Corneal topography with upper eyelid platinum chain implantation using the pretarsal fixation technique.

PURPOSE: To determine the effect of upper eyelid platinum chain implantation, with the pretarsal fixation technique, on corneal astigmatism. METHODS: This is a prospective, cohort study. Fifteen eyes of 15 patients underwent upper eyelid platinum chain implantation, with the pretarsal fixation technique, for facial nerve palsy. Information recorded included patient demographics, etiology for facial palsy, weight of the implant, time from onset of paresis to upper eyelid platinum chain implantation, associated surgical procedures, and preoperative and postoperative corneal topography measurements. RESULTS: Of the 15 patients studied, 10 were male and five were female. The mean age was 55.9 ± 13.8 years (range, 33-87 years). The most common etiology for facial palsy was acoustic neuroma. The weight of the implant ranged from 0.6 to 1.6gr (median 1.2gr). The time from onset of paresis to upper eyelid platinum chain implantation varied from 1 week to 3 months (median 1 month). Four patients had an associated procedure to correct the effect of paralytic ectropion. There was no statistically significant difference in with the rule astigmatism before and after platinum chain implantation. CONCLUSIONS: Upper eyelid platinum chain implantation, with the pretarsal fixation technique, does not appear to cause significant change in corneal astigmatism. This is contrary to data for pretarsal gold weight implantation, which does induce significant with the rule astigmatism.

Pharmacokinetics of intravitreal bevacizumab (Avastin®) in rabbits.

PURPOSE: To describe the pharmacokinetics of intravitreal bevacizumab (Avastin®) in rabbits. METHODS: The right eye of 20 rabbits was injected intravitreally with 1.25 mg/0.05 mL bevacizumab. Both eyes of four rabbits each time were enucleated at days 1, 3, 8, 15, and 29. Bevacizumab concentrations were measured in serum, aqueous humor, and vitreous. RESULTS: Maximum vitreous (406.25 µg/mL) and aqueous humor (5.83 µg/mL) concentrations of bevacizumab in the right eye were measured at day 1. Serum bevacizumab concentration peaked at day 8 (0.413 µg/mL) and declined to 0.032 µg/mL at 4 weeks. Half-life values in right vitreous, right aqueous humor, and serum were 6.61, 6.51, and 5.87 days, respectively. Concentration of bevacizumab in the vitreous of the noninjected eye peaked at day 8 (0.335 ng/mL) and declined to 0.218 ng/mL at 4 weeks. In the aqueous humor of the noninjected eye, maximum concentration of bevacizumab was achieved at day 8 (1.6125 ng/mL) and declined (to 0.11 ng/mL) at 4 weeks. CONCLUSION: The vitreous half-life of 1.25 mg/0.05 mL intravitreal bevacizumab was 6.61 days in this rabbit model. Maximum concentrations of bevacizumab were reached at day 1 in both vitreous and aqueous humor of the right eye and at day 8 in the serum. Very low concentrations of bevacizumab were measured in the fellow noninjected eye.

Choroidal neovascularization due to punctate inner choroidopathy: long-term follow-up and review of literature.

INTRODUCTION: The aim of the article was to report on the long-term follow-up of choroidal neovascularization (CNV) cases secondary to punctate inner choroidopathy (PIC) either treated with photodynamic therapy (PDT) or followed without treatment. A comprehensive review of existing literature on the various treatment modalities is incorporated. METHODS: Nine eyes of 8 female patients with CNV due to PIC were followed retrospectively for an average of 105 months (range, 36-162 months). Mean age of the patient cohort on presentation was 28 years (range, 21-39). Four eyes were treated with PDT, whereas in 4 patients, including 1 with bilateral involvement, the disease followed its natural course without treatment. Snellen visual acuity and the extent of neovascularization and subretinal fibrosis were evaluated on presentation and at the end of the follow-up period. RESULTS: Improvement of vision was observed in 6 eyes (66.7%), including all cases treated with PDT, and in 2 of 5 eyes left untreated. The size of the neovascular lesion, including both CNV and subretinal fibrosis, increased in all cases left untreated (55.6%) and remained stable in all cases treated with PDT (44.4%). CONCLUSION: Without treatment, the CNV due to PIC is slowly progressive. Our short cohort appears to have benefited from PDT in terms of maintaining visual acuity and stabilizing the extent of CNV and fibrosis.

