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

2. 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

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

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

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

6. 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

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

8. 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

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

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

11. 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
x175
x045
Tried RGP, then Scleral Lens

12. 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

13. 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
Cyclopentolate 1% TID,
Pred Forte QID
Bandage contact lens

14. 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

15. Treatment
Day 55
VA HM
Intracameral gas injection.

16. 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

17. 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.

18. 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.

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

20. 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

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

22. 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

23. 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

24. 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

25. 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

26. 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

27. 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

28. 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

29. 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

30. 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)

31. Acute corneal hydrops in keratoconus - new perspectives.
Am J Ophthalmol, (5): p
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).

32. Advisable to first measure the dimensions of the DM tear with AS-OCT
Acute corneal hydrops in keratoconus - new perspectives.
Am J Ophthalmol, (5): p
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.

33. Acute corneal hydrops in keratoconus.
Indian J Ophthalmol, (8): p

34. THANK YOU

35. References External Disease and Cornea- BCSC 2015-2016
Maharana, P.K., N. Sharma, and R.B. Vajpayee, Acute corneal hydrops in keratoconus. Indian J Ophthalmol, (8): p
Fan Gaskin, J.C., D.V. Patel, and C.N. McGhee, Acute corneal hydrops in keratoconus - new perspectives. Am J Ophthalmol, (5): p

36. Acknowledgments
Dr. S. Balakrishnan
Dr. S. Reddy