Primary Mucinous Adenocarcinoma of the Lower Eyelid

Purpose@#We report a case of a primary mucinous adenocarcinoma of the lower eyelid diagnosed in patient with a painless mass.Case summary: A 62-year-old male visited our clinic with a painless mass of the left lower eyelid of increasing the size for 2 months. Orbital computed tomography and magnetic resonance imaging revealed an approximately 2 cm well-defined ovoid, homogeneous enhancing mass in the left lower eyelid. A biopsy was performed under general anesthesia, and histopathologic examination confirmed mucinous adenocarcinoma. Final diagnosis was made as primary mucinous adenocarcinoma since torso positron emission tomography and computed tomography found no abnormal findings and extensive resection was performed for complete resection of the tumor. @*Conclusions@#Primary mucinous adenocarcinoma that occurs in the eyelid is a very rare malignant tumor, and a systemic examination to determine whether it is metastatic is essential, and due to its high local recurrence, regular follow-up after extensive resection is required.

Rhino-orbito-cerebral Actinomycosis Infection

Purpose@#To report a case of actinomycosis infection originating in the orbitonasal cavity that quickly invaded the cerebrum.Case summary: A-57-year-old male with a history of right tooth extraction 7 days before the first visit presented with right eyelid swelling and pain that had developed 4 days prior to the visit and had become increasingly worse. The best-corrected visual acuity was light perception. The intraocular pressure of the patient was 37 mmHg in the right eye. Eyelid abscess, subconjunctival hemorrhage, and severe chemosis was observed. Orbital computed tomography showed multiple air pockets and enhancing soft tissue along the periorbital, maxillary, and ethmoid sinus. Emergent endoscopic sinus surgery, lateral canthotomy, and intravenous antifungal treatment were performed due to suspicion of mucormycosis infection. Two days after treatment, exenteration combined with otolaryngology and neurosurgery were performed, as brain magnetic resonance imaging scans indicated that intraorbital lesions had invaded the dura and frontal sinus. On Day 8 of treatment, Actinomyces odontolyticus was identified and intravenous Ampicillin was administered. Despite systemic antibiotic treatment, the infection could not be controlled. The patient died 28 days after treatment due to persistent intracranial hemorrhage and brain edema. @*Conclusions@#For patients with severe eyelid swelling with a history of tooth extraction, actinomycosis infection should be considered. Delays in diagnosis and treatment of this infection could lead to serious consequences.

Rhino-orbito-cerebral Actinomycosis Infection

Purpose@#To report a case of actinomycosis infection originating in the orbitonasal cavity that quickly invaded the cerebrum.Case summary: A-57-year-old male with a history of right tooth extraction 7 days before the first visit presented with right eyelid swelling and pain that had developed 4 days prior to the visit and had become increasingly worse. The best-corrected visual acuity was light perception. The intraocular pressure of the patient was 37 mmHg in the right eye. Eyelid abscess, subconjunctival hemorrhage, and severe chemosis was observed. Orbital computed tomography showed multiple air pockets and enhancing soft tissue along the periorbital, maxillary, and ethmoid sinus. Emergent endoscopic sinus surgery, lateral canthotomy, and intravenous antifungal treatment were performed due to suspicion of mucormycosis infection. Two days after treatment, exenteration combined with otolaryngology and neurosurgery were performed, as brain magnetic resonance imaging scans indicated that intraorbital lesions had invaded the dura and frontal sinus. On Day 8 of treatment, Actinomyces odontolyticus was identified and intravenous Ampicillin was administered. Despite systemic antibiotic treatment, the infection could not be controlled. The patient died 28 days after treatment due to persistent intracranial hemorrhage and brain edema. @*Conclusions@#For patients with severe eyelid swelling with a history of tooth extraction, actinomycosis infection should be considered. Delays in diagnosis and treatment of this infection could lead to serious consequences.

