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Probiotics have been considered as preventive agents for the control of inflammatory bowel disease (IBD). In this study, we assessed the immunomodulatory effect of Bifidobacterium bifidum BGN4 on the control of IBD using the CD4(+) CD45RB(high) T cell transfer disease model. The mice were fed for 4 weeks with either a conventional diet containing only skim milk or a diet containing skim milk with 0.3% (w/w) BGN4. The BGN4-fed mice showed normal weight growth, fewer clinical symptoms such as thickened wall and inflammatory cell infiltration, and lower levels of CD4(+) T lymphocyte infiltration and inflammatory cytokine productions than the skim milk-fed mice with IBD in the large intestine. Suppression of these cytokine productions, particularly IFN-gamma and MCP-1, through BGN4 treatment was also observed in the in vitro co-culture between intestinal epithelial cells and T cells. These findings suggested that a BGN4 supplemented diet could be helpful for the control of aberrant immune responses in the intestinal tissue.

It is well established that intraepithelial T lymphocytes (IELs) are derived from conventional single-positive (SP) thymocytes, as well as unconventional double-negative (DN) thymocytes and CD103+CD8alphabeta recent thymic emigrants (RTEs). We show that IELs can be divided into two groups according to their dependency on sphingosine 1-phosphate (S1P) for trafficking into the intestines. CD4 or CD8alphabeta naive lymphocytes originating from SP thymocytes express high levels of type 1 S1P receptor (S1P(1)), and their preferential migration into the large intestine is regulated by S1P. In contrast, RTEs migrate exclusively into the small intestine, whereas DN thymic IEL precursors expressing either TCRalphabeta or TCRgammadelta migrate into both the small and large intestines. S1P does not play a role in the migration pathways of these unconventional thymic IEL precursors. Thus, down-regulation of S1P(1) expression or disruption of the S1P gradient halted conventional CD4 or CD8alphabeta IEL trafficking into the intestines, but did not affect the trafficking of unconventional thymic IEL precursors. These data are the first to demonstrate that a lipid-mediated system discriminates IELs originating from conventional and unconventional thymic precursors.

Background Ocular adnexal IgG4-related sclerosing disease (IgG4-SD) has been categorized as a novel disease entity. It is characterized by stromal sclerosis and an infiltration of mass-forming lymphoplasmic cells containing many IgG4-positive plasma cells. Although ocular adnexal tissue involvement has been increasingly reported, a focal nodular sub-brow mass is not typical in an IgG4-SD presentation. We report a rare case of probable ocular adnexal IgG4-SD that clinically mimicked eyelid pilomatrixoma. Case presentation A 42-year-old woman presented with a nodular mass in her left sub-brow area. The initial clinical impression of her lesion was eyelid pilomatrixoma. However, the final pathologic diagnosis was IgG4–SD, but extranodal marginal zone B-cell lymphoma could not be excluded. The patient underwent testing to determine tumor malignancy and systemic IgG4-SD involvement. Laboratory testing showed normal IgG and IgG4 serum levels and imaging revealed no remarkable findings. Oral prednisolone was administered and slowly tapered to manage the possible remnant lesion and to prevent disease recurrence. Two years after initiating therapy, there was no evidence of relapse. The patient is under close surveillance for signs of recurrence, systemic involvement, and potential malignant transformation. Conclusions We found an unusual case of probable ocular adnexal IgG4-SD, which presented as a unilateral restricted mass involving the sub-brow area. Although the mass was surgically removed, systemic steroid treatment and long-term surveillance were initiated due to the possibility of recurrence, the potential association with systemic disease, and the potential development of extranodal mucosa-associated lymphoid tissue (MALT) lymphoma.

Purpose To compare genome-wide DNA methylation profiles according to Chlamydophila psittaci (Cp) infection status and the response to doxycycline treatment in Korean patients with ocular adnexal extranodal marginal zone B-cell lymphoma (EMZL). Methods Twelve ocular adnexal EMZL cases were classified into two groups (six Cp-positive cases and six Cp-negative cases). Among the 12 cases, eight were treated with doxycycline as first-line therapy, and they were divided into two groups according to their response to the treatment (four doxy-responders and four doxy-nonresponders). The differences in the DNA methylation states of 27,578 methylation sites in 14,000 genes were evaluated using Illumina bead assay technology. We also validated the top-ranking differentially methylated genes (DMGs) with bisulfite direct sequencing or pyrosequencing. Results The Infinium methylation chip assay revealed 180 DMGs in the Cp-positive group (74 hypermethylated genes and 106 hypomethylated genes) compared to the Cp-negative group. Among the 180 DMGs, DUSP22, which had two significantly hypomethylated loci, was validated, and the correlation was significant for one CpG site (Spearman coefficient=0.6478, p=0.0262). Regarding the response to doxycycline treatment, a total of 778 DMGs were revealed (389 hypermethylated genes and 336 hypomethylated genes in the doxy-responder group). In a subsequent replication study for representative hypomethylated (IRAK1) and hypermethylated (CXCL6) genes, the correlation between the bead chip analysis and pyrosequencing was significant (Spearman coefficient=0.8961 and 0.7619, respectively, p

Purpose There is a paucity of data in the literature on the surgical management of lower eyelid marginal entropion. In this study, the authors report outcomes of a surgical technique of eyelid margin splitting and anterior lamellar reposition in patients with lower eyelid marginal entropion. Methods The medical records for 30 eyelids from 22 patients with lower eyelid marginal entropion who had undergone eyelid margin splitting and anterior lamellar repositioning at Seoul National University Hospital from January 2004 to December 2012 were retrospectively reviewed. Success was defined as the lack of any lash in contact with the globe, no need for a second procedure, the complete resolution of symptoms, and acceptable cosmesis at the final follow up. Results The mean follow-up duration was 16.7 months. The split eyelid margin exhibited good wound healing in each case, but trichiasis recurred postoperatively in 3 of 30 eyelids, even though the eyelid margin was ultimately well positioned. That is, the overall success rate was 90%. Of the 3 eyelids with recurrent trichiasis, 2 required additional electrolysis to remove irritated cilia, but the other did not require to be treated. In the latter case, the trichiasis observed was fine, focal, and unaccompanied by symptoms or corneal lesions. There was no significant complication such as secondary ectropion or eyelid retraction. Conclusions Eyelid margin splitting and anterior lamellar repositioning achieve success with a low rate of complications among patients with lower eyelid marginal entropion.

