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Acute Anterior Uveitis with HypopyonRyan Ngo, O.D.
Spokane Mann-Grandstaff VA Medical Center
1
Disclosure
´ The presenter and Organizer for:
´ “Acute Anterior Uveitis with Hypoypon”
´ By Dr. Ryan Ngo has no financial relationship with any company or productsmentioned in this presentation.
2
Case Study
´ 29 year old Caucasian male walk in visit
´ CC: Significant left eye pain (8/10) that started 3 days ago
´ Associated symptoms: Significant photosensitivity and blurred vision
3
Ocular History
´ Patient’s Ocular history
´ Anterior uveitis OS x 2018 – Initial
´ Anterior uveitis OD x 2019 – work up ordered, but not completed
´ Medical history´ History of uncontrolled Type 1 diabetes , Last A1C: 12.5%
´ Erectile dysfunction
´ Eczema
´ Medication
´ Insulin, sildenafil, triamcinolone acetenoid 0.1% cream for eczema
4
Patient’s 2019 Lab Work Up
Test ResultsHLA b27 NegativeRheumatoid Factor NegativeReactive plasma regain (RPR)
Non-reactive
Angiotensin converting enzyme (ACE)
55
QuantiFERON Negative
5
Examination
´ Entrance Examination:
´ VA: 20/20 OD, 20/60 OS PH: 20/25
´ IOPs: 13mmHg OD, 15mmhg OS
´ Pupils: PERLL (-) APD
´ Slit Lamp Examination OS:´ Conjunctiva: 3-4+ conjunctival and episcleral
injection
´ Cornea: 2+ diffuse fine keratic precipitates
´ A/C: 4+ Cell and 3+ Flare with 500um hypopyon
´ Dilated Fundus Examination:
´ Unremarkable; no vitritis or macular edemahttps://www.aaojournal.org/article/S0161-6420(03)01745-7/pdf
6
Assessment/Plan
´ Assessment: ´ Recurrent Nongranulomatous Anterior Uveitis OS
´ Plan´ Significant improvement in symptoms (pain and photosensitivity) with loading
dose of 1gtt Pred Forte every 5 minutes for 30 minutes in office
´ Prescribed Pred Forte Q1H and atropine BID while awake
´ RTC 1 week for follow up
7
Anterior Uveitis
´ Inflammation of the anterior chamber
https://www.google.com/url?sa=i&url=http%3A%2F%2Fwww.ishwareyecentre.com%2Fuveitis%2F&psig=AOvVaw0fKnB-t-qoo7uO11f--ZM5&ust=1588213479819000&source=images&cd=vfe&ved=0CAIQjRxqFwoTCJCi07XKjOkCFQAAAAAdAAAAABAI
8
Anterior Uveitis: Epidemiology
´ 5th leading cause of blindness in the worldwide
´ Most common inflammation eye practitioners will see
´ 15/100,000 affected, 45,000 new cases every year
´ Common between second and fourth decades
´ 50% Idiopathic, HLA b27 is the most common type
9
Anterior Uveitis: Pathophysiology
´ Break down of blood aqueous barrier ´ Releases cells and flares
´ Keratic Precipitates´ Hypopyon
´ TM damage
´ Cataract´ Macular Edema
´ Iris becomes swollen ´ PAS & AS à Secondary glaucoma
´ Other´ Iris changes (Atrophy, heterochromia,
koeppe/busacca nodules)´ Circumlimbal injection
´ Fibrinous materials
https://www.researchgate.net/profile/Jithesh_Sivadasan/publication/303802215/figure/fig1/A S:369255934644225@ 1465048883280/M ain-parts-of-the-hum an-eye-Source-wwweyediologyoptic ianscouk.png
10
Acute Anterior Uveitis: Symptoms
´ Symptoms´ Pain
´ Redness
´ Photophobia
´ Lacrimation
´ Mild decreased vision
11
Hypopyon
´ Indication of severe inflammation inthe anterior chamber
´ Consist of tissue debris, inflammatorybyproducts, and recruited leukocytes
´ Uncommon finding in uveitis
h t tp s :/ / e n .w ik ip e d ia .o rg / w ik i/ H y p o p y o n
12
Types of Hypopyons
https://webeye.ophth.uiowa.edu/eyeforum/atlas/pages/leukemic-pseudohypopyon.htmhttp://www.willseyeonline.org/Media/ViewCourse.aspx?id=2804PowerPoint slide presented by Dr. Dunn, M.D., at Wills Eye Hospital CME event on May 2019.https://www.intechopen.com/books/advances-in-the-diagnosis-and-management-of-uveitis/behcet-s-disease
13
Classification of Causes of Hypopyon by Ramsay
Ramsay A., Lightman S. Hypopyon Uveitis. Surv Ophthalmol. 2001;46(1):1-18. Doi: 10.1016/S0039-6257(01)00231-4
14
Differential Diagnosis: HLA-b27 uveitis ´ HLA-b27 antigen is a class I major histocompatibility complex
´ Second most common noninfectious etiology of anterior uveitis up to 50%acute anterior uveitis with 70% recurrence
´ Signs more severe in presentation
´ Ages 20-40 years of age
´ 2.5x F>M
´ Usually recurrent unilateral, bilateral, or alternating nongranulomatousanterior uveitis and may have fine endothelial KPS
15
HLA-b27 associated diseases
´ Ankylosing Spondylitis (80%)
´ 25% risk anterior uveitis
´ Reactive arthritis´ 12% risk of anterior uveitis
´ Inflammatory bowel disease (Crohn’s & ulcerative)´ 5-10% risk
´ Psoriatic arthritis´ 7% risk of anterior uveitis
16
Differential Diagnosis: Bechet's Disease ´ Chronic, recurrent, and multisystem
mucocutaneous inflammatory disorder
´ Classic triad signs: genital/oralulcers, skin lesions, iritis
´ Found along ancient silk road extending from eastern Asia to the Mediterranean basin
´ 10-15% of patients, uveitis is the initialmanifestation of the disease
´ Affect ages between 20-40s
https://w w w .celgene.com /behcets-sym ptom s/
17
Bechet's Uveitis with Hypopyon
´ Usually presents with acute nongranulomatous anterior and/or posterior uveitis
´ About 19-31% cases present with hypopyon
´ Considered as “cold” hypopyon
´ Shifts with head positioning
´ *HLA hypopyon considered as “hot” sticky
´ Hypopyon forms and dissolves rapidly
18
Infectious causes of hypopyon
´ Toxocariasis
´ Syphilis
´ Leprosy
´ Herpetic uveitis
´ Tuberculosis uveitis
19
Diabetic Anterior Uveitis
´ Several study suggest patients with diabetes have a higher incidence ofacute uveitis, while those with type 1 and poor glycemic control are at higher risk of developing eye inflammation
´ Studies have shown positive correlation between hyperglycemia andinflammation in anterior chamber in patients with anterior uveitis
´ Behaviors of uveitis in these patients is more aggressive and occurs moreoften bilaterally
20
When to order lab work up ´ Uveitis unresponsive to steroids
´ Bilateral
´ Alternate, Recurrent
´ Positive review of system or systemicexamination
Hua, Len V., Yudcovitch, Lorne B. Anterior Uveitis: Teaching Case Reports. Optometric Education, 36 (2), 2011.
