Microphthalmia with a Large Orbital Cyst: A Case Report

Purpose@#We present the case of a 4-month-old boy with a large orbital cyst that caused lateral displacement of the optic nerve resulting in progressive exophthalmos of the left eye since birth.Case summary: A 4-month-old boy presented with progressive exophthalmos of the left eye since birth. Physical examination revealed marked left eye proptosis. Magnetic resonance imaging revealed microphthalmia and a 2.2 × 1.9 cm orbital cyst in the retrobulbar region. Subsequently, an anterior orbitotomy of the left eye was performed to dissect and excise the cyst. Histopathological examination revealed an orbital cyst containing neuroectodermal tissue. No complications or recurrences were observed during the 3-month post-surgery period. @*Conclusions@#Congenital microphthalmia with orbital cysts is a rare condition that does not always require surgical intervention. However, timely surgical intervention in cases of severe exophthalmos can result in favorable functional and cosmetic outcomes. We present a case that exhibited significant improvement following surgical removal of the orbital cyst.

Clinical Results of Prosthetic Eye Wear in Patients with Congenital Microphthalmos

Purpose@#To report the long-term clinical outcomes of non-surgical treatment involving prosthetic eye wear in patients diagnosed with congenital microphthalmos. @*Methods@#A retrospective review of the medical records of 20 patients was conducted. In total, 21 eyes were diagnosed with congenital microphthalmos between May 2008 and December 2022 at Seoul St. Mary’s Hospital in Korea. @*Results@#This study included 20 patients (12 males and 8 females) with an average age of 4 months at diagnosis. The observed ophthalmic anomalies included two cases of congenital cataract, one of posterior embryotoxon, one of corneo-iris strand, four of iris coloboma, five of central corneal opacity, one of Peter's anomaly, and one of retrobulbar cyst. Accompanying systemic abnormalities were noted, such as lateral ventricle atrophy, corpus callosum atrophy, patent ductus arteriosus, atrial septal defect, and developmental language disorder. Genetic anomalies included anti SS-A/Ro antibody positivity, a 1:100 titer of anti-nuclear antibody, and a PAX6 mutation identified through next-generation sequencing. No specific family histories or pregnancy-related factors were noted. The average follow-up duration was 5.94 years (range: 1 month to 18 years), the average corneal diameter was 4.6 mm, the average axial length was 17.44 mm, the average age for first artificial eye trial was 5.96 years (range: 7 months to 19 years), and the average interval for artificial eye replacement was 22 months (range: 4 months to 5 years and 8 months). @*Conclusions@#Twenty patients with congenital microphthalmos underwent gradual expansion of their prosthetic eyes by regular replacement and size increase without severe complications. This approach led to aesthetically and emotionally positive outcomes for the patients.

A Case of Endocrine Mucin-Producing Sweat Gland Carcinoma of the Eyelid

Purpose@#We report an endocrine mucin-producing sweat gland carcinoma diagnosed by biopsy in a patient who presented with an eyelid mass.Case summary: A 64-year-old male presented with a 3 × 3 mm solitary, painless pinkish mass on the right lower eyelid that had developed over the past year. The mass was excised and a biopsy was performed. The pathological findings included basaloid nodules composed of cells with eosinophilic cytoplasm, focal mucin production, and occasional glandular structures. Immunohistochemical examination was positive for cytokeratin 7 (CK-7), tumor protein 63 (P63), and the androgen receptor (AR). The patient was thus diagnosed with an endocrine mucin-producing sweat gland carcinoma. The lesion healed, and there has been no sign of recurrence over 6 months of follow-up. @*Conclusions@#An endocrine mucin-producing sweat gland carcinoma is a very rare low-grade glandular malignancy that has not been reported in Korea previously. The prognosis is good (i.e., there is no recurrence) when the lesion is completely surgically excised. We thought it would be useful to report this very rare case.