A 37-year-old woman presented with a 2-month history of fullness and ptosis of the left upper eyelid. Examination revealed a 6-cm x 2-cm mass in the left brow and upper eyelid, and a diffuse mass in the lower eyelid. Marked ptosis of the left upper eyelid and elevation of the left lower eyelid were noted. CT showed masses with a bone-like density in the left eyelid and periorbital soft tissue. A through history revealed that the patient had received calcium hydroxylapatite filler injection for nose augmentation 3 days prior to the development of the eyelid masses. The eyelid masses were excised and pathologically confirmed as calcium hydroxylapatite microspherules surrounded by collagen and histiocytes. Two months after surgery, the eyelid masses and ptosis of the left upper eyelid were completely resolved. To our knowledge, this is the first reported case of eyelid mass after injection of calcium hydroxylapatite facial filler for nose augmentation.

Aim To evaluate the surgical outcome after the insertion of smooth surface tunnel porous polyethylene orbital implants (Medpor SST) in children with retinoblastoma. Methods 44 consecutive children with retinoblastoma who underwent primary enucleation and Medpor SST implantation at Seoul National University Hospital from November 2004 to August 2009, with at least 24 months of follow-up were included. A retrospective review of cases was performed. Results Mean age at the time of surgery was 24.7 months (range 1–65 months). The diameter of the spherical implant was 20 mm in 36 patients (81.8%) and 18 mm in 8 patients (18.2%). During a mean follow-up period of 60.1 months (range 26–93 months), there were no cases of implant exposure, extrusion or infection. Transient conjunctival thinning developed in three patients, but all resolved with conservative treatment. Anophthalmic socket complications such as lower lid malposition (retraction or entropion) (n=10, 22.7%), blepharoptosis (n=8, 18.2%) and enophthalmos (n=2, 4.5%) developed, but most showed acceptable cosmesis. Conclusions Medpor SST is relatively safe, allowing for a mean follow-up of 5 years in terms of implant exposure, and may be a good choice of orbital implant for children with retinoblastoma.

Purpose To evaluate the clinical characteristics and treatment outcomes of patients with congenital membranous punctal obstruction. Methods The medical records of patients who underwent surgical treatment for congenital membranous punctal obstruction between 1999 and 2011 were retrospectively reviewed. The demographic data and clinical presentations of the patients were analyzed. Surgical intervention methods and treatment results were also evaluated. Results A total of 51 puncta in 31 eyes of 23 patients with congenital membranous punctal obstruction were included. Of the 31 eyes, 20 had both upper and lower punctal obstruction and 11 had single punctal obstruction. Up to 2008, 10 patients underwent simultaneous punctoplasty and silicone tube intubation; 7 were under 10 years of age and required general anesthesia for the procedure. All showed patent puncta without epiphora postoperatively. Of the 13 patients treated after 2008, 11 children underwent punctoplasty and lacrimal irrigation under general anesthesia. Nasolacrimal duct obstruction (NLDO) was found in 6 of the 11 patients, and combined silicone tube intubation was performed; 2 adults with both upper and lower punctal obstruction underwent only punctoplasty under local anesthesia. All puncta were opened sufficiently, but epiphora remained in 1 patient due to NLDO, requiring subsequent silicone intubation. Overall, NLDO was found in 7 of 13 patients. Conclusions Congenital membranous punctal obstruction can be successfully treated by punctoplasty alone. Because this is often associated with NLDO, the nasolacrimal system should also be thoroughly evaluated.

A 40-year-old woman presented with horizontal diplopia and right proptosis for 1 month. She had esotropia and right abducens nerve palsy. Pupils were small and equal and there was no relative afferent pupillary defect. A cutaneous capillary malformation, hypertrophy of bone and soft tissue, and varicose veins on lower extremities were consistent with the diagnostic triad of Klippel-Trenaunay syndrome (KTS) (figure 1).1 Orbital CT revealed multiple giant intracranial aneurysms (figure 2).

Objective To evaluate the incidence and associated factors of functional failure of anatomically patent external dacryocystorhinostomy (DCR). Design Prospective, nonrandomized case series. Participants Thirty-four consecutive patients who underwent external DCR at the oculoplasty clinic in the Department of Ophthalmology, Seoul National University Hospital between December 2008 and July 2009. Methods All patients underwent external DCR for primary acquired nasolacrimal duct obstruction. The silicone tubes were removed around 6 months after the DCRs, and patients were examined 1 month after tube removal. Anatomical patency was evaluated by a nasal endoscopic dye test and lacrimal syringing test. Tearing symptoms were evaluated using Munk’s scoring system. Patients were divided into 2 groups according to the presence of epiphora symptom; a functional success and functional failure group, and the incidence of functional failure were calculated. Potential associated factors were also compared between 2 groups. Results This study involved 50 eyes of 34 patients, and 8 eyes of 6 patients showed significant epiphora in spite of anatomical patency (16% functional failure). We compared associated factors between 42 functionally successful and 8 functionally failed DCRs. Among variables studied, demographic factors and intraoperative surgical findings were not statistically different between the 2 groups. With respect to intranasal endoscopic findings, the shape of the rhinostomy showed significant differences between the 2 groups (p = 0.03, Fisher’s exact test). In the functional failure group, there were no flat shape rhinostomies. Three showed alcove shape rhinostomies, and the other 5 showed cavern shape rhinostomies. Conclusions Among anatomically patent DCRs, the incidence of persistent epiphora was 16%. The shape of rhinostomy is a possible associated factor for functional failure after external DCR.