21
Lab work up Lab test Diagnosis Colonoscopy Inflammatory bowel disease
(Chrons & ulcerative) Chest X-ray Sarcoidosis & TB Sarcoiliac X-Ray Ankylosing spondylitis HLA-B27 Inflammatory Bowel disease
(Chrons & ulcerative) Reactive arthritis Psoriatic Arthiritis
HLA-B51 Behcet’sANA Juvenile Idiopathic Arthritis PPD/Quantiferon TubercolosisRPR/VDRL & FTA-ABS/TPPA/MHA-TP or dark field microscopy
Syphillis
ELISA Lyme Disease CBC General health status
(anemeia, infection, leukemia) CRP Determine cause or location of
inflammation of the body ESR With CRP, detect
inflammation, serves as a monitor for underlying etiology
https://www.aoa.org/documents/optometrists/CPG-7.pdf)
22
Goals for managing Acute Anterior Uveitis ´ 1.) Aggressively resolve ocular inflammation to prevent potential of visual
loss & relieve ocular pain
´ Prevent PAS and prevent and/or break PS
´ Reduce risk of recurrence
´ 2.) Determine underlying systemic etiology and when indicated, makeappropriate referral for evaluation and treatment of condition
23
Conventional Treatment for Acute Anterior Uveitis ´ 1. Topical corticosteroids
´ Prednisolone acetate 1%
´ Difluprednate 0.05%
´ 2. Cycloplegics´ Atropine 1% BID & cyclopentolate 1% TID
´ Homatropine and scopolamine
´ *Treat any infectious etiologies
24
Immunosuppressants ´ Indications:
´ Noninfectious Uveitis
´ Mainly for recalcitrant cases that are unresponsive to conventional therapy
´ Autoimmune diseases (HLA-B27 associated uveitis, Bechet’s,…)
https://www.retina-specialist.com/article/when-to-consider-systemic-treatments
25
Adalimumab (Humira, AbbVie) ´ Biologic proteins ´ Tumor necrosis factor (TNF) blocker
´ specific source of inflammation that appears to have a role in uveitis
´ FDA approved in 2016
´ By blocking it, the inflammatory effect of uveitis is reduced
´ Requires baseline screening for tuberculosis and hepatitis, should be avoided in patientswith demyelinating disorders
´ Cost: $4,500 per month
26
Follow up #1
´ CC: worsening vision OS, but improvement in redness & pain
´ Current ocular medications: Pred Forte Q1H and Atropine BID only
27
Follow up #1: Examination
´ Entrance Examination:
´ VA: 20/20 OD, 20/320 PHNI OS
´ IOPs: 11mmHg OD, 29mmhg OS
´ Pupils: dilated
´ Slit Lamp Examination OS:´ Conjunctiva: 3+ diffuse injection greater
circumlimbal
´ Cornea: 2+ diffuse fine keraticprecipitates
´ A/C: 2+ Cell 1+ Flare, (-) hypopyon,significant exudative fibrinous materials over visual axis
´ Iris: 10:00 large exudative fibrinmembrane protruding base
https://www.aaojournal.org/article/S0161-6420(03)01745-7/pdf
28
Assessment/ Plan
´ Assessment: ´ Alternating recurrent non-granulomatous anterior uveitis, OS
´ DDX: HLA-b27,, Bechet's, Sarcoidosis, TB, Syphilis, Diabetes
´ Plan:
´ Switched to Durezol q1h while awake
´ Continue Atropine
´ Rxed brimonidine BID to assist with elevated IOPs
29
Follow up visits #2: day 7´ Assessment:
´ Alternating recurrent non-granulomatous anterior uveitis, OS
´ Significant improvement in symptomsVA 20/100
´ 2+ Cell with fibrin membrane overpupil but no hypopyon, superiormembrane resolved
´ Plan:´ Continue Durezol hourly, brimonidine
bid &, prescribed Tobradex ung qhs´ Ordered lab work up: Chest X-ray &
blood draws
30
Follow up visits #3: day 10
´ Assessment: Alternating recurrent non-granulomatous anterior uveitis, OS
´ Significant improvement in symptoms VA 20/40
´ 1+ Cell with small amount of fibrin membrane over pupil but no hypopyon
´ States he has issues with dry skin, but no actual lesions. Has had one mouth ulcer butthat was when he had a wisdom tooth abscess
´ Plan:
´ Continue Durezol hourly, brimonidine bid &, continue tobradex ung qhs
´ Did not get chest x-ray or labs, recommended lab work up today
´ RTC 1 week for follow up
31
Follow up visits
´ Cancelled and no showed 4x
32
Conclusion
´ Hypopyon uveitis is an uncommon occurrence in uveitis
´ Result of inflammatory, infective, neoplastic, or therapeutic stimuli
´ Run appropriate lab work
´ Treat aggressively with topical corticosteroids and cycloplegics
´ Immunosuppressants remains a mainstay in management of severe uveitis
´ Requires close communication and co-management with rheumatologist
33
Special Thanks
´ Doctors of Spokane VAMC
´ Dr. Jeffrey Urness
´ Dr. Chad Gosnell
´ Dr. Len Koh
´ Dr. Megan McChesney
´ Dr. Tom Kollodge
´ Dr. Lindsay Kleinschmit
´ Dr. Anna Wells
´ Dr. Allison Makadia
34
References
´ Hua, Len V., Yudcovitch, Lorne B. Anterior Uveitis: Teaching Case Reports. Optometric Education, 36 (2), 2011. ´ Watanabe T, Keino H, Nakayama K, Taki W, Echizen N, Okada AA. Clinical features of patients with diabetic anterior uveitis. Br J Ophthalmol. 2019;103(1):78-82. doi:10.1136/bjophthalmol-2017-311453´ Bartlett, Jimmy D., Jaanus, Siret D. Clinical Ocular Pharmacology. Boston: Butterworth, 2008´ Sowka, Joseph W., Kabat, Alan G. Master Uveitis Prescribing. Review of Optometry October 2017; 110-111. ´ Duica, I., Voinea, L. M., Mitulescu, C., Istrate, S., Coman, I. C., & Ciuluvica, R. (2018). The use of biologic therapies in uveitis. Romanian journal of ophthalmology, 62(2), 105–113.´ Kopplin, L. When to consider systemic treatments (2020). Retinal Specialist A publication by Review of Ophthalmology, 6(3).´ Ramsay A., Lightman S. Hypopyon Uveitis. Surv Ophthalmol. 2001;46(1):1-18. Doi: 10.1016/S0039-6257(01)00231-4´ Lambert J, Wright V. Eye inflammation in psoriatic arthritis. Annals of the Rheumatic Diseases, 1976;35(4):354-6.´ Friedman, N. Kaiser, J. The Massachusetts Eye and Ear Infirmary. 3rd edition. Elsevier, 2009. ´ D'Alessandro, L. P., Forster, D. J., & Rao, N. A. (1991). Anterior uveitis and hypopyon. Transactions of the American
Ophthalmological Society, 89, 303–311.
