Grand RoundsGrand Rounds

Raafay Sophie, M.D.Raafay Sophie, M.D.

9/4/20159/4/2015

University of LouisvilleUniversity of Louisville

Department of Ophthalmology and Department of Ophthalmology and Visual SciencesVisual Sciences

Patient PresentationPatient Presentation CCCC: : Blurry Vision and Painful Eye OSBlurry Vision and Painful Eye OS

HPIHPI: : 33 yr old AAF, woke up in the morning 33 yr old AAF, woke up in the morning

with blurry vision and severe pain OS.with blurry vision and severe pain OS. Hx of contact lens use OSHx of contact lens use OS Complained of photophobia and epihora.Complained of photophobia and epihora. Denied any trauma, flashes, floaters, Denied any trauma, flashes, floaters,

scotomas or pain on eye movements scotomas or pain on eye movements

HistoryHistory• PMHx:PMHx: Migraines, AnemiaMigraines, Anemia

• FAMHx:FAMHx: UnremarkableUnremarkable

• ROS: ROS: UnremarkableUnremarkable

• MEDS:MEDS: NoneNone

• ALLERGIESALLERGIES: : NKDA NKDA

ExamExam

VASC TP P

20/80

20/CF@4ft

14

Firm no RAPD

EOM: full OU CVF: full OD, could not assess OS

4→3

4→3

External PhotosExternal PhotosOD OS

Slit Lamp Photos Slit Lamp Photos OD OS

Slit Lamp Photos Slit Lamp Photos OS OS

Exam 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

HistoryHistoryPOHx: POHx:

• Keratoconus OUKeratoconus OU• Pachymetry 394/358

• Previously tried Rigid Gas Permeable (RGP) and Previously tried Rigid Gas Permeable (RGP) and then Scleral contact lens then Scleral contact lens OSOS

• Corneal scar OSCorneal scar OS

• Severe irregular astigmatism OU Severe irregular astigmatism OU • -4.50 +3.25 x175-4.50 +3.25 x175• -5.25 +4.25 x045-5.25 +4.25 x045

AssessmentAssessment

DIAGNOSIS:DIAGNOSIS:Acute Corneal Acute Corneal

Hydrops 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.

Treatment Treatment First Visit

VA CF@4m

Day 4VA 20/400

Cyclopentolate 1% BID,NaCl 5% ointment QID,Pred Forte BID, Pressure patch for 24 hrs

Same Regimen

Day 11VA CF@4m

Cyclopentolate 1% TID,

Pred Forte QID

Bandage contact lens

Treatment Treatment

Day 20VA HM

Medrol (methylprednisolone) dose pack

Day 26VA HM

Pred Forte Q3h Tramadol PRN for pain

Day 33VA HM

Cosopt BID

Day 18 VA HM

Pred Forte 6x daily NaCl 5% drops QID

Treatment Treatment Day 55

VA HM

Keratoconus (KC)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

KeratoconusKeratoconus

• No hereditary pattern• 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

KeratoconusKeratoconus• 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

KeratoconusKeratoconus• Clinical Findings

KeratoconusKeratoconus• Clinical Findings

KeratoconusKeratoconus• Evaluation

• Computerized videokeratography

KeratoconusKeratoconus• 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

Acute Corneal HydropsAcute 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

Acute Corneal HydropsAcute 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 HydropsAcute Corneal Hydrops• Epidemiology

• 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 HydropsAcute Corneal Hydrops• Clinical Presentation

• Epiphora• Markedly reduced visual acuity• Intense photophobia• Pain

• Slitlamp examination• Marked stromal and epithelial microcystic

edema• Intrastromal cyst/clefts • Conjunctival hyperemia

Acute Corneal HydropsAcute 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

Acute Corneal HydropsAcute Corneal Hydrops• Imaging

• Ultrasound biomicroscopy (UBM)• In vivo confocal microscopy (IVCM)• Anterior segment optical coherence

tomography (AS-OCT)

Acute Corneal HydropsAcute Corneal Hydrops• Treatment

• 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

Acute Corneal HydropsAcute 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. Acute corneal hydrops in keratoconus - new perspectives. Am J Ophthalmol, 2014. 157(5): p. 921-8 Am J Ophthalmol, 2014. 157(5): p. 921-8

• 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).

Intracameral gas

Acute corneal hydrops in keratoconus - new perspectives. Acute corneal hydrops in keratoconus - new perspectives. Am J Ophthalmol, 2014. 157(5): p. 921-8 Am J Ophthalmol, 2014. 157(5): p. 921-8

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.

Intracameral gas

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

THANK YOU

References 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.

AcknowledgmentsAcknowledgments

• Dr. S. Balakrishnan

• Dr. S. Reddy