To investigate the physiological role of substance P innervation (SPI) in the nasal mucosa from inhaled irritant macromolecules, SPI in the rat nasal mucosa was selectively stimulated with capsaicin and the degree of horseradish peroxidase (HRP) permeability across the nasal epithelium was evaluated by enzyme immunoassay or by histochemical electron microscopy. The serum level of HRP was reduced significantly in capsaicin-administered rats compared to control rats along the time course with quantitative analysis of the enzyme immunoassay. Control rats showed heavy permeation of HRP across the epithelium, but capsaicin-administered rats showed weak permeation of HRP across the epithelium with histochemical electron microscopy. The epithelial lining and its tight junctions were left intact as judged by electron microscopy. In conclusion, selective stimulation of SPI of the rat nasal mucosa with capsaicin decreased the epithelial absorption permeability to HRP without compromising the epithelial integrity. These findings imply that the physiological role of capsaicin-sensitive SPI in the rat mucosa is to protect the airway from inhaled irritant macromolecules.

Epiblepharon is known to be bilateral and symmetric. However, asymmetric epiblepharon is frequently observed in patients with head tilt and, to date, this condition has not been investigated.To evaluate the clinical features of epiblepharon in patients with head tilt and to analyze the association between the direction of head tilt and epiblepharon asymmetry.The medical records of 1074 Korean children who received a diagnosis of epiblepharon between January 1, 2006, and October 31, 2013, at Seoul National University Bundang Hospital were retrospectively reviewed. The data collected included sex, age, extent and asymmetric presentation of epiblepharon, and direction and cause of head tilt.The presence of epiblepharon asymmetry in patients with head tilt as well as the association between the direction of head tilt and the side having more severe epiblepharon.Of 1074 patients (536 boys; mean [SD] age, 5.4 [2.6] years) with epiblepharon, 38 individuals (3.5%) showed head tilt. The causes of head tilt were superior oblique palsy (18 patients [47.4%]), congenital muscular torticollis (10 [26.3%]), dissociated vertical deviation (3 [7.9%]), and unknown (7 [18.4%]). Asymmetric epiblepharon was more common in children with vs without head tilt (34 [89.5%] vs 80 [7.7%]; P .001, Fisher exact test). Of the 34 patients with head tilt and asymmetric epiblepharon, the direction of head tilt was consistent with the side having more severe epiblepharon in 29 individuals (85.3%) and inconsistent in 5 individuals (14.7%) (P = .009).These data suggest that, in patients with epiblepharon and head tilt, epiblepharon is most often asymmetric and is severe in the head-tilted side. These results suggest that patients with asymmetric epiblepharon should be evaluated for head tilt.

Purpose: To investigate periorbital lipogranuloma cases that developed after autologous fat injection and to determine various treatment outcomes from these cases. Methods: This retrospective study involved 27 patients who presented with periocular mass (final diagnosis of lipogranuloma) and had history of facial autologous fat injection. The collected data included information on patient sex, age, clinical presentation, number and site of fat injections, interval between injections, duration from injection to symptom onset, fat harvesting site, use of cryopreservation, and treatment outcome. Results: The most common presenting symptom was palpable mass (92.6%), followed by blepharoptosis and eyelid edema. The mean time from injection to symptom onset was 13.6 ± 29.2 months (range, 2 to 153 months). Patients were managed by intralesional triamcinolone injection (six patients) and surgical excision (three patients); 18 patients were followed without treatment. Among the six patients who underwent intralesional triamcinolone injection, five showed complete resolution, and one showed partial resolution. Among the 18 patients who were followed without management, three showed spontaneous resolution over a 5-month follow-up period. Conclusions: Lipogranuloma can develop in the eyelid after autologous fat injection into the face. Both surgical excision and intralesional triamcinolone injection yield relatively good outcomes. Simple observation can be a good option because spontaneous resolution can occur in a subset of patients.

서울대학교병원 안과, 서울대학교 의과대학 안과학교실, 분당서울대학교병원 안과, 서울대학교병원운영 서울특별시보라매병원 안과 Department of Ophthalmology, Seoul National University Hospital, Seoul, Korea Department of Ophthalmology, Seoul National University College of Medicine, Seoul, Korea Department of Ophthalmology, Seoul National University Bundang Hospital, Seongnam, Korea Department of Ophthalmology, Seoul Metropolitan Government Seoul National University Boramae Medical Center, Seoul, Korea

The aim of this work is to investigate the prevalence and clinical characteristics of congenital lacrimal fistula in Down syndrome patients. The medical records of 198 Down syndrome patients who were referred to a tertiary ophthalmology clinic from 2000 to 2010 were retrospectively reviewed to identify patients with congenital lacrimal fistula. The demographic data, clinical features, clinical management, and clinical outcomes were recorded. The main outcome measures were the presence and laterality of fistula, accompanying adnexal and oculomotor abnormalities including congenital nasolacrimal duct obstruction (NLDO), the type of surgery performed, and surgical outcome. The prevalence of congenital lacrimal fistula in Down syndrome patients was calculated upon this data. Congenital lacrimal fistula was identified in 8/198 (4.04 %) patients, 4 (2.02 %) of whom presented with bilateral lacrimal fistula. All patients that had lacrimal fistula complained of tearing from their eyes. Congenital NLDO was observed in seven of eight patients with lacrimal fistula. Five patients underwent excision of the lacrimal fistula for the improvement of cosmesis, and three of these patients also underwent lacrimal silicone intubation for NLDO. Another patient received lacrimal silicone intubation for NLDO without excision of the lacrimal fistula. Excision of the lacrimal fistula was successful in all patients; however, tearing persisted after surgery in two patients with uncorrected NLDO. Congenital lacrimal fistula occurs more frequently in Down syndrome patients and therefore these patients should be thoroughly examined for this abnormality. Down syndrome patients with congenital lacrimal fistula should be also examined for NLDO, because this condition is frequently observed in these patients.

