Corneal melt as the initial presentation of primary Sjögren's syndrome.

Corneal melting is a rare complication of S ogren's syndrome (SS). Previously reported cases of corneal ulceration occurred in patients with established SS, usually secondary to RA. We describe the first case of corneal ulceration with stromal melting as the initial presentation of primary SS. A 79-year-old man without prior sicca symptoms developed a large sterile corneal ulcer that required extensive treatment over several months with ocular lubricants, systemic immunosuppressives, and surgical repair. Evaluation for an underlying connective tissue disease revealed positive antinuclear antibodies (1:640 speckled) and anti-SSA antibody. A lip biopsy established the diagnosis of SS. Ulceration later occurred in the contralateral eye. Two years after the last corneal ulcer and no longer taking prednisone, the patient's ocular disease remained quiescent taking azathioprine 175 mg and hydroxychloroquine 400 mg daily. This case highlights the potential for primary SS to present with serious ocular complications despite lack of a priori sicca symptoms, as well as the importance of immunosuppressive therapy in the treatment of this complication.

Spontaneous corneal perforation in pellucid marginal degeneration.

PURPOSE: We present a report of a spontaneous corneal perforation in a patient with pellucid marginal corneal degeneration. METHODS: We describe the presentation and management of a 74 year old male with spontaneous corneal perforation related to pellucid marginal corneal degeneration. RESULTS: The corneal perforation was repaired with a 3 mm penetrating patch graft. The cornea has remained stable for 7 years with no further progression of corneal ectasia in either eye. CONCLUSIONS: Although rare, spontaneous corneal perforations in patients with pellucid marginal corneal degeneration can occur, and patients should be cautioned about this possibility.

Delayed-onset fungal keratitis after endophthalmitis.

PURPOSE: This study aimed to present two patients with delayed-onset, endogenous fungal keratitis after treatment for fungal endophthalmitis after cataract surgery. METHODS: The authors retrospectively reviewed the clinical course of two patients in whom deep stromal fungal keratitis developed 2 weeks and 3 months, respectively, after apparent successful aggressive therapy for fungal endophthalmitis. Before the onset of the keratitis, both patients underwent vitrectomies with intraocular injection of 7.5 to 10.0 mcg amphotericin B, as well as treatment with systemic fluconazole and topical antifungal therapy. In case 1, a pre-existing prosthetic intraocular lens and lens capsular bag were removed at the time of vitrectomy, whereas in case 2, the intraocular lens was left in place and a posterior capsulectomy was performed. RESULTS: The keratitis worsened in both patients, despite intensive systemic and topical antifungal therapy. Both patients underwent therapeutic penetrating keratoplasties. In case 1, this resulted in successful resolution of the infection and no recurrences 3 months after the transplant. The corneal transplant was complicated by an expulsive choroidal hemorrhage in the other patient. Fusarium solani was cultured from the initial vitrectomy specimen in patient 1, and although it was not cultured from the keratitis, septate hyphal elements were present on histopathologic examination. The causative organism in case 2 was Acremonium kiliense, which was cultured from both the original vitrectomy specimen and the deep corneal stromal infiltrate. CONCLUSIONS: Fungal organisms may not be eradicated completely from eyes with endophthalmitis despite aggressive therapy and apparent initial successful treatment. These patients need to be monitored for prolonged periods, and treatment should be reinitiated at the earliest sign of recrudescence of infection.

Successful visual rehabilitation after neonatal penetrating keratoplasty.

BACKGROUND: Penetrating keratoplasty in infancy and childhood has traditionally met with limited visual success due to a combination of unique physiology and technical problems in this patient population. With the advances in microsurgical instrumentation, corneal preservation, and visual developmental physiology ophthalmologists are finding increasing indications for penetrating keratoplasty in the childhood population. The long term results of neonatal penetrating keratoplasty in two patients with unilateral congenital corneal opacification are reported. METHODS: Penetrating keratoplasty was performed on one eye in each of two infants within the first 3 weeks of life. Amblyopia treatment and optical therapy have been continued since surgery. RESULTS: After 6 years both grafts have remained clear. One patient developed the infantile esotropia syndrome. Visual development using Snellen optotypes is age normal for both transplanted eyes. CONCLUSIONS: Penetrating keratoplasty when combined with optical correction and amblyopia therapy may restore and preserve vision in selected patients with congenital corneal opacification if performed in the neonatal period.