35
Symblepharon: A Case StudySymblepharon: A Case Study
Lora Cretella, O.D.
Spokane VAMC
June 12th, 2018
Lora Cretella, O.D.
Spokane VAMC
June 12th, 2018
Course ObjectivesCourse Objectives
1. Recognize various clinical presentations of symblepharon
2. Estimate risk of progression and sight loss based on clinical history and presentation
3. Understand mechanism of symblepharon formation and most common underlying conditions
4. Know appropriate treatment and referral
5. Explain different treatment courses which might be employed
1. Recognize various clinical presentations of symblepharon
2. Estimate risk of progression and sight loss based on clinical history and presentation
3. Understand mechanism of symblepharon formation and most common underlying conditions
4. Know appropriate treatment and referral
5. Explain different treatment courses which might be employed
DisclosuresDisclosures
The presenter and organizers for “Symblepharon: A Case Study” by Dr. Lora Cretella has no financial relationship with any company or products mentioned in this presentation
The presenter and organizers for “Symblepharon: A Case Study” by Dr. Lora Cretella has no financial relationship with any company or products mentioned in this presentation
The PatientThe Patient
CC: 74 yo WM presents for DFE F/U
POH: CE/IOL 7/2019 OU , H/O Drance Heme
PMH: DM, Coronary Artery Disease, BPH, Hypertension, Dyslipidemia, Colon Polyps
FMH: Unremarkable
FOH: Unremarkable
SH: Unremarkable
CC: 74 yo WM presents for DFE F/U
POH: CE/IOL 7/2019 OU , H/O Drance Heme
PMH: DM, Coronary Artery Disease, BPH, Hypertension, Dyslipidemia, Colon Polyps
FMH: Unremarkable
FOH: Unremarkable
SH: Unremarkable
The PatientThe Patient
Meds:
AT’s Refresh + Genteal
Aspirin 81 mg
Atorvastatin
Hydrochlorothiazide
Losartan
Metformin
Metoprolol
Triamcinolone cream
Allergies - PCN , Fosinopril
A1C: 6.7
Meds:
AT’s Refresh + Genteal
Aspirin 81 mg
Atorvastatin
Hydrochlorothiazide
Losartan
Metformin
Metoprolol
Triamcinolone cream
Allergies - PCN , Fosinopril
A1C: 6.7https://www.airforcemedicine.af.mil/News/Art/igphoto/2001267649/
The ExamThe Exam
BCVA: OD: 20/20-- OS: 20/20--
IOP: OD: 15 mmHg OS:15 mmHg
CVF: FTFC OU
PUPILS: PERRL
EOM: FROM
BCVA: OD: 20/20-- OS: 20/20--
IOP: OD: 15 mmHg OS:15 mmHg
CVF: FTFC OU
PUPILS: PERRL
EOM: FROM
https://www.airforcemedicine.af.mil/News/Art/igphoto/2001267649/
Anterior Segment ExamAnterior Segment Exam
FINDING OD OS
ADNEXA: Unremarkable
LIDS: Mild ectropion, complete voluntary closure
CONJ: White and quiet
Fornix shortened temporally
Symblepharon of palpebral conjunctiva near lid margin and inf temp bulbar conjunctiva
Associated inf temporal subconjunctival bulbar (3-6 o’clock) and palpebral hemorrhage
CORNEA: Clear
ANT CHAMBER: D/Q
IRIS: Flat
LENS: PCIOL Clear + Centered
Posterior Segment ExamPosterior Segment Exam
FINDING OD OS
C/D: 0.40/0.45
NERVES: distinct margins, good color
MACULA: flat, even pigmentation
POSTERIOR POLE: unremarkable
A/V RATIO: 2/3
PERIPHERY: flat 360, no holes/tears/detachments
VITREOUS: PVD Grossly Clear
BLOOD PRESSURE:
RAS: 196/88 @ 954 am *Patient reports not taking his 9am pill today*
RAS: 177/82 @ 1058 am *after Instructed to take hypertensive med*
BLOOD PRESSURE:
RAS: 196/88 @ 954 am *Patient reports not taking his 9am pill today*
RAS: 177/82 @ 1058 am *after Instructed to take hypertensive med*
AssessmentAssessment
1. Symblepharon OS>OD
New
Asymptomatic
First encounter
Unclear underlying etiology
1. Symblepharon OS>OD
New
Asymptomatic
First encounter
Unclear underlying etiology
2. Subconjunctival Hemorrhage
New
Asymptomatic
Likely HTN related
Potentially related to symblepharon formation
2. Subconjunctival Hemorrhage
New
Asymptomatic
Likely HTN related
Potentially related to symblepharon formation
PlanPlan
1. Symblepharon
PredForte QID OU x 10 days then BID until F/U
RTC 1 month w/ in house Ophthalmologist
Pt ed on condition and need for F/U testing
1. Symblepharon
PredForte QID OU x 10 days then BID until F/U
RTC 1 month w/ in house Ophthalmologist
Pt ed on condition and need for F/U testing
2. Subconjunctival Hemorrhage
Pt ed on blood pressure today, risks of uncontrolled BP, and need for PCP consult.
