Diagnosis, Differential ; Endoscopy ; Granuloma, Plasma Cell/diagnosis* ; Humans ; Orbital Pseudotumor ; Skull Base

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.
Biopsy ; Collagen ; Diagnosis ; Fibrosis ; Humans ; Immunoglobulin G ; Immunoglobulins* ; Lacrimal Apparatus ; Lymphocytes ; Medical Records ; Orbit* ; Orbital Pseudotumor* ; Phlebitis ; Plasma Cells ; Recurrence ; Retrospective Studies

Orbital compartment syndrome (OCS) is a visual threatening ocular emergency. We report a 50-year-old male with acute presentation of OCS, a rare manifestation of idiopathic orbital inflammatory disease. At presentation, high intraocular pressure was reduced by prompt lateral canthotomy and cantholysis. The disease responded to systemic steroids and treatment resulted in good visual outcome. Detail evaluation and early detection and treatment are mandatory to prevent permanent vision loss.
Orbital Pseudotumor

PURPOSE: To report an unusual case of mucosa-associated lymphoid tissue (MALT) lymphoma localized to the left inferior rectus muscle. CASE SUMMARY: A 52-year-old male presented with double vision 6 months in duration, which was exacerbated in the down-gaze. On initial examination, 6 and 30 prism diopters (PDs) of left hypertropia were observed in primary gaze and down gaze, respectively. Prominently limited infraduction was also observed in his left eye. Computed tomography (CT) revealed contrast enhancing mass on the left inferior rectus muscle. He was diagnosed with suspicious orbital pseudotumor and treated with systemic steroid for 2 months. Double vision and limited infraduction was improved and the mass size was decreased on CT. After 21 months, the patient revisited the clinic with the same symptoms. In the down-gaze, 35 PDs of hypertropia were observed in his left eye along with limited infraduction. CT revealed an enlarged mass and left superior rectus muscle recession along with an incisional biopsy of the left inferior rectus muscle were performed. Infiltration by small lymphoid cells was detected with hematoxylin-eosin and immunohistochemical stainings. Subsequently, the patient was diagnosed with MALT lymphoma localized to the left inferior rectus muscle. CONCLUSIONS: This is the first description of MALT lymphoma with inferior rectus muscle invasion in a Korean patient. In a patient with strabismus and limited duction, the possibility of extraocular muscle tumor including lymphoma should be considered. Homogenously contrast-enhanced mass on orbit CT can help in to make a diagnosing extraocular muscle lymphoma.
Biopsy ; Diplopia ; Humans ; Lymphocytes ; Lymphoid Tissue ; Lymphoma ; Lymphoma, B-Cell, Marginal Zone* ; Male ; Middle Aged ; Orbit ; Orbital Pseudotumor ; Strabismus

A 2-year-old girl presented with a one-day history of acute-onset bilateral painful, swollen eyes and a two‑month history of loose stools. Physical examination revealed a right eyelid swelling with proptosis. Magnetic resonance imaging revealed a right orbital pseudotumour. The patient responded well to treatment with intravenous antibiotics and nonsteroidal anti-inflammatory drugs. However, three weeks later, she was readmitted with a vasculitic lesion over her left upper chest, with mucous-bloody diarrhoea. Histopathology confirmed the diagnosis of ulcerative colitis. The patient was treated with intravenous pulse methylprednisolone and sulphasalazine. Two weeks after discharge, she was readmitted for cutaneous vasculitis and worsening diarrhoea. The patient's bowel and extraintestinal diseases resolved upon addition of infliximab to her treatment regimen. Her inflammatory markers also normalised. Azathioprine was subsequently added. Infliximab was discontinued after four doses and prednisolone was tapered off. The patient remained well without any flare-up after 24 months of follow-up.
Azathioprine ; therapeutic use ; Child, Preschool ; Colitis, Ulcerative ; diagnosis ; drug therapy ; Drug Therapy, Combination ; Female ; Gastrointestinal Agents ; therapeutic use ; Humans ; Immunosuppressive Agents ; therapeutic use ; Infliximab ; therapeutic use ; Magnetic Resonance Imaging ; Orbital Pseudotumor ; diagnosis ; drug therapy

No abstract available.
Blepharoptosis ; Myositis* ; Orbital Myositis ; Orbital Pseudotumor

PURPOSE: To evaluate the clinical features and treatment outcomes of steroid therapy for orbital inflammatory pseudotumor. METHODS: Sixty-four patients diagnosed with orbital inflammatory pseudotumor were reviewed retrospectively. Patients with a follow-up period of less than 6 months were excluded from the study. The pseudotumor was classified into myositic, lacrimal, anterior, diffuse, or apical type according to orbital computed tomography findings. All patients were initially treated with systemic corticosteroids and evaluated for response to the treatment. Treatment outcome was considered a "success" if the patient had complete relief of symptoms with no recurrence, and a "failure" if the patient had no or only partial relief of symptoms or showed relapse. Factors affecting the treatment outcome were analyzed. RESULTS: The most frequent lesion subtype was myositis. Periorbital edema was the most common symptom and was evident in 53.1% of the patients. Thirty-eight patients (59.4%) showed treatment success. Age, sex, bilaterality, and mean follow-up length did not correlate with the treatment outcome. A short interval from symptom onset to treatment time and apical subtype were significantly associated with good steroid response (p < 0.05). CONCLUSIONS: In orbital inflammatory pseudotumor, myositis was the most common subtype. A short interval from symptom onset to treatment time and apical subtype were associated with good steroid response.
Adrenal Cortex Hormones ; Edema ; Follow-Up Studies ; Humans ; Myositis ; Orbit ; Orbital Pseudotumor ; Recurrence ; Retrospective Studies ; Treatment Outcome