Purpose To investigate the prevalence and clinical features of acquired lower eyelid epiblepharon in Korean patients with thyroid-associated ophthalmopathy (TAO) and compare the pathogenic features of acquired and congenital epiblepharon. Design Retrospective, nonrandomized, comparative case series, cross-sectional study. Participants A total of 494 Korean patients with TAO and 845 Korean patients with congenital lower eyelid epiblepharon. Methods The medical records were reviewed, and the presence, location, and extent of epiblepharon were identified. Clinical features of TAO (lower eyelid retraction, exophthalmos, and elevation limitation) were compared between patients with TAO with and without epiblepharon. Acquired epiblepharon was classified into 3 types according to the location and extent. The prevalence of epiblepharon types was determined and evaluated for associations with TAO clinical features. Comparisons were made between the prevalence of epiblepharon types in acquired and congenital epiblepharon. Main Outcome Measures Prevalence, location, and extent of epiblepharon; association with clinical features of TAO; and difference from congenital epiblepharon. Results An acquired lower eyelid epiblepharon was found in 42 (8.5%) of 494 patients with TAO. The mean age of patients with TAO with epiblepharon (34.2±13.5 years) was significantly lower than that of patients with TAO without epiblepharon (46.5±14.1 years) ( P = 0.000). Lower eyelid retraction (0.78±1.11 mm) was more severe in patients with TAO with epiblepharon than in patients without epiblepharon (0.30±0.73 mm) ( P = 0.000). Lower eyelid retraction was more severe in diffuse-type acquired epiblepharon than in central-type epiblepharon ( P = 0.012). Elevation limitation was more severe in central-type acquired epiblepharon than in medial-type epiblepharon ( P = 0.001). The occurrence of central-type epiblepharon was higher in TAO-associated acquired epiblepharon (20 eyelids, 30.8%) than in congenital epiblepharon (27 eyelids, 1.9%, P = 0.000). Conclusions Acquired lower eyelid epiblepharon is one of the clinical features of patients with TAO. The association between lower eyelid retraction and acquired epiblepharon may lead to a better understanding of the cause of acquired epiblepharon in patients with TAO. Financial Disclosure(s) The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Purpose: To describe a series of patients with lower eyelid epiblepharon associated with lower eyelid retraction. Methods: We retrospectively reviewed the medical records of patients who underwent surgery for lower eyelid retraction, epiblepharon, or thyroid-associated ophthalmopathy (TAO) between October 1999 and March 2007. Patients with both lower eyelid retraction and epiblepharon on preoperative examination were included in this study. Results: Twenty-seven eyelids of 20 patients with both lower eyelid retraction and epiblepharon were enrolled. The underlying causes of lower eyelid retraction included congenital retraction (seven eyelids), congenital fibrosis of the extraocular muscles (CFEOM; seven eyelids), TAO (seven eyelids), post-operative cicatricial retraction (five eyelids), and facial nerve palsy (one eyelid). Eight of 27 eyelids were successfully corrected after the repair of retraction without the repair of epiblepharon, regardless of the cause of lower eyelid retraction. Another four eyelids with epiblepharon associated with TAO resolved after only orbital decompression. Cilia-everting sutures were additionally applied for epiblepharon in another 14 eyelids, 12 of which did not require the excision of a skin fold or the orbicularis muscles. Only one eyelid with mild retraction and epiblepharon underwent simple epiblepharon repair. Recurrence of retraction or epiblepharon developed in three eyelids during follow-up. Conclusions: In cases with both lower eyelid retraction and epiblepharon, the retraction should be repaired first, and then the epiblepharon can be corrected selectively according to the severity of the case.

Purpose To evaluate treatment results of canalicular curettage through the punctum after 1-snip punctoplasty for primary canaliculitis. Design Retrospective, interventional case series. Participants Thirty patients with primary canaliculitis who were treated at Seoul National University Hospital between February 2005 and January 2008. Methods All patients underwent 1-snip punctoplasty and canalicular curettage through the punctum, and granules or concretions were removed. After the procedure, oral and topical antibiotics were used for 3 weeks. Demographic data and clinical presentations of the patients were analyzed. The resolution of symptoms and inflammatory signs and complications were evaluated 3 weeks after the procedure. Main Outcome Measures Resolution rate of the canaliculitis and complications after the procedure. Results There was a female predominance in the study group (23:7). Common symptoms of canaliculitis included discharge, tearing, and pus or concretions from the punctum. During the procedure, concretions, granules, or discharges were drained in all patients. In 25 (83.3%) of 30 patients, the symptoms and signs of canaliculitis completely resolved 3 weeks after 1 treatment. Two patients (6.7%) required additional curettage. Symptomatic canalicular strictures developed in 2 patients. Conclusions Materials were removed easily from the canaliculus using a 1-snip punctoplasty and curettage through the punctum. This procedure, combined with systemic and topical antibiotic therapy, can be a highly effective treatment for primary canaliculitis. Financial Disclosure(s) The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Aims: The aim of the study was to evaluate the results of the frontalis sling operation using silicone rod for the correction of ptosis in chronic progressive external ophthalmoplegia patients. Methods: Chronic progressive external ophthalmoplegia patients who received the frontalis sling operation using silicone rods from 1999 to 2006 were included in this study. The medical records were retrospectively reviewed and the clinical characteristics and postoperative surgical results of these patients were analysed. This study was a retrospective, non-randomised, interventional case series and the main outcome measures were margin reflex distance, eyelid contour and corneal status. Results: Seven patients were recruited (one male and six female). The mean age at the time of operation was 29.6 (range 15-62) years. Two patients had unilateral ptosis and five patients had bilateral ptosis. The mean follow-up period was 22.7 (range 1-61) months. Satisfactory lid height was achieved in all patients. Although corneal erosions were detected in five patients 1 month after surgery, these findings eventually resolved in three patients 2 months later, after the use of artificial tear eyedrops and ointments. Conclusion: The frontalis sling operation using silicone rod can safely and effectively correct ptosis in chronic progressive external ophthalmoplegia patients without serious corneal complications.

