Mohs Surgery for Periocular Basal Cell Carcinoma Without a Mohs Surgeon: The First Series in Hong Kong.

Purpose To report the first series of Mohs micrographic surgery (MMS) in Hong Kong, where the roles of a Mohs surgeon were shared and coordinated by a "mobile" surgeon. METHODS DESIGN: Prospective non-comparative interventional case series. SUBJECTS: 20 consecutive Chinese patients (10 male, age 78.5+10.4 years, range 55-91 years) with primary periocular basal cell carcinoma (pBCC) referred to the university oculoplastic unit between October 2007 and August 2013. INTERVENTION: MMS were conducted according to a streamlined standard operating procedure emphasizing surgeon-driven mapping, specimen orientation, and on-site clinico-histological correlation with the dermatopathologist at the frozen-section laboratory. MAIN OUTCOME MEASURES: Clinical and histological characteristics of tumors, layers of Mohs procedures, complications, and biopsy-confirmed recurrence at the same location. Results All 20 patients received MMS as planned. Sixteen pBCCs (80%) were diffusely pigmented, and three (15%) were focally pigmented. Sixteen were also nodular. The average tumor diameter was 7+3 (3-15) mm. Seven (35%) were within 2 mm of the punctum. Histologically, 11 (55%) were nodules, and four (20%) were superficial. An average of 1.8+0.8 Mohs levels were performed. Apart from the initial two patients, who required four and three levels, respectively, seven (35%) patients were cleared after the first level of MMS using a 1mm clinical margin. The remaining 11 patients required two levels with an additional 1-2mm margin, but only focally as guided histologically. Defects in 16 patients (80%) were reconstructed by local flaps, two by direct closure, and two with pentagon closure. Among the seven patients with pericanalicular BCC, three patients had their remaining canaliculi successfully intubated, while two developed stenotic upper and two lower punctae postoperatively. One patient had prolonged wound healing. Three patients had lid margin notching, two had medial ectropion, one had medial canthal rounding, and two had lateral canthal dystopia. No recurrence was detected at a mean follow-up of 80+23 months (43 to 113 months) in all patients. Conclusions MMS was successfully introduced in Hong Kong without a Mohs surgeon. Providing complete microscopic margin control and preserving tissues, it was proven to be a valuable treatment option for pBCC. Our multidisciplinary protocol demonstrated that these merits are possible and warrant validation in other resource-limited healthcare settings.

Malignancies in Immunoglobulin G4-related ophthalmic disease.

PURPOSE: Clinical phenotypes in Immunoglobulin G4-related disease (IgG4-RD) according to the patterns of affecting organs have different risks of malignancies. We attempt to determine the association of malignancies with IgG4-related ophthalmic disease (IgG4-ROD). DESIGN: Retrospective cohort study. METHODS: Review of medical records, orbital images and histopathology reports in a territory-wide cohort of biopsy proven IgG4-ROD patients from 2005-2019. FINDINGS: Among 122 patients who had biopsies taken from adnexal lesions including lacrimal glands (n = 108), orbital mass (n = 30), infiltrated orbital fat (n = 10), conjunctiva (n = 2) or extraocular muscles (n = 3), 13% (16/122) developed malignancies over 73 ± 48months' follow-up. There were 9 cases of ocular adnexal lymphoma (OAL) and 7 extra-orbital malignancies. Compared with the general population, the incidence of OAL was significantly higher (standardized incidence ratios, SIRs = 10.0, 95%CI = 4.5-17.6) while that of extra-orbital malignancies was similar. The SIRs was highest within the first year (SIR = 46.7, 95%CI = 18.5-87.6) when 7 OAL were concomitantly diagnosed. Patients who developed OAL or extra-orbital malignancies were older than other patients at IgG4-ROD diagnosis (64.9 ± 7.1, 68.3 ± 8.5 versus 55.2 ± 15.0 years, P < 0.05). Asymmetric lacrimal gland enlargement (78% versus 13%), lack of frontal (0% versus 12%) or infraorbital nerve enlargement (0% versus 36%) were associated with OAL (all P < 0.05). Pre-treatment serum IgG4 level or extra-orbital IgG4-RD involvement was similar among patients with or without malignancies. CONCLUSION: In this biopsy-proven IgG4-ROD cohort, 7% developed OAL which was 10 times higher than the general population. Patients with asymmetric lacrimal gland enlargement or without trigeminal nerves involvement radiologically were associated with OAL.

Treatment outcomes and their determinants of IgG4-related ophthalmic disease: a territory-wide cohort study.

