A Case of Orbital Infarction Syndrome in a Patient with Hemophagocytic Lymphohistiocytosis

Purpose@#To report a case of orbital infarction syndrome in a patient with hemophagocytic lymphohistiocytosis (HLH).Case summary: A 70-year-old woman with diabetes mellitus and hypertension was referred to the Department of Ophthalmology for sudden-onset left upper eyelid ptosis after being diagnosed with HLH. After 3 days, the best corrected visual acuity in the right eye was 0.8, while there was no light perception in the left eye. In the left eye, the ocular motility examination showed limitations in all fields of gaze. On fundus examination, optic disc pallor, retinal hemorrhage, and narrowed retinal arteries were observed in the left eye. Fluorescein angiography showed no blood flow in the retinal arteries and veins in the left eye. On neck computed tomography angiography, the left distal internal carotid artery was narrowed. Orbit computed tomography showed exophthalmos and extraocular muscle hypertrophy in the left eye. Orbit magnetic resonance imaging confirmed optic nerve edema, enhancement of the optic nerve sheath, and high signal intensity of the intraocular fat in the left eye. Slit-lamp examination revealed ischemia of the anterior segment and ischemic necrosis of the eyelid in the left eye. The patient was diagnosed with orbital infarction syndrome. @*Conclusions@#Several factors may lead to ischemia of the orbital tissues in patients with HLH, including coagulopathy, an increased incidence of blood clots, and inflammation in the orbit. It is necessary to consider the possibility of orbital infarction syndrome when HLH patients present with visual loss, ocular movement limitations, and anterior segment ischemia.

Retinal Microvascular Abnormalities in Patients with Type I Neurofibromatosis

Purpose@#To evaluate the findings and frequencies of retinal microvascular abnormalities observed in patients with type 1 neurofibromatosis. @*Methods@#Fundus photographs of 61 patients with type 1 neurofibromatosis and 61 controls without systemic disease or ophthalmic abnormalities were retrospectively compared and analyzed. The presence or absence of retinal microvascular abnormalities in the form of simple vascular tortuosity, corkscrew retinal vessels, and moyamoya-like patterns was confirmed, and the diagnostic sensitivity, diagnostic specificity, positive predictive value, negative predictive value, and diagnostic accuracy for type 1 neurofibromatosis were analyzed. @*Results@#Retinal microvascular abnormalities were found in 19.7% (12 patients) of the patient group, There was no cases in the control group, thus. The difference between the patient group and the control group was significant (p = 0.0003). Of the 12 patients with abnormalities, 10 exhibited simple vascular tortuosity, one had corkscrew retinal vessels, and one exhibited both findings. The diagnostic sensitivity of retinal microvascular abnormalities for type 1 neurofibromatosis was 23.53%, the diagnostic specificity was 100%, the positive predictive value was 100%, and the negative predictive value was 61%. The diagnostic accuracy was 65.18%, which was slightly lower than the 79.5% diagnostic accuracy for the Lisch nodule, but the diagnostic accuracy was comparable to that of neurofibroma (68.03%). @*Conclusion@#Retinal microvascular abnormalities were observed in 19.7% of type 1 neurofibromatosis patients, of which simple vascular tortuosity was the most common. Considering that retinal microvascular abnormalities were not observed at all in the control group, and the diagnostic accuracy was 65.18%, this type of abnormality could be included as a new ophthalmic clinical feature of type 1 neurofibromatosis.

The Role of Orbital Computed Tomography as a Prognostic Indicator for Open Globe Injury

Purpose@#To study the clinical role of preoperative orbital computerized tomography (CT) in determining initial surgery in patients with open globe injuries. @*Methods@#This is a retrospective study that evaluated patients who underwent an operation for single-eye open-globe injury after orbital CT and were followed up for at least one year. Patients were divided into two groups: a group with anatomical failure that underwent enucleation, evisceration, or phthisis bulbi and a group with anatomical success. The correlations with preoperative CT findings and clinical outcomes were compared between the two groups. @*Results@#A total of 122 cases of open globe injury were included. Comparing preoperative CT results between the two groups, anatomical failure was correlated with the CT findings of intraocular hemorrhage (odds ratio, 5.0; 95% confidence interval [CI], 2.0-15.4), orbital wall fracture, and a reduction in eyeball volume (all p < 0.05). In the anatomical failure group, the average volume and axial length of the eyeball decreased by 28% and 16%, respectively, in traumatic eyes compared with non-traumatic eyes. @*Conclusions@#Orbital CT is a useful tool for evaluating the extent of open globe injury. If the prognostic factors indicate poor results, including intraocular hemorrhage, orbital wall fracture, or a severe decrease in the volume of the injured eye in orbital CT scans, primary enucleation or evisceration should be considered carefully, based on consultations with patients and their caregivers.

