyes had primary orbital implants. Indications for evisceration included endophthalmitis (18/26, 69%), microbial keratitis with corneal perforation (4/26, 15%), non-infectious corneal perforation (3/26, 12%), and recent trauma (1/26, 4.8%). The implants used were acrylic (15/24, 63%), MEDPOR (5/24, 21%), and silicone (4/24, 17%). The follow-up period was 15 months to 14 years. Implant exposure occurred in two (8.3%), managed with implant exchange and scleral reformation in one, and implant removal with dermis fat grafting in the other. One patient (4.2%) had conjunctival wound dehiscence with spontaneous healing. Six (25%) required further surgery for minor complications as follows: conjunctival prolapse, upper lid ptosis with slight sulcus loss, lower lid entropion with shortened fornix, and lower lid ectropion. The systematic literature review showed that the mean rate of orbital implant exposure/extrusion in this subset of patients was 7.8% (95% CI: 2.7%, 12.9%, SD 8.0%), range 0-27%. CONCLUSIONS: In acutely infected/inflamed eyes, the implant exposure/extrusion rate following orbital evisceration with primary implant placement is acceptable.

Neuro-Ophthalmic Complications of Vestibular Schwannoma Resection: Current Perspectives.

Vestibular schwannomas (VSs), also called acoustic neuromas, are benign intracranial neoplasms of the vestibulocochlear (VIII) cranial nerve. Management options include "wait-and-scan," stereotactic radiosurgery and surgical resection. Due to the proximity of the VIII nerve to the facial (VII) nerve in the cerebello-pontine angle, the VII nerve is particularly vulnerable to the effects of surgical resection. This can result in poor eye closure, lagophthalmos and resultant corneal exposure post VS resection. Additionally, compression from the tumor or resection can cause trigeminal (V) nerve damage and a desensate cornea. The combination of an exposed and desensate cornea puts the eye at risk of serious ocular complications including persistent epithelial defects, corneal ulceration, corneal vascularization, corneal melting and potential perforation. The abducens (VI) nerve can be affected by a large intracranial VS causing raised intracranial pressure (a false localizing sign) or as a result of damage to the VI nerve at the time of resection. Other types of neurogenic strabismus are rare and typically transient. Contralaterally beating nystagmus as a consequence of vestibular dysfunction is common post-operatively. This generally settles to pre-operative levels as central compensation occurs. Ipsilaterally beating nystagmus post-operatively should prompt investigation for post-operative cerebrovascular complications. Papilledema (and subsequent optic atrophy) can occur as a result of a large VS causing raised intracranial pressure. Where papilledema follows surgical resection of a VS, it can indicate that cerebral venous sinus thrombosis has occurred. Poor visual function following VS resection can result as a combination of all these potential complications and is more likely with larger tumors.

Eyelid skin trichilemmoma and underlying local malignancy: is an aggressive treatment necessary?

OBJECTIVE: Trichilemmoma is a benign tumour derived from the outer root sheath of hair follicles. Trichilemmoma can be associated with basal cell carcinoma (BCC), either as a collision lesion or from malignant transformation. This study evaluates malignancy associated with eyelid trichilemmoma and principles of treatment. METHODS AND ANALYSIS: Retrospective study involving biopsy-proven eyelid trichilemmoma cases over 14 years encountered at a tertiary referral centre. Presenting features, differential diagnosis, type and number of operations required and histopathological features including coexisting BCC were analysed. RESULTS: We identified 36 cases with an average age of 66 years. The clinical differential diagnoses were mainly BCC (44%), papilloma (36%) and squamous cell carcinoma (SCC) (3%). Three patients (8%) had trichilemmoma with associated BCC. Of the 19 cases (53%) of trichilemmomas without BCC with equivocal surgical margins, seven patients (19%) opted for further excision while 12 patients (33%) opted for observation and were discharged. A patient re-presented two years later with invasive BCC. Overall, 11% of our biopsy-proven eyelid trichilemmoma cases were associated with BCC. CONCLUSION: Patients should be informed that a proportion of incompletely excised eyelid trichilemmomas may conceal underlying BCC. Therefore, further surgery to achieve clear surgical margins should be offered. Patients who opt for observation should be offered 6-monthly follow-up for three to five years. Alternatively, they can be discharged with advice to report any recurrence of lumps, skin changes or loss of lashes at the site of previous lesion.

Management of recurrent sebaceous gland carcinoma.

OBJECTIVE: To evaluate the incidence and management of recurrent periocular sebaceous gland carcinoma at a tertiary ocular oncology service in the United Kingdom. METHODS: This was a retrospective cohort study of 62 patients with sebaceous gland carcinoma treated between 2004 and 2017. A total of 10 eyes were treated for local recurrence. The following variables were recorded: age and sex of patient; tumour location, histological subtype; recurrence type; treatment and outcome. RESULTS: Of the 62 cases with eyelid SGC, 10 (16%) had recurrences during the study period and satisfied inclusion criteria. There were six (60%) females and four males in the recurrent group. The mean time interval between initial excision and tumour recurrence was 37 months (median 23 months; range 4 to 84 months). Four patients received cryotherapy to the lids and conjunctiva to control recurrent disease and two patients were treated with topical or intralesional chemotherapy. Four patients (40%) underwent orbital exenteration during the study period. Metastasis occurred in 20% over a mean follow-up of 113 months (median 106; range 47-184 months). CONCLUSIONS: The risk factors for local recurrence of SGC after wide excision with paraffin section control were reported, and an approach to these recurrent lesions was proposed. The results of this study will help guide surgeons dealing with the medical and surgical conundrum of recurrent disease. The risk of recurrence is highest in the first 2 years after initial excision.

Staged excision of primary periocular basal cell carcinoma: absence of residual tumour in re-excised specimens: a 10-year series.

AIMS: The aim is to study staged periocular basal cell carcinoma (BCC) excision in a tertiary oculoplastic referral centre in Sheffield, UK. In particular, we examined patients with close or positive margins and no tumour seen on re-excision to identify demographics and tumour characteristics in this population. METHODS: A retrospective review of medical records of 437 cases of staged periocular BCC excisions over a 10-year period (2007-2017) was carried out. Patients had surgical excision with 3 mm clinically clear margins. Staged excision was performed for all cases included in this study. Standard reconstruction techniques were employed. Histopathology was analysed for tumour type, subtype and stage. RESULTS: Over the 10-year period, of the 437 periocular BCCs, 156 had close or involved margins. Residual tumour was found in 29 (18.6%), whereas in 122 eyelids of 120 patients (78.2%) no residual tumour was identified on histological examination. Micronodular (54.1%) and nodular (23.7%) growth patterns of BCC, as well as lower eyelid location (72.1%), were the most prevalent in this population. Two patients (1.6%) had recurrence of BCC over a mean follow-up of 57 months (range 1-125 months). CONCLUSIONS: A significant proportion of BCCs transected on initial excision show no residual tumour in the re-excision specimens. In the interval between initial excision and re-excision, there may be eradication of the residual tumour. The exact mechanisms for this are unclear, however, and re-excision remains the appropriate recommended course in the presence of involved surgical margins of periocular BCC, particularly when high-risk tumour subtypes are encountered.