Grand Rounds Raafay Sophie, M.D. 9/4/2015 University of Louisville Department of Ophthalmology and Visual Sciences

Patient Presentation CC: Blurry Vision and Painful Eye OS HPI: 33 yr old AAF, woke up in the morning with blurry vision and severe pain OS. Hx of contact lens use OS Complained of photophobia and epihora. Denied any trauma, flashes, floaters, scotomas or pain on eye movements

History PMHx: Migraines, Anemia FAMHx: Unremarkable ROS: Unremarkable MEDS: None ALLERGIES: NKDA

Exam VASC TP P 14 Firm 4→3 EOM: full OU 20/80 20/CF@4ft 14 Firm 4→3 no RAPD EOM: full OU CVF: full OD, could not assess OS

External Photos OD OS OS photo demonstrates central corneal edema and +1 injection

Slit Lamp Photos OD OS Cone shaped cornea, thinner in apex in cross section- finding consistent with KC stromal and epithelial edema with microcysts and bullae

Slit Lamp Photos OS OS OS photo demonstrates corneal edema, microcysts and +1 injection

Exam OD OS LIDS/LASHES WNL WNL CONJ WNL +1 injection CORNEA cone shaped stromal and epithelial edema with microcysts and bullae, break in descemet IRIS WNL WNL LENS clear could not visualize

Corneal topography- irregular astigmatisim in the form of inferior steepening. Marked difference btwn inf and superior, big difference between steep and flat Ks

Topography shows significant ectasia with very steep Ks in mid 70s- the marked steepening and irregularity make the scan technically difficult

History Pachymetry 394/358 POHx: Keratoconus OU Previously tried Rigid Gas Permeable (RGP) and then Scleral contact lens OS Corneal scar OS Severe irregular astigmatism OU -4.50 +3.25 x175 -5.25 +4.25 x045 Tried RGP, then Scleral Lens

Assessment DIAGNOSIS: Acute Corneal Hydrops 33 yr old AAF, hx of keratoconus, with blurry vision, severe pain, photophobia, and watering eye OS. Exam shows severe corneal edema and 1+injection. DIAGNOSIS: Acute Corneal Hydrops

Treatment First Visit VA CF@4m Day 4 VA 20/400 Day 11 VA CF@4m Cyclopentolate 1% BID, NaCl 5% ointment QID, Pred Forte BID, Pressure patch for 24 hrs Day 4 VA 20/400 Same Regimen Day 11 VA CF@4m Cyclopentolate 1% TID, Pred Forte QID Bandage contact lens

Treatment Day 18 VA HM Day 20 VA HM Day 26 VA HM Day 33 VA HM Pred Forte 6x daily NaCl 5% drops QID Day 20 VA HM Medrol (methylprednisolone) dose pack Day 26 VA HM Pred Forte Q3h Tramadol PRN for pain Day 33 VA HM Cosopt BID

Treatment Day 55 VA HM Intracameral gas injection.

Keratoconus (KC) Progressive, noninflammatory ectatic corneal disorder characterized by central/paracentral corneal thinning, protrusion, and irregular myopic astigmatism. Prevalence of 1 in 2000 Increased prevalence in Down Syndrome Atopy Marfan syndrome Floppy Eyelid syndrome Leber congenital hereditary optic neuropathy Mitral valve prolapse

Keratoconus No hereditary pattern Environmental factors 6-8% have positive family history Multiple chromosome loci reported, but exact gene unknown Environmental factors Eye rubbing Inflammation Hard contact lens wear Oxidative Stress Clinically unaffected first degree relatives have highger chance of subclinical topographic abnormailities.

Keratoconus Clinical Findings Mostly B/L- usually one eye worse Progression in mid 20’s to 30’s Apical thinning of cornea Scissoring of red reflex on retinoscopy Mostly bilateral, with one eye being more severely affected.

Keratoconus Clinical Findings Rizutti sign-conical reflection of nasal cornea Munson sign- protrusion of the lower eyelid on downward gaze

Keratoconus Clinical Findings Fleischer ring- iron deposits within the epithelium using cobalt blue filter Vogt's striae are vertical (rarely horizontal) fine, whitish STRESS lines in the deep/posterior stroma and Descemet's membrane

Keratoconus Evaluation Computerized videokeratography Helpful in early diagnosis, following progression, fitting for contact lens.