Successful Treatment of Dacryocystocele due to Secondary Nasolacrimal Duct Obstruction by Intraosseous Cavernous Hemangioma

Purpose@#Here, we report the clinical features and successful treatment of a very rare case of dacryocystocele due to secondary nasolacrimal duct obstruction by cavernous hemangioma in an adult patient.Case summary: A 78-year-old man had a painless mass on the right medial canthus and epiphora for 5-6 years. The mass on the lower area of the right medial canthus had a well-defined border, was fixed in position, and smooth. Lacrimal irrigation via the lower punctum showed reflux through the opposite punctum without nasal passage. Orbital computed tomography showed multicystic mass formation on the right lacrimal sac and a bony lesion with ground glass opacity on the anteromedial side of the right nasolacrimal duct causing duct narrowing. Orbital magnetic resonance imaging showed a multicystic, fluid-filled structure in the right lacrimal sac. An intranasal bony lesion 14 mm in diameter was also observed. The patient was diagnosed with dacryocystocele due to secondary nasolacrimal duct obstruction by an intranasal mass. Endoscopic transnasal removal of two lacrimal cystic masses and the intranasal mass, and dacryocystorhinostomy (DCR) were performed. Pathological examination revealed chronic inflammation with lymphoid follicles of the tear bag and cavernous hemangioma of the nasal cavity. @*Conclusions@#Acquired dacryocystocele in adults is most commonly idiopathic. Here, we report a case of a dacryocystocele due to secondary nasolacrimal duct obstruction by cavernous hemangioma that was treated successfully by endoscopic transnasal tumor removal and DCR.

Clinical Manifestations and Computed Tomography Findings of Trapdoor Type Medial Orbital Wall Blowout Fracture

PURPOSE: To report the clinical manifestations and computed tomography (CT) findings of patients with a trapdoor type medial orbital wall blowout fracture.METHODS: From March 2009 to October 2016, the clinical records and computed tomography findings of patients who underwent surgical treatment for a trapdoor type medial orbital wall blowout fracture were retrospectively analyzed.RESULTS: A total of eight patients (six males and two females) were enrolled with a combined mean age of 14.4 years. Clinical manifestations were eyeball movement limitation (abduction and adduction) and ocular motility pain (eight patients, 100%), diplopia (seven patients, 87.5%), and nausea and vomiting (four patients, 50%). On CT, the distance from the orbital apex to the fracture site was an average of 22.0 mm and occurred in the middle position of the entire wall. Two patients had missed rectus completely dislocated into the ethmoid sinus through the fracture gap and six patients had definite involvement in the fracture gap and edema of the medial rectus muscle. The medial rectus muscle cross-sectional area was 47.7 mm² which was edematous compared to the contralateral eye (40.1 mm²). Orbital wall reconstruction was performed an average of 4.1 days after the injury. In all patients with oculocardiac reflex-like nausea and vomiting immediately improved after surgery. Six out of eight patients who had eyeball movement limitations (abduction and adduction) preoperatively showed adduction limitation after surgery. The eyeball movement limitation and diplopia disappeared 11.7 days and 46.7 days after surgery, respectively.CONCLUSIONS: Patients with trapdoor type medial wall blowout fracture showed characteristic computed tomographic findings and clinical manifestations such as eyeball movement limitation, ocular motility pain, diplopia, and oculocardiac reflex. An understanding of clinical findings and quick surgical treatment are therefore required. The type of eyeball movement limitation was abduction and adduction limitation preoperatively and adduction limitation postoperatively.

360° Circumferential Hemorrhagic Lymphangiectasia of the Conjunctivae

PURPOSE: We report a case of hemorrhagic lymphangiectasia of the conjunctiva with a 360° connected circumference, which recovered spontaneously.CASE SUMMARY: A 44-year-old female patient presented with congestion of the right eye 1 day prior to her visit. There was no history of any systemic disease or trauma, but she had experienced relapses of the same episode three times before the visit. There were no accompanying symptoms such as decreased vision or pain. Blood analysis, orbital computed tomography, and angiographic findings showed no remarkable finding. Slit lamp examination showed circumferential lymphatic dilatation extending 360° under the conjunctiva of the eye at a distance of about 6 mm behind the limbus of the right eye, which was accompanied by intralymphatic bleeding. Irregular local lymphatic dilatations were observed on the bulbar conjunctiva at 4 and 8 o'clock of the left eye. The bleeding spontaneously resolved in about 2 weeks, but the translucent enlarged lymphatic vessels were still observed on slit lamp examination and anterior segment optical coherence tomography.CONCLUSIONS: This is the first reported case of hemorrhagic lymphangiectasia involving 360° of the bulbar conjunctiva in Republic of Korea.

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.