Long-term Efficacy of Dacryoendoscopy-guided Recanalization and Silicone Tube Intubation

Purpose@#To investigate the long-term efficacy of dacryoendoscopy-guided recanalization and silicone tube intubation in patients with obstruction in the lacrimal drainage system and to identify factors related to surgical outcome. @*Methods@#We retrospectively reviewed the medical records of patients with primary nasolacrimal duct obstruction and canalicular obstruction who underwent dacryoendoscopy-guided recanalization and silicone tube intubation between August 2014 and March 2016. Factors related to surgical outcome were examined and compared between the success group (eyes with complete response and partial response) and the failure group. Kaplan-Meier survival analysis and multivariable logistic regression analysis were used to analyze the success rate according to the factors found to have statistical significance. @*Results@#The study included 74 eyes of 51 patients. The mean age of the patients was 60.3 ± 10.0 years (range, 34–80 years). The success group consisted of 66 eyes (89.2%) (complete response, 56 eyes, 75.7%; partial response, 10 eyes, 13.5%) and the failure group consisted of eight eyes (10.8%). The median follow-up period was 58 months (range, 6.5–72 months), and the overall success rate was 89.2%. Compared to the eyes with preoperative lacrimal irrigation test of partial passage, the eyes with no passage were associated with a lower success rate (95.9% vs. 76.0%, p = 0.01). Postoperative inflammation was also associated with a lower success rate (96.6% vs. 60.0%, p < 0.001). @*Conclusions@#Dacryoendoscopy-guided recanalization and silicone tube intubation is effective and can be considered a first choice of treatment for eyes which show partial passage in the lacrimal irrigation test. The management of postoperative inflammation is essential to ensure surgical success.

Comparing Changes in Corneal Astigmatism Using Scheimpflug Camera after Epiblepharon Correction Surgery

Purpose@#To evaluate the changes in corneal astigmatism before and after epiblepharon correction surgery with a Scheimpflug camera. @*Methods@#From April, 2019, to June, 2020, 48 eyes of 24 patients underwent epiblepharon correction surgery. The patients were divided into two subgroups by age and their best corrected visual acuity (BCVA) and corneal anterior and posterior astigmatism were compared before and after correction surgery. @*Results@#There was no difference in BCVA after the correction surgery. To assess the changes in the keratometric value and astigmatism after epiblepharon surgery, a Scheimpflug camera was used. The anterior flat and mean keratometry changed from 42.10 ± 1.46 to 42.43 ± 1.10 D (p = 0.035) and from 42.97 ± 1.26 to 43.21 ± 1.15 D (p = 0.012), respectively, while the posterior corneal astigmatism changed from 0.44 ± 0.31 to 0.35 ± 0.17 D (p = 0.047). There were no significant changes in steep keratometry (p = 0.191) or anterior corneal astigmatism (p = 0.126). @*Conclusions@#There were significant changes after epiblepharon correction surgery in anterior keratometry reading and posterior corneal astigmatism.

Clinical Outcomes of Orbital Exenteration in Korean Patients with Orbital Malignancies

Purpose@#We report the clinical outcomes of Korean patients who were diagnosed with orbital malignancies and underwent orbital exenteration. @*Methods@#We retrospectively reviewed the tumor origins, histopathological diagnoses, local/regional recurrences, distant metastases, surgical margin clearances, overall and event-free survivals, and adjuvant chemotherapy or radiation therapy statuses of 14 patients who underwent orbital exenteration in our center from February 2009 to March 2020. @*Results@#We enrolled seven men and seven women of mean age at the time of exenteration of 68 years (range, 37 to 80 years). The mean follow-up period was 44.6 months (range, 10 to 133 months). Most tumors had arisen in the eyelid (seven cases, 50.0%). The most common pathological diagnosis was malignant melanoma (five cases, 35.7%). We observed no local or regional recurrence after exenteration, but distant metastases developed in seven cases, of which four were malignant melanomas (80% of all melanomas). Positive surgical margins were observed in six cases (42.9%). The distant metastasis rate was 42.9%; the overall survival rate was 60%. The 1-year overall survival rate was 100%, the 2-year survival rate was 81.8%, and the 5-year survival rate was 56.1%. The 1-year event-free survival (EFS) rate was 100%, the 2-year EFS rate was 72.7%, and the 5-year EFS rate was 49.9%. Nine patients received adjuvant radiation or chemotherapy and six patients received combined chemoradiation. @*Conclusions@#Patients underwent orbital exenteration to treat orbital malignancies and received postoperative chemotherapy and/or radiation exhibited differences in clinical outcomes and survival rates depending on the tumor type.

