Uveitic glaucoma and Rosai-Dorfman disease (sinus histiocytosis)

PURPOSE: To report a novel association of uveitic glaucoma with Rosai-Dorfman disease. METHODS: Case report. RESULTS: A 67-year-old Caucasian woman presented with a chronic bilateral granulomatous uveitis which did not respond to conventional topical steroid therapy. She also had raised intraocular pressures, glaucomatous optic disc changes and diffuse nodular fibrous skin lesions. Subsequent skin biopsy immuno-cytochemistry showed S-100 staining, consistent with Rosai-Dorfman disease. The uveitis and glaucoma were highly resistant to standard medical treatments, but completely resolved together with the systemic features of the disease after six months. CONCLUSIONS: Rosai-Dorfman disease has not previously been reported to cause uveitic glaucoma and should be considered in non-responsive cases presenting with a rash. The disease is entirely self-limiting and early diagnosis may therefore avoid unnecessary trabeculectomy and/or systemic immune suppression.     

Racial Influences of Uveitic Glaucoma: Consolidation of Current Knowledge of Diagnosis and Treatment

ABSTRACT

Introduction: The etiology, frequency, manifestation, and treatment of uveitis and uveitic glaucoma are commonly influenced by race. Materials and Methods: This study aims to review the effect of race on the diagnosis and treatment of patients with common diseases of uveitis that may develop into glaucoma. Results: Race affects the presentation and prevalence of uveitis. Sarcoidosis, for example, is more common in Blacks than in Whites in the United States. Furthermore, Blacks more commonly have anterior segment involvement, while Whites more commonly have posterior segment involvement. In addition, treatments—such as steroid implants and trabeculectomy—in the setting of uveitis and uveitic glaucoma may have higher risk of failure in Black patients. Discussion: Over the last half-century, we have increased our understanding of the role of race in uveitis and uveitic glaucoma. Major gaps remain and further study is needed.     

Atypical Ocular Presentations of Rosai-Dorfman Disease

Purpose: To report atypical ocular findings of Rosai-Dorfman disease and to determine association with parvovirus 19. Design: The study is an observational case series of three patients that had atypical ocular presentations of Rosai-Dorfman disease. Methods: A multicenter, retrospective case series of 3 patients was evaluated for varied ocular complaints, including enlarging epibulbar masses and uveitis. Histologic specimens were examined retrospectively for parvovirus 19 antigen. Results: Patients presented with ocular findings prior to or concurrently with systemic findings, with and without associated lymphadenopathy. Two cases presented with epibulbar masses, one with the mass as the only sign of disease while the other exhibited multinodal involvement with bilateral epibulbar masses and anterior granulomatous inflammation. Parvovirus B19 antibody staining was negative in these cases. The final case exhibited bilateral anterior granulomatous inflammation and choroidal infiltrates. Conclusions: Ocular findings may be the sole or presenting complaint in Rosai-Dorfman disease prior to recognition of systemic disease and should be considered in the differential diagnosis of epibulbar masses as well as anterior and posterior granulomatous inflammation.     

Sclerouveitis Associated with Rosai-Dorfman Disease in an HIV-infected Patient

ABSTRACT

Purpose: To describe a case of Rosai-Dorfman-associated sclerouveitis in an HIV-infected patient.

Design: Case report.

Methods: A 49-year-old man with HIV had bilateral eye pain, redness, photophobia, and multiple systemic complaints. He underwent serial ocular examinations, abdomen/pelvis CT scan, and lymph node biopsy.

Results: Ophthalmologic evaluation revealed bilateral, nongranulomatous anterior uveitis and anterior scleritis. Biopsy of a retroperitoneal lymph node identified on CT revealed mixed inflammatory infiltrate composed of histiocytes, lymphocytes, and plasma cells involving fibroadipose tissue, indicating Rosai-Dorfman disease.

Conclusions: This is the first report of sclerouveitis in Rosai-Dorfman disease. Rosai-Dorfman is typically self-limiting, occurring rarely in HIV.     