PCP notified.
Educated on expected resolution.
RTC if not resolving.
2. Subconjunctival Hemorrhage
Pt ed on blood pressure today, risks of uncontrolled BP, and need for PCP consult.
PCP notified.
Educated on expected resolution.
RTC if not resolving.
PlanPlan
3. HVF / Pachymetry / Glaucoma Work up.
4. Monitor
5. Monitor Annually
6-7. Cont AT’s QID.
3. HVF / Pachymetry / Glaucoma Work up.
4. Monitor
5. Monitor Annually
6-7. Cont AT’s QID.
3. H/O Drance heme OD
4. Pseudophakia
5. Diabetes Mellitus w/ no retinopathy
6. Ectropion OU
7. Dry Eye
3. H/O Drance heme OD
4. Pseudophakia
5. Diabetes Mellitus w/ no retinopathy
6. Ectropion OU
7. Dry Eye
Follow up #1 -- FindingsFollow up #1 -- Findings
All findings stable/resolution of subconjunctival hemorrhage.
Inferior symblepharon OU
OD: broad sheet temporally
OS: 2 separate strands temporal and nasal
Subconjunctival hemorrhage resolved
All findings stable/resolution of subconjunctival hemorrhage.
Inferior symblepharon OU
OD: broad sheet temporally
OS: 2 separate strands temporal and nasal
Subconjunctival hemorrhage resolved
PhotodocumentationPhotodocumentation Follow up #1 -- AssessmentFollow up #1 -- Assessment
Symblepharon OS>OD
Asymptomatic
no intervening trauma other than cataract surgery
no other skin changes to suggest SJS
referral to corneal specialist for biopsy to look for ocular cicatricial pemphigoid
Symblepharon OS>OD
Asymptomatic
no intervening trauma other than cataract surgery
no other skin changes to suggest SJS
referral to corneal specialist for biopsy to look for ocular cicatricial pemphigoid
Top differentialsTop differentials
Traumatic scarring secondary to cataract surgery
Progressive cicatrization due to ocular pemphigoid
Cicatrization due to medication reaction
Traumatic scarring secondary to cataract surgery
Progressive cicatrization due to ocular pemphigoid
Cicatrization due to medication reaction
https://www.reviewofophthalmology.com/article/intravitreal-injections-safety-guidelines
ConjunctivaConjunctiva
Mucous membrane covering the globe and inner eyelids
Clear tissue
Contains goblet cells
Mucous membrane covering the globe and inner eyelids
Clear tissue
Contains goblet cells
https://europepmc.org/article/PMC/4049531
FornixFornix
Caucasians:
15.6 mm upper
10.9 mm lower
(~0.3 mm less for females)
Progressive decline with age
Caucasians:
15.6 mm upper
10.9 mm lower
(~0.3 mm less for females)
Progressive decline with age
https://www.sciencephoto.com/media/703697/view/conjunctiva-illustration-illustration
SymblepharonSymblepharon
Adhesion of palpebral to bulbar conjunctiva
Can eventually involve cornea
Adhesion of palpebral to bulbar conjunctiva
Can eventually involve cornea
https://www.ophthalmologyreview.org/articles/causes-of-symblepharon
Signs of Conjunctival Scarring
Visible adhesions, conjunctival fibrosis, and fornix shortening
https://www.reviewofoptometry.com/article/corneal-manifestations-of-systemic-diseases
https://entokey.com/fibrosing-conjunctivitis/
https://link.springer.com/chapter/10.1007/978-3-319-23754-1_15
SymptomsSymptoms
EARLY:
Often none
Nonspecific: redness, stinging, dryness, watering
(Goblet cells affected)
LATE:
Restriction of eye movements
Poor cosmesis
Reduced vision
Discomfort from eyelid malposition
ComplicationsComplications
Dry eye
Injection
Inadequate blinking
Eyelid malposition / entropion /ectropion
Restricted range of motion
Corneal involvement w/ reduced vision
Dry eye
Injection
Inadequate blinking
Eyelid malposition / entropion /ectropion
Restricted range of motion
Corneal involvement w/ reduced vision
https://www.drballitch.com/uncategorized/entropion
https://www.thegeniusprof.com/types-and-treatment-of-symblepharon/
Predicting?Predicting?
The more conjunctival area injured, the more likely
More common in areas in apposition
More likely inferior, can be superior
The more conjunctival area injured, the more likely
More common in areas in apposition
More likely inferior, can be superior
https://www.semanticscholar.org/paper/Ocular-surface-reconstruction-with-keratolimbal-for-Farid-Lee/02fff44fbb29337a5b4732c8a6fd6bbea84c2178/figure/2
Important clinical questionsImportant clinical questions
Underlying cause?
Systemic or ocular?
Self limiting vs. progressive?
Is this a blinding condition?
Surgical intervention?
Underlying cause?
Systemic or ocular?
Self limiting vs. progressive?
Is this a blinding condition?