Purpose: We aimed to evaluate the effectiveness of bicanalicular double silicone intubation in dacryocystorhinostomy (DCR) and canaliculoplasty for distal or common canalicular obstruction. Methods: We reviewed the medical records of patients with distal or common canalicular obstruction who had undergone bicanalicular double silicone intubation (insertion of two tubes into each canaliculus) during external DCR and canaliculoplasty. We reviewed the records of subjects who had undergone the same surgery with a bicanalicular single intubation as control material. The tubes were removed at around 6 months after surgery. Anatomical and functional success rates of patients who were followed up for > 2 months after the removal of tube(s) were evaluated. In addition, complications related to the silicone tube were evaluated. Results: Data for 60 eyes of 45 patients in the double-intubation group and 69 eyes of 65 patients in the single-intubation group were retrieved. The double-intubation group showed higher anatomical success rates (96.5%) than the single-intubation group (85.5%). Functional success was achieved by 53 (88.3%) of 60 eyes in the double-intubation group and 56 (81.2%) of 69 eyes in the single-intubation group. There was no significant difference in complication rates between the two groups. Conclusions: Bicanalicular double silicone intubation for DCR and canaliculoplasty may be an effective mode of treatment for patients with distal or common canalicular obstruction.

To evaluate the cytologic features of conjunctival epithelium in anophthalmic sockets with an ocular prosthesis, using an impression cytology technique and to determine the clinical factors associated with these changes.In a prospective case-controlled study, 40 consecutive unilateral anophthalmic patients who wore an ocular prosthesis were recruited. A questionnaire on the care of the prosthesis included total wearing period, frequency of cleaning, frequency of polishing, cleaning solution, and eye drop use. The degree of inflammation of the anophthalmic conjunctival socket was evaluated. Impression cytology specimens were taken from the upper tarsal conjunctiva, the bulbar conjunctiva, and the lower tarsal conjunctiva of each socket, and from the contralateral eye (to serve as an internal matched control). The goblet cell density and the nucleus-to-cytoplasm ratio of the epithelial cells were measured. The relevance of these conjunctival cytologic features to the various factors of prosthesis care or conjunctival inflammation was analyzed.In the anophthalmic sockets, the conjunctiva showed squamous metaplasia at all 3 areas sampled. The goblet cell density was significantly decreased and the nucleus-to-cytoplasm ratio of the epithelial cells was significantly increased compared with the control eyes (p0.05, on all 3 areas sampled, Wilcoxon signed rank test). These conjunctival cytologic changes were not significantly associated with total wearing time, frequency of polishing, cleaning solution, or eye drops use (p0.05, Spearman's correlation test).Squamous metaplasia with decreased goblet cell density and increased nucleus-to-cytoplasm ratio occurred in anophthalmic conjunctival sockets but was not associated with particular aspects of prosthesis care.

Purpose: To describe the technique of splitting the lid margin combined with the excision of redundant skin and muscle during the surgical correction of epiblepharon and to report its clinical outcome. Methods: A combined procedure that included splitting the lid margin to repair lower eyelid epiblepharon was performed on 31 eyes of 19 consecutive patients. Lid margin splitting was performed along the grey line on the medial third or half of the lower eyelid by making a 1 mm-deep incision. Having made a transverse subciliary skin incision and a dissection between the tarsus and the orbicularis oculi muscle, the subcutaneous tissue of the superior edge of the incision was secured to the tarsus with interrupted sutures to evert the cilia. An excision of the redundant skin and orbicularis tissue was made and the skin was closed. The patients were followed for direct inspection of the wound, the split lid margin, the direction of the lashes and the status of the cornea. Results: The mean postoperative follow-up period was 29.4 weeks. Symptoms disappeared in all patients. In 30 eyelids of 19 patients the cilia did not touch the cornea, even in the down-gaze. In one eyelid the cilium touched the medial conjunctiva, but not the cornea. The cosmetic outcome of the lower lid was satisfactory in all cases and the wounds of the split lid margin healed without scarring. To date, there have been no complications such as wound dehiscence, ectropion or eyelid retraction. Conclusions: The lamellar splitting of the lid margin is a beneficial addition to the repair of prominent lower lid epiblepharon, especially on the medial aspect of the eyelid. This simple technique ensures easier eversion of the cilia in epiblepharon repair, without disturbing the posterior lamella or causing unfavourable results.

External dacryocystorhinostomy (DCR) is an effective surgical treatment for nasolacrimal duct obstruction, and consistently yields a success rate greater than 90%.1,2 However, epiphora might remain in some patients even after anatomically successful DCR.3,4 Moreover, as far as we are aware, no effective means of treating these functional failures has been reported. Our interest in this topic arose when one of our patients underwent primary external DCR and silicone intubation, which successfully resolved epiphora postoperatively, but which recurred after the silicone tube was removed at 4 months postoperatively, as had been planned. The authors relocated the silicone tube in this patient, which again resolved the epiphora. This experience suggested that silicone tubes intubated into DCR sites might play a significant role in lacrimal drainage, even when a patent bypass route has been established by DCR. The authors therefore considered that silicone intubation might resolve epiphora in cases of anatomically patent but functionally failed DCR. Thus, we began to more confidently utilize this technique of silicone intubation into DCR sites to address persistent or recurrent epiphora after successful DCR. This study was conducted to evaluate the efficacy of this procedure for the correction of epiphora in patients that have received an anatomically patent but functionally failed DCR.