Coding of glaucoma for patients with uveitis in the medicare database

PURPOSE: To investigate possible causes for, and implications of, variations in coding of glaucoma for patients with uveitis in the Medicare database. PATIENTS AND METHODS: We identified 6,391 patients coded with one of several ICD-9 codes designating a form of uveitis from a 5% sample of the 1999 Medicare database. The proportion of patients with uveitis codes who also had codes for uveitic glaucoma, primary open-angle glaucoma (POAG), or unspecified open-angle glaucoma (OAG) were determined. The proportion with codes for argon laser trabeculoplasty (ALT), trabeculectomy, visual field examinations, fundus photographs, and gonioscopy were calculated in three groups: patients with both uveitis and uveitic glaucoma; those with both uveitis and POAG; and those with both uveitis and unspecified open-angle glaucoma. Summaries of the total allowed reimbursement related to eye visits and to eye codes were determined. RESULTS: Of the 6,391 patients with uveitis who were studied, 1,260 (19.7%) also had the code for POAG; 412 (6.4%) had the code for OAG; and 117 (1.8%) had the code for uveitic glaucoma. Visual field testing was less common among patients with uveitic glaucoma (37%) than among those with POAG/uveitis (46%) or OAG/uveitis (44%) (P < 0.001). There were no statistically significant differences among the three groups in the amount of total reimbursement related to eye services or eye codes. CONCLUSIONS: The code for POAG appears to be used in lieu of uveitic glaucoma in many cases. Discrepancies are probably not influenced by expected reimbursements, as much as by a lack of understanding about the available ICD-9 codes.     

Pattern of Uveitic Hypotony in a Tertiary Eye Hospital in India

Purpose: To report pattern of uveitic patients with hypotony in Indian population. Methods: Retrospective medical record review of 30 eyes of 21 consecutive patients with uveitic hypotony. Patient demographics, ocular findings and systemic association were recorded. Results: Mean age of the patients was 29.5 years (range 7–71 years) and 80.9%(17 patients) were below the age of 40 years. Most common complaint associated with the development of hypotony was diminution of vision (26 eyes; 86.6%), and cells in anterior vitreous (25 eyes, 83.3%) were the most common slit-lamp finding. Most common type of ocular inflammation causing hypotony was panuveitis (14, 46.6%), and ocular tuberculosis (5; 23.8%) was the most common cause of uveitis in these patients. Conclusion: Ocular hypotony is a rare but dreaded complication of uveitis. The etiology of uveitic hypotony is not uniform across the world, and tuberculosis remains an important cause of uveitic hypotony in tuberculosis endemic country like India     

Fibrinous Anterior Uveitis Due to Cysticercus Cellulosae

Purpose: To report a case of fibrinous anterior uveitis due to cysticercus cellulosae in anterior chamber. Design: Retrospective, interventional case study. Methods: Patient underwent complete ophthalmic and systemic evaluation with relevant investigations. Viscoexpression of anterior chamber cysts were done under general anaesthesia. Results: A 10-year-old male child presented as unilateral fibrinous iridocylitis with secondary glaucoma. Two cysts of cysticercus cellulosae were seen after control of inflammation. Complete resolution of uveitis occurred once the cysts were removed by viscoexpression. Conclusion: Cysticercus cellulosae can present as fibrinous anterior uveitis with secondary glaucoma. Removal of the cyst can cause complete resolution of uveitis.     

Extensive chorioretinal atrophy in Vogt-Koyanagi-Harada disease.

PURPOSE: To report extensive chorioretinal atrophy during the long-term course of Vogt-Koyanagi-Harada (VKH) disease not treated properly in the initial phase. CASES: Four patients with VKH disease were examined more than 10 years after onset of the disease. OBSERVATIONS: They presented initially with classic features of VKH disease, except 1 patient who had developed bilateral, acute angle-closure glaucoma as the initial sign. Two patients received systemic corticosteroid therapy at the acute phase of the disease. During the follow-up of 13-34 years subsequent to onset, these patients had chronic recurrent anterior uveitis with apparently stable depigmented fundus. Eventually, they developed diffuse, extensive chorioretinal atrophy that resulted in severe visual loss. One patient had an unusual familial occurrence of the disease. CONCLUSIONS: Failure to prescribe proper corticosteroid therapy in the initial phase of VKH disease may lead to chronic recurrent uveitis. Long-standing uveitic reactions may eventually result in severe visual loss due to extensive chorioretinal degeneration.     