Chronic localized conjunctival chemosis.

Conjunctival chemosis is a commonly encountered clinical finding stemming from the presence of excess fluid in the conjunctiva. It is typically self-limiting or reversible if the underlying condition is treated. The authors present a series of seven cases of chronic localized conjunctival chemosis. Each patient had a localized area of dependent conjunctival edema for >or= 6 months. Evaluation of each patient included clinical examination, laboratory studies, and neuroimaging to attempt to elucidate the pathogenesis of the chemosis. Conjunctival biopsy was performed in six of the seven patients. In all of the patients studied, clinical examination uncovered no definitive signs of local inflammation. Laboratory evaluation was normal, and neuroimaging failed to confirm obstruction of venous or lymphatic drainage. Conjunctival biopsies showed chronic tissue inflammation or lymphangiectasia. The diagnosis of chronic localized conjunctival chemosis (CLCC) can be made if localized conjunctival edema persists for 6 months. Evaluation of biopsy specimens supports the theory that CLCC may result from scarring and structural alteration of conjunctival lymphatics, which appears to irreversibly affect the distribution of fluid in the conjunctiva.

Corneal endothelial polymerization of histoacryl adhesive: a report of a new intraocular complication.

Tissue adhesive are commonly used in ophthalmology to seal small corneal perforations. We report a case of inadvertent instillation of cyanoacrylate adhesive (Histoacryl) in the anterior chamber resulting in polymerization of the glue on the corneal endothelial surface, with iridocorneal and iridolenticular adhesion.

Capsulorhexis contracture in phacoemulsification surgery.

We report two cases of capsulorhexis contracture and eventual complete closure after phacoemulsification surgery. The closures significantly decreased vision, but vision improved in both cases following successful Nd:YAG laser treatment.

Epithelial downgrowth following the removal of iris inclusion cysts.

We present three patients in whom epithelial downgrowth occurred following the excision of iris inclusion cysts. The sheet-like downgrowth was aggressively treated and successfully eradicated in two of the three patients. Early identification and prompt therapy are essential.

Bell's palsy in Moebius syndrome.

We report a case of unilateral transient and reversible facial paresis, which was superimposed on a congenital bifacial palsy in a young adult with Moebius syndrome. Our case illustrates the potential for two conditions, both affecting the facial nerve and both of unknown etiology, to be juxtaposed in a single individual. Worsening of facial palsy in the Moebius syndrome may not signify progressive disease.

Keratoconus associated with corneal endothelial dystrophy.

Keratoconus is found in conjunction with many ocular and nonocular disorders. We describe five patients with concurrent keratoconus and corneal endothelial dystrophy.

Class II alloantigen induced on corneal endothelium. Role in corneal allograft rejection.

The expression of Class II major histocompatibility complex (MHC) antigens on corneal cells can be increased in vitro by (gamma-interferon) and in vivo in inflammatory reactions. The expression of Class II MHC by corneal endothelium of New Zealand White (NZW) rabbits during the rejection of corneal allografts was demonstrated by immunoperoxidase staining. Class II MHC expression by corneal endothelial cells may facilitate rejection of corneal allografts.

The unexpected guest: problem of iris reposition.

A 31-year-old male presented with a corneal laceration and iris prolapse in the left eye. The surgical repair was complicated by epithelial ingrowth on the lens capsule. The appropriate management of both the initial trauma and subsequent complication is discussed.

Ocular lubricants and corneal injury during anesthesia.

Seventy-six patients undergoing general endotracheal anesthesia were studied prospectively to determine whether simply taping the eyelids closed during the surgical procedure as opposed to taping the lids after instilling a bland lubricating ointment had any different effect on corneal or conjunctival epithelium. No significant difference was found.