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Biopsies and ANCA testing for limited forms of granulomatosis with polyangiitis (GPA) are frequently non-diagnostic. We characterized gene expression in GPA and other causes of orbital inflammation. We tested the hypothesis that a sub-set of patients with non-specific orbital inflammation (NSOI, also known as pseudotumor) mimics a limited form of GPA. Formalin-fixed, paraffin-embedded orbital biopsies were obtained from controls (n=20) and patients with GPA (n=6), NSOI (n=25), sarcoidosis (n=7), or thyroid eye disease (TED) (n=20) and were divided into discovery and validation sets. Transcripts in the tissues were quantified using Affymetrix U133 Plus 2.0 microarrays. Distinct gene expression profiles for controls and subjects with GPA, TED, or sarcoidosis were evident by principal coordinate analyses. Compared with healthy controls, 285 probe sets had elevated signals in subjects with GPA and 1472 were decreased (>1.5-fold difference, false discovery rate adjusted p<0.05). The immunoglobulin family of genes had the most dramatic increase in expression. Although gene expression in GPA could be readily distinguished from gene expression in TED, sarcoidosis, or controls, a comparison of gene expression in GPA versus NSOI found no statistically significant differences. Thus, forms of orbital inflammation can be distinguished based on gene expression. NSOI/pseudotumor is heterogeneous but often may be an unrecognized, localized form of GPA.

Uri Soiberman,1 Hirohiko Kakizaki,2 Dinesh Selva,3 Igal Leibovitch11Division of Oculoplastic and Orbital Surgery, Department of Ophthalmology, Tel-Aviv Medical Center, Tel-Aviv University, Tel-Aviv, Israel; 2Department of Ophthalmology, Aichi Medical University, Nagakute, Japan; 3South Australian Institute of Ophthalmology and Discipline of Ophthalmology and Visual Sciences, University of Adelaide, South Australia, AustraliaAbstract: Acquired punctal stenosis is a condition in which the external opening of the lacrimal canaliculus is narrowed or occluded. This condition is a rare cause of symptomatic epiphora, but its incidence may be higher in patients with chronic blepharitis, in those treated with various topical medications, including antihypertensive agents, and especially in patients treated with taxanes for cancer. The purpose of this review is to cover the medical literature, focusing in particular on definition, incidence, risk factors, etiology and treatment options.Keywords: acquired punctal stenosis, definition, epiphora, etiology, treatment

Hirohiko Kakizaki1, Yasuhiro Takahashi1, Akihiro Ichinose2, Masayoshi Iwaki1, Dinesh Selva3, Igal Leibovitch41Department of Ophthalmology, Aichi Medical University, Nagakute, Aichi, Japan; 2Department of Plastic Surgery, Kobe University, Kobe, Hyogo, Japan; 3South Australian Institute of Ophthalmology and Discipline of Ophthalmology and Visual Sciences, University of Adelaide, South Australia, Australia; 4Division of Oculoplastic and Orbital Surgery, Department of Ophthalmology, Tel Aviv Medical Center, Tel Aviv University, Tel Aviv, IsraelPurpose: To evaluate the surgical outcome of deep lateral orbital decompression with or without rim removal.Design: Retrospective case series.Methods: Thirty-two patients (47 orbits) with Graves’ orbitopathy who underwent simple deep lateral decompression or balanced lateral plus medial decompression. Of the 14 patients (24 orbits) who underwent simple deep lateral decompression, 8 (13 orbits) had temporary rim removal and in 6 (11 orbits) the rim was left intact. Of the 18 patients (23 orbits) who underwent a balanced decompression, 7 (9 orbits) had temporary rim removal and in 11 (14 orbits) the rim was left intact. The amount of postoperative reduction in proptosis was compared among these four groups.Results: The average reduction in proptosis in the simple deep lateral decompression group was 5.73 mm (range: 4.0–8.0 mm) in the rim removal group and 4.09 mm (range: 2.5–6.0 mm) in the intact rim group (P = 0.005). The average reduction in proptosis in the balanced decompression group was 6.39 mm (range: 5.0–8.5 mm) in the rim removal group and 5.07 mm (range: 3.0–8.0 mm) in the intact rim group (P = 0.039). There was no statistically significant difference in proptosis reduction between the simple deep lateral decompression with rim removal group and the balanced decompression with an intact rim group (P = 0.220).Conclusion: The rim removal approach allows a more effective decom

