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AAO GRAND ROUNDS
Unilateral peripheral corneal ectasia following Bell’s palsy
Wednesday 12th November 2014, 9.00am – 10.00am
Dr Laura E Downie
BOptom, PhD, PGCertOcTher, FACO, FAAO, DipMus(Prac), AMusA
Department of Optometry and Vision Sciences, University of Melbourne, Parkville, VIC
Australia 3010.
The author does not have any conflicts of interest to disclose.
Case History
•
August 2013: 55-year old Caucasian male attends for an eye examination with regard
to an apparent deterioration in his vision over the past three years (OS > OD)
•
Medical history: systemic hypertension (treated with oral perindopril/amlodipine,
5mg/5mg, once daily, qd), osteoarthritis (managed with glucosamine sulphate,
1500mg, qd).
•
Family history: none.
•
Ocular history: negative for allergy, trauma or surgery. Bell’s palsy (2009) of
unknown
aetiology;
eight-week
period
of
left
facial
paralysis.
Residual
malpositioning of the left inferior eyelid, due to compromised function of the
orbicularis oculi, resulted in a mild ectropion and intermittent epiphora. The reduced
lid tension was also reported to be problematic for the patient in his occupation as a
tram maintenance technician. In this dusty workplace environment, he reported that
particulate matter would frequently enter the left cul de sac, causing irritation and
resulting in him vigorously rubbing the affected eye.
Pertinent Findings
Spectacle visual acuities (VAs) of OD: 20/20, OS counting fingers (CFs) were achieved with
a refraction of: OD +1.00/-1.75x69, OS balance.
Earlier clinical data (2007) indicated that VAs of OD: 20/20+, OS 20/20 were previously
achieved with OD +0.50/-1.75x70, OS +1.25/-1.75x80.
2
Slit lamp examination: normal biomicroscopic appearance (OD); narrow, crescentic band of
corneal stromal thinning, extending from 4 to 8 o’clock and positioned approximately two
millimetres (mm) superior to the inferior limbus (OS). Sodium fluorescein assessment
revealed moderate tear instability (OU) and a whorl-like pattern of corneal epithelial
disruption along the zone of corneal thinning (OS).
Corneal topography demonstrated significant inter-ocular asymmetryThe right cornea showed
two dioptres (D) of regular, against-the-rule astigmatism. In the left eye, corneal
topographical abnormalities were evident; flattening along the vertical meridian was
associated with mid-peripheral, inferior oblique meridians resulting in a classic ‘crab-claw’
pattern and over 14D of irregular astigmatism. Central-corneal thicknesses were within
normative ranges (OD 540µm, OS 539µm). All other aspects of the comprehensive ocular
health assessment were within normal limits.
Differential Diagnosis
Based upon the clinical signs, the most pertinent differential diagnoses are the corneal
thinning conditions keratoconus, Terrien’s marginal degeneration and pellucid marginal
degeneration (PMD). Keratoconus is unlikely due to the proximity of the corneal ectatic
changes to the inferior limbus, topographical pattern of corneal irregularity and the absence
of other pathognomonic signs, such as Vögt’s striae and a Fleischer’s ring. Terrien’s marginal
degeneration, while a peripheral corneal disease, typically induces superior corneal thinning
initially which may progress to circumferential involvement; superficial pannus is also often
present. The ‘crab-claw’ phenotype of the corneal topography and slit lamp biomicroscopic
signs are considered most consistent with pellucid marginal degeneration (PMD).
Diagnosis and Discussion
PMD is a rare, idiopathic corneal ectasia that results in progressive corneal irregularity and
reduced spectacle VA. PMD is regarded as a bilateral disease, with only a handful of
unilateral cases having been documented.3 To my knowledge, this is the first report
describing the development of unilateral peripheral corneal ectasia, consistent with the
clinical appearance of PMD, following Bell’s palsy. The aetiology of PMD is uncertain; it
has been proposed to represent a peripheral phenotype of the more common form of corneal
ectasia, keratoconus.4 Chronic habits of abnormal eye rubbing are recognised as a major risk
factor for the development of corneal ectatic disorders.5 In the case reported here, the residual
impairment in facial nerve innervation and resultant lower lid laxity are hypothesized to have
3
predisposed this patient to the entrapment of foreign matter and dust in the inferior
conjunctival sac, which would provide a stimulus for chronic rubbing of the affected eye.
Treatment and Management
A left corneo-scleral gas permeable (GP) contact lens fitting was performed. A bi-toric
custom, large-diameter (13.5mm) GP lens, was successfully fitted and enabled VA of
OS 20/25+. Within one week of commencing contact lens wear, the patient reported a
comfortable wearing period in excess of 14 hours per day. Ongoing success with this contact
lens modality continues.