Thelazia Callipaeda Infestation with Tarsal Ectropion

PURPOSE: To report a case of Thelazia callipaeda infestation with tarsal ectropion.CASE SUMMARY: A 79-year-old man presented with epiphora and a sensation of a foreign body in his right eye that had persisted for several months. On the initial examination, he had conjunctival injection, conjunctival follicles, telangiectasia of the lid margins, meibomian gland dysfunction, and tarsal ectropion. We performed a lateral tarsal strip operation to treat the tarsal ectropion. During surgery, we identified and removed four parasites (about 1–2 cm in length and 0.5 mm in width) that had not been apparent on slit lamp examination. The parasites were identified as Thelazia callipaeda. The patient's symptoms subsequently improved considerably; he has followed up to the present time without recurrence.CONCLUSIONS: In the presence of continuous extraocular inflammation, as with infestation by Thelazia callipaeda, ectropion can be induced by several possible causes. Involutional ectropion can be accelerated mechanically through rubbing. Other causes include inferior force from inflammation of the connective tissue and temporary blepharospasm.

Efficacy and Safety of Letibotulinum Toxin A for the Treatment of Essential Blepharospasm

PURPOSE: To evaluate the efficacy and safety of BOTULAX® in subjects with essential blepharospasm.METHODS: In this study, a total of 250 subjects with essential blepharospasm were enrolled at 15 investigational sites and a total of 220 subjects completed the study. The efficacy and safety were evaluated at weeks 4 and 16 after treatment compared with baseline. In total, 240 subjects were enrolled, treated with the investigational product, and evaluable for the primary efficacy assessment at week 4 after treatment; these subjects were included in the intention-to-treat (ITT) population. With the ITT set as the main efficacy set, efficacy assessment included Jankovic rating scale (JRS), functional disability score, investigator evaluation of global response and quality of life. Safety assessment including the incidence of adverse events was also performed.RESULTS: In terms of the primary efficacy endpoint (i.e., change in JRS total score at week 4 after treatment from baseline [ITT set]), mean change indicated a statistically significant reduction (p < 0.0001) and demonstrated the non-inferiority of the test drug to similar drugs. In terms of the secondary efficacy endpoints, mean change in JRS total score at week 16 after treatment and mean change in functional disability score at weeks 4 and 16 after treatment both exhibited a statistically significant reduction compared with baseline (p < 0.0001 for all). Among the 249 subjects treated with the investigational product in this study, 44 (17.67%) experienced 76 treatment emergent adverse events but no serious adverse events were observed.CONCLUSIONS: Based on the study results, BOTULAX® is considered to be an effective and safe treatment for essential blepharospasm.

Treatment for Functional Failure after Primary Endonasal Dacryocystorhinostomy

Purpose@#To analyze the results of secondary silicone tube insertion in patients exhibiting persistent epiphora despite acquiringanatomical patency after primary endonasal dacryocystorhinostomy. @*Methods@#We conducted a retrospective review of 23 patients (37 eyes) seen at our department from December 2006 to April2018 for primary endonasal dacryocystorhinostomy. These patients exhibited anatomical patency but experienced persistentepiphora, requiring secondary silicone tube re-insertion. @*Results@#The average age at reintubation with a silicone tube was 59.8 years, and the average follow-up was 14.8 months. Theinterval between the primary endonasal dacryocystorhinostomy and secondary silicone tube re-insertion was approximately24.5 months. The silicone tube re-insertion, was successful for 33 of 37 eyes with four eyes exhibiting partial improvement. Thus,there were no failed outcomes based on subjective symptoms. @*Conclusions@#After primary endonasal dacryocystorhinostomy, when tearing symptoms persist despite no evidence of anatomicalproblems, our results show that secondary silicone tube re-insertion is a safe and effective procedure for patients experiencingfunctional failure.