Hirohiko Kakizaki1, Yasuhiro Takahashi1, Masayoshi Iwaki1, Akihiro Ichinose2, Dinesh Selva3, Igal Leibovitch41Department of Ophthalmology, Aichi Medical University, Nagakute, Aichi, Japan; 2Department of Plastic Surgery, Kobe University, Chuo, Kobe, Hyogo, Japan; 3South Australian Institute of Ophthalmology and Discipline of Ophthalmology and Visual Sciences, University of Adelaide, South Australia, Australia; 4Division of Oculoplastic and Orbital Surgery, Department of Ophthalmology, Tel-Aviv Medical Center, Tel-Aviv University, Tel-Aviv, IsraelPurpose: To assess the effectiveness of the upper eyelid double motion sign in Graves’ orbitopathy (GO) in detecting thyroid dysfunction or a positive level of thyroid-related autoantibodies.Methods: GO was defined when more than two GO-related eyelid symptoms, including the double motion sign, existed with at least one positive thyroid-related blood test. Blood tests were performed in patients with more than two GO-related eyelid symptoms. The double motion was defined when the upper eyelid stopped at least once during downward eye movement. Fifty patients without GO or other eyelid diseases were used as controls.Results: There were 353 patients who showed more than two GO-related eyelid symptoms including the upper eyelid double motion sign. Of these, 300 patients were diagnosed with GO (300/353, 85.0%). The double motion sign was demonstrated in 267 patients (75.6%). A pause in double motion was typically seen around the anterosuperior direction of gaze. Double motion was not seen in any of the control eyelids. Although only 7.0% were hyperthyroid and 8.6% were hypothyroid, thyroid related autoantibodies were shown in 73.9% of patients. When the double motion sign was removed from the diagnostic criteria of GO, 263 patients had more than two thyroid-related eyelid symptoms, including 223 patients diagnosed as GO (25.7% reduction), although the rate of a correct diagnosis was almost the same (84.8%).Conclusions

Lucy Goold1, Hirohiko Kakizaki1,2,3, Raman Malhotra3, Dinesh Selva11South Australian Institute of Ophthalmology and Discipline of Ophthalmology and Visual Sciences, University of Adelaide, Australia; 2Department of Ophthalmology, Aichi Medical University, Nagakute, Aichi, Japan; 3Corneoplastic Unit and Eye Bank, Queen Victoria Hospital NHS Trust, East Grinstead, West Sussex, United KingdomAbstract: Classical anatomical teaching reports the presence of the lateral palpebral raphe formed at the union in the preseptal and orbital parts of the orbicularis oculi muscle, or by the tendon adhering these to the underlying zygomatic bone. The lateral palpebral raphe has been shown to be absent in Asian cadavers. The current study uses both evidence from the anatomical dissection of five eyelids from three Caucasian cadavers, and histological assessment of the lateral canthus of 13 eyelids from seven Caucasian cadavers to illustrate the absence of the lateral palpebral raphe in Caucasian population.Keywords: lateral palpebral raphe, orbicularis oculi muscle, Caucasian, cadavers

Hirohiko Kakizaki1, Igal Leibovitch2, Yasuhiro Takahashi3, Dinesh Selva41Department of Ophthalmology, Aichi Medical University, Nagakute, Aichi 480-1195, Japan; 2Division of Oculoplastic and Orbital Surgery, Department of Ophthalmology, Tel-Aviv Medical Center, Tel-Aviv University, Tel-Aviv, Israel; 3Department of Ophthalmology and Visual Sciences, Osaka City University Graduate School of Medicine, Osaka 545-8585, Japan; 4South Australian Institute of Ophthalmology and Discipline of Ophthalmology and Visual Sciences, University of Adelaide, AustraliaPurpose: To evaluate the effect of redundant lower eyelid skin on the eyelash direction in epiblepharon.Materials and methods: Asian patients with epiblepharon participated in this study. The lower eyelid skin was pulled downward in the upright position with the extent just to detach from eyelash roots, and the direction of the eyelashes was examined. These evaluations were repeated before surgery while the patients were lying supine under general anesthesia. Results: The study included 41 lower eyelids of 25 patients (17 females, 8 males, average age; 5.6 years, 16 cases bilateral, 9 unilateral). In the upright position, without downward traction of the skin, the eyelashes were vertically positioned and touching the cornea. The redundant skin touched only the eyelash roots and had minimal contribution to eyelash inversion. With downward skin traction, there was no significant change in the eyelash direction. In the supine position, the eyelashes were touching the cornea, and there was marked redundant skin that was pushing the eyelashes inward. With downward skin traction, there was no significant change.Conclusions: The direction of lower eyelashes in patients with epiblepharon was less influenced by lower eyelid skin redundancy than previously considered. The redundant skin is only a possible aggravating factor to epiblepharon.Keywords: epiblepharon, skin redundancy, upright, eyelash

Hirohiko Kakizaki1,2, Weng Onn Chan2, Yasuhiro Takahashi3, Dinesh Selva21Department of Ophthalmology, Aichi Medical University, Nagakute, Aichi, Japan; 2South Australian Institute of Ophthalmology and Discipline of Ophthalmology and Visual Sciences, University of Adelaide, Australia; 3Department of Ophthalmology and Visual Sciences, Osaka City University Graduate School of Medicine, Osaka, Japan Abstract: We aimed to microscopically examine whether Caucasian eyelids demonstrate overriding of preseptal orbicularis oculi muscle (OOM) over the pretarsal OOM in both lower and upper eyelids. Full thickness sections of 13 lower eyelids and 11 upper eyelids from seven Caucasian cadavers were examined. In the lower eyelids, all 13 specimens demonstrated clear overriding of preseptal OOM over the pretarsal OOM. The overriding part extended almost to the level of lower eyelid margin. However, in the upper eyelids, only one of the 11 eyelids demonstrated overriding, and the overriding part only extended to the level of mid-tarsal plate. Our result strongly supports the hypothesis of overriding of the preseptal OOM over the pretarsal OOM as an etiology of involutional lower eyelid entropion. The relatively low frequency of upper eyelid overriding preseptal OOM in our study reflects and may explain the rare occurrence of involutional upper eyelid entropion.Keywords: Caucasian, overriding, orbicularis oculi muscle, preseptal, pretarsal