Ranibizumab for diabetic macular edema difficult to treat with focal/grid laser.

BACKGROUND: To evaluate the efficacy of intravitreal injections of ranibizumab in patients with diabetic clinically significant macular edema (CSME), when further focal or grid laser was considered to be unsafe. METHODS: In this retrospective, interventional case study, intravitreal injections of ranibizumab were performed in 16 eyes (ten patients) suffering from diabetic retinopathy with CSME. All patients had been treated in the past with focal or grid laser. Additional photocoagulation could not be performed because the leaking points were very close to the avascular zone, and there was also a perifoveal capillary dropout. The patients underwent three injections (months 0, 1 and 2) and were followed monthly. Reinjection was performed if central retinal thickness (CRT) was ≥250 µm associated with fluorescein leakage involving the center of the macula. RESULTS: The patients underwent a median of seven injections (range 6-9) and the median follow-up time was 11 months (range 9-15). The median best-corrected visual acuity (BCVA) was 0.85 logMAR at baseline and 0.54 logMAR at the end of the follow-up time (p = 0.018). BCVA improved in seven eyes (43.75%), remained stable in eight (50%) and decreased in one eye (6.25%). The median CRT decreased from 409.5 µm at baseline to 272 µm at the end of the follow-up time (p = 0.0002). No ocular or systemic adverse events were reported. CONCLUSIONS: For a median follow-up time of 11 months, the treatment with intravitreal injections of ranibizumab resulted in stabilization or improvement of the visual acuity in the vast majority of patients with diabetic maculopathy and CSME, when further focal or grid laser was considered to be unsafe.

Central retinal vein occlusion secondary to clomiphene treatment in a male carrier of factor V Leiden.

We report a case of a 35-year-old previously healthy man treated with clomiphene for infertility, who presented with blurred vision in his left eye due to ocular vein occlusion as documented by fluorescein angiography. The patient was heterozygous for the factor V Leiden (FV Leiden) mutation and for the 1298 A-C polymorphism of the methylene-tetrahydrofolate reductase (MTHFR) gene. He was treated with clopidogrel and is now free of symptoms. Because congenital thrombophilia is a moderate risk factor for central retinal vein occlusion and the administration of clomiphene may trigger this process, we recommend screening of young patients for FV Leiden before clomiphene treatment.

Bevacizumab (Avastin) for the management of anterior chamber neovascularization and neovascular glaucoma.

PURPOSE: To establish the efficacy and safety of intravitreal bevacizumab in reducing iris and anterior chamber angle neovascularization and managing neovascular glaucoma. DESIGN: Prospective interventional case series. PATIENT AND METHODS: Eleven eyes of 11 patients with iris and anterior chamber angle neovascularization with refractory intraocular pressure were treated with intravitreal injection of 1.25 mg bevacizumab (Avastin((R))). The study group included eight males and three females aged 23 to 77 years (average, 62 years). Out of the 11 cases, five had proliferative diabetic retinopathy, of whom two had undergone vitrectomy for tractional retinal detachment and vitreous hemorrhage, and six were secondary to ischemic central retinal vein occlusion (CRVO). All patients were followed for eight to 16 months (average, 10 months). RESULTS: Iris and anterior chamber angle neovascularization receded in all eyes after one to three injections at monthly intervals. In five eyes, neovascularization recurred during the follow-up period. The intraocular pressure normalized in one eye. Four eyes were controlled with anti-glaucoma drops. A cyclodestructive procedure was required in two eyes. An Ahmet drainage valve was implanted in four eyes, including one controlled with additional antiglaucoma drops and one in which the intraocular pressure remained high while on maximum antiglaucoma medication and a cyclodestructive procedure was scheduled. CONCLUSIONS: Bevacizumab appears to be effective in reducing iris and anterior chamber angle neovascularization and is likely to extend our therapeutic options in the management of neovascular glaucoma.

