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Karen G. Carrasquillo, OD, PhD, FAAO, FSLS, FBCLA

Innovation | Education

Scleral Lenses, Irregular Corneas and Ocular Surface Disease

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• VP of Clinical and Professional Affairs, BostonSight

• Adjunct Clinical Faculty• New England College Of 

Optometry

• Adjunct Clinical Faculty• MCPHS University School of 

Optometry

• Advisory Board Member• Gas Permeable Lens Institute

• Fellow Scleral Lens Education Society

• Fellow of the British Contact Lens Association

Disclosure

Karen G. Carrasquillo, O.D., Ph.D., FAAO, FSLS and all doctors of the BostonSight are salaried, full-time employees of a 501(c)3 non-profit organization.

• Neither Dr. Carrasquillo nor any of the BostonSight providers have proprietary interest in BostonSight PROSE treatment or BostonSight SCLERAL

• www.bostonsight.org

Brief History

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Earliest descriptions of CL – published late 1800s

Scleral Shells 19-21mm made out of blown or ground glass - 1889

Impression molding –1930s

Translation of Mueller, Fick, and Kalt’s work -

published in 1980s

Ezekiel publishes first description of gas

permeable scleral lens –1983

Rosenthal develops Fluid-Ventilated scleral lens –

1986

“A recent search of scleral lens designs included on the Gas Permeable Lens Institute’s website yielded a list of 17 manufacturers currently offering 41 different scleral lens designs”

From: Schornack, M. (2014) Scleral Lenses: A Literature Review, Eye and Contact Lens, (41) N1 3-11

Terminology

• Intralimbal

• Corneoscleral

• Miniscleral

• Scleral

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Scleral Lenses

• Diameter varies between 17.0 to 23.0+

• Vaults over the cornea

• Bearing of lens completely on sclera

Scleral Lenses

Scleral Lens Features

• Avoids all contact with the cornea• Fluid reservoir• Modern high Dk materials• Rests entirely on the conjunctival

tissue overlying sclera

Scleral Lens Examples

• BostonSight SCLERAL• Jupiter• TruForm• Europa• Custom Stable • EyePrint PRO

Indications for Scleral Lens Use

• Correct irregular astigmatism• Dry eye symptom relief• Protection of ocular surface• Maintenance and support of the

ocular surface

Scleral Lens Evaluation/Assessment

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• Measure central clearance first

- With and without NaFl

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Vault

Identify the front and back surface of the lens with an optic section

Vault

Make sure there is no corneal contact (unless you are fitting a corneosclerallens)

Confirm both the front and back surface reflex of the lens

Insert the lens with Fluorescein and look for narrowing of vault or contact

Vault Diameter•Selection may be arbitrary•May be multifactorial•Fitting set parameters

•Palbebral fissure size•Patient apprehension/

cooperation•Anatomical Obstacles

Diameter

Anatomical Obstacle: Pinguecula

Working Inside Out

• Then monitoring the limbus

Superiorly

Inferiorly

Nasally

Most likely to be problematic Temporally

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Working Inside Out

• Edge Lift

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Excessive Edge Lift

Note the air meniscus

Working Inside Out

• Compression

• Blanching

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Mid-Haptic Compression

Compression and Impingement Working Inside Out

• Impingement

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ImpingementHaptic Evaluation

•Goal is haptic alignment to sclera•Things to avoid

•Excessive Edge Lift•Compression 360°

•Monitor for Toricity

Toricity

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Working Inside Out

• Hooding/Conjunctival Prolapse

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Large diameter vs. Small diameter

• Large bearing area on sclera to support higher corneal clearances

- Distributes weight over a larger area

- Easier to clear the limbal area

- Provides more coverage in dry eye conditions

• Smaller diameters may minimize toricity

- May have to fit under symblephera

- Lower corneal clearance

- Smaller eyes or difficult applications

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Evaluation after settling

Allow lenses to settle

Smaller lenses settle more than larger lenses

More critical in corneal scleral lenses

3 hours settling

- At least 20 minutes

End of day appointments

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Caroline, P, Andre, M. (2012) “Scleral Lens Settling”, Contact Lens Spectrum

Effect of settling over time

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EVIDENCED-BASE/DATA-DRIVEN DESIGN

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Contraindications and Non-candidates

Determined on a case by case basis

Ankyloblepharon

Symblepharon too close to the limbus

Glaucoma tube shunts – tricky

Glaucoma Blebs

Entering with corneal edema

Unique fits Graft Failure: Fenestration

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Training Challenges Training Challenges

Application and Removal

• Supplies

Cleaning and Disinfection

Saline Solutions

SCLERAL LENS INDICATIONS

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Irregular Astigmatism Correction

Keratoconus

Pellucid Marginal

Degeneration

Post Penetrating Keratoplasty

Post Refractive Surgery

Keratoglobus

Keratoglobus

Apex Thinning

Keratoconus Pellucid Marginal Degeneration

Apex

Thinning

Baseline

Cressey, A., Jacobs, D.S., and Carrasquillo, K.G. (2012) Management of vascularized limbal keratitis (VLK) with prosthetic replacement of the ocular surface system. Eye and Contact Lens 38(2):137-40