The Clinical Utility of Routine Histological Biopsy during Dacryocystorhinostomy

Purpose@#We assessed the clinical utility of routine histopathological evaluation of the lacrimal sac during dacryocystorhinostomy. @*Methods@#From April 2009 to April 2018, we included 1,619 eyes of 1,266 patients who underwent dacryocystorhinostomy in our hospital. All lacrimal sacs were histopathologically examined. We excluded cases in which malignant lacrimal sac tumors had been preoperatively diagnosed. We retrospectively analyzed the medical records in terms of clinical manifestations, histological findings after lacrimal sac biopsy, and malignant tumors. We recorded the age, extent of bone marrow involvement, stage, and prognosis of patients with malignancies. @*Results@#We treated 217 males and 1,049 females of a mean age at diagnosis of 58.8 ± 12.3 years. The biopsy data showed that chronic inflammation with fibrosis (n = 1,026 [81.0%]) was the most common condition, followed by fibrosis (n = 133 [10.5%]), chronic inflammation (n = 94 [7.4%]), reactive lymphoid hyperplasia (n = 5 [0.4%]), malignant tumors (n = 4 [0.3%]), tubular adenomas (n = 2 [0.2%]), and papillomas (n = 2 [0.2%]). All malignant tumors were mucosal-associated lymphoid tissue lymphomas. Two of these four patients underwent additional imaging tests, but malignancies were not apparent. The other two had no specific complaints other than tearing. In addition, no abnormalities were evident on slit lamp examination or the syringing test. All four patients were cured by chemotherapy. @*Conclusions@#No clinical manifestation, physical examination or imaging data, or intraoperative finding in patients with nasolacrimal duct obstructions reliably identify a malignancy; but histological examination does.

The Clinical Utility of Routine Histological Biopsy during Dacryocystorhinostomy

Purpose@#We assessed the clinical utility of routine histopathological evaluation of the lacrimal sac during dacryocystorhinostomy. @*Methods@#From April 2009 to April 2018, we included 1,619 eyes of 1,266 patients who underwent dacryocystorhinostomy in our hospital. All lacrimal sacs were histopathologically examined. We excluded cases in which malignant lacrimal sac tumors had been preoperatively diagnosed. We retrospectively analyzed the medical records in terms of clinical manifestations, histological findings after lacrimal sac biopsy, and malignant tumors. We recorded the age, extent of bone marrow involvement, stage, and prognosis of patients with malignancies. @*Results@#We treated 217 males and 1,049 females of a mean age at diagnosis of 58.8 ± 12.3 years. The biopsy data showed that chronic inflammation with fibrosis (n = 1,026 [81.0%]) was the most common condition, followed by fibrosis (n = 133 [10.5%]), chronic inflammation (n = 94 [7.4%]), reactive lymphoid hyperplasia (n = 5 [0.4%]), malignant tumors (n = 4 [0.3%]), tubular adenomas (n = 2 [0.2%]), and papillomas (n = 2 [0.2%]). All malignant tumors were mucosal-associated lymphoid tissue lymphomas. Two of these four patients underwent additional imaging tests, but malignancies were not apparent. The other two had no specific complaints other than tearing. In addition, no abnormalities were evident on slit lamp examination or the syringing test. All four patients were cured by chemotherapy. @*Conclusions@#No clinical manifestation, physical examination or imaging data, or intraoperative finding in patients with nasolacrimal duct obstructions reliably identify a malignancy; but histological examination does.

Clinical outcomes and pathological characteristics of immunoglobulin G4-related ophthalmic disease versus orbital inflammatory pseudotumor