Severe Upper and Lower Tarsal Ectropion with Immunoglobulin G4-related Ophthalmic Disease

Purpose@#To present the first case of severe upper and lower tarsal ectropion associated with immunoglobulin G4 (IgG4)-relatedophthalmic disease.Case summary: A 37-year-old male presented with relapsed eyelid swelling and periocular inflammation in the left eye for7 years. He had severe upper and lower tarsal ectropion and a thickened upper eyelid with prolapsed conjunctival fornix. Orbitalcomputed tomography showed a poorly defined anterior orbital mass involving the lacrimal gland and upper and lower eyelids onthe left eye. The severe upper and lower eyelid ectropion was corrected by a modified spindle procedure and lateral tarsal strip.Hard, ill-defined fibrotic tissue and a mass around the lateral upper tarsal plate were discovered that required excision of themass and a biopsy. Hematologic and histopathologic findings of the orbital mass indicated IgG4-related ophthalmic disease withsevere upper and lower ectropion caused by the disease. @*Conclusions@#In cases of severe upper and lower eyelid ectropion caused by chronic inflammation, as seen in this case, not onlyeyelid tightening with excision of the causative mass but also a modified spindle procedure is required to prevent rotation of theeyelid.

Blepharoptosis Secondary to Local Conjunctival and Tarsal Amyloidosis

Purpose@#To report a case of primary conjunctival and tarsal amyloidosis with blepharoptosis.Case summary: A 73-year-old woman presented with left blepharoptosis and foreign body sensation that worsened over a1-year period prior. She presented with severe blepharoptosis on the left upper lid, with slightly reduced levator function. Duringthe operation, a fragile yellowish-pink mass-lesion was observed, widely involving palpebral conjunctiva and the space betweenthe levator aponeurosis and tarsal plate. While levator resection was withheld, the mass was excised as much as possible andblepharoplasty was performed. After surgery, the blepharoptosis improved slightly, and histopathologic examination revealedamyloid deposits. Because there were no abnormal findings in systemic examinations including blood tests, chest X-rays, andabdominal ultrasonography, the patient was diagnosed as primary conjunctival and tarsal amyloidosis. @*Conclusions@#Although local amyloidosis of the conjunctiva and tarsal plate is rare, it should be considered in differential diagnosisof patients with involutional blepharoptosis.

Clinical and Demographic Characteristics of Blepharoptosis in Korea: A 24-year Experience including 2,328 Patients

PURPOSE: To describe the demographics, relative incidence of subtypes, and clinical characteristics of blepharoptosis in Korean patients. METHODS: This is a retrospective, observational case series consisting of 2,328 patients who underwent ptosis surgery from 1991 to 2014 at a tertiary referral hospital in Korea. The patients were classified according to the type of ptosis and the evaluation of clinical characteristics including levator muscle function (LF) and degree of ptosis. RESULTS: Of the 2,328 patients, 1,815 (78%) had congenital ptosis and 513 (22%) had acquired ptosis. Simple congenital ptosis is the most common type overall (73.7%), and aponeurotic ptosis is the most common acquired type. More than three-quarters of eyes with congenital ptosis were affected in a moderate (34.4%) to severe degree (41.3%), and most of these eyes had fair (33.7%) to poor LF (60.1%). Among eyes with acquired ptosis, approximately three-quarters were affected in a mild (33.3%) to moderate degree (41.0%), with 63.3% of these eyes having good LF. The most widely used surgical technique was frontalis suspension (55.1%), followed by levator resection (29.0%) and aponeurosis repair (14.8%). At 3 years after the first surgery, 15.7% of patients with congenital ptosis and 10.4% of patients with acquired ptosis underwent reoperation. CONCLUSIONS: Although the prevalence has decreased from previous years, the proportion of cases with congenital ptosis was higher in this study than has been shown in research conducted in the West. The majority of eyes with congenital ptosis was affected to a severe degree and had poor LF, while those with acquired ptosis were affected to a moderate degree and had good LF. More cases with acquired ptosis presented with fair to poor LF, and frontalis suspension surgery was performed more commonly compared to previous studies. The reoperation rate was higher in congenital ptosis compared to acquired ptosis.

Periosteal Fixation Applied to Patients with Large-angle Paralytic Strabismus

PURPOSE: To evaluate the effect of periosteal fixation in patients with large-angle paralytic strabismus that was not corrected through conventional strabismus surgery. METHODS: Four eyes of three patients with large-angle paralytic strabismus who underwent periosteal fixation from June 2014 to August 2014 were examined. All patients presented with exotropia > 50 prism diopters (PD). Two of them showed exotropia caused by chronic complete oculomotor nerve palsy; the other two showed exotropia caused by medial rectus muscle injury during endoscopic sinus surgery. RESULTS: The mean preoperative exodeviation using the Krimsky test was 58 ± 29 PD. The postoperative values were 6.5 ± 9.4 PD at 1 week, and 11.25 ± 2.5 PD at 6 months. The mean surgical effect of exodeviation was 43.75 ± 21.36 PD. CONCLUSIONS: Periosteal fixation is an effective surgery for the management of paralytic strabismus that was not corrected through conventional strabismus surgery.