Conclusion
This case is clinically significant as it demonstrates the need to monitor the ocular health of
patients with Bell’s palsy beyond the acute phase of the neuropathy, in order to ensure the
timely diagnosis and management of potential long-term ocular sequelae. The report
highlights scope for enhanced counselling with regard to the functional impact of impaired
lid tension, and the avoidance of eye rubbing, which may otherwise predispose patients to
secondary corneal degenerative disease.
References
1.
Martyn CN, Hughes RA. Epidemiology of peripheral neuropathy. J Neurol Neurosurg
Psychiatry 1997;62:310-8.
2.
Peitersen E. The natural history of Bell’s palsy. Am J Otol 1982;4:107-11.
3.
Jinabhai A, Radhakrishnan H, O'Donnell C. Pellucid corneal marginal degeneration:
A review. Cont Lens Anterior Eye 2011;34:56-63.
4.
Krachmer MJ, Feder RS, Belin MW. Keratoconus and related non inflammatory
corneal thinning disorders. Surv Ophthalmol 1984;28:293-322.
5.
McMonnies CW. Management of chronic habits of abnormal eye rubbing. Cont Lens
Anterior Eye 2008;31:95-102.
7/30/14 Case Presenta*on A Case of Recalcitrant Conjunctivitis
Erich A. Hinel, OD, MS
•  10 year old male presents with a 4
month history of a red, irritated,
and itchy right eye.
Disclosure Statement:
•  Nothing to disclose
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Unauthorized recording of this
session is prohibited.
•  Mom reports a long history
involving “many doctors” and
“many drops” with no improvement.
#aaoptom14
Initial Presentation to PCP (4 months ago).
•  Diagnosed as Red Eye – treated for 10 days with Vigamox. No
relief.
•  Then treated with Pataday resulting in no improvement
1 month later presented to ophthalmologist
•  Complaints of tearing; itching; crusting of the right eye. No
improvement with Vigamox or Pataday.
•  Diagnosed with Allergic Conjunctivitis – treated with Lotemax tid
(loteprednol etabonate) OD x 3 week.
Call to Doctor’s Office 3 weeks later:
•  Parent states: “So much better after d/c Vigamox and starting
on Lotemax -- completely resolved!”
•  Cancelled follow-up appointment
1 month later presented to a different ophthalmologist:
–  Complaints of tearing; itching; crusting of the right eye
•  Reports history of no improvement with Vigamox,
Pataday
•  Redness and symptoms return every time lotemax is
stopped
–  Diagnosed as possible medicamentosa
•  Questionable BAK Allergy – Start Lotemax Ointment
qhs (loteprednol etabonate)
3 weeks later – phone call to doctor’s office
–  No improvement with Lotemax ointment
–  Cornea consult requested
#aaoptom14
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Ophthalmic Examina*on Right Eye Le3 Eye Visual Acuity 20/25 20/20 Pupils Round, Equal, and Reac6ve to Light Round, Equal, and Reac6ve to Light Extra-‐Ocular Muscles Full, No Diplopia Full, No Diplopia Confronta*onal Visual Fields Full to Finger Count Full to Finger Count Intra-‐Ocular Pressure 16mmHg Slit Lamp Examina*on…… 17mmHg #aaoptom14
#aaoptom14
1 7/30/14 #aaoptom14
Papillae
Follicles or Papillae?? #aaoptom14
Follicles
•  Dilated central vessels with surrounding edema and
inflammation cells
•  Papillae are found only where the conjunctiva is
attached to the underlying tissue via anchoring septae
•  Round elevations of conjunctiva produced by a
lymphocytic response
•  Vessels surround the follicule, but the central portion is
avascular
–  Giant Papillae develop when anchoring septae break down
Source: American Academy of Ophthalmology: BCSC Sec6on 8 Source: American Academy of Ophthalmology: BCSC Sec6on 8 A papillary response is a rela6vely non-‐speciﬁc sign of conjunc6val inﬂamma6on #aaoptom14
Rela6vely speciﬁc inﬂammatory response with a well deﬁned diﬀeren6al diagnosis #aaoptom14
#aaoptom14
Follicles or Papillae?? #aaoptom14
2 7/30/14 Differential Diagnosis of
Follicular Conjunctivitis
Differential Diagnosis of
Follicular Conjunctivitis
Adenovirus Conjunctivitis
•  May present as epidemic keratoconjunctivitis (EKC), pharyngeal
conjunctival fever (PCF), or a non-specific follicular conjunctivitis.