To evaluate the effect of mechanical trauma induced by cilia on the conjunctival epithelium in patients with epiblepharon or entropion and to evaluate changes in epithelium after surgical correction in patients with entropion.Case-control study.One hundred and seven eyes of 61 patients were enrolled in this study and were divided into three groups: the epiblepharon group (n = 59), the entropion group (n = 17), and the age-matched control group for the epiblepharon group (n = 31). Impression cytologic specimens were obtained from nasal and temporal bulbar conjunctiva of the epiblepharon and control groups immediately after the induction of general anesthesia. In the entropion group, these were obtained before and one month after surgical correction. Conjunctival changes were graded using the Tseng method and goblet cell densities were compared.Cytologic scores were significantly higher in the epiblepharon group than in the control group (P.001), and goblet cell densities of nasal and temporal conjunctiva were significantly lower in the epiblepharon group than in the control group (P = .044 and P = .018, respectively). In the entropion group, postoperative scores were significantly lower than preoperative scores in both conjunctival areas (P = .033 and P = .003, respectively). No statistically significant difference was found between nasal and temporal conjunctiva in the three groups.The persistent mechanical trauma by cilia in patients with epiblepharon or entropion can induce squamous metaplasia of the conjunctival epithelium. However, these conjunctival changes can be reversed by surgical correction.

Purpose To investigate the expression of cancer stem cell (CSC) marker proteins in eyelid sebaceous gland carcinoma and evaluate the clinical significance. Methods Archival tissue blocks from 50 cases of eyelid sebaceous gland carcinoma were tested via immunohistochemistry for 16 putative CSC markers. Levels of protein expression were analyzed alongside various clinicopathologic parameters such as metastasis-free survival time. Results Ten patients (20%) showed nodal or distant metastasis during the follow-up period (median, 35.2 months; range, 1-128 months) without any mortality in our series. Among the 16 markers, ALDH1, CD44, CD133, ABCG2, Sox4, Sox9, and slug were selected for candidates of CSC markers because they were frequently and predominantly found in the tumor cells compared with control tarsus cells, which showed negative or very low expression. Univariate analysis revealed that ALDH1, CD133, and ABCG2 were significantly associated with metastasis; patients with ALDH1- or CD133-positive tumors developed metastasis more frequently than patients with tumors that were negative for these markers (log-rank test, P = 0.014, P = 0.013, respectively), and diffuse expression of ABCG2 was associated with significantly shorter metastasis-free survival (log-rank test, P = 0.010). Multivariate Cox proportional hazard model revealed that ALDH1 (hazard ratio [HR] = 5.682, P = 0.038) was significantly associated with metastasis. Conclusions Development of metastasis in eyelid sebaceous gland carcinoma might be attributed to increased number of CSCs or acquisition of dedifferentiated phenotype. Our findings suggest that CSCs are involved in the disease progression of eyelid sebaceous gland carcinoma, and in particular, expression of ALDH1 is a predictor of a poor outcome.

3 , Seoul, Korea Purpose: Basal cell carcinoma is the most common malignant neoplasm of the skin. Periorbital basal cell carcinoma needs to be treated for cosmetic improvement and prevention of intraorbital invasion. Although surgical excision is a standard treatment, nonsurgical treatment using imiquimod cream, a topical immunomodulator, has been attempted. The authors report the first case of periorbital basal cell carcinoma successfully treated with imiquimod cream; to the best of our knowledge, similar cases have not been published to date in the Korean ophthalmology journals. Case summary: A 73-year-old female visited our hospital for treatment of a biopsy-proven basal cell carcinoma in the right eyebrow. A flat, elevated, 1 x 1 cm-sized mass having central indentation was observed in the centromedial area of the right eyebrow. She was treated with 5% imiquimod cream once daily at night, 5 days per week for 6 weeks. Eight weeks after the treat- ment with imiquimod cream was discontinued, the mass was flattened and the cilia began to grow on the mass site. Under the impression of a partial regression, an additional 4 weeks of application was recommended. The mass disappeared more and her eyebrow became more normal. At the final follow-up 31 months after treatment, her right eyebrow appeared normal. Conclusions: Imiquimod cream may be a useful medication which can be used for complex periorbital basal cell carcinomas that are difficult to reconstruct after resection or surgical candidates with poor general condition. J Korean Ophthalmol Soc 2015;56(11):1789-1793

Dear Editor, Posttraumatic cerebrospinal fluid (CSF) leakage is one of the well-documented sequelae of skull base fracture. Typically, it is presented as rhinorrhea, and less frequently, headache, otorrhea, dysgeusia, facial numbness, and discharge through orbit [1]. To the best of our knowledge, conjunctival prolapse due to CSF leakage has not been previously reported. Herein, we report a patient who experienced delayed severe conjunctival prolapse after skull fracture. A 3-year-old girl visited the emergency room for a fallingdown injury. Brain computed tomography (CT) showed multiple comminuted skull fractures, including both frontal bones and left orbital roof (Fig. 1a). Periorbital tissue swelling was severe, and no other abnormal finding was noted in the anterior segment and fundus. She was admitted for close observation. After a few days, her eyelid swelling became more severe, and left superior forniceal conjunctiva became prolapsed (Fig. 1b). Orbit CT showed brain parenchymal herniation through the left orbital roof, implying injury of dura mater. She underwent open reduction of orbital roof and frontal bone with repair of dura mater. Five days after the operation, conjunctival prolapse got worse and a yellow fluid level was seen in the prolapsed conjunctiva. Spontaneous regression couldn’t be expected, and we decided to perform reductive surgery. Under general anesthesia, prolapsed conjunctiva was incised, and fluid was drained, collected, and sent to lab for analysis. The shrunken tissues were securely anchored to the superior fornix by fullthickness eyelid sutures through silicone 240 retinal band (MIRA, USA) lying in the superior fornix (Fig. 1c). Laboratory analysis of the drained fluid revealed that red blood cell (RBC) density was 27,648/mm, and white blood cell (WBC) density was 6/mm. There were no cells other than RBC or WBC. Glucose level was 98 mg/dl. Reference ranges in CSF are 50–80 mg/dl of glucose, negative for RBCs, and 0–3 WBCs [2]. We could rule out the possibility of blood and conjunctival fluid, because their cell densities are 4–5 million/mm and about half a million/mm respectively. This fluid was diagnosed as CSF based on the cell density and glucose level [2–4]. Two months after the surgery, superior fornix was well-formed and maintained without recurrent conjunctival prolapse (Fig. 1d). Skull base fracture can cause CSF leaks and more than 60% of patients with CSF leaks present with CSF rhinorrhea [5]; however, orbital CSF accumulation can also develop if skull base fracture is accompanied with superior orbital wall J. Lee :N. Kim :H.-K. Choung : S. I. Khwarg Department of Ophthalmology, College of Medicine, Seoul National University, Seoul, Korea

