WHAT IS YOUR DIAGNOSIS? By ADREA R. BENKOFF M.D.

WHAT IS YOUR DIAGNOSIS? By ADREA R. BENKOFF M.D.

Anterior Chamber Inflammation and Iris Depigmentation Noted 25 Years After Cataract Extraction

Decreasing Vision Over a 5- Year Period 64 year old white woman had uncomplicated cataract surgery in her right eye 25 years ago 20/25 vision gradually decreased to 20/50 over the last 5 years

Background Diffuse iris atrophy Enlarged and slightly irregular pupil Posterior capsular opacification YAG capsulotomy performed with low grade iritis found after the procedure. Pt. was placed on prednisolone acetate 1% 5X/day and scopalamine daily with no improvement in the iritis or vision

Background Fuchs corneal dystrophy Blepharitis Lymes disease 9 months before presenting as Bell s palsy, treated with antibiotics for 1 month Rosacea Irritable bowel syndrome Lumbar spondyloarthropathy Rare cold sores Meds: included metronidazole cream and omeprazole

Examination Va: OD 20/80, OS 20/30 Myopic prescription and 2.5 D of astigmatism OD only Pupil OD: 5 mm and fixed without a relative afferent pupillary defect EOM normal IOP normal

Examination Slit Lamp: rosacea, mild blepharitis, 3+ guttata without corneal edema OU, Light brown irises with diffuse depigmentation OD and transillumination defects, trace cell and flare OD, sulcus IOL well positioned OD with an open posterior capsule, mild nuclear sclerotic cataract OS Fundus exam: unremarkable

Examination Full work- up by a rheumatologist was done High ANA Elevated SSA and SSB antibodies Mildly elevated Rheumatoid factor High- normal Lysozyme titer

WHAT IS YOUR DIAGNOSIS?

Differential Diagnosis INFLAMMATORY CAUSE Fuch s Heterochromic Uveitis Posner- Schlossman Syndrome Chronic Autoimmune Uveitis Related to Lyme disease or Lupus

Differential Diagnosis OTHER CAUSES Trauma: IOL causing sectoral chafing of the iris Herpes Iritis Congenital Horner s Syndrome Asymmetrical Pigment Dispersion Syndrome

Discussion FUCHS HETEROCHROMIC UVEITIS Accounts for 3.5% to 8% of all uveitis Characterized by chronic low- grade uveitis without formation of synechiae or acute exacerbations Iris heterochromia (atrophy in blue irises appears hyperchromic due to exposure of the iris posterior pigment epithelium) Early cataract formation

Heterochromia

Discussion Small stellate keratic precipitates Glaucoma Unilateral (in 90% to 95% of cases) Vitreous opacities Amsler Sign: a hyphema occurs after cataract surgery or paracentesis, gonioscopy or applanation tonometry Unilateral astigmatism

Stellate Keratic Precipitates

Discussion Pts. typically present in 3 rd to 4 th decade of life with complaint of decreased vision and floaters No gender or racial predisposition Cataract is the most common complication Higher risk of PSC opacification and IOL precipitates Glaucoma is the most serious complication Not as responsive to medical or surgical therapy

Cataract Formation

Discussion Etiology of Fuchs Uveitis - - not well known Recent research implicates the rubella virus Also associated with CMV, toxoplasmosis and herpes simplex virus Syndrome may be the result of a variety of infectious agents Prognosis Good for majority of pt. who do not develop glaucoma, maintaining Va of 20/40 or better

Treatment Topical Steroids, chronically No Treatment Some pts. continue to show a small amount of inflammation despite chronic use of topical steroids

Woman Presents with Light Sensitivity and Blurry Vision

History 68 year old white woman has 1 week of bilateral light sensitivity and blurry vision associated with redness and pain Recent diagnosis of pneumonia 1 week before ocular symptoms and was treated with a 7 day course of oral Avelox (moxifloxacin) and steroids Pts. ocular and medical history unremarkable Family history of colon cancer and heart failure

Examination Va: 20/40 OD, 20/30 OS Pupils: Fixed and atonic bilaterally EOMs: Full IOP: 26 OD, 28 OS SLE: Mild conjunctival injection diffusely OU, Clear cornea, 1+ darkly pigmented keratic precipitates inferiorly OU, 4+ fine pigmented cells in the AC OU

Pigmented Keratic Precipitates

Examination SLE cont.: Pigmented granules were present along the pupillary margin, transillumination defects and extensive posterior synechiae were found in the mid- peripheral iris Gonioscopy: Deep open angles with diffuse pigment deposition OU, no peripheral anterior synechiae or concave iris configuration Fundus: normal OU

Transillumination Defects

Gonioscopy

WHAT IS YOUR DIAGNOSIS?