Keratoconus Management Glasses Rigid or Gas permeable contact lenses Intrastromal rings and collagen crosslinking flatten cone and stabilize progression Corneal transplant ( PK vs DALK) Contact lens intolerance Poor vision with comfortable lens Unstable contact lens fit Progressive thinning to periphery approaching limbus nodulectomy

Acute Corneal Hydrops Development of marked corneal edema caused by a break in Descemet membrane (DM) and endothelium, allowing aqueous to enter the corneal stroma and epithelium. Significant complication of non-inflammatory ectatic disorders Keratoconus (2.6%–2.8%) Pellucid marginal corneal degeneration (6%-11%) Keratoglobus (11%) Rarely- Post refractive keratectasia PMCD- peripheral usually inferior corneal thinning with protrusion usually above area of maximum thinning KB

Acute Corneal Hydrops Pathology DM break (trauma? Such as eye rubbing) Elastic DM retracts or coils due to tension. Accumulation of the aqueous leads to the separation of the collagen lamellae Formation of large fluid-filled stromal pockets. Postulated repair mechanism DM has to reattach to the posterior stroma- the time for this depends on the depth of the detachment. Endothelium has to migrate over the gap- the time for this depends on the dimensions of the DM break

Acute Corneal Hydrops Epidemiology Risk Factors 2nd or 3rd decade Males> Females No racial predisposition Risk Factors Poorer Snellen visual Steeper keratometry Earlier age at onset of KC Eye rubbing Vernal keratoconjunctivitis (VKC) Atopy Down's syndrome

Acute Corneal Hydrops Clinical Presentation Slitlamp examination Epiphora Markedly reduced visual acuity Intense photophobia Pain Slitlamp examination Marked stromal and epithelial microcystic edema Intrastromal cyst/clefts Conjunctival hyperemia

Acute Corneal Hydrops Clinical Course Most cases resolve spontaneously over 2-4 months Secondary flattening of the cornea (improved contact lens fitting) central corneal scarring typically (mandates corneal transplantation) corneal neovascularization may occur (increased risk if break involves limbus) area of corneal involvement duration for the edema to resolve, risk of neovascularization chance poorer visual outcome Other complications: Infection, pseudocyst formation, malignant glaucoma, corneal perforation. Greater likelihood of episodes of endothelial graft rejection after penetrating keratoplasty Pseudocysts are acquired fluid-filled cavities lined by fibro-connective tissue that differ from true cysts because they lack an epithelial lining malignant glaucoma is characterised by a shallow anterior chamber associated with raised intraocular pressure and in the presence of a patent iridotomy

Acute Corneal Hydrops Imaging Ultrasound biomicroscopy (UBM) In vivo confocal microscopy (IVCM) Anterior segment optical coherence tomography (AS-OCT) -corneal edema and the presence of a large intrastromal fluid filled cleft -Resolved- Central corneal stromal scarring and a focal detachment of Descemet membrane are demonstrated

Acute Corneal Hydrops Treatment Medical Conservative Observation + topical lubrication for comfort ± Pressure patching and bandage contact lens Medical Topical hypertonic saline (5%) to reduce intrastromal edema, Topical corticosteroids to reduce inflammation and prevent neovascularization Cycloplegic agents to reduce pain Antiglaucoma medications to lessen the hydrodynamic force on the posterior cornea Topical antibiotics are used where there is epithelial defect or the risk of such is high

Acute Corneal Hydrops Surgical - Intracameral Air/gas Injection Provides tamponade effect which prevents aqueous penetration into the stroma and also by unrolling the torn ends of ruptured DM Air 20% sulfur hexafluoride (SF6) 14% perflouropropane (C3F8)

Acute corneal hydrops in keratoconus - new perspectives. Am J Ophthalmol, 2014. 157(5): p. 921-8 Intracameral gas Approximately a 1 month faster resolution No significant difference in terms of final BCVA or need for corneal transplantation. “Using isoexpansile gases with caution” Frequent follow-up due to serious complications pupil block glaucoma intrastroml migration of gas, possible cataract and endothelial cell loss. Supine positioning required after surgery- from 24 hours up to 2 weeks. Repeated injections are frequently necessary (except for C3F8).

Advisable to first measure the dimensions of the DM tear with AS-OCT Acute corneal hydrops in keratoconus - new perspectives. Am J Ophthalmol, 2014. 157(5): p. 921-8 Intracameral gas When to use? “Might” be recommended for individuals who are highly compliant and motivated Perfluoropropane gas of choice (least number of reinjections, safe for endothelial preservation) Advisable to first measure the dimensions of the DM tear with AS-OCT Further studies are required to validate the area and depth of the tear, beyond which intracameral gas injection is unhelpful.

Acute corneal hydrops in keratoconus. Indian J Ophthalmol, 2013. 61(8): p. 461-4.

THANK YOU

References External Disease and Cornea- BCSC 2015-2016 http://www.eyerounds.org/ Maharana, P.K., N. Sharma, and R.B. Vajpayee, Acute corneal hydrops in keratoconus. Indian J Ophthalmol, 2013. 61(8): p. 461-4. Fan Gaskin, J.C., D.V. Patel, and C.N. McGhee, Acute corneal hydrops in keratoconus - new perspectives. Am J Ophthalmol, 2014. 157(5): p. 921-8.

Acknowledgments Dr. S. Balakrishnan Dr. S. Reddy