6 mos s/p PROSE 6 mos s/p PROSE 2 yrs s/p PROSE 4 yrs s/p PROSE 2 yrs s/p PROSE

Complications

• Scarring

• Hydrops

• High Order Aberrations

• Rupture

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Hydrops

50.8/ 58.9 @ 143

August 2004

66.5/ 73.4 @ 122

December 2007

31.0/ 39.2 @ 172

August 2009

Signs and symptoms of graft edema

• Complications of older grafts

- Microcystic Epithelial Edema

- Hazy Vision

- Sattler’s veil:

• rainbows around light sources from corneal edema

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Microcystic Edema(our view in)

Sattler’s Veil(pt’s view out)

Endothelial cell counts decrease

• Typical endothelial counts range from 2400 – 3200 cells/mm2

• Cell counts decrease with:

- Age of patient

- Age of graft

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Failing Grafts and Scleral lenses

• Etiology

- Hypoxia

• Epithelial edema resulting from oxygen deprivation

- Suction with Sclerals

• Suction increases with wearing time

• Both poor epithelial cell junctions and low endothelial reserve may be contributory to edema

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Fitting Strategies

• Decrease Hypoxic Stress

- High Dk material

• XO2 with Dk 141

- Thin center thickness

• Design Strategies to Decrease Suction

- Large diameters

- Fenestrations

- Channels

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Case

• 52 year old female s/p PKP (OD 1998, OS 1991) secondary to keratoconus

• Presented to clinic having been fit with scleral lenses in the past for vision OU

- Intolerant; required frequent removal and re-insertion throughout the day secondary to discomfort

• Trial BostonSight® PROSE devices

- Appreciated comfort and VA (20/20 OD, 20/20 OS)

• Proceeded to start treatment

• During treatment reported hazy vision and rainbows around lights

- Corroborated by objective observation of epithelial edema

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PROSE Strategies: Large and Loose

• PROSE Device #1:

- Vault: 4.9, BC: 8.2, Power: -0.50, Diameter: 18.5

- Wearing times of 2 hours before haze, rainbows and epithelial edema

• PROSE Device #2:

- Vault: 5.1, BC: 8.2, Power: -0.50, Diameter: 20.5

- No haze, rainbows or edema after 9 hours of wear

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PROSE Strategies: Large and Loose

• Goal to decrease hypoxia and suction

• Some keys to fitting:

- Apical touch

- Bubble management

• Size

• Placement

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Fitting Strategies – Fenestrations in Optic Zone Fitting Strategies

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PROSE Strategies: Decrease Suction

• Channels

- Radial grooves in device to disrupt the seal of the device on the globe

- Encourages fluid ventilation

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PROSE Strategies: Decrease Suction

• Channels

- Radial grooves in device to disrupt the seal of the device on the globe

- Encourages fluid ventilation

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Systemic Associations with Ectasia conditions

Down’s SyndromeAtopyConnective Tissue Disorders

Ocular Surface

The ocular surface• cornea • conjunctiva• lacrimal glands• tear film• lid margins

from Gipson, Joyce, ZieskeSmolin and Thorf’s, The Cornea, 2005

Ocular Surface Disease

• Corneal Stem Cell Deficiencies• Severe Dry Eye• Anesthetic Corneas• Exposure• Diseases of the Epidermis

Dry Eye Syndrome

Exposure Keratoconjunctivitis

Neurotrophic Keratopathy

Limbal Stem Cell Deficiency

Ocular Exposure

OS pre-lens OS 4.5 hours lens wear

GVHD

OD pre-lens OS pre-lens

OD 6 hours lens wear OS 6 hours lens wear

Stem Cell Deficiencies

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CORNEAL STEM CELL DEFICIENCIES

• Stevens-Johnson Syndrome

• Ocular Pemphigoid

• Chemical/Thermal Injuries

• Aniridia

• Radiation Keratopathy

Stevens-Johnson Syndrome

Trichiasis & Distichiasis

Stevens-Johnson Syndrome

Conjunctivalization

Stevens-Johnson Syndrome

Keratinization

Stevens-Johnson Syndrome

June 2007 November 2007

SEVERE STEVENS 

JOHNSON SYNDROME

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Neurotrophic Keratopathy

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Neurotrophic Keratopathy - A degenerative corneal condition resulting from impaired corneal sensation

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Poor healing

Decreased reflex blinking//tearing

Ulceration

Infection

Corneal melting

Perforation

Permanent vision loss

NEUROTROPHIC CORNEAS

Goals of Treatment

• Protection

• Lubrication

• Re-epithelialization

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Neurotrophic Keratopathy - Etiology varies (and is often multi-factorial)

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HSAN Type III (Familial DysautonomiaRiley Day Syndrome)

HZO

CNV and CNVII neuropathys/p resection of acoustic neuroma

Congenital

Familial Dysautonomia

HSAN

Acquired

HSV

Multiple surgeries

Medicamentosa

Neurological – HZO

Neurosurgical -Meningioma, Acoustic Neuroma, Trigeminal Ablation

Systemic – DM, Sjorgrens

Re-epithelialization of Persistent Epithelial Defects

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Exposure Keratopathy

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Exposure Keratoconjunctivitis