Differential Diagnosis for Acute Anterior Uveitis INFLAMMATORY DISORDERS HLA- B27 with or without associated ankylosing spondylitis Inflammatory bowel disease Psoriatic arthritis (commonly bilateral but alternating and non- simultaneous) Vogt- Koyanagi- Harada Syndrome Behcet s disease Juvenile idiopathic arthritis (insidious onset with chronic course)

Differential Diagnosis for Acute Anterior Uveitis INFECTIOUS ETIOLOGIES Herpetic eye disease ( usually unilateral) Syphilis TB Lyme disease TUMORS (mimic anterior uveitis) Retinoblastoma Lymphoma Iris melanoma Metastasis

Differential Diagnosis of Acute Anterior Uveitis SYSTEMIC MEDICATIONS Rifabutin (antibiotic used in treatment of TB in AIDS) Cidofovir (antiviral used in treatment of CMV retinitis in AIDS) Sulfonamide antibiotics Pamidronate (treatment for osteoporosis) Fluoroquinolone antibiotics

Differential Diagnosis cont. IRIS DEPIGMENTATION AND ATROPHY Pigment dispersion syndrome After radiation treatment Fuchs heterochromic iridocyclitis Unilateral, mild anterior chamber reaction with no posterior synechiae.

Diagnosis FURTHER WORK- UP Anterior chamber paracentesis: negative for Herpes Simplex virus ACE and Lysozyme levels : normal Syphilis and Lyme antibody testing: normal

Discussion FLUOROQUINOLONE- ASSOCIATED UVEITIS Rare 40 known cases 48% were bilateral Transillumination defects, atonic pupils and findings of pigment dispersion 63% assoc. with oral Moxifloxacin treatment Mechanism for uveitis is currently unknown

Treatment TOPICAL STEROIDS Pred Forte q 1 hour initially CYCLOPLEGIC AGENTS Cyclogyl 3X/day IOP- LOWERING MEDICATIONS Combigan 2X/day

Unilateral Pain, Photophobia and Blurry Vision in a Pediatric Patient

History 5 year old girl complains of pain, photophobia and blurry vision OD for 2 weeks She is being treated with erythromycin ophthalmic ointment 4X/day OD She has no other medical or ocular history

Examination Va: 20/20 OD, 20/15 OS PERRLA with no afferent pupillary defect EOM full IOP: 12 OD, 13 OS Slit Lamp OD: 1 to 2+ conjunctival injection Numerous fine, linear corneal stromal opacities less than 1 mm in size, some were associated with surrounding subepithelial infiltrates

Examination Slit Lamp OD cont.: Linear opacities were dispersed nasally but were also found superiorly and inferiorly in the paracentral and peripheral cornea. No epithelial defect or anterior chamber reaction were found Slit Lamp OS: Unremarkable Fundus Exam: Unremarkable OU

Stromal Corneal Opacities

Subepithelial Infiltrates

WHAT IS YOUR DIAGNOSIS?

Differential Diagnosis For Linear Stromal Opacities Corneal Foreign Body Acute onset of symptoms Unilateral presentation Fine linear appearance of the opacities is consistent with animal or insect hairs

Differential Diagnosis For Linear Stromal Opacities Infectious Keratitis Etiologies such as: EKC, bacterial or fungal keratitis Would cause subepithelial infiltrates and conjunctival injection Would cause opacities with feathery not sharp borders Contact Lens Wear Trauma

Differential Diagnosis For Linear Stromal Opacities Corneal Dystrophies Lattice: Long, branching lines with intervening haze Granular: Crumb- like opacities that fuse to form stellate or linear structures. Crystalline: Diffuse and bilateral Positive family history

Diagnosis Further History: Pt. had been playing with her cousin s pet CHILEAN ROSE TARANTULA when the symptoms began Re- examination: No superficial or exposed tarantula hairs discovered Hairs were found deeply embedded in posterior stroma of cornea and could not be removed

Treatment Topical Pred Forte drops every 4 hours OD and tapered slowly to 4X/day Besivance drops (besifloxacin 0.6%) 3X/day OD for 10 days 1 month follow- up Resolved stromal infiltrates, no uveitis present Resolved symptoms of tearing, pain and photophobia

Treatment 2 month follow- up Tarantula hairs migrated and extruded into anterior chamber Anterior chamber reaction KPs formed around the hairs Pt. started on Lotemax 4X/day and followed closely for anterior chamber inflammation

Discussion OPHTHALMIA NODOSA Described in 1904 by Saemisch A term that encompasses inflammatory ocular reactions to animal and vegetable hairs or hair- like structures Includes exposure to caterpillar and tarantula hairs, insect stingers and wings Tarantula hairs have caused: conjunctival granulomas, corneal stromal infiltrates, anterior uveitis, vitritis and retinochoroiditis

Conjunctival Granulomas

Discussion Tarantulas Expulsion of dorsal hairs is a primary defense mechanism 10,000 hairs/mm located on its dorsal abdomen Hairs are barbed and are 0.6mm to 1.5mm in length Barbs facilitate penetration and intraocular migration No toxins

Tarantula Hair

Discussion Treatment Topical steroids and antibiotics initially Slit lamp removal of superficial hairs Long- term steroid therapy for persistent anterior uveitis, vitritis or retinochoroiditis

STAY AWAY FROM TARANTULAS!!!