Orbital cellulitis, orbital subperiosteal and intraorbital abscess: report of three cases and review of the literature.

INTRODUCTION: Orbital cellulitis is usually a complication of paranasal sinus infection. Either the infection may dissect under the periosteum and lead to subperiosteal abscess (SPA) or intraorbital abscess may be formed secondary to a progressive and localized cellulitis. Without appropriate treatment orbital infection may lead to serious complications, even death. REPORT OF CASES: Three cases are described, one of orbital cellulitis, one of SPA and one of intraorbital abscess and the literature is being reviewed. CONCLUSION: Prompt treatment is mandatory to avoid visual loss or intracranial complications. Initially, IV antibiotics may be administered, but if no improvement appears within 48h, surgical drainage of the orbit and the affected sinuses must be performed. In medial or medial-inferior SPA a transnasal approach is used, but in superior orbital abscess an external incision is required.

Sclerokeratitis and facial skin lesions: a case report of pyoderma gangrenosum and its response to dapsone therapy.

PURPOSE: This documents the evaluation and management of a patient with an unusual destructive external ocular disorder in the context of progressively disfiguring dermatitis. METHODS: Observational case report including ophthalmologic examination with conjunctival and corneal scraping, radiologic studies of the sinuses and chest, skin, conjunctival, and scleral biopsies, and serologic studies. RESULTS: Biopsies, cultures, and serologic testing led to the conclusion that the patient had pyoderma gangrenosum. Therapy with dapsone resulted in preservation of vision and resolution of the oculocutaneous inflammation. CONCLUSIONS: Pyoderma gangrenosum is a rare autoimmune disorder that can affect the eye. Biopsy of affected tissue is the key to diagnosis. Immunomodulating therapy can be a useful adjunct to the traditional steroid therapy.

Mooren ulcer: an immunopathologic study.

PURPOSE: To determine the pattern and distribution of mononuclear cells, adhesion, and co-stimulatory molecules in the conjunctiva of patients with Mooren ulcer. METHODS: Conjunctival biopsy specimens were obtained from 6 patients with Mooren ulcer and 6 healthy individuals. Immunohistochemistry was performed on frozen sections of the cryopreserved human conjunctivas using monoclonal antibodies directed against CD1alpha, CD3, CD4, CD8, CD20, CD25, CD57, and CD68 cells; the adhesion molecules E-selectin, vascular cell adhesion molecule-1 (VCAM-1), very late activation-4 (VLA-4), ICAM-1, and LFA-1; and the co-stimulatory molecules CD28, B7-1, B7-2, and CTLA-4. RESULTS: Differences in expression on the conjunctival epithelium from patients with Mooren ulcer and normal subjects were noted only for VCAM-1, VLA-4, ICAM-1, and LFA-1. The ratio of CD4+/CD8+ cells in Mooren ulcer specimens was significantly higher (3.5-fold). However, in the substantia propria, Mooren ulcer specimens revealed significantly increased numbers of CD1alpha+, CD3+, CD4+, CD20+, CD28+, B7-1+, B7-2+, and CD68+ cells. The ratios of CD4+/CD8+ cells and B7-2+/antigen-presenting cells in Mooren ulcer specimens were significantly higher (5-fold). All tested adhesion molecules showed significant up-regulation in the patients' conjunctivas. Mooren ulcer vascular endothelial cells prominently expressed E-selectin, VCAM-1, VLA-4, and ICAM-1 compared with normal conjunctiva. CONCLUSION: The simultaneous presence of multiple types of inflammatory cells, adhesion, and co-stimulatory molecules in Mooren ulcer conjunctiva suggests that their interaction may contribute to a sustained immune activation as at least part of the pathogenic mechanism of this disorder.

Obliterative microangiopathy presenting as chronic conjunctivitis in a patient with relapsing polychondritis.