BACKGROUND: Oral corticosteroid remains the first-line treatment of IgG4-related ophthalmic disease, but steroid-dependence is common and serious. Factors associated with steroid dependence and relapse have to be further explored. STUDY POPULATION: A city-wide, biopsy-proven, Chinese cohort. METHODS: Retrospective, masked review of medical records, orbital images and histopathology reports. RESULTS: There were 101 patients with at least 24-month follow-up. Up to 82% (82/101) received oral corticosteroid as first-line treatments, and 7 of them received also concomitant steroid-sparing agents (SSA)/biological agents as primary treatment. There was 61% (50/82) of patients required long-term corticosteroid (alone=23, with SSA=27) after 1.9±0.7 (range 1-5) relapses. When compared with the 21% (17/82) of patients who tapered corticosteroid successfully for 24 months, steroid dependence was associated with elevated baseline serum IgG4 level (94% vs 65%, p<0.01) and Mikulicz syndrome (46% vs 18%, p<0.05). Up to 13% (11/82) of patients tolerated residual disease after tapering off corticosteroid. There was 17% (17/101) of patients did not require any medications after biopsies. They were more likely to have debulking surgeries (71% vs 40%, p<0.05), discrete orbital lesions (65% vs 26%, p<0.05), normal baseline serum IgG4 level (24% vs 6%, p<0.05) and no Mikulicz syndrome (94% vs 61%, p<0.05). CONCLUSION: In this cohort, 60% of patients required long-term maintenance oral corticosteroid. Elevated pretreatment serum IgG4 level and Mikulicz syndrome were associated with steroid dependence. Debulking surgery is an alternative for a subgroup of patients with discrete orbital lesions, normal baseline IgG4 level and no Mikulicz syndrome.

Radiological Determinants of Complicated Immunoglobulin G4-Related Ophthalmic Disease: A Territory-Wide Cohort Study.

PURPOSE: To evaluate the presenting radiological features of immunoglobulin G4-related ophthalmic disease (IgG4-ROD) and their associations with IgG4-related optic neuropathy (IgG4-RON), and IgG4-related ocular adnexal lymphoma (IgG4-ROL). METHODS: A territory-wide, biopsy-proven, Chinese cohort. Masked review of orbital images, medical records, and histopathology reports. RESULTS: A total of 115 (94%) of the 122 patients in our cohort had preoperative orbital images (computed tomography=105, magnetic resonance imaging=40). Among them, 103/115 (90%) showed enlarged lacrimal glands, and 91 (88%) were bilateral. Nerve enlargement was observed: infraorbital in 31/115 (27%) patients and frontal in 17/115 (15%), 10 and 9 being bilateral, respectively. At least 1 or more extraocular muscle (EOM) enlargement was found in 41/115 (37%) patients, bilaterally in 20. Lateral rectus occurred in 30 (73%) of these 41 EOM patients and inferior rectus in 28 (68%). Two adjacent EOMs (inferior and lateral recti in 11 patients, inferior and medial recti in 7 patients) or multiple EOMs (at least 3) were enlarged in 23/41 (56%) and 13/41 (32%) of the patients, respectively. Intraconal lesions (67% vs 11%, P<0.05), infraorbital (83% vs 23%, P<0.005), or frontal (50% vs 15%, P<0.05) nerve enlargement was significantly associated with IgG4-RON (6 patients) by univariate analyses. Asymmetric lacrimal gland enlargement and discrete orbital mass (both P<0.05) were associated with IgG4-ROL (9 patients) by multivariate analyses. CONCLUSIONS: In this IgG4-ROD cohort, most patients had bilateral enlarged lacrimal glands, and the lateral rectus is the most frequently involved EOM. For the first time, unique radiological patterns associated with the development of IgG4-RON and IgG4-ROL are found.

Comparative study of stenting and ostium packing in Endoscopic Dacryocystorhinostomy for Primary Acquired Nasolacrimal Duct Obstruction.

In this retrospective study, we compared the efficacy and safety of mechanical adjuvants in mucosal-sparing, mechanical endoscopic dacryocystorhinostomy (MMED) for primary acquired nasolacrimal duct obstruction (PANDO). 116 adult patients (90 female) aged 61 ± 11 received one of the following after MMED without topical mitomycin: no stenting or packing (group 1, n = 25), 1-week ostium packing by ribbon gauze (group 2, n = 29) or non-medicated absorbable gelatin sponge (group 3, n = 25), 8-week bicanalicular stenting (group 4, n = 28). 104 patients(92%) provided 12-month outcomes. Number of patients, age, gender, surgeon, and osteotomy size were comparable among groups (p = 0.4-0.9). Marginal significance was found in anatomical (group 1:80%, group 2:96.6%, group 3:96%, group 4:96.4%, p = 0.05) but not functional success (group 1:85%, group 2:85.7%, group 3:83.3%, group 4:88.9%, p = 0.75) at postoperative 12-month. Patients receiving any packing or stenting achieved better anatomical (96% versus 80%, p = 0.015) but not functional success (85% versus 86%, p = 0.90) compared to those receiving none. More patients receiving stenting developed postoperative granuloma than those who did not (87% versus 63%, p = 0.04). 1-week ostium packing was found to be as effective as 8-week bicanalicular intubation in improving anatomical outcome after MMED for PANDO. Functional outcome, however, did not differ among patients receiving mechanical adjuvant or not.