Recurrent superior orbital fissure syndrome associated with VEXAS syndrome.

PURPOSE: To describe a case of recurrent orbital inflammation and superior orbital fissure syndrome associated with VEXAS (vacuoles, E1 enzyme, X-linked, auto-inflammatory, somatic) syndrome. CASE PRESENTATION: VEXAS syndrome is a recently identified multi-system inflammatory disease of late adult onset. The authors describe the case of a 76-year-old man who presented with recurrent episodes of orbital inflammation, with superior orbital fissure syndrome, dacryoadenitis and orbital myositis. He had a constellation of systemic disorders including recurrent chest infections, congestive cardiac failure, pulmonary emboli and skin rashes. The underlying diagnosis of VEXAS syndrome was confirmed by genetic testing, which revealed the UBA1 mutation. CONCLUSION: VEXAS syndrome should be considered in the differential diagnosis of orbital inflammatory disease associated with multi-system inflammatory disorders.

Periocular basal cell carcinoma recurrence following surgical treatment: Safe surveillance time.

BACKGROUND: To report the rate of primary periocular BCC recurrence following surgical excision in low-risk and high-risk BCCs, and to propose long term follow up guidelines. METHODS: Retrospective case series of primary BCC treated with surgical excision (Mohs micrographic surgery [MMS], wide local excision [WLE] or fast paraffin excision) who have histologically-confirmed BCC subtype and histologically-measured tumour clearance margins. RESULTS: 77 patients (78 eyelids) were included. Mean age was 72.0 ± 12.8 years with a female predominance (42, 54.5%). Most common histological BCC subtype was nodular (39, 50.0%). 44 (56.47.1%) patients underwent MMS. Tumour clearance was achieved in 59 (75.6%) eyelids after one surgery. 9 had further surgery to achieve tumour clearance while 10 were monitored. There was no statistical significance between recurrence rates in patients who had tumour clearance compared with patients with incomplete tumour clearance after initial surgery (p = 0.15). In patients with incomplete tumour clearance, there was no statistical significance between recurrence rates in those who underwent further surgery versus those monitored (p = 0.47). Average follow-up duration was 37.9 ± 17.2 months. Three (3.9%) cases had recurrent BCC. All three cases had high-risk BCC features (infiltrative subtypes and/or incomplete tumour excision after initial surgery). CONCLUSIONS: There was no evidence of recurrence of completely excised, low-risk BCCs at three years, regardless of type of surgical excision. We recommend patients with completely excised, primary BCCs without high-risk features be monitored for one year. Patients with any high-risk BCC features, such as incompletely excised tumours or high-risk histological subtypes, should be monitored for five years.

Bilateral Giant Fornix Syndrome Associated with Serratia Marcescens.

An 82-year-old woman with macular degeneration receiving intravitreal bevacizumab, presented with 5-months history of muco-purulent blepharo-kerato-conjunctivitis, resistant to multiple topical antibiotics. Visual acuities were 20/25 right and 20/30 left. She had patent nasolacrimal systems, bilateral blepharoptosis, deep superior fornices (21mm right, 22mm left) with muco-purulent discharge, upper tarsal papillae, and punctate keratitis. Bilateral giant fornix syndrome (GFS) was diagnosed, and forniceal swabs grew Serratia marcescens and Pseudomonas aeruginosa, despite current use of intensive topical moxifloxacin. Topical preservative-free Povidone-Iodine 5% twice-daily, two-hourly weaning course of Prednisolone acetate 1% and Ciprofloxacin 250 mg twice-daily were added with complete resolution within 1 week and no recurrence after 3 months. Serratia conjunctivitis has not been previously reported in association with GFS. Conjunctival flora alteration following previous peri-procedural antibiotics and antiseptics, in the setting of a large fornix with redundant, inflamed conjunctiva, may explain the colonization with this opportunistic organism. Topical povidone-iodine 5% appears to be effective for multi-drug resistant opportunistic Serratia conjunctivitis in association with GFS.

Comparison of a manometric tear duct irrigation test with dacryoscintigraphy in the investigation of epiphora.

PURPOSE: Assessment of lacrimal resistance in patients with patent lacrimal systems is important in determining whether dacryocystorhinostomy (DCR) surgery is appropriate. Current techniques, including lacrimal syringing and dacryoscintigraphy (DSG), can be unreliable. We compare the results of a manometric tear duct test (mTDT) irrigation technique with DSG in these patients. METHODS: All symptomatic patients in a specialist lacrimal clinic had full work-up apart from syringing. Lacrimal resistance was assessed using mTDT which applies a fixed head of fluid pressure via a cannula sealed to punctum. Conventional syringing was also performed in cases with abnormal mTDT. Symptomatic patients with delayed tear clearance, no external cause for watering and patent lacrimal systems had DSG. MTDT and DSG results were compared, including in asymptomatic fellow eyes. RESULTS: 105 tear ducts were examined, 85 symptomatic. Symptomatic eyes had a significantly higher mean mTDT resistance (p = .0003) and more had abnormal mTDT (52%) compared to asymptomatic eyes (10%). Although mean tear duct resistance was higher in those with nasolacrimal duct stenosis (NLDS) than freely patent (FP) on syringing (8.5 vs 10.8 dpm), this was not significant (p = .6). High proportions of both FP and NLDS groups had no flow at all on mTDT (60% and 53%, respectively). DSG showed no significant difference in the symptomatic versus asymptomatic eyes (p = .36), nor between those with a normal or abnormal mTDT (p = .25), nor between FP and NLDS groups (p = .25). CONCLUSIONS: In contrast to DSG, the mTDT provides valuable, objective information on lacrimal resistance that can guide a decision to DCR surgery.

Assessment of lacrimal resistance using a manometric tear duct irrigation system.

Lacrimal surgery aims to provide a low-resistance tear drainage passage. An assessment of lacrimal resistance guides decisions on surgery. We present results of a modified tear duct irrigation system that reliably measures lacrimal outflow resistance. Patients in a specialist lacrimal clinic had a full work-up to the point of tear duct syringing. The tear ducts were irrigated using a manometric system, which applied a fixed, known head of fluid pressure to a lacrimal cannula. Fluid flow is recorded and the lacrimal resistance derived as fluid pressure/fluid flow (units cmH20 secml-1, for simplicity presented as drops per minute, dpm). Patient groups were: A: Asymptomatic, A1: subgroup where the fellow symptomatic eye had a visible cause for watering, B: external visible cause for watering (ocular surface/lid/punctum), C: no externally visible cause, D: post op DCR, E: post syringing and probing, F: mixed/other. 444 tear ducts were examined. Mean flows (dpm) were: A1 (n = 19) 55; B (n = 183) 46; C (n = 142) 22: D (n = 38) 52. Excluding complete obstruction (n = 29), tear duct syringing only detected 48% of those with impaired manometric flow. Of those with a normal tear duct syringing, 53% had impaired manometric flow; 34% had a flow of 0 dpm. Differences in A1 versus C; B versus C and pre versus post dacryocystorhinostomy were all statistically significant (p < 0.05). The manometric system presented reliably measures lacrimal resistance and provides a substantial increase in sensitivity and specificity over conventional lacrimal syringing.