Metoprolol responding uveitis

BACKGROUND: Noninfectious uveitis is usually managed by topical and systemic corticosteroids and in refractory cases by immunosuppressive drugs. OBJECTIVE: To describe a patient with noninfectious anterior and posterior uveitis, refractory to corticosteroids, and immunosuppressive therapy, which responded to systemic metoprolol. PATIENT AND METHODS: A 49-year-old patient was treated for 3 years with topical and systemic corticosteroids and systemic cyclosporin A for a bilateral anterior and posterior uveitis of unknown origin. The treatment did not result in resolution of the uveitis. A bilateral uveitic glaucoma developed and necessitated neodymium : YAG laser iridotomies and antiglaucoma medications. A systemic beta-blocker, metoprolol tartrate 50 mg b.i.d., was administered for palpitations because of idiopatic paroxysmal supraventricular tachycardia and short ventricular tachycardia. RESULTS: Following administration of metoprolol tartrate, the bilateral uveitis resolved. The corticosteroids and the cyclosporin A were withdrawn after 6 weeks without any recurrence. A trial to discontinue metoprolol after 6 months resulted in flare-up of the disease and only following its readministration the inflammation resolved. The patient is currently under metoprolol for a year without flare-ups. CONCLUSIONS: The use of metoprolol tartrate in this patient resulted in resolution of bilateral noninfectious uveitis. This is the first report of non-antiinfectious, antiinflammatory, or immunosuppressive drug effective for uveitis. It is possible that a subgroup of resistant uveitis may respond to drugs other than the traditional drugs, such as metoprolol, and that other forms of uveitis of unidentified origin exist.     

Glaukom und Uveitis

Uveitic secondary glaucoma poses one of the most difficult problems for differential diagnostics and therapeutics in the field of clinical glaucomatology. The prevalence of glaucoma among uveitis patients ranges between 5 and 20% with great fluctuations depending on the underlying disease, duration of the disease, and the patient's age. Based on slit-lamp examination, uveitis can be classified as granulomatous and non-granulomatous. The effects of uveitic glaucoma on the quality of life and central visual acuity are particularly serious during childhood. Surgical reduction of eye pressure in cases of uveitic secondary glaucoma should only be performed if medical treatment of the underlying disease or ocular inflammation does not lower the eye pressure level and if in the presence of glaucomatous damage to the optic nerve the level of intraocular pressure is so high that a drastic decrease in pressure that cannot be achieved by medication becomes necessary.     

Long-term Follow-up of Patients with Uveitis Associated with Juvenile Idiopathic Arthritis: A Cohort Study

Purpose: To analyze the clinical and visual outcome of 55 consecutive patients with juvenile idiopathic arthritis (JIA)-associated uveitis examined 7 and 24 years after the onset of the eye disease. Methods: A retrospective study. Results: Fifty-five out of 350 patients with JIA developed uveitis (15.7%). Forty-six (84%) of these had oligoarthritis, 6 (11%) had polyarthritis, and 3(5%) had systemic disease. Cataract was present in 42% at 7 years and in 51% at 24 years. Uveitic glaucoma was present in 5% at 7 years and in 22% at 24 years. After 24 years, 27 patients (49%) still had active uveitis. Conclusions: In half of these patients the uveitis was still active after 24 years of eye disease. In the group of patients with active disease, cataract and secondary glaucoma increased significantly between years 7 and 24 of disease.     

Glaucoma and uveitis. Causes of and treatment options for increased intraocular pressure in cases of inflammatory ophthalmology

Uveitic secondary glaucoma poses one of the most difficult problems for differential diagnostics and therapeutics in the field of clinical glaucomatology. The prevalence of glaucoma among uveitis patients ranges between 5 and 20% with great fluctuations depending on the underlying disease, duration of the disease, and the patient's age. Based on slit-lamp examination, uveitis can be classified as granulomatous and non-granulomatous. The effects of uveitic glaucoma on the quality of life and central visual acuity are particularly serious during childhood. Surgical reduction of eye pressure in cases of uveitic secondary glaucoma should only be performed if medical treatment of the underlying disease or ocular inflammation does not lower the eye pressure level and if in the presence of glaucomatous damage to the optic nerve the level of intraocular pressure is so high that a drastic decrease in pressure that cannot be achieved by medication becomes necessary.     