서울대학교 의과대학 안과학교실, 분당서울대학교병원 안과, 서울대학교병원운영 서울특별시보라매병원 안과, 서울대학교병원 임상의학연구소 서울인공안구센터 Department of Ophthalmology, Seoul National University College of Medicine, Seoul, Korea Department of Ophthalmology, Seoul National University Bundang Hospital, Seongnam, Korea Department of Ophthalmology, Seoul Municipal Government–Seoul National University Boramae Medical Center, Seoul, Korea Seoul Artificial Eye Center, Seoul National University Hospital Clinical Research Institute, Seoul, Korea

PURPOSE We investigated the aberrant promoter methylation status of known or suspected tumor suppressor genes in ocular adnexal lymphoma (OAL) and the possible association with clinical characteristics and Chlamydophila psittaci infection. METHODS Thirty-five cases of ocular adnexal mucosa-associated lymphoid tissue (MALT) lymphoma cases were examined for the methylation status of nine genes using methylation-specific PCR and for the detection of C. psittaci DNA using PCR. The medical records were reviewed retrospectively. Patient demographics, clinical characteristics including the response of the lymphoma to the therapy, and C. psittaci infection status were evaluated for possible association with methylation frequencies. RESULTS CpG island methylation in nine genes was variously found as follows; DAPK (94.3%), ECAD (77.1%), MT1G (48.6%), THBS1 (37.1%), RAR-β (31.4%), p16 (20%), MGMT (5.7%), p14 (0%), and RASSF1A (0%). Methylation was not observed in any of 13 control cases. C. psittaci DNA was observed in 25 (75.8%) of 33 patients with available tumor tissues, and ECAD hypermethylation was significantly higher in C. psittaci-positive cases (P = 0.041). Promoter hypermethylation status was not correlated with clinical characteristics. CONCLUSIONS Aberrant CpG island methylation of tumor suppressor genes is a frequent event in ocular adnexal MALT lymphoma. In particular, high frequencies of DAPK and ECAD methylation may be strongly correlated with ocular adnexal MALT lymphomagenesis in South Korea. Furthermore, ECAD hypermethylation is closely associated with C. psittaci infection, which may shed light on the mechanisms of bacterium-induced oncogenesis.

Case summary: A 37-year-old woman presented with a mass around the punctum in the right lower eyelid margin, which had developed several years earlier and was increasing in size. The mass was a light pinkish nodule consisting of a few smooth-surface papillary lobules. The pathologic findings of the specimen obtained from a shaving biopsy showed a papil- lary projection which was covered with epithelium and communication with duct-like structures showing glandular config- uration in the deep portion of the lesion. EMA-positive cells were found at the invaginated epithelium, implying a glandular epithelial origin. GCDFP-15-positive cells were found in the deep portion of the lesion, implying apocrine differentiation. The findings were compatible with syringocystadenoma papilliferum. Conclusions: Syringocystadenoma papilliferum should be considered as a differential diagnosis of a solitary eyelid tumor. J Korean Ophthalmol Soc 2012;53(7):1023-1026

Purpose To demonstrate the use of the autogenous dermis-fat graft for the treatment of porous polyethylene implant exposure and volume augmentation in postenucleation retinoblastoma children. Design Retrospective, interventional case series. Methods Ten children who received a dermis-fat graft at Seoul National University Hospital between July 1, 2005 and January 31, 2010 were included in this study. The patients had undergone unilateral enucleation for retinoblastoma, and received a subconjunctival dermis-fat graft to repair implant exposure and simultaneously correct enophthalmos. The clinical characteristics of the patients, rate of graft survival, and complications were analyzed. The cosmetic outcome was assessed using a grading system. Results All patients had enucleation using porous polyethylene implant as a primary orbital implant. The average time to exposure was 89.1 ± 22.4 months and the median size of defect was 2 × 3 mm. With a mean follow-up of 28 months, 9 of 10 patients showed well-survived graft without re-exposure. One patient who experienced a graft failure managed with implant rotation and a scleral graft. Seven patients showed significant improvement of enophthalmos. Conclusions Implantation of an autogenous dermis-fat graft is a procedure that can be effectively used to treat porous polyethylene implant exposure and simultaneously correct enophthalmos. Use of this procedure can be particularly advantageous for pediatric postenucleation patients.