PURPOSE: To report histopathologic findings and treatment of obliterative microangiopathy in a patient with chronic conjunctivitis due to relapsing polychondritis (RP). METHODS: This interventional case report describes a 50-year-old woman with relapsing polychondritis was referred to the Ocular Immunology and Uveitis Service for bilateral tearing due to refractory chronic conjunctivitis. Ocular examination revealed the presence of bilateral follicular conjunctivitis. Conjunctival biopsy of the inferior palpebral conjunctiva was performed, and the histopathologic findings guided the therapeutic intervention. RESULTS: The pathology of the conjunctival biopsy included granulomatous obliterative microangiopathy with numerous eosinophils, plasma cells, lymphocytes, and epithelioid cells in the substantia propia. The chronic conjunctivitis resolved with systemic methotrexate therapy. CONCLUSIONS: Microangiopathy may cause chronic conjunctivitis in patients with RP and may be a harbinger of evolving nonocular problems as a consequence of incomplete control of this autoimmune disorder. Immunomodulatory therapy should be considered in such cases to prevent possible cardiovascular, renal, respiratory, and neurologic complications of vasculitis. Although methotrexate has been used in treatment of RP-related necrotizing scleritis with poor results, it can be sufficient for the conjunctivitis with microangiopathy associated with RP.

Eosinophil activation in Wegener's granulomatosis: a harbinger of disease progression?

PURPOSE: To investigate the relation between eosinophil activation in tissue from patients with an active, limited form of Wegener's granulomatosis (WG) affecting the eye and subsequent systemic disease activity. METHODS: Analysis of ocular specimens obtained from 10 patients was performed. Sections were probed with antibodies to assess the presence of major basic protein (MBP) and eosinophil cationic protein (ECP). RESULTS: Four of the 10 specimens demonstrated the presence of MBP and ECP. WG progressed to the complete form in two of these patients, who received no or inadequate treatment. The other two, treated with cyclophosphamide for one year, did not progress to the complete form of WG during observation after therapy. CONCLUSIONS: Activated eosinophils in sclera or conjunctiva of patients with ocular limited WG may predict progression to complete WG.

Outcomes of treatment with immunomodulatory therapy in patients with corticosteroid-resistant juvenile idiopathic arthritis-associated chronic iridocyclitis.

PURPOSE: To describe the clinical outcome of patients with juvenile idiopathic arthritis (IA)-associated chronic iridocyclitis unresponsive to conventional therapy, in whom inflammation was eventually controlled using immunomodulatory therapy (IMT), and to determine if patients treated early with IMT have better visual acuity outcomes than those treated with corticosteroid alone. METHODS: Patients with JIA-associated chronic iridocyclitis receiving immunomodulatory therapy at the Ocular Immunology and Uveitis Service of the Massachusetts Eye and Ear Infirmary between 1981 and 2001 were studied. INCLUSION CRITERIA: JIA with chronic corticosteroid-dependent uveitis; IMT for a minimum of one year; minimum follow-up of three years; inflammation eventually controlled for a minimum of six consecutive months by IMT. RESULTS: At last visit, 51% of eyes (23 eyes) had acuity of 20/20 to 20/40, 16% (7 eyes) had 20/50 to 20/100, and 33% (15 eyes) had 20/200 or less. In patients whose chronic inflammation was eventually controlled within three years from onset of the uveitis, final bilateral vision was maintained within 20/20 to 20/30, except for a patient whose one eye had poor vision at initial consultation. When acuities at the end of the follow-up period of patients treated early with IMT were compared with acuities at the initial consultation of patients treated late with IMT, with duration of uveitis matched for each patient, visual acuities of those treated early were statistically significantly better than those treated late with IMT (p < 0.005 right and left eyes pooled; p = 0.0075 best eyes; p = 0.0375 worst eyes). CONCLUSIONS: We noted improvement or maintenance of visual acuity (86%) during the course of follow-up of patients with treatment-resistant JIA-associated uveitis treated with effective IMT. However, only IMT given early in the disease course was noted to be associated with bilateral visual acuity of 20/30 or better.

Ocular cicatricial pemphigoid, keratomycosis, and intravenous immunoglobulin therapy.