•  Starts unilateral but quickly spreads to the other eye.
–  Leading cause of follicular conjunctivitis
–  Topical steroids can mask epithelial infiltrates and lengthen
duration.
•  Self limiting
Primary Herpes Simplex Virus
•  Associated with vesicular lesion of the lid margin
–  Within 2 weeks of onset, 50% of patients will develop corneal
involvement
•  Watery discharge and preaurcular lymphademopathy,
•  Self limiting
#aaoptom14
Chlamydia Conjunctivitis (Adult Inclusion)
•  Commonly unilateral and self-limiting, but can last up to 6-9 months
in untreated adults
•  Can involve mild lid swelling and mild mucopurulent discharge
•  Usually found in younger, sexually active adults
Toxic Conjunctivitis
•  Chronic exposure to topical medications, eye make-up,
environmental pollutants
–  Benzalkonium Chloride is known to contribute to punctate
keratitis and papillary reactions – but only rarely causes a
follicular response
•  Failure to recognize toxic follicular conjunctivitis can result in
progressive punctal stenosis and subconjunctival scars
#aaoptom14
Differential Diagnosis of
Follicular Conjunctivitis
Uncommon Causes of Follicular Conjunctivitis:
•  Epstein Barr Virus
–  May cause either a follicular or membranous conjunctivitis
Let’s Take a Second Look…..
•  Morexella
–  Large gram negative diplobacilli
•  Parunaud’s Oculoglandular Syndrome
–  Unilateral granulomatous conjunctivitis with a localized
follicular response
•  Lyme Disease
–  Can cause a bilateral follicular conjunctivitis and eyelid
swelling may be seen in the early stages of the disease
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Molluscum Contagiosum
•  Lesions are produced by DNA poxvirus.
•  Spread through direct contact with infected individuals, usually
in children and sexually active adults
–  Casual contact should not cause spread of the virus
–  Fomites have been suggested to be another source of the
infection
–  Disease is much more common in developing countries
•  Can involve the trunk, face, and extremi6es. •  The virus may spread to neighboring areas of skin through autoinocula6on. •  A molluscum nodule has a smooth umbilicated central core. Possible Diﬀeren*als? #aaoptom14
#aaoptom14
3 7/30/14 Molluscum Contagiosum
Molluscum Contagiosum
•  Children with atopic dermatitis are predisposed to molluscum
contagiosum due to their impaired skin barrier. Tends to be more
chronic.
(Br. K Dermatol 2004; 75: 1210-1211)
•  Acquired Immune Deﬁciency Pa6ents (HIV) •  Immunocompromised patients are susceptible to opportunistic
molluscum contagiosum which tend to be larger, more diffuse, and
recurrent.
(Am J. Ophthalmol 1997; 124; 2: 240-‐201) •  Acquired Immune Deﬁciency Pa6ents (HIV) •  Immunosuppressed Pa6ents (TNF alpha-‐an6bodies and methotrexate) (Am J. Ophthalmol 1997; 124; 2: 240-‐201) (Am J. Ophthalmol 2002; 134: 270-‐271) •  Immunosuppressed Pa6ents (TNF alpha-‐an6bodies and methotrexate) (Am J. Ophthalmol 2002; 134: 270-‐271) #aaoptom14
#aaoptom14
• Eyelid nodules release viral par6cles into the tear ﬁlm and conjunc6val sac causing a toxic or hypersensi6vity reac6on resul6ng in a follicular conjunc6vi6s • In rare and chronic cases may also cause epithelial kera66s, pannus forma6on, and conjunc6val scarring. The lesions are classically described as round, white-‐ish, domed-‐shaped nodules, 2-‐3 mm in diameter. Characteris6cally have an umbilicated center. #aaoptom14
Treatment of Molloscum Contagiosum
Eyelid is the most common site for lesions resul6ng in ocular sequelae, but there are reports of: • Epibular Molluscum Conjunc6vi6s (Am J. Ophthalmol 1998; 125: 394-‐396) • Intraocular Molluscum Contagiosum ader Corneoscleral Lacera6on (Am J. Ophthalmol 1997; 124: 560-‐561) • Molluscum Contagiosum of the Palpebral Conjunc6va #aaoptom14
Management • 
In immuncompetent host, molluscum contagiosum is self limiting with resolution in 3 to
12 months – sometimes longer.
•  Con6nue with Lotemax ointment qhs for now. • 
Molluscum present elsewhere on the body may not require treatment if asymptomatic or
if only a few lesions are present.