Optic Nerve Sheath Meningocele Diagnosed in Patients with Exophthalmos

Purpose@#To report a rare case of bilateral optic nerve sheath meningocele diagnosed in a patient with exophthalmos.Case summary: A 33-year-old male visited our clinic with bilateral exophthalmos for 6 months. The patient had been diagnosed with hypertension 3 years previously; however, the condition had been poorly controlled. He also had a history of treatment associated with retinal vein occlusion, macular edema, and papilledema 2 years earlier. There was no limitation in his ocular movement. A Hertel exophthalmometry test showed bilateral exophthalmos of 20 mm in both eyes. Visual field tests showed an inferior arcuate visual field defect in the right eye and a superotemporal peripheral field defect in the left eye. In orbital magnetic resonance imagery, cerebrospinal fluid space widening along the optic nerve and flattening of the bilateral posterior pole of the eye were evident. The patient was diagnosed with optic nerve sheath meningocele. @*Conclusions@#Optic nerve sheath meningocele should be considered as a differential diagnosis of exophthalmos patients. Because it is a disease that can affect visual function in a manner similar to that of a visual field defect, rapid diagnosis through imaging study and thorough regular follow-up examinations are essential.

Optic Nerve Sheath Meningocele Diagnosed in Patients with Exophthalmos

Purpose@#To report a rare case of bilateral optic nerve sheath meningocele diagnosed in a patient with exophthalmos.Case summary: A 33-year-old male visited our clinic with bilateral exophthalmos for 6 months. The patient had been diagnosed with hypertension 3 years previously; however, the condition had been poorly controlled. He also had a history of treatment associated with retinal vein occlusion, macular edema, and papilledema 2 years earlier. There was no limitation in his ocular movement. A Hertel exophthalmometry test showed bilateral exophthalmos of 20 mm in both eyes. Visual field tests showed an inferior arcuate visual field defect in the right eye and a superotemporal peripheral field defect in the left eye. In orbital magnetic resonance imagery, cerebrospinal fluid space widening along the optic nerve and flattening of the bilateral posterior pole of the eye were evident. The patient was diagnosed with optic nerve sheath meningocele. @*Conclusions@#Optic nerve sheath meningocele should be considered as a differential diagnosis of exophthalmos patients. Because it is a disease that can affect visual function in a manner similar to that of a visual field defect, rapid diagnosis through imaging study and thorough regular follow-up examinations are essential.

Apocrine Hidrocystoma Presenting as a Lacrimal Gland Mass

Purpose@#To report a case of apocrine hidrocystoma in the lacrimal gland.Case summary: A 51-year-old woman presented with a left upper eyelid mass that occurred two years before presenting tohospital. Upon physical examination, a painless palpable mass on the left upper eyelid was observed. Based on slit-lamp examination,a pinkish mass with a well-circumscribed border on the left upper temporal conjunctiva was observed. Orbital computedtomography showed a well-defined contrast enhanced mass of 8 × 9 × 9 mm in the lacrimal lesion. The mass was removed completelyunder local anesthesia. Biopsy confirmed that the mass was an apocrine hidrocystoma. There was no recurrence at oneyear postoperatively. @*Conclusions@#This is the first report of an apocrine hidrocystoma in a lacrimal lesion in Korea. Apocrine hidrocystoma is rarelyfound on lacrimal gland lesions, but it should be considered in the differential diagnosis of a lacrimal gland mass.

Longitudinal Evaluation of Retinal Structure in Patients with Traumatic Optic Neuropathy Using Optical Coherence Tomography

PURPOSE: We used optical coherence tomography (OCT) for longitudinal evaluation of structural changes in the peripapillary retinal nerve fiber layer (RNFL), the macular ganglion cell-inner plexiform layer (GC-IPL), and the macula in patients with traumatic optic neuropathy. METHODS: From May 2012 to April 2015, the medical records of 20 patients with monocular traumatic optic neuropathy who were followed up for over 6 months were retrospectively analyzed. Best-corrected visual acuity was checked and Cirrus high-definition optical coherence tomography (HD-OCT) was used to measure the thicknesses of the peripapillary RNFL, macular GC-IPL, and macula of both eyes at the first visit (within 4 weeks after trauma), at 10 and 24 weeks after trauma, and at the final visits. The differences over time in the parameters of the traumatic and fellow eyes were analyzed. RESULTS: The final best-corrected visual acuities of the traumatic and fellow eyes differed significantly from those at the first visit (p = 0.007). The average thicknesses of the peripapillary RNFL, the macular GC-IPL, and the macula differed significantly between the traumatic and fellow eyes commencing 10 weeks after trauma (p < 0.001, p = 0.002, p = 0.003, respectively). CONCLUSIONS: Significant changes in visual acuity preceded structural changes in the retina. Objective assessment of retinal structural changes using OCT yields helpful information on the clinical course of patients with traumatic optic neuropathy.

