Transcript Acute Hydrops - University of Louisville Ophthalmology

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
14
20/80
VASC
TP
20/CF@4ft
4→3
P
Firm
4→3
no RAPD
EOM: full OU
CVF: full OD, could not assess OS
External Photos
OD
OS
Slit Lamp Photos
OD
OS
Slit Lamp Photos
OS
OS
Exam
OD
OS
LIDS/LASHES
WNL
WNL
CONJ
WNL
+1 injection
CORNEA
cone shaped
IRIS
WNL
LENS
clear
stromal and epithelial edema
with microcysts and bullae,
break in descemet
WNL
could not visualize
History
POHx:
•
Keratoconus OU
•
Pachymetry 394/358
•
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
Assessment
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
Same Regimen
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
Medrol (methylprednisolone) dose pack
Pred Forte Q3h
Tramadol PRN for pain
Cosopt BID
Treatment
Day 55
VA HM
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
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Down Syndrome
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Atopy
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Marfan syndrome
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Floppy Eyelid syndrome
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Leber congenital hereditary optic neuropathy
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Mitral valve prolapse
Keratoconus
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No hereditary pattern
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6-8% have positive family history
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Multiple chromosome loci reported, but exact gene unknown
Environmental factors
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Eye rubbing
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Inflammation
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Hard contact lens wear
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Oxidative Stress
Keratoconus
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Clinical Findings
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Mostly B/L- usually one eye worse
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Progression in mid 20’s to 30’s
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Apical thinning of cornea
•
Scissoring of red reflex on
retinoscopy
Keratoconus
•
Clinical Findings
Keratoconus
•
Clinical Findings
Keratoconus
•
Evaluation
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Computerized videokeratography
Keratoconus
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Management
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Glasses
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Rigid or Gas permeable contact lenses
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Intrastromal rings and collagen crosslinking
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•
flatten cone and stabilize progression
Corneal transplant ( PK vs DALK)
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Contact lens intolerance
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Poor vision with comfortable lens
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Unstable contact lens fit
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Progressive thinning to periphery approaching limbus
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
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Keratoconus (2.6%–2.8%)
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Pellucid marginal corneal degeneration (6%-11%)
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Keratoglobus (11%)
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Rarely- Post refractive keratectasia
Acute Corneal Hydrops
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Pathology
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DM break (trauma? Such as eye rubbing)
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Elastic DM retracts or coils due to tension.
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Accumulation of the aqueous leads to the separation of the
collagen lamellae
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Formation of large fluid-filled stromal pockets.
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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
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Epidemiology
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2nd or 3rd decade
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Males> Females
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No racial predisposition
Risk Factors
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Poorer Snellen visual
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Steeper keratometry
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Earlier age at onset of KC
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Eye rubbing
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Vernal keratoconjunctivitis (VKC)
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Atopy
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Down's syndrome
Acute Corneal Hydrops
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Clinical Presentation
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Epiphora
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Markedly reduced visual acuity
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Intense photophobia
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Pain
Slitlamp examination
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Marked stromal and epithelial microcystic edema
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Intrastromal cyst/clefts
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Conjunctival hyperemia
Acute Corneal Hydrops
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Clinical Course
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Most cases resolve spontaneously over 2-4 months
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Secondary flattening of the cornea (improved contact lens fitting)
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central corneal scarring typically (mandates corneal transplantation)
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corneal neovascularization may occur (increased risk if break involves limbus)
area of corneal involvement
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duration for the edema to resolve,
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risk of neovascularization
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chance poorer visual outcome
Other complications:
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Infection, pseudocyst formation, malignant glaucoma, corneal perforation.
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Greater likelihood of episodes of endothelial graft rejection after penetrating
keratoplasty
Acute Corneal Hydrops
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Imaging
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Ultrasound biomicroscopy (UBM)
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In vivo confocal microscopy (IVCM)
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Anterior segment optical coherence tomography (AS-OCT)
Acute Corneal Hydrops
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Treatment
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Conservative
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Observation + topical lubrication for comfort ±
Pressure patching and bandage contact lens
Medical
•
•
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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 Hydrops
•
Surgical - Intracameral Air/gas Injection
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Provides tamponade effect which prevents
aqueous penetration into the stroma and also by
unrolling the torn ends of ruptured DM
•
•
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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).
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