PURPOSE: To report the case of a patient developing fungal keratitis in the context of uncontrolled ocular cicatricial pemphigoid (OCP), which, despite intravenous immunoglobulin (IVIg) and other immunomodulatory therapy, progressed to end-stage pemphigoid, with corneal opacification, ankyloblepharon, and xerosis. Keratoprosthesis (KPro) restored functional vision for the patient. METHODS: A 39-year-old man presented with uncontrolled CP and corneal ulcer in the left eye. Conjunctival biopsy diagnosed OCP; corneal scraping and biopsy diagnosed the cause of the corneal ulcer. OCP was treated with systemic steroids, immunosuppressive drugs, and IVIg. Visual rehabilitation was accomplished with Ahmed valve and a type II Dohlman KPro. RESULTS: Immunohistology of the biopsied conjunctiva showed IgG at the epithelial basement membrane zone, confirming the clinical diagnosis of OCP. Microbiologic studies of the corneal biopsy specimen were negative for Acanthamoeba and herpes but positive for Aspergillus niger. The patient's keratomycosis resolved with topical antifungal therapy. Treatment with Dapsone, intravenous-pulse steroid, oral cyclophosphamide, and intravenous immunoglobulin (IVIg) failed to control the OCP, with resultant complete conjunctivization of the cornea. Keratoprosthesis improved the patient's visual acuity from hand movements to 20/20. CONCLUSIONS: Patients with uncontrolled OCP are at increased risk of corneal infection. The difficulty in diagnosing keratomycosis and the relatively rare occurrence of OCP explain the uniqueness of our reported case. OCP may progress to "end-stage" disease despite therapy. Keratoprosthesis can restore vision in selected otherwise seemingly hopeless cases.

Ocular manifestations and concepts of systemic vasculitides.

Vasculitic disorders are relatively rare. Their etiology and pathophysiology remain enigmatic, leading to confusing nomenclature and multiple classification schemes. Untreated vasculitis can be fatal. Early diagnosis is the key to successful treatment and better prognosis. However, early diagnosis can be difficult; vasculitic conditions usually present with non-specific symptoms for a long period before clinically overt manifestations occur. Ophthalmologists should be familiar with the ocular manifestations of the vasculitic disorders because they may not only be sight-threatening, but more importantly could be the presenting manifestations of active, potentially lethal systemic disease. This review summarizes clinical and ocular manifestations of systemic vasculitic disorders. Furthermore, it discusses general concepts in diagnosis and treatment of these diseases in an effort to provide a practical framework for the ophthalmologist evaluating patients with vasculitis.

Human mast cell subtypes in conjunctiva of patients with atopic keratoconjunctivitis, ocular cicatricial pemphigoid and Stevens-Johnson syndrome.

PURPOSE: Investigate mast cell (MC(S)) subtypes in atopic keratoconjunctivitis (AKC), ocular cicatrical pemphigoid (OCP), and Stevens-Johnson Syndrome (SJS). METHODS: MC(S) subtypes were determined by immunohistochemistry of conjunctiva (obtained from 34 patients--9 AKC, 9 OCP, 9 SJS and 7 normal) using monoclonal antibodies directed against chymase (MC(C)) and tryptase (MC(T)). Double staining was used to distinguish MC(S) as positive for both chymase and tryptase (MC(TC)). RESULTS: The number of MC(S) was significantly increased in AKC, OCP and SJS patients, compared to normal subjects. MC( C) were especially high in AKC, and moderately high in OCP. MC(T ) and MC(TC) were similar in each disease group. CONCLUSIONS: While the AKC findings were not surprising, the result in OCP and SJS suggests that MC(S) play an underappreciated role in the inflammatory process of these disorders. Disparate proportions of MC(S) subtypes in these diseases may imply differential functions of MC(S) in these disorders.

Long-term progression, prognosis, and treatment of patients with recurrent ocular manifestations of Reiter's syndrome.