A Case of Eyelid Desmoplastic Trichilemmoma

PURPOSE: Desmoplastic trichilemmoma is a rare variant of trichilemmoma first described in 1990. Desmoplastic trichilemmoma on the eyelid has not been previously reported in Korea. We report our experience with a case of desmoplastic trichilemmoma of the eyelid. CASE SUMMARY: A 72-year-old male patient presented with a round mass on the upper eyelid, which was noticed 1 year prior and caused irritation and itching. Excisional biopsy of the mass was performed. Histopathological examination revealed findings suggestive of a desmoplastic trichilemmoma. There was no local recurrence during the 2-year follow-up. CONCLUSIONS: Desmoplastic trichilemmoma should be considered when determining the type of eyelid mass.

Multiple Solitary Plasmacytomas Presenting with Painful Erythematous Swelling of the Upper Eyelid

PURPOSE: To report a case of multiple solitary plasmacytomas that presented with painful erythematous swelling of the upper eyelid. The patient was diagnosed with extramedullary plasmacytoma, and was later found to have multiple metastases of the bone and soft tissue during follow up. CASE SUMMARY: A 55-year-old female patient presented with painful erythematous swelling of the left upper eyelid that persisted for 1 month prior to examination. Under suspicion of lacrimal gland inflammation, anti-inflammatory medication was started but the symptoms worsened. Orbital computed tomography showed that a mass infiltrated the left lacrimal gland. We performed incisional biopsy of the mass via eyelid crease incision. Based on histopathological examination, the mass was diagnosed as extramedullary plasmacytoma and the patient was treated with radiation. After a 10-month follow-up period, multiple metastases on the left parotid gland, thoracic spine, lumbar spine and pelvic bone were observed. Finally, we diagnosed the patient with multiple solitary plasmacytomas. CONCLUSIONS: We report the first case of multiple solitary plasmacytomas presenting with upper eyelid painful erythematous swelling. This condition should be considered for patients presenting with eyelid inflammation. In addition, long term follow up should be conducted to detect metastasis or recurrence.

A Case of Eruptive Hair Cyst Developed on the Eyelid

PURPOSE: Eruptive vellus hair cysts (EVHC) are benign lesions that affect the pediatric population and are rarely seen congenitally or in young adults. EVHCs are small, cystic papules that usually occur on the chest and proximal extremities. EVHCs of the eyelids have been reported infrequently. We experienced a case of solitary EVHC that developed on the eyelid in a middle-aged male. Herein, we present our case with a brief review of the literature. CASE SUMMARY: A 44-year-old male presented with a history of an asymptomatic, palpable mass in the right upper eyelid that had been slowly growing for 2 years. Physical examination revealed non-tender, firm and round mass in right upper eyelid. Computed tomography scan of the orbit showed a 7.0 x 9.0 x 9.5 mm-sized focal bulging contour in the right upper eyelid. The patient underwent sub-brow incision and excisional biopsy of the eyelid mass. Histopathological examination revealed a 7.0 x 6.0 x 4.0 mm-sized cystic structure lined by squamous epithelium containing laminated keratinous materials and multiple vellus hair shafts. There was no local recurrence during the postoperative follow-up. CONCLUSIONS: We experienced a rare case of EVHC that developed on the eyelid. Based on our experience, EVHC should be considered when determining an eyelid mass, especially around hair follicles.