BACKGROUND/AIMS: This study investigated the clinical and pathological features of immunoglobulin G4 (IgG4)-related ophthalmic disease. To clarify the features, we compared IgG4-related ophthalmic disease and orbital inflammatory pseudotumor. METHODS: We retrospectively reviewed the medical records of 103 patients who were initially diagnosed with orbital inflammatory pseudotumor, and identified 16 cases in which the diagnosis was based on surgical biopsy and for which data in medical records were sufficient for analysis. Immunohistochemical staining of pathological specimens for IgG and IgG4 was performed. Finally, six of IgG4-related ophthalmic disease patient and 10 of orbital inf lammatory pseudotumor patient were analyzed. RESULTS: The IgG4-related ophthalmic disease group had more IgG4-positive plasma cells and a higher IgG4/IgG plasma cell ratio than the orbital inflammatory pseudotumor group. Collagenous fibrosis and lacrimal gland involvement were significantly more frequent in the IgG4-related ophthalmic disease group. Dense lymphocyte infiltration, obliterative phlebitis, and bilateral lesions were more frequent in IgG4-related ophthalmic disease, but the differences were not significant. The recurrence-free period was shorter in the IgG4-related ophthalmic disease group (p = 0.035). CONCLUSIONS: The location of the lesion (lacrimal gland), count and ratio of IgG4-positive plasma cells, and collagenous fibrosis aid the diagnosis of IgG4-related ophthalmic disease in patients with idiopathic orbital mass-like lesions. In addition, maintenance therapy should be considered in patients with IgG4-related ophthalmic disease to prevent recurrence.

Clinical Results of Anti-adhesion Adjuvants after Endonasal Dacryocystorhinostomy

PURPOSE: Various absorbable anti-adhesion agents have been used to prevent postoperative synechia formation after endonasal surgery. The purpose of this study was to evaluate the anti-adhesion effects of HyFence and Mediclore after endonasal dacryocystorhinostomy (DCR) compared to a mixed solution of hyaluronic acid and sodium carboxymethylcellulose (Guardix-Sol). METHODS: In this retrospective study, endonasal DCR and silicone tube intubation were performed on 198 eyes of 151 patients. Three different anti-adhesion adjuvants were applied to the osteotomy site in the nasal cavity after standard endonasal DCR procedures. The subjects were classified into three respective groups: group A (71 eyes, Guardix-Sol 1.5 g), group B (89 eyes, HyFence 1.5 mL), and group C (38 eyes, Mediclore 1 cc). The three groups were evaluated by asking patients about subjective symptoms and by performing lacrimal irrigation tests and endoscopic examinations. RESULTS: There were no statistically significant differences in age, sex, timing of tube removal, or follow-up period among the three groups. There were no statistically significant differences in success rates among the three groups (p = 0.990, 91.5% [65 / 71], 92.1% [82 / 89], and 92.1% [35 / 38], respectively). CONCLUSIONS: HyFence and Mediclore are safe and effective adjunctive modalities following endonasal DCR compared to Guardix-Sol. Therefore, these agents can be considered good alternatives to Guardix-Sol to increase the success rate of endonasal DCR in treating patients with poor prognosis.

Fibroepithelial Polyp on the Eyelid Margin: A Case Report

PURPOSE: Fibroepithelial polyps on the eyelid or orbit have not been previously reported in Korea. Herein, we report our experience with a case of a primary localized fibroepithelial polyp of the eyelid. CASE SUMMARY: A 78-year-old male patient presented with a round mass on the lower eyelid which has been noticed 1 week prior. There was no pain and no tenderness. The mass was removed and excisional biopsy was performed. Histopathologic examination revealed findings suggestive of a fibroepithelial polyp. There was no local recurrence during 4 months of follow-up. CONCLUSIONS: Fibroepithelial polyp should be considered when determining the type of eyelid mass.

Alternating and Relapsing Migratory Orbital Myositis

No abstract available.

A Case of Fungal Ball Causing Acute Dacryocystitis

PURPOSE: We report a case of fungal ball after Endoscopic Dacryocystorhinostomy (DCR) in a 40-year-old female patient. CASE SUMMARY: A 40-year-old female patient was admitted to our hospital for left lower eyelid tenderness and bloody discharge from the lacrimal punctum. During a planned endoscopic DCR, the sac was opened after the osteotomy, and 2 fungal balls were found in the lacrimal sac. The masses were 7 × 5 mm and, 9 × 5 mm sized, irregularly shaped, and red in color. Aspergillus fumigatus was diagnosed pathologically. Postoperative paranasal sinus magnetic resonance imaging showed no residual fungal ball. During follow-up, the patient showed patent rhinostomy opening, and there was no evidence of fungal infection on nasal endoscopic finding. CONCLUSIONS: Although Aspergillus fumigatus is a rare cause of canalicular obstruction, fungal ball development in the lacrimal sac can cause acute dacryocystitis.