Purpose: To report a case of periorbital infantile capillary hemangioma treated with propranolol. Case summary: A 6-month-old girl visited our clinic for a mass on the right upper eyelid, which had been present since birth. A dome-shaped, red-purple colored mass occupying almost the entire right upper eyelid was observed, and overlying branch-shaped telangiectases were also detected. The visual axis of the right eye was partially obscured, due to the ptotic eyelid, and her ability to fix and follow was poor in the right eye. The patient was diagnosed with infantile capillary hemagioma. Because amblyopia in her right eye was suspected, treatment with propranolol was initiated. After 2.5 months of propranolol treatment, the hemangioma decreased in size and volume dramatically, changed from red-purple to light red color, and softened. The ptosis of the right upper eyelid improved, as well as the ability of the right eye to fix and follow. No significant adverse effects (e.g. hypotension, bradycardia, hypoglycemia, bronchospasm, or elevated liver enzymes) were observed, except transient QTc prolongation during cotreatment with ibuprofen, due to an upper respiratory infection. Conclusions: Propranolol should be considered as a treatment option for periorbital infantile capillary hemangioma. Further clinical studies are required to establish the optimal guidelines and long-term outcome.

Purpose: To report 3 cases of hyper-IgG4 syndrome involving ocular adnexa. Case summary: A 66-year-old woman presented with swelling of the bilateral upper eyelids with ocular pain that began 1 year before. Nodular episcleral injection of the left eye and other generalized symptoms, such as cough, decreased hearing ability, multiple nodular lesions of the bilateral lungs and right kidney, together suggested rheumatic disease. Orbital computed tomographic images revealed diffuse swelling of the bilateral lacrimal glands. After immunostaining a surgically-biopsied specimen from the lacrimal gland for IgG4 expression, 15% of infiltrated lymphoplasmacytic cells were IgG4-positive. Similar findings were shown with biopsied specimens from the lung and kidney; therefore, the patient was diagnosed with Hyper-IgG4 syndrome. A 49-year-old woman complained of a mass in the left upper eyelid that began 4 years earlier. Orbital computed tomographic images showed a 5-mm-sized mass in the left upper eyelid. Ocular adnexal Hyper-IgG4 syndrome was confirmed by the immunostained biopsy from the left upper eyelid, showing infiltration of IgG4-positive lymphoplasmacytic cells. A 51-year-old woman presented with swelling of the bilateral lacrimal glands. Enlargement of the bilateral lacrimal glands were apparent in orbital computed tomographic images. After anti-IgG4 antibody staining of a biopsied specimen from the right lacrimal gland, dense infiltration of IgG4-positive lymphoplasmacytic cells was observed. The patient was also diagnosed with Hyper-IgG4 syndrome.

Purpose: The authors report a case of orbital mucocele lined with two types of histological epithelial cells developed after repair of the orbital wall fractures. Case summary: A 53-year-old man presented with proptosis of the left eye for two years. The patient had a history of left orbital inferomedial wall fracture repair ten years earlier at a different hospital. Examination revealed 4 mm proptosis and superior globe displacement of the left eye. Restriction of left ocular movements on elevation, depression and adduction were observed. A computed tomography scan demonstrated a large, non-enhancing, cystic tumor in the left inferior orbit with the inferior and medial wall displaced toward the paranasal sinus. An orbital cystic tumor was excised with the removal of previously-inserted orbital implant via a transconjunctival and transcaruncular approach. The inferior, and medial orbital walls were reconstructed using a MEDPOR TITAN implant. The initial pathologic diagnosis was epidermal cyst. Histopathologic re-review revealed an orbital cyst lined with both stratified squamous epithelium and pseudostratified ciliated columnar epithelium, thus diagnosis was changed to orbital mucocele. Proptosis and restriction in ocular motility improved postoperatively. Conclusions: Mucocele formation should be considered in patients in whom a cyst developed after orbital fracture repair.

Purpose: To report a large-angle exotropia, limited adduction, epiblepharon, high myopia and no pupillary light reflex in a patient with hydranencephaly. Case summary: A ten-year-old girl with mental retardation presented with exotropia. The patient could fix only with the right eye and was unable to follow with either eye. The Krimsky test revealed 95 prism diopters of exotropia, and adduction was severely limited in both eyes. Pupillary light reflex was absent in both eyes. Cycloplegic refraction showed high myopia in both eyes. Slit lamp examination revealed lower lid epiblepharon and inferior corneal opacity in the right eye. No abnormal findings in the fundus examination were detected. A computed tomogram of the brain showed that the cerebral hemispheres were replaced by a cystic space filled with cerebrospinal fluid, compatible with hydranencephaly. Recession of the lateral rectus muscle and resection of the medial rectus muscle with epiblepharon repair of the lower lid were performed in both eyes. One week postoperatively, the epiblepharon was corrected, and the Krimsky test showed 16 prism diopters of left intermittent exotropia at near. Conclusions: When a combined manifestation of mental retardation, limited adduction, no pupillary light reflex and a large-angle exotropia is present, the possibility of a congenital developmental anomaly of the central nervous system including hydranencephaly should be suspected.

Purpose: To report the clinical features and treatment of canaliculitis associated with SmartPlug punctal plug insertion. Methods: Case selection criteria included patients with canaliculitis, who were managed at Seoul National University Hospital from January 2006 to October 2008, presenting with a history of punctal plug insertion. The operation reports were reviewed to identify patients in whom SmartPlug was discovered during the operation. Six patients (8 eyes) were identified, and a retrospective chart review was performed for all the patients. Results: The mean age of the patients was 34.38.6 years, and there were 1 men and 5 women. Common symptoms were mucous discharge (6 eyes) and conjunctival injection (2 eyes). The mean time from insertion of the plug to onset of symptoms was 27.027.0 months (range 4 to 77 months). All patients underwent surgical removal of the punctal plug by one-snip punctoplasty, canalicular retrograde compression using 2 cotton-tipped applications (2 eyes), or canalicular curettage (6 eyes). All patients had resolution of symptoms after the procedure. Conclusions: Canaliculitis should be considered when there is conjunctival discharge or injection in patients with SmartPlug. One-snip punctoplasty and retrograde compression of canaliculus can be attempted preferentially as a minimally-invasive treatment option.