Surgical intervention?
http://eyerounds.org/cases/192-Stevens-Johnson.htm
CategorizingCategorizing
Self limited
Chemical/thermal burns
Infectious diseases
Adenovirus
Herpes virus
Chlamydial conjunctivitis
Self limited
Chemical/thermal burns
Infectious diseases
Adenovirus
Herpes virus
Chlamydial conjunctivitis
Progressive
“Cicatrizing conjunctivitis”
Mucous Membrane Pemphigoid (MMP)
Stevens-Johnson syndrome (SJS)
Toxic Epidermal Necrolysis (TEN)
Progressive
“Cicatrizing conjunctivitis”
Mucous Membrane Pemphigoid (MMP)
Stevens-Johnson syndrome (SJS)
Toxic Epidermal Necrolysis (TEN)
Rarely…Rarely…
Atopic keratoconjunctivitis (AKC) Ocular rosacea Lichen planus Lupus
Neoplastic growth Host vs. graft disease Sarcoid Sjogren's Ectodermal dysplasia Porphyria cutanea Xeroderma pigmentosum Squamous papilloma of the conjunctiva
Atopic keratoconjunctivitis (AKC) Ocular rosacea Lichen planus Lupus
Neoplastic growth Host vs. graft disease Sarcoid Sjogren's Ectodermal dysplasia Porphyria cutanea Xeroderma pigmentosum Squamous papilloma of the conjunctiva https://eyecyte.com/causes-and-treatment-of-symblepharon/
Ocular BurnsOcular Burns
7.7-18% of all ocular trauma
Alkali more penetrating and traumatic (ammonia, lye)
Superior burn can still permeate inferior damage
Healing takes weeks. Occurs in phases.
Conjunctival sac volume decreases
Fornix shortens
Symblepharon in late phase healing, 3-4 weeks
Larger defect more necrosis delayed epithelization
more extensive scarring
7.7-18% of all ocular trauma
Alkali more penetrating and traumatic (ammonia, lye)
Superior burn can still permeate inferior damage
Healing takes weeks. Occurs in phases.
Conjunctival sac volume decreases
Fornix shortens
Symblepharon in late phase healing, 3-4 weeks
Larger defect more necrosis delayed epithelization
more extensive scarring
https://www.nature.com/articles/s41598-019-50286-x
“Cicatrizing Conjunctivitis”“Cicatrizing Conjunctivitis”
Progressive form of conjunctival scarring associated with complications
Most commonly Mucous Membrane Pemphigoid (60%)
Can be many etiologies, but most commonly linked to OCP, SJS, TEN
Progressive form of conjunctival scarring associated with complications
Most commonly Mucous Membrane Pemphigoid (60%)
Can be many etiologies, but most commonly linked to OCP, SJS, TEN
https://www.reviewofoptometry.com/article/an-atlas-of-conjunctival-and-scleral-anomalies
Mucous Membrane Pemphigoid (MMP)Mucous Membrane Pemphigoid (MMP)
Same as Ocular Cicatricial Pemphigoid (OCP)
60-80% present with ocular /conjunctival involvement
Subset of systemic autoimmune pemphigoid diseases
Affects all mucous membranes :
genital, oral, ocular
Same as Ocular Cicatricial Pemphigoid (OCP)
60-80% present with ocular /conjunctival involvement
Subset of systemic autoimmune pemphigoid diseases
Affects all mucous membranes :
genital, oral, ocular
https://www.dentalcare.com/en-us/professional-education/ce-courses/ce541/mucous-membrane-pemphigoid-mmp
https://ostrowon.usc.edu/2019/03/26/mucous-membrane-pemphigoid/
Mucous Membrane Pemphigoid (MMP)Mucous Membrane Pemphigoid (MMP)
No racial predilection
Onset age 60-80
2:1 women: men
HLA-DR4 gene increases susceptibility
diagnosis often delayed due to unfamiliarity
No racial predilection
Onset age 60-80
2:1 women: men
HLA-DR4 gene increases susceptibility
diagnosis often delayed due to unfamiliarity https://www.researchgate.net/figure/a-Involvement-of-conjunctivae-in-mucous-membrane-pemphigoid-before-application-of_fig1_23983870
Mucous Membrane Pemphigoid (MMP)Mucous Membrane Pemphigoid (MMP)
Symptoms vary widely: burning/dryness to scarring / blindness
Usually starts unilaterally, bilateral in 2 years
Early: recurrent inflammation (mimics dry eye, conjunctivitis)
Late: fornix shortening / symblepharon
End: pannus / blindness
75% untreated will progress
42% will progress even with no clinical inflammation “White inflammation”
Symptoms vary widely: burning/dryness to scarring / blindness
Usually starts unilaterally, bilateral in 2 years
Early: recurrent inflammation (mimics dry eye, conjunctivitis)
Late: fornix shortening / symblepharon
End: pannus / blindness
75% untreated will progress
42% will progress even with no clinical inflammation “White inflammation”
https://uveitis.org/recent-research-relating-to-cicatricial-pemphigoid-and-the-use-of-intravenous-immunoglobulin-ivig/
https://webeye.ophth.uiowa.edu/eyeforum/cases/122-limbal-OCP.htm
MMP: MechanismMMP: Mechanism
Type 2 hypersensitivity: cytotoxic attack on
conjunctival basement membrane
Unclear exact antigen:
protein BP180
alpha-6 beta-4 integrin of hemidesmosomes
confirmed by biopsy w/ direct immunofluorescence microscopy (DIF)
dermatologist/ophthalmologist
linear deposition of IgG / complement C3 / IgA along basement membrane
Type 2 hypersensitivity: cytotoxic attack on
conjunctival basement membrane
Unclear exact antigen:
protein BP180
alpha-6 beta-4 integrin of hemidesmosomes
confirmed by biopsy w/ direct immunofluorescence microscopy (DIF)
dermatologist/ophthalmologist
linear deposition of IgG / complement C3 / IgA along basement membrane
https://www.researchgate.net/figure/Direct-IMF-displaying-linear-deposition-of-IgG-IgA-and-C3-at-the-basement-membrane-zone_fig1_298331611
MMP StagingMMP Staging
https://eyewiki.aao.org/Ocular_cicatricial_pemphigoid
MMP StagingMMP Staging
https://www.researchgate.net/figure/Clinical-presentation-of-patients-with-conjunctival-fibrosis-Patients-presenting-with_fig4_280908481
Mondino’s Classification SystemMondino’s Classification System
Stage I: up to 25% inferior forniceal depth loss
Stage II: 25-50% inferior forniceal depth loss
Stage III: 50-75% inferior forniceal depth loss
Stage IV: > 75% inferior forniceal depth loss
Stage I: up to 25% inferior forniceal depth loss