PURPOSE: To investigate the spectrum of ocular involvement, to examine the clinical outcome, and to analyze the influence of treatment in patients with chronic ocular manifestations of Reiter's syndrome (RS) referred to a tertiary care ocular immunology service. DESIGN: Retrospective, noncomparative, interventional case series. PARTICIPANTS: Twenty-five patients with RS evaluated at the Ocular Immunology and Uveitis Service of the Massachusetts Eye and Ear Infirmary from 1981 through 2001. METHODS: Charts of patients were reviewed and data on age, gender, follow-up time, ocular symptoms, extraocular involvement, ocular complications, therapy, and visual acuities were recorded. MAIN OUTCOME MEASURES: Visual acuity, ocular complications, disease progression, clinical outcome, and systemic treatment. RESULTS: Twenty-five patients (20 male and 5 female) diagnosed with RS, with a mean age at presentation to our service of 37 years, were studied. The mean follow-up was 48.5 months. Eighty-five percent of patients tested were positive for human leukocyte antigen B27. Sixty-four percent of patients had a positive family history. All patients had oligoarthritis and enthesitis, most commonly affecting the back (56%), Achilles tendon (52%), and sacroiliac joint (24%). Eighty percent had a history of infection, most frequently urethritis (68%). Forty-four percent had a history of mucocutaneous lesions. All patients demonstrated ocular involvement at the time of diagnosis (68% with unilateral and 32% with bilateral disease), 84% had evidence of uveitis, 3% had scleritis, 2% had conjunctivitis, and 1% had pars planitis and iridocyclitis. During follow-up, the ocular complications included conjunctivitis (96%), anterior uveitis (92%), posterior uveitis (64%), keratitis (64%), cataract (56%), intermediate uveitis (40%), scleritis (28%), cystoid macular edema (28%), papillitis (16%), and glaucoma (16%). Systemic treatment for ocular inflammation was initiated in all patients. Ninety-six percent were treated with nonsteroidal anti-inflammatory agents. Eighty-eight percent were treated with corticosteroids, 64% requiring systemic prednisone. Immunosuppressive therapy was initiated in 52% of patients, with all receiving methotrexate. Seven patients required more than one immunosuppressive agent. The mean initial visual acuity was 20/25 in the right eye and 20/30 in the left eye. The mean final visual acuity was 20/25 in the right eye and 20/25 in the left eye. CONCLUSIONS: Reiter's syndrome may be associated with chronic recurrent ocular inflammation. Systemic therapy (including immunosuppressive treatment) typically is required to control the ocular inflammation and to prevent progressive visual loss.

Mycophenolate mofetil as an immunomodulatory agent in the treatment of chronic ocular inflammatory disorders.

PURPOSE: To evaluate the outcomes of patients with chronic ocular inflammatory disease treated with mycophenolate mofetil as an immunosuppressive and steroid-sparing agent. DESIGN: Retrospective noncomparative interventional case series. PARTICIPANTS: All patients with ocular inflammatory disease treated with mycophenolate mofetil at a single institution between 1998 and 2001. METHODS: Charts of patients seen on the Ocular Immunology and Uveitis Service at the Massachusetts Eye and Ear Infirmary were reviewed. Patients with chronic ocular inflammatory disease were included in the study. MAIN OUTCOME MEASURES: Control of inflammation, steroid-sparing effect, visual acuity, and adverse reactions were measured. RESULTS: A total of 54 patients were evaluated. Control of ocular inflammation with mycophenolate mofetil as monotherapy was achieved in 35 patients (65%) and in 67 eyes (62%), and a steroid-sparing effect was achieved in 29 (54%) patients. Visual acuity was maintained or improved in 51 patients (94%) and in 97 eyes (90%). Side effects requiring discontinuation of medication occurred in 10 patients (18%). There was neither long-term morbidity nor mortality due to mycophenolate mofetil. CONCLUSIONS: Mycophenolate mofetil is effective in the treatment of patients with steroid-dependent or -resistant chronic ocular inflammatory disorders that fail to respond to conventional steroid treatment. It is a safe and effective steroid-sparing immunomodulatory agent and can be considered an important addition to our armamentarium in the care of patients with ocular inflammatory disease.