PURPOSE: To describe the clinical manifestations, radiologic findings, and treatment outcomes of pediatric pseudotumors. METHODS: A retrospective chart review of patients diagnosed with pediatric pseudotumor (age under 20 years old) from August 2008 to February 2012 was performed. RESULTS: Thirteen patients (16 eyes) were included in this study. The mean age of the subjects was 14.2 years (5-20 years). Swollen eyelid (56.3%), ptosis (43.8%), conjunctival injection (18.8%), localized mass (18.8%), limitation of ocular movement (12.5%), proptosis (6.3%), and decreased visual acuity (6.3%) were found initially. Dacryoadenitis (62.5%), myositis (18.8%), anterior orbital inflammation (18.8%), and diffuse type (6.3%) were observed on orbital computed tomography (CT). Among the 13 patients (16 eyes), 8 patients (10 eyes) were administered oral systemic corticosteroids, 2 patients (2 eyes) received IV systemic corticosteroid, 1 patient (1 eye) received systemic corticosteroids combined with NSAID, and 2 patients (3 eyes) were prescribed NSAIDs only. Symptoms improved 4.1 days after initiation of treatment. CT scans revealed that one patient experienced diffuse-type disease recurrence twice. CONCLUSIONS: Orbital pseudotumors, which are rare in younger people, are likely to respond very well to corticosteroid treatment, and the recurrence of orbital inflammation is rare.
Adrenal Cortex Hormones ; Anti-Inflammatory Agents, Non-Steroidal ; Dacryocystitis ; Exophthalmos ; Eyelids ; Humans ; Inflammation ; Korea ; Myositis ; Orbit ; Orbital Pseudotumor ; Recurrence ; Retrospective Studies ; Visual Acuity

PURPOSE: To assess the efficacy of levator recession under local anesthesia to treat upper eyelid retraction. METHODS: Records of 12 patients (12 lids) were reviewed retrospectively. Postoperative cosmetic results were assessed as good, fair, or poor based on the upper lid height and symmetry. Preoperative and postoperative marginal reflex distance (MRD1, mm), upper eyelid asymmetry, lid lag, lagophthalmos, and ocular exposure symptoms were compared. The reoperation rate was also evaluated. RESULTS: Causes accounting for upper lid retraction were Graves ophthalmopathy (9 lids, 75.0%), orbital pseudotumor (2 lids, 16.7%), and hypercorrection from previous ptosis operation (1 lid, 8.3%). At a mean +/- standard deviation of 27.7 +/- 24.0 months follow-up (range, 5-60 months), 11 patients (91.7%) showed significantly better cosmetic results. MRD1 decreased an average of 3.1 +/- 1.3 mm from 6.3 +/- 1.5 mm preoperatively to 3.2 +/- 0.9 mm postoperatively (p < 0.001). Upper lid asymmetry, lagophthalmos, and lid lag were also reduced significantly (p < 0.001). Overcorrection occurred in 3 lids (25%) and required levator advancement. Eleven patients (91.7%) experienced complete resolution of dry eye symptoms following levator recession. CONCLUSIONS: Levator recession showed good cosmetic results up to 2 years after surgery for upper eyelid retraction.
Accounting ; Anesthesia, Local ; Cosmetics ; Eye ; Eyelids ; Follow-Up Studies ; Graves Ophthalmopathy ; Humans ; Orbital Pseudotumor ; Reflex ; Reoperation ; Retrospective Studies

BACKGROUND: An orbital pseudotumor typically presents with periorbital pain, cranial nerve palsies and proptosis. Although visual deterioration is not unexpected in this pathology, its presentation solely with visual loss is unusual. CASE REPORT: In this short report, we summarize a case of orbital pseudotumor which presented solely with a decrease in visual acuity, and discuss the clinical and radiological findings. CONCLUSIONS: This atypical presentation likely resulted from the orbital pseudotumor originating in the optic foramen, leaving the neurovascular structures of the superior orbital fissure untouched initially. In the early clinical period, an orbital pseudotumor may manifest itself solely by visual loss. It should therefore be included in the differential diagnosis of visual pathologies-even in the absence of orbital pain and symptoms related to ocular movements.
Diagnosis, Differential ; Exophthalmos ; Headache ; Orbit ; Orbital Pseudotumor ; Paralysis ; Visual Acuity