Stage II: 25-50% inferior forniceal depth loss
Stage III: 50-75% inferior forniceal depth loss
Stage IV: > 75% inferior forniceal depth loss
https://www.nature.com/articles/eye2016128
MMP: TreatmentMMP: Treatment
75% progress without treatment
10% progress despite treatment
Anti-inflammatory and immunomodulatory
Dapsone for mild
Corticosteroids if moderate/severe
Rituximab/infliximab
2-3 years of stability, perhaps D/C medications
22% of patients relapse
Surgical repair
75% progress without treatment
10% progress despite treatment
Anti-inflammatory and immunomodulatory
Dapsone for mild
Corticosteroids if moderate/severe
Rituximab/infliximab
2-3 years of stability, perhaps D/C medications
22% of patients relapse
Surgical repair
https://www.aao.org/image/osteoodonto-keratoprosthesis
http://www.djo.harvard.edu/site.php?url=/physicians/oa/1319
Steven’s Johnson Syndrome (SJS)Steven’s Johnson Syndrome (SJS)
Autoimmune Inflammation of skin + mucous membranes
Adverse reaction to medications:
Usually sulfonamides, also NSAIDS, anticonvulsants, antigout
Acute, life-threatening blistering and necrosis, followed by chronic scarring
Autoimmune Inflammation of skin + mucous membranes
Adverse reaction to medications:
Usually sulfonamides, also NSAIDS, anticonvulsants, antigout
Acute, life-threatening blistering and necrosis, followed by chronic scarring
https://www.pinterest.com/pin/309129961915610437/
Toxic Epidermal Necrolysis Toxic Epidermal Necrolysis
More severe, toxic variant of SJS
SJS: <10% of surface area
TEN: >30% of surface area
Rare: SJS + TEN = < 8 cases in 1 million yearly
More severe, toxic variant of SJS
SJS: <10% of surface area
TEN: >30% of surface area
Rare: SJS + TEN = < 8 cases in 1 million yearly
Early: Mucopurulent conjunctivitis, chemosis, hyperemia
Late: scarring
Early: Mucopurulent conjunctivitis, chemosis, hyperemia
Late: scarring
https://www.aao.org/eyenet/article/management-of-stevensjohnson-syndrome-toxic-epider-2
Topical Treatment?Topical Treatment?
Each case is different and based on underlying etiology
No proven improvement to MMP with topical medications / steroids
Lubrication to help relieve symptoms
Each case is different and based on underlying etiology
No proven improvement to MMP with topical medications / steroids
Lubrication to help relieve symptoms
Prevention?Prevention?
1 ounce of prevention = a pound of treatment!
Consider Symblepharon rings
Prokera lens similar effect
Anti-inflammatory and anti-scarring
Large conjunctival defects, especially inferior, consider Prokeralens
If very large, can consider tissue transplant
Long – term visual effect
1 ounce of prevention = a pound of treatment!
Consider Symblepharon rings
Prokera lens similar effect
Anti-inflammatory and anti-scarring
Large conjunctival defects, especially inferior, consider Prokeralens
If very large, can consider tissue transplant
Long – term visual effect
https://lasikkansascity.com/corneal-procedures/prokera-renewal-for-damaged-eyes/
https://www.guldenophthalmics.com/products/index.php/orbital-implants-conformers/symblepharon-rings-large-24mm.html
https://www.biotissue.com/wp-content/uploads/sites/3/2019/09/prokera-insert_PI-BT-003E_V3.pdf
Surgical TreatmentSurgical Treatment
Historically, glass rod to separate
Surgical repair
restoring the fornix, separate conjunctiva, cover defects
limbal graft from opposing eye, amniotic membrane, or oral mucosa transplant
Outcome depends on stability of inflammation.
Stability before surgery
Historically, glass rod to separate
Surgical repair
restoring the fornix, separate conjunctiva, cover defects
limbal graft from opposing eye, amniotic membrane, or oral mucosa transplant
Outcome depends on stability of inflammation.
Stability before surgery
https://www.sciencedirect.com/science/article/pii/S0002939408002596
Clinical PearlsClinical Pearls
Any signs of conjunctival scarring, closely examine BOTH eyes!
Educate patient on many possible causes, but need to rule out the most visually devastating.
Any signs of conjunctival scarring, closely examine BOTH eyes!
Educate patient on many possible causes, but need to rule out the most visually devastating.
https://pxhere.com/en/photo/1602644
https://www.google.com/search?q=clam+shell+pearl&sxsrf=ALeKk02B17PcdJW20NWPnJvrXEkzN5VpgA:1588998149986&source=lnms&tbm=isch&sa=X&ved=2ahUKEwj3iJ2y96XpAhXNjp4KHWUBBO8Q_AUoAnoECBAQBA&biw=639&bih=701#imgrc=8NmHCCF7I0J06M
Special ThanksSpecial Thanks
Doctors of Spokane VAMC
Dr. Jeffrey Urness
Dr. Megan McChesney
Dr. Ryan Ngo
Dr. Tom Kollodge
Dr. Lindsay Kleinschmidt
Dr. Anna Wells
Dr. Len Koh
Dr. Allison Coit
Dr. Chad Gosnell
Doctors of Spokane VAMC
Dr. Jeffrey Urness
Dr. Megan McChesney
Dr. Ryan Ngo
Dr. Tom Kollodge
Dr. Lindsay Kleinschmidt
Dr. Anna Wells
Dr. Len Koh
Dr. Allison Coit
Dr. Chad Gosnell
https://www.pngkey.com/download/u2a9o0i1i1e6o0y3_thank-you-clipart-holiday-christmas-day/
ReferencesReferences
1. Kang, Y., Li, S., Liu, C., Liu, M., Shi, S., Xu, M., He, J., & Zhang, T. (2019). A rabbit model for assessing symblepharon after alkali burn of the superior conjunctival sac. Scientific Reports,9(1), 13857.
2. Mannis, M. J. (2016). Kanski’s Clinical Ophthalmology: A Systematic Approach. Eighth Edition. Cornea, 35(2), e2.
3. Ong, H. S., Minassian, D., Rauz, S., Mehta, J. S., & Dart, J. K. (2020). Validation of a clinical assessment tool for cicatrising conjunctivitis. The Ocular Surface, 18(1), 121–129
4. Ocular cicatricial pemphigoid. (2019, October 4). Retrieved April 23, 2020, from https://eyewiki.aao.org/Ocular_cicatricial_pemphigoid
5. Lee, B. W. H., Tan, J. C. K., Radjenovic, M., Coroneo, M. T., & Murrell, D. F. (2018). A review of scoring systems for ocular involvement in chronic cutaneous bullous diseases. Orphanet Journal of Rare Diseases, 13(1), 83.