The Relationship between Visual Outcome and Ocular Trauma Score after Open Globe Injuries in Children

PURPOSE: We evaluated the prognostic factors of open globe injuries in children and adolescents, and compared the ocular trauma score (OTS) and pediatric penetrating ocular trauma score (POTS). METHODS: We performed a retrospective review of 77 children under 18 years of age who visited our clinic with open globe injuries between May 1993 and April 2014. We investigated the factors that may affect final visual acuity. We also compared the OTS and POTS using receiver operating characteristic curves as a method to predict final visual acuity. RESULTS: By univariate analysis, an initial visual acuity less than 20/200, globe rupture, wound size greater than 7.0 mm, retinal detachment, lens dislocation, and total number of operations contributed to worse visual outcomes (<20/200). Conversely, central corneal involvement, traumatic cataract, wound size less than 7.0 mm, and initial visual acuity greater than 20/200 were better prognostic indicators (≥20/32). Both OTS and POTS had diagnostic value as a predictor of final visual acuity, although there were no statistically significant differences between the two scoring systems. CONCLUSIONS: Initial visual acuity and wound size are important prognostic factors for the final visual acuity in children and adolescent, following open globe injuries. Both OTS and POTS are reliable prognostic models for open globe injuries in children and adolescents.

Toxic Optic Neuropathy Caused by Chlorfenapyr Poisoning

PURPOSE: To report a case of toxic optic neuropathy caused by chlorfenapyr ingestion accompanied by central nervous system involvement. CASE SUMMARY: A 44-year-old female visited our clinic complaining of reduced visual acuity in both eyes for 7 days. She had ingested a mouthful of chlorfenapyr for a suicide attempt 2 weeks prior to the visit. Gastric lavage was performed immediately after ingestion at the other hospital. Her best-corrected visual acuity was finger count 30 cm in the right eye and hand motion in the left eye. Both pupils were dilated by 5.0 mm and the response to light was sluggish in both eyes. A relative afferent pupillary defect was detected in her left eye. Funduscopy revealed optic disc swelling in both eyes. Magnetic resonance imaging of the brain showed a symmetric hyper-intense signal in the white matter tract including the internal capsule, corpus callosum, middle cerebellar peduncle, and brainstem. The patient was diagnosed with toxic optic neuropathy induced by chlorfenapyr ingestion, and underwent high-dose intravenous corticosteroid pulse therapy. Three days later, the best-corrected visual acuity was no light perception in both eyes. Three months later, optic atrophy was observed in both eyes. Optical coherence tomography revealed a reduction in the thicknesses of the retinal nerve fiber layer and ganglion cell and inner plexiform layer in the macular area. CONCLUSIONS: Ingestion of even a small amount of chlorfenapyr can cause severe optic nerve damage through the latent period, despite prompt lavage and high-dose steroid treatment.

Incipient Ocular Mucosa-associated Lymphoid Tissue Lymphoma in IgG4-related Orbital Disease

No abstract available.

Evaluation of Bony Nasolacrimal Ducts in Koreans with Primary Acquired Nasolacrimal Duct Obstruction

PURPOSE: To evaluate the morphometric differences of bony nasolacrimal ducts (BNLDs) using computed tomography (CT) in Koreans with primary acquired nasolacrimal duct obstruction (PANDO). METHODS: From March 2014 to March 2016, 40 unilateral PANDO patients and 40 control subjects were retrospectively reviewed. The axial, sagittal, and coronal planes of CT were used for image evaluation. The proximal, minimal, and distal transverse diameters (TDs) of the BNLD were assessed. The length, sagittal orientation angle of BNLD, relative lacrimal sac-BNLD angle, nasal floor-BNLD angle, and turbinate angle were investigated. In addition, the distance between the bilateral BNLD and inter-frontozygomatic suture distance were also measured. RESULTS: There were no morphologic differences between the PANDO and non-PANDO sides within PANDO patients. The proximal and minimum BNLD TD measurements were significantly narrower in the PANDO patients, as compared with the control group (p = 0.010 and p = 0.017, respectively). The lacrimal sac-BNLD angle, nasal floor-BNLD angle, and turbinate angle also exhibited statistically significant differences between the PANDO patients and the control group (p = 0.019, p = 0.001, and p < 0.001, respectively). CONCLUSIONS: Although this study was performed in a small group, the narrow proximal and minimum BNLD TD in PANDO patients (in both the PANDO and non-PANDO sides) may be associated with PANDO development in Koreans. Additionally, the narrow lacrimal sac-BNLD, nasal floor-BNLD, and turbinate angle might be possible causative factors of PANDO.