Eyelid Mass as Initial Presentation of Breast Cancer: A Case Report

PURPOSE: We report a case in which eyelid mass was the initial presentation of breast cancer. The diagnosis of breast cancer was made after lid biopsy. CASE SUMMARY: A 41-year-old female patient presented with a painful mass on the left lower lid after 1 month. There was a pinkish mass in the lateral portion of the tarsal conjunctiva, and computed tomography revealed a mass with an irregular margin on the lower lid connected to the left lacrimal gland. Biopsy was performed at the tarsal conjunctiva of the left eye, and histopathologic examination was suggestive of malignant epithelial cell neoplasm, especially metastatic carcinoma in the breast. The patient was diagnosed as having invasive ductal cancer, for which she is currently receiving chemotherapy. CONCLUSIONS: Metastases to the eyelid are very rare, accounting for less than 1% of all malignant eyelid lesions. We report a patient who presented with an eyelid mass as the initial presentation of breast cancer, which was diagnosed with metastatic breast cancer.

Introduction of ophthalmic plastic and reconstructive surgery

No abstract available.

Analysis of treatment outcomes for primary tonsillar lymphoma

PURPOSE: Although each Waldeyer’s ring sub-site is considered an independent prognostic factor, few studies have assessed the prognosis and treatment of tonsillar lymphoma. Treatment outcomes were analyzed in patients with primary tonsillar lymphoma who were treated with chemotherapy and radiotherapy (RT). MATERIALS AND METHODS: Nineteen patients with diffuse large B-cell lymphoma were evaluated, with a median follow-up of 53 months. Age, sex, and histology, amongst other factors, were reviewed. Progression-free survival (PFS) and overall survival (OS) rates were analyzed. RESULTS: Most patients had Ann Arbor stage I-II (94.7%), IPI score of 0 (89.5%), and complete remission after chemotherapy (89.5%). The 5-year PFS and OS rates were 74.6% and 80%, respectively. In univariate analysis, the rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) regimen resulted in a better PFS than the cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) regimen (88.9% vs. 50.0%; p = 0.053). RT dose was related to the survival outcome (p = 0.010 for PFS, p = 0.044 for OS). Patients were classified into the CHOP + RT (>40 Gy) group and R-CHOP + RT (≤40 Gy) group. The 5-year PFS rates were 50% in the CHOP + RT group, and 100 % in the R-CHOP + RT group (p = 0.018). The 5-year OS rates were 66.7% and 100%, respectively (p = 0.087). CONCLUSION: Primary tonsillar lymphoma patients typically have favorable outcomes. Chemotherapy (R-CHOP) combined with relatively lower dose consolidative RT may be safe and effective for primary tonsillar lymphoma.

A Case of Lacrimal Gland MALT Lymphoma in a Patient with Primary Sjögren's Syndrome

PURPOSE: To report a case of lacrimal gland mucosa-associated lymphoid tissue (MALT) lymphoma in a patient with primary Sjögren's syndrome and Behcet's disease. CASE SUMMARY: A 49-year-old female patient with primary Sjögren's syndrome and Behcet's disease presented with a one-year history of painless upper and lower eyelid swelling in her right eye. Lacrimal gland incisional biopsy was performed, and the patient was diagnosed with malignant lymphoma (extranodal marginal zone B cell lymphoma of MALT). No distant metastases were detected on whole-body computed tomography or positron emission tomography, and the patient was treated with Rituximab, Cyclophosphamide, Vincristine, Prednisone (R-CVP) regimen chemotherapy. After 8 consecutive chemotherapy cycles, her eyelids appeared normal externally, and partial regression was found radiologically. CONCLUSIONS: The possibility of MALT lymphoma should be considered as a differential diagnosis if patients with autoimmune diseases such as primary Sjögren's syndrome show eyelid swelling or palpable mass.