6. Jutley, G., Carpenter, D., Hau, S., Booth, D.,
Jasim, H. A., Tay, E., Daniel, C., & Saw, V. (2016). Upper and lower conjunctival fornix depth in healthy white caucasian eyes: a method of objective assessment. Eye , 30(10), 1351–1358.
7. Akkaya, S., & Ozkurt, Y. B. (2016). Persistent Symblepharon in an Infant Following Epidemic Keratoconjunctivitis. Medical Hypothesis, Discovery & Innovation Ophthalmology Journal, 5(3), 74–77.
8. Ophthalmology Review. (2016, February 22). Causes of Symblepharon. Retrieved April 23, 2020, from https://www.ophthalmologyreview.org/articles/causes-of-symblepharon
9. Arnold, J. (n.d.). Causes and Treatment of Symblepharon. Retrieved April 23, 2020, from https://eyecyte.com/causes-and-treatment-of-symblepharon
10. Feizi, S., & Roshandel, D. (2019). Ocular Manifestations and Management of Autoimmune Bullous Diseases. Journal of Ophthalmic & Vision Research, 14(2), 195–210.
11. Various Causes of Cicatrizing Conjunctivitis. (n.d.). Retrieved April 23, 2020, from https://www.aao.org/focalpointssnippetdetail.aspx?id=606f80a7-5898-4b19-a868-9e202d0a7aef
12. Park, A. J., Webster, G. F., Penne, R. B., & Raber, I. M. (2002). Porphyria cutanea tarda presenting as cicatricial conjunctivitis. American Journal of Ophthalmology, 134(4), 619–621.
13. Duong, H.-V. (2019, July 28). Conjunctival Papilloma. Retrieved April 23, 2020, from https://eyewiki.aao.org/Conjunctival_Papilloma
14. Tam, P. M. K., Cheng, L. L., Young, A. L., & Lam, P. T. H. (2009). Paraneoplastic pemphigus: an uncommon cause of chronic cicatrisingconjunctivitis. BMJ Case Reports, 2009. https://doi.org/ 10.1136/bcr.12.2008.1306
15. Thorne, J. E., Anhalt, G. J., & Jabs, D. A. (2004). Mucous membrane pemphigoid and pseudopemphigoid. Ophthalmology, 111(1), 45–52
1. Kang, Y., Li, S., Liu, C., Liu, M., Shi, S., Xu, M., He, J., & Zhang, T. (2019). A rabbit model for assessing symblepharon after alkali burn of the superior conjunctival sac. Scientific Reports,9(1), 13857.
2. Mannis, M. J. (2016). Kanski’s Clinical Ophthalmology: A Systematic Approach. Eighth Edition. Cornea, 35(2), e2.
3. Ong, H. S., Minassian, D., Rauz, S., Mehta, J. S., & Dart, J. K. (2020). Validation of a clinical assessment tool for cicatrising conjunctivitis. The Ocular Surface, 18(1), 121–129
4. Ocular cicatricial pemphigoid. (2019, October 4). Retrieved April 23, 2020, from https://eyewiki.aao.org/Ocular_cicatricial_pemphigoid
5. Lee, B. W. H., Tan, J. C. K., Radjenovic, M., Coroneo, M. T., & Murrell, D. F. (2018). A review of scoring systems for ocular involvement in chronic cutaneous bullous diseases. Orphanet Journal of Rare Diseases, 13(1), 83.
6. Jutley, G., Carpenter, D., Hau, S., Booth, D.,
Jasim, H. A., Tay, E., Daniel, C., & Saw, V. (2016). Upper and lower conjunctival fornix depth in healthy white caucasian eyes: a method of objective assessment. Eye , 30(10), 1351–1358.
7. Akkaya, S., & Ozkurt, Y. B. (2016). Persistent Symblepharon in an Infant Following Epidemic Keratoconjunctivitis. Medical Hypothesis, Discovery & Innovation Ophthalmology Journal, 5(3), 74–77.
8. Ophthalmology Review. (2016, February 22). Causes of Symblepharon. Retrieved April 23, 2020, from https://www.ophthalmologyreview.org/articles/causes-of-symblepharon
9. Arnold, J. (n.d.). Causes and Treatment of Symblepharon. Retrieved April 23, 2020, from https://eyecyte.com/causes-and-treatment-of-symblepharon
10. Feizi, S., & Roshandel, D. (2019). Ocular Manifestations and Management of Autoimmune Bullous Diseases. Journal of Ophthalmic & Vision Research, 14(2), 195–210.
11. Various Causes of Cicatrizing Conjunctivitis. (n.d.). Retrieved April 23, 2020, from https://www.aao.org/focalpointssnippetdetail.aspx?id=606f80a7-5898-4b19-a868-9e202d0a7aef
12. Park, A. J., Webster, G. F., Penne, R. B., & Raber, I. M. (2002). Porphyria cutanea tarda presenting as cicatricial conjunctivitis. American Journal of Ophthalmology, 134(4), 619–621.
13. Duong, H.-V. (2019, July 28). Conjunctival Papilloma. Retrieved April 23, 2020, from https://eyewiki.aao.org/Conjunctival_Papilloma
14. Tam, P. M. K., Cheng, L. L., Young, A. L., & Lam, P. T. H. (2009). Paraneoplastic pemphigus: an uncommon cause of chronic cicatrisingconjunctivitis. BMJ Case Reports, 2009. https://doi.org/ 10.1136/bcr.12.2008.1306
15. Thorne, J. E., Anhalt, G. J., & Jabs, D. A. (2004). Mucous membrane pemphigoid and pseudopemphigoid. Ophthalmology, 111(1), 45–52
Limbal Dermoid
A CASE REPORT & CLINICAL REVIEW
THOMAS KOLLODG E, O.D.
OCULAR DISEASE RESIDENT
MANN-GRA NDSTA FF VA MEDICAL CENTER
SPOKA NE, WA SHINGTON
DisclosuresThe Presenter and Organizers for:
“Limbal Dermoid: A Case Report and Clinical Review”
By Dr. Thomas Kollodge has no financial relationship with any company or products mentioned in this presentation.