Management of inflammatory glaucomas

PURPOSE OF REVIEW: To review the epidemiology, pathogenesis and management of uveitic glaucoma in the light of significant studies published during the review period (October 2002-2003). RECENT FINDINGS: The visual prognosis of glaucoma secondary to certain types of uveitis, for example Juvenile Idopathic Arthritis, remains poor because of late presentation and a high rate of corticosteroid responsiveness. Production of Myocilin is intimately linked with outflow resistance and corticosteroid responsiveness. Although Myocilin gene expression is widespread in the eye, corticosteroid-induction is specific for trabecular meshwork. The hypotensive effect of some glaucoma medications such as Latanoprost and Brimonidine may be partially blocked by concurrent administration of NSAIDs, though the risk of redcrudescence of uveitis with Latanoprost appears to be much lower than initially feared. Use of Mitomycin C in uveitic trabeculectomy has not clearly translated into better long-term IOP control, though this may be due to an absence of prospective studies. Glaucoma drainage devices appear to be more successful in uveitic glaucoma than in other recalcitrant types. SUMMARY: The management of uveitic glaucoma requires a careful balance between adequate anti-inflammatory therapy and appropriate intraocular pressure (IOP)-lowering to prevent long-term visual loss. In the foreseeable future, successful elucidation of the function of Myocilin is most likely to lead to improvements in the management of corticosteroid-induced and hence uveitic glaucoma.     

Somatostatin for Uveitic Cystoid Macular Edema (CME)

Purpose: To report the favorable response of bilateral recalcitrant uveitic cystoid macular edema (CME) to treatment with a somatostatin analog. Methods: Medical ophthalmic history and the results of ophthalmic examinations were recorded. Fluorescein angiography (FA) studies were reviewed. Results: A 52-year-old white female with intermediate uveitis developed bilateral recalcitrant CME. Treatment with subcutaneous injections of the somatostatin analog octreotide resulted in partial resolution of the CME and improvement of visual acuity. Conclusions: Somatostatin may play a role in the treatment of CME secondary to uveitis.     

Overcoming diagnostic and treatment challenges in uveitic glaucoma

Uveitic glaucoma is a range of disorders that results in optic nerve damage from elevated intraocular pressure secondary to intraocular inflammation. As compared to primary open angle glaucoma, uveitic glaucoma is associated with a more aggressive disease course caused by very high intraocular pressure levels that wax and wane. Diagnosis is often based on clinical presentation, disease course, and associated systemic manifestations. Diagnostic imaging plays an important role in both diagnosis and management. While the mechanisms of uveitic glaucoma vary, treatment requires strict control of the inflammation and may involve additional intraocular pressure lowering techniques. Management often dictates an interdisciplinary approach as systemic association and treatment is common. When topical management does not slow the progression of optic nerve damage and vision loss, surgical intervention is required. A significant portion of patients with uveitic glaucoma will eventually require surgical intervention and the appropriate referrals should be made. By nature, success rates of surgical intervention in uveitic glaucoma patients are lower than non‐inflammatory causes of elevated intraocular pressure and glaucomatous damage. Chronic inflammation, multiple mechanisms, systemic associations, and unpredictable response to treatment make uveitic glaucoma challenging to manage. This review will discuss the pathophysiology, diagnosis, and management of uveitic glaucoma to provide a guide for eye‐care providers.     

Atypical Rosai-Dorfman Disease with Lacrimal Gland Involvement

Abstract

Purpose: Rosai-Dorfman disease is a rare, benign, idiopathic histocytic proliferative disorder that typically presents in young adults with painless cervical lymphadenopathy. Here we report an atypical case of Rosai-Dorfman disease involving orbit tissue and lacrimal gland, unilaterally. Case: A 69-year-old Asian women developed a painless palpable mass with local edema over the left upper eyelid over several months. Computed tomography (CT) showed an orbital mass with homogenous soft tissue density over the left lacrimal gland and superior orbital area. The patient underwent complete excision of the orbital tumor. Observations: The histopathology revealed diffuse and nodular infiltrations of S-100 positive histiocytes, plasma cells and lymphocytes. Emperipolesis (lymphocytophagocytosis) was also noted. These findings were consistent with Rosai-Dorfman disease. Chest CT revealed hilar lymphadenopathy. Three months after excision of the orbital mass, the patient developed lymphadenopathy in the extremities that resolved spontaneously over a few weeks. There were no complications or recurrence without systemic treatment after the complete excision. Conclusions: Orbital Rosai-Dorfman disease is a rare disorder, especially in Asia. Though there is no consensus on therapeutic choices, including corticosteroids, chemotherapy, radiation therapy, and surgical excision, the complete surgical excision performed in this case was without complication and had a favorable outcome.     