–  Most physicians recommend treatment should be initiated at the time of diagnosis to
decrease autoinoculation and spread of the infection
•  Oculoplas6c consult requested for removal and biopsy. • 
Treatment of Molluscum Contagiosum:
–  Excision
–  Cautery (thermal or chemical w/ 10% potassium hydroxide)
–  Cryotherapy
–  Topical 5% Imiquimod Cream
•  FDA has ruled this treatment as ineffective
–  Tea Tree Oil (melaleuca alternifolia) and betadine
–  Cantharidin
•  Derived from blister beetles – “beetle juice”
Pathology Results #aaoptom14
#aaoptom14
4 7/30/14 Molluscum Contagiosum - Pathology
Ophthalmic Mollusum Contagiosum: Clinical and
Immunopatholgic Features
Bri6sh Journal of Ophthalmology 1995; 79:476-‐481 •  A retrospective study from the Manchester Royal Eye Hospital
from 1981-1993 (35 cases).
–  60% percent of cases (21) were diagnosed at the original visit
(although 14 were referred for an eyelid lesion).
•  The other 14 cases took as few as 1 and as many as 7
visits to make the diagnosis (mean of 3.1 -- a delay of 11.1
weeks).
#aaoptom14
Cl and Exp. Optometry 89.6 (2006): 390-‐93. •  Duration of symptoms were 1 week to 18 months.
–  19 patients (54%) had a conjunctivitis (although only identified
as follicular conjunctivitis in 13 patients)
•  11 of these patients also had corneal involvement
•  Subepitheal opacities in 3 cases
#aaoptom14
Follow-‐up •  Patient presents for 4 month followup s/p excision of molluscum lesions
(x2) from right upper lid.
•  “Much better, but eye is starting to
become occasionally itchy and red
over the last month.”
•  Slit Lamp Examination….
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•  Recurrences can
appear 6-8 weeks after
removal of the lesion.
•  “A topical lesion begins
small, round, and firm.
The virus replicates in
the epithelial cells as
the lesion grows.
Cellular destruction
eventually occurs
resulting in central
umbilication.”
#aaoptom14
#aaoptom14
5 7/30/14 References:
Follow-‐up •  Removal and biopsy confirms
molluscum lesion.
•  Patient will have to be followed closely
for recurrence.
1.  Krachmer, Jay H., Mark J. Mannis, and Edward J. Holland. Cornea. Philadelphia: Elsevier/Mosby, 2005. Print. 2.  Basic and Clinical Science Course 2013-‐2014. [San Francisco, Calif.]: American Academy Of Opthalmology, 2013. Print. 3.  Charteris DG, Bonshek RE, Tullo AB. Ophthalmic Mollusum Contagiosum: Clinical and Immunopatholgic Features: Bri6sh Journal of Ophthalmology 1995; 79:476-‐481 4.  Ingraham HJ, Schoenleber DB, Epibular Molluscum Conjunc6vi6s Am J. Ophthalmol 1998; 125: 394-‐39 5.  Ryan EH Jr, Cameron JD, Carpel E. Intraocular Molluscum Contagiosum ader Corneoscleral Lacera6on Am J. Ophthalmol 1997; 124: 560-‐561 6.  Ophthalmic Atlas Images by EyeRounds.org, The University of Iowa 7.  American Academy of Dermatology: hjp://www.aad.org 8.  Markum E, Baillie J (2012). "Combina6on of essen6al oil of Melaleuca alternifolia and iodine in the treatment of molluscum contagiosum in children". J Drugs Dermatol.v11 (3): 349–54. 9.  Schornack, Muriel M., Dennis W. Siemsen, Elizabeth A. Bradley, Diva R. Salomao, and Harold B. Lee. "Ocular Manifesta6ons of Molluscum Contagiosum." Clinical and Experimental Optometry 89.6 (2006): 390-‐93. 10.  Hason D, Diven DG “Molluscum contagiosum” Dermatology Online Journal 2003 9:2 11.  Lerbaek A, Agner T. Facial erup6on of molluscum contagiosum during topical treatment of atopic derma66s with tacrolimus. Dr, J Dermatol, 2004; 150: 1210-‐1211 #aaoptom14
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6 2014‐07‐31
Disclosure
A unique case of viral infection leading to
neurotrophic cornea
Dr Langis Michaud O.D. M.Sc. FAAO (Dipl) FSLS, FBCLA
Professor
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Subjective
• 47 years old Caucasian Female
• Referred by her cornea specialist for c.l.