A Case of Angiolymphoid Hyperplasia with Eosinophilia (ALHE) of the Eyelid

PURPOSE: The authors of the present study describe a rare case of angiolymphoid hyperplasia with eosinophilia (ALHE) of the eyelid. CASE SUMMARY: A 63-year-old male who was diagnosed with ALHE based on biopsy of an inguinal mass presented with an eyelid mass of 1 month duration. A light brown, solitary, 1.0 x 0.5 cm-sized mass involved the right upper eyelid. There was no lymphadenopathy, but eosinophilia was present. An excisional biopsy of the mass was performed for diagnosis and management. Macroscopic examination of the excised mass revealed a well-defined, smooth, firm, yellowish-red colored lesion measuring 1.0 x 0.6 x 0.5 cm. Histopathology showed the proliferation of small blood vessels, many of which were lined by enlarged endothelial cells with uniform ovoid nuclei and intracytoplasmic vacuoles. The distinctive endothelial cells were described as having a cobblestone appearance. In addition, a perivascular and interstitial infiltrate composed primarily of lymphocytes and eosinophils was present. ALHE was finally confirmed with clinical and microscopic examination. CONCLUSIONS: The authors of the present study report a rare case of ALHE of the eyelid and suggest that a differential diagnosis should be considered.

Unilateral Ptosis Due to Isolated Levator Myositis

PURPOSE: To present a rare case of idiopathic orbital myositis involving levator palpebrae superioris. CASE SUMMARY: A 27-year-old male presented with a 1-week history of redness, discomfort, swelling, and drooping of his left upper eyelid. A computed tomography scan showed isolated enlargement of the right superior rectus/levator muscle complex. On examination, there was a left blepharoptosis, although eye movements were normal. The authors treated the patient with 3rd-generation cephalosporin; however, after 3 days, the symptoms did not improve. Subsequently, the patient was diagnosed with idiopathic orbital myositis and treated with oral corticosteroids for 1 month; the symptoms gradually resolved. CONCLUSIONS: Idiopathic orbital myositis is a subtype of nonspecific orbital inflammation primarily involving the extraocular muscles. Although the exact cause of orbital myositis is unknown, an immune-mediated pathophysiologic mechanism appears to be one of the causes. Medial rectus myositis is the most common, and isolated levator muscle myositis is very rare. The authors of the present study reported a case of orbital myositis involving the levator palpebrae superioris which should be considered a differential diagnosis of blepharoptosis with eyelid swelling.

A Case of Isolated Squamous Cell Carcinoma of the Orbit

PURPOSE: To report a case of isolated squamous cell carcinoma of the orbit. CASE SUMMARY: A 75-year-old man with over a 50 pack-year history of smoking presented discomfort and visual disturbance of the left eye for several months. His best-corrected visual acuity was 0.3, intraocular pressure was 9 mm Hg, and extraocular movements were normal. Slit-lamp examinations revealed no specific findings in the anterior segment in the left eye. However, retinal exams showed an oval-shaped, juxtapapillary mass-like lesion associated with retinal folding in the left eye. A huge, distorted echoic mass with an initial prominent spike and low-to-medium internal reflectivity with diminishing amplitude was observed on ocular ultrasonography. Enhanced CT revealed a highly-intense, irregular-circumscribed heterogeneous mass (2.0 x 2.0 x 1.5) in the superomedial quadrant of the left eye. Metastatic workups, including bone scan and CT of the head, neck, chest, and abdomen, were unremarkable. One week after the initial visit, the patient experienced pain and reduced visual acuity (light perception) in the left eye. Following the diagnosis, enucleation with tumor resection and hydroxyapatite implantation was performed. Histopathologic examination revealed a moderated-differentiated squamous cell carcinoma invading the sclera. The patient subsequently underwent radiation treatment and no evidence of recurrence was reported 6 months after surgery.

An Association Study of Apolipoprotein E Gene Polymorphism and Cataracts

PURPOSE: To evaluate the association of apolipoprotein E (APOE) polymorphism and cataracts in the Korean population. METHODS: The present research included participants from a population-based study in Incheon, Korea. A sample of 126 adults genotyped for polymorphisms of APOE underwent a medical interview, an eye examination which included visual acuity testing, slitlamp cataract evaluation and fundus examination. The APOE polymorphism was determined using a polymerase chain reaction method. RESULTS: Eighty-eight participants (69.8%) were diagnosed with cataracts or had undergone cataract surgery in 1 or both eyes, and 38 participants (30.2%) demonstrated no signs of cataract. The frequencies of the APOE genotypes and alleles were not significantly different from the cataract and the control group. APOE epsilon2 carriers were less likely to have cataracts than non-epsilon2 carriers with an odds ratio of 0.367 which was almost statistically significant with the multiple logistic regression analysis (p = 0.052). CONCLUSIONS: There was no significant correlation of APOE genotype and cataracts. However, a slight negative association of APOE epsilon2 and cataracts were found in the Korean population.