Incidence and Risk Factors for Developing Glaucoma Among Patients with Uveitis in a University-based Tertiary Referral Center in Riyadh,Saudi Arabia

Purpose: To investigate the incidence and risk factors of secondary glaucoma among uveitis patients.

Methods: Retrospective review of medical records of 642 patients (1220 eyes).

Results: Glaucoma was diagnosed in 169 (13.9%) eyes and was most common in eyes with anterior uveitis (19.1%) (p?p?Conclusions: Incidence of glaucoma differed depending on anatomic and etiologic diagnoses of uveitis. There is a significant association between severity of inflammation at presentation and development of glaucoma.     

Efficacy and safety of deep sclerectomy in uveitic glaucoma

Purpose To evaluate the safety and efficacy of deep sclerectomy with implant and mitomycin C in uveitic glaucoma. Design Prospective, noncomparative case study. Patients and methods Nine patients (13 eyes) with uncontrolled uveitic glaucoma underwent deep sclerectomy with implant from 2002 to 2006. All patients had their uveitis controlled before and after surgery with anti-inflammatory therapy. Main outcome measures Control of intraocular pressure. A secondary outcome measure was the number of antiglaucoma medications required to achieve the desired intraocular pressure. Visual acuity and complication associated with the surgery were monitored. Results Mean follow-up was 21 months (range 12–54 months). Intraocular pressure (IOP) was reduced from a mean preoperative value of 28.7 mmHg to a mean postoperative value of 13.85 mmHg (Wilcoxon signed rank test P = 0.005). At the most recent visit, complete success was obtained in 84.6%, qualified success was obtained in 7.7%, and complete failure in 7.7%. Mean number of antiglaucoma medications was reduced from 3.07 to 0.2 (Wilcoxon signed rank test P = 0.001). Neodymium:YAG goniopuncture was performed in two eyes. Postoperative complications included transient hypotony with maculopathy in one eye, shallow choroidal effusions in two eyes, and progression of cataract in four eyes. Conclusion Deep sclerectomy with implant in uveitic glaucoma appeared to be effective in controlling the IOP at short-term follow-up with no serious postoperative side-effects.     

The Use of Prostaglandin Analogs in the Uveitic Patient

Glaucoma is a disease process characterized by progressive optic nerve damage and corresponding visual field loss. It may be further categorized into either primary open-angle glaucoma or secondary glaucoma. These secondary glaucomas include glaucomas associated with uveitis and inflammation. Prostaglandin analogs (PGA) have been used to help lower intraocular pressure (IOP) in these often difficult to manage eyes. However, controversy exists concerning their use in uveitic patients due to the theoretically higher risk of anterior uveitis, development of cystoid macular edema (CME), and reactivation of herpes simplex keratitis (HSK). There is little evidence that PGA disrupt the blood-aqueous barrier and only anecdotal evidence suggesting an increased risk of these rare findings. PGA may be used in uveitic glaucoma when other topical treatments have not lowered IOP to the patient’s target range.     

Interferon alpha 2b in the Treatment of Uveitic Cystoid Macular Edema

Purpose: To determine the efficacy of interferon alpha 2b in the treatment of refractory, uveitic cystoid macular edema (CME).

Methods: Retrospective chart review of 4 patients attending the uveitis clinic at the Casey Eye Institute, Oregon Health & Science University.

Results: All 4 patients had uveitis and refractory CME, resistant to a variety of immunosuppressants. All patients, except one with severe scleral thinning, had tried and failed therapy with locally injected corticosteroids. Treatment with systemic interferon alpha 2b produced dramatic improvement in CME (central macular thickness: 563 to 267?µm, p?=?.002) and visual acuity (logMAR: +0.81 to +0.45, p?=?.0004) in all 4 cases. All patients have been able to reduce the interferon dosage, but none has discontinued it completely. All patients had some mild adverse response that did not necessitate stopping therapy.

Conclusions: Interferon alpha 2b is an effective option to treat refractory CME secondary to uveitis.