• Result of any condition that causes abnormal lid function or reduction in the natural blink reflex

• Most commonly associated with cases of orbital disease or cranial nerve palsy

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Causes of Ocular Surface Exposure

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Congenital Lagophthalmos

Craniofacial Trauma

Craniofacial Abnormalities

Cranial Nerve Palsy

• Acoustic neuroma

• Bell palsy

• CPEO

• Möbius syndrome

• Stroke

Infectious

Nocturnal Lagophthalmos

Orbital disease

• Graves’ disease

• Orbital tumors

Post-operative

• Botox

• Blepharoplasty

• LASIK

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Chronic Exposure

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Congenital Craniofacial Abnormality

Facial Nerve Palsy (s/p acoustic neuroma resection)

Chronic Exposure Treatment Options

Depends on underlying cause

May Include

- Aggressive Lubrication

- Punctal Occlusion

- Lid Weights

- Contact Lenses (Soft or Scleral)

- Amniotic Membrane

- Surgical Correction of Lid Function

- Tarsorrhaphy

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Complications from Chronic Exposure

• Corneal scarring

• Corneal neovascularization

• Epithelial Breakdown

• Stromal thinning

• Ulceration

• Perforation

Bad Blepharoplasty

Bad Blepharoplasty Chronic Exposure: Case 1

• 34 yo female Crouzon’s Syndrome

• Arnold Chiari Malformation

• Blepharoplasty OD

• Entropion repair OS

• s/p mucus membrane grafting OU

• Strabismus surgery OU

• Tarsorrhaphy OU –successful OD

• Failed lid weight OS

• 20/20 OD

• 20/80 OS96

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2017

H/O epithelial breakdown 2’ chronic exposure

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2009

CASE 2

• 36 yo (at the time) male soldier

• Sustained severe burns from an improvised explosive device (IED) 11 months prior to referral

• Despite multiple reconstructive surgeries chronic exposure keratopathy OU from cicatricial lagophthalmos

• OS – Corneal thinning and irregularity – 80% tarsorrhaphy to protect surface

• OD – Pt reported Sx of dryness and photophobia

• UCVA OD 20/25 20/80 OS

• PROSE Tx OD Only

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SymptomsUCVA

BCVA20/25

20/15No PAINPHOTOPHOBIA

Managed effectively A/R despite using only one hand

VAULT - CENTRALLY

What about central clearance?

Vault

• Relationship to corneal surface oxygen tension

1. Certainly less oxygen at K surface with higher vaults

• Compan et. al. 2016, Giasson, et. al. 2017

2. But doesn’t necessarily correlate with negative corneal sequelae

• Berkeley studies (UC Berkeley Clinical Research Center) don’t show a correlation between increased vault and corneal swelling

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Case #1a

• 45 year old WF• h/o RK, AK OU 1993• Salzmann Nodules removal 2009

• PROSE treatment: 2011• Vault in lenses ~ 500 um

Case #1a

2011 2017

Case #2

• Fit originally February 25, 2008

PROSE Device OD

BC: 7.90 mm

Power -1.50 D

Diam: 22.0 mm

Vault: > 500 um

PROSE device OS

BC: 7.90 mm

Power: -1.25 D

Diam: 22.0 mm

Vault: > 500 um

Diameter and Vault

• Smaller diameter certainly needs a lower vault

- Small haptic landing area cannot sustain higher vaults

- Generally smaller diameters result in a closed system, more suction

- More suction occurs in smaller diameters with less fluid exchange requiring a lower vault

• Large diameter affords more flexibility:

- Inherent toricity will result in more fluid exchange

- Can have wider range of vault

Vault

• Relationship between vault and corneal physiology needs to be established

• Even when there’s evidence that higher vault results in lower oxygen tension levels at the cornea, there may not be a direct correlation to negative effects on corneal physiology

• More studies needed

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PEDIATRIC CORNEA

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Kendra Phillis, OD, Dan Brocks, MD, and Karen G. Carrasquillo, OD, PhD, FAAO, FSLS, FBCLA

Use of PROSE treatment for traumatic  lid

ptosis  in a pediatric patient: Case Report

Submitted to OVS, Jan 2020

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Medically Necessary Scleral Lenses

Is it always about the piece of plastic?

H/O GVHD post trasplante de células

madres 2016. PROSE since 2017

H/O “steroid response”

At the time ‐ 30mg/d oral prednisone (slow taper)

Cosopt (Dorzolamide/Timolol –without preservatives) 1 gtt BID 

OU

Loteprednol ung QHS OR

Prednisolone acetate 1% 1 gtt QID OD

CASE

Graft vs Host Disease

19mm OD/OS

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2 week evaluation

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Without any revisions to the lens!

When everything seems to fail…..

Boston Keratoprosthesis Type I and II

There is always hope…

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Thank you for your attention!

www.bostonsight.org

QUESTIONS?

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