Efficacy of etanercept in preventing relapse of uveitis controlled by methotrexate.

OBJECTIVE: To evaluate the efficacy of etanercept vs placebo in preventing relapses of uveitis in patients taking methotrexate with control of uveitis and whose methotrexate dosage was being tapered. METHODS: Patients with chronic or recurrent noninfectious uveitis with inflammation controlled by low-dose methotrexate were randomized to either the drug or placebo group in a double-masked manner, given a methotrexate taper schedule, and followed for 24 weeks. The main outcome measures were control of inflammation, visual acuity, and adverse reactions. Data were analyzed both as an attempt-to-treat analysis and an analysis only of those patients who completed the study. RESULTS: A total of 20 patients were randomized to the drug and placebo groups. Relapse of uveitis occurred in 3 of 10 patients in the treatment group and 5 of 10 patients in the control group. Two patients in the treatment group withdrew prematurely from the study due to adverse effects. There was no significant difference between the treatment and placebo groups with regard to the rate of relapse and the final visual acuity. No patient suffered from any irreversible, long-term morbidity or mortality. CONCLUSION: Etanercept has no significant efficacy over placebo in preventing relapses of uveitis in patients being tapered from methotrexate.

Sectorial keratitis and uveitis: differential diagnosis.

PURPOSE: The purpose of this study was to examine the importance of considering the differential diagnosis for patients with sectorial keratitis and uveitis by case summary and literature review. METHODS: A retrospective review of patients with sectorial keratitis and uveitis seen at the Ocular Immunology and Uveitis Service of the Massachusetts Eye and Ear Infirmary and a summary of the diagnoses of cases with similar ocular findings that have been reported in the literature. RESULTS: Data on six patients with sectorial keratitis and uveitis were reviewed. Four patients were female and two were male, ages 21-50 years. All were eventually diagnosed with herpes simplex viral stromal sectorial keratitis. The corneal infiltrates were most common in the superior corneal quadrants, located in the posterior corneal layers. Anterior non-granulomatous uveitis was present in all cases. One case had bilateral ocular involvement. Five of the six patients responded to topical steroids and antiviral treatment. Immunomodulation with methotrexate, cyclosporine, and systemic prednisone was required in one patient. Glaucoma was a complication in six of the seven eyes. Five patients developed corneal scarring, localized in the anterior and mid-stroma. CONCLUSION: The differential diagnosis of sectorial keratitis and uveitis is limited. The entities included in the differential are diverse; some of them threaten not only vision but also life. It is important for the ophthalmologist to be familiar with these entities, in order to pursue pertinent diagnostic investigations and arrive at an accurate diagnosis and institute appropriate management.

Relapsing polychondritis: a clinical review.

OBJECTIVE: This study comprehensively reviews the literature related to relapsing polychondritis (RP). METHODS: A detailed search via MEDLINE (PubMed) was performed using relapsing polychondritis as the key term. Relevant articles were analyzed with a focus on history, epidemiology, etiology, pathogenesis, clinical manifestations, diagnosis, treatment, and prognosis of RP. RESULTS: RP is a rare episodic and progressive inflammatory disease of presumed autoimmune etiology first described in 1923. RP affects cartilage in multiple organs, such as the ear, nose, larynx, trachea, bronchi, and joints. In addition, it can affect proteoglycan-rich tissues, such as the eyes, aorta, heart, and skin. The diagnosis of RP is based on the presence of clinical criteria. A standardized therapeutic protocol for RP has not been established. Nonsteroidal anti-inflammatory drugs, dapsone and/or colchicine, may control disease activity in some patients. In other patients, immunosuppressive drugs and prednisone have been effective. RP is a potentially lethal disease; pulmonary infection, systemic vasculitis, airway collapse, and renal failure are the most common causes of death. Earlier studies indicate survival rates between 70% at 4 years and 55% at 10 years. In a recent study, a survival rate of 94% at 8 years may be due to improved medical and surgical management. CONCLUSIONS: RP is a rare, multisystemic, and potentially fatal disease. The pathogenesis and optimal therapeutic approach to patients with RP is poorly understood.