fitting to improve her visual acuity
• Complaints of blurred vision, discomfort
and eye dryness
Subjective (3)
• Had consulted a cornea specialist is 2012
– Because of her ophthalmologist retirement
– The new MD ordered c.l. cessation and put her
on steroids medication x 2 years (tapered to DIE
in 2014)
•
•
•
•
Increased scarring
Neovascularization
Instructed her to consult for c.l. refits
Corneal graft is not an option
• Honorarium /Speaker fees / Research Grants
–
–
–
–
–
Alcon / Ciba
B&Lomb (Valeant)
Cooper Vision
Blanchard Labs
Genzyme Canada
Subjective (2)
• Reported severe bilateral keratitis (>15 years
ago)
– Viral infection and/or contact lens abuse as a
potential cause (unkonown diagnosis)
– Left both corneas highly irregular
– Known presence of corneal scarring
– Fitted thereafter by a general ophthalmologist
• « homemade » hydrid lenses
– Low DK – poor wettability
Visual needs
• Visual acuity in glasses are OD 20/80 OS 20/120
• Under medical leave since 2012
– Before, was an executive assistant
• Computer use (>8h00/day)- actually very limited)
• Systemic Hx: no other medication, no pain killer
• Family hx: negative
1
2014‐07‐31
Objective
Slit lamp
• BCVA
– -2,00 -1,75 x 30 OD 20/80 PH 20/30
– -2,75 -2,25 x 115 OS 20/120 PH 20/50
• DFE
– Normal retinal tissue OU
Topo maps
• Very THIN (inferiorly) and Irregular corneas
C.L. Fitting goals
• To restore v.a. by compensation of the
corneal irregular surfaces
• To maintain high level of oxygen delivery
• Compatibility with topical medication
• To contribute to cornea healing over time
OS‐ OD is even thinner (276 um)
C.L. options for irregular corneas
• RGPs
– Small diameter
• With or without piggy-back system
Trials
• Small RGP in Menicon Z
– To minimize impact on corneal health
– Not tolerated because of discomfort, even with a piggy
back system (DD- SIHy)
• Patient did not want to handle 2 types of lenses
– Mini/ Full sclerals
• Hybrids
– High DK
• Customized soft lenses
• Hybrids
– Patient was sceptic about the long-term outcome
– Negative past experience with hydrid lenses
– Not aimed to restore neurotrophic corneas as sclerals do
2
2014‐07‐31
Trials
• Mini-scleral lenses
– Preferred to full scleral to increase oxygen
delivery to the corneal tissue
– Fitted with the use of elevation map
(Medmont)
– Diameter of 14.9 mm selected
– Optimal initial clearance targetted @ 180 um
CL design
• ONE FIT P&A (Blanchard
Labs)
– BFS + 0.6 mm
– Initial clearance optimized with
OCT
– Final parameters
BC 6.7 Diam 14.6
Power -9.00 OU
Boston XO2
#aaoptom14
Delivery
• Over-refraction
–
–
–
–
OD +0,25 -1, 50 x 45 20/25
OS +0,25 – 1,50 x 25 20/30
Add : +1,75
Provided in progressive add glasses
Follow-ups (2012-2014)
• Over the weeks, was able to reach 8h00/day OD
and 6h00/day OS
• Resume working- part time then full time
• Corneal condition improved over the months
• Wear schedule
– 2h00 / day with an increase of 1h00/day every week
– Prednisolone acetate 1% switched for loteprednol
0.5% DIE before lens wear
Follow-up
• In December 2013, viral infection flared up
OD
– Good clinical response with trifluridine 1%
– Resume contact lens wear x 2 wks
– Loteprednol 0.2 % ceased
• OS did the same July 2014
– Same clinical approach
– Considered oral anti-viral medication but cornea specialist
delayed this option
• IOP well controlled
– Loteprednol 0.5% switched for 0.2 % after 3 months.
• Cyclosporine A introduced with success June 2013
Long-term
• Over 24 months
– Corneal tissue restoration
• Reduced irregularity, scarring and neovasc.
– Visual acuity improved
– Corneal thickness remained unchanged
– Viral infection remains an issue
• Needs regular follow-up
3
2014‐07‐31
Right eye
October 2012
October 2012
Left Eye
July 2014
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July 2014
Ghost vessels
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Conclusion
• Mini-scleral contact lenses can help to
restore visual acuity and to heal altered
corneal tissue
– Associated with more oxygen delivery
– Handling is not a problem for someone used
to hybrids
– Can be used in conjunction with topical meds
– Easiest to fit and wear
4