Course ObjectivesRecognize and identify common clinical features of limbal dermoids
Describe various associated conditions and abnormalities
Differentiate between limbal dermoids and other anterior segment pathology
Explain when to refer for surgical management
OverviewCase CJ
Histology
Clinical presentation
Grading
Epidemiology
Ocular associations
Systemic associations
Differential diagnoses
Management
Summary video
Conclusion
Case LJ71 year old Caucasian male
Comprehensive eye exam, no complaints
Medical history:◦ Bilateral sensorineural hearing loss◦ Subjective tinnitus
Not taking systemic medications
Family history: Non-contributory
Case LJ – Entrance TestingRefraction/BCVA◦ OD: +0.25 -0.75 x 097 20/20◦ OS: -0.25 sphere 20/20
Pupils: ERRL, no APD OD/OS
EOMs: FROM OU
Confrontations: FTFC OD/OS
No gross abnormalities of head, neck, or ears
Case LJ – Anterior SegmentOcular adnexa: Unremarkable OD/OS
Lids: Unremarkable, no coloboma OD/OS
Conjunctiva: Clear and quiet OD/OS
Iris: Flat without coloboma OD/OS
Anterior chamber: Deep and quiet OD/OS
IOP: ◦ OD: 13 mmHg◦ OS: 12 mmHg
Case LJ – Anterior SegmentSclera: ◦ OD: White and quiet◦ OS:
◦ Non-moveable, superficial, elevated mass within palpebral fissure, partially on temporal cornea and sclera◦ Located at ~4 o’clock relative to the cornea◦ Round, pale yellow to white in color, and approximately 4.5 mm in diameter◦ Extended approximately 2 mm onto the cornea◦ No abnormal vasculature
Cornea: ◦ OD: Clear◦ OS: Band of haze parallel to the lesion’s encroachment
Picture
Corneal haze
Corneoscleral mass
Case LJ – Anterior SegmentPt reported lesion present and stable his entire life
Case LJ– Posterior SegmentLens: Trace nuclear sclerosis and trace cortical spoking OD/OS
Optic nerve: Pink and perfuse, no coloboma OD/OS
C/D ratio:◦ OD: 0.20/0.20◦ OS: 0.15/0.15
Macula: Flat with even pigmentation OD/OS
Posterior pole:◦ OD: Unremarkable◦ OS: Flat, round, darkly pigmented, 1/8th disc-diameter CHRPE inferior/nasal to optic
nerve
Blood vessels: Healthy, 2:3 artery:vein ratio OD/OS
Periphery: Flat and attached 360 OD/OS
Vitreous: Clear OD/OS
Case LJAssessment:
1. Limbal dermoid OS2. Cataract OU3. CHRPE OS4. Refractive error OU
Plan:1. Monitor2. Defer surgery until functional vision worse3. Monitor4. Spectacle Rx released
Limbal Dermoid HistologyChoristoma: Tumor composed of normal tissues in abnormal location
Congenital benign tumor derived from mesoderm and ectoderm
Form during embryogenesis when cells are accidentally captured inside developing tissues
Made up of dense connective/collagenous tissues with pilosebaceous units
Covered with stratified squamous epithelium
Can contain sweat glands, fat, lacrimal gland, and neurologic tissues
No known malignancy potential
Limbal Dermoid Histology
Subepithelial fibrous tissue resembling reticular dermis with skin appendages
Limbal Dermoid Histology
Lacrimal gland tissue within a limbal dermoid
Island of cartilage within a limbal dermoid
Limbal Dermoid HistologyH & E staining of various lesions
Top Left (A): Limbal dermoid, yellow arrow presents collagen fibers
Top Right (B): Lipodermoid, green arrow shows collagen fiber and adipose tissue
Bottom Left (C): Complex choristoma, red arrow indicates collagen fiber and smooth muscle
Bottom Right (D): Epibulbar osseous choristoma, black arrow shows collagen fiber and bone tissue
Typical Clinical PresentationElevated, smooth, white/yellow, solid, soft, well-circumscribed sub-conjunctival mass
Found at birth or during early childhood
Unilateral or bilateral
Inferotemporal corneoscleral limbus
Can partially or completely involve the cornea
Can have hair protruding from their surface
Size: Hardly observable to 5+ mm
Corneal lipid deposition can occur near the dermoid edge
Growth is uncommon, could occur during puberty
GradingGrade I: Superficial and smaller than 5 mm in diameter
Grade II: Extend into corneal stroma and Descemet’s membrane, typically larger than grade I
Grade III: Affect the entire cornea, extend into anterior chamber, and disrupt all structures between the pigmented epithelium of the iris to the anterior surface of the eye
Grading
I IIIII
EpidemiologyRare
Choristomas are the most common category of epibulbar tumors of young children and infants
Limbal dermoids are the most frequent epibulbar choristoma, occurring in approximately 1 out of 5,000-10,000 people
Ocular AssociationsIris coloboma
Eyelid coloboma
Lacrimal abnormalities
Corneal staphyloma
Scleral staphyloma
Aniridia
Microphthalmos
Systemic AssociationsGoldenhar syndrome – craniofacial disorder
Treacher Collins syndrome – craniofacial disorder
Linear nevus sebaceous of Jadassohn – genetic condition
SCALP syndrome - nevus sebaceous, CNS malformations, aplasia cutis congenita, limbal dermoid, and pigmented nevus
Differential DiagnosesConjunctival dermolipoma◦ Choristoma◦ Similar cellular composition to a dermoid◦ Typically more yellow in color due to
increased fat◦ Most often superotemporal bulbar
conjunctiva
Differential DiagnosesOrbital fat prolapse◦ Similar appearance to dermolipoma◦ Moveable◦ Yellow-colored mass◦ Sub-conjunctival◦ Superotemporal location◦ From weakening of Tenon’s capsule due to
surgical trauma or aging
Differential DiagnosesPinguecula◦ Area of thickening of the bulbar conjunctiva◦ Fatty appearance◦ Usually occur nasally within the palpebral
fissure◦ Associated with increased age and
ultraviolet light exposure
Differential DiagnosesPterygium◦ Fibrovascular growth extending from
conjunctiva onto cornea◦ More often within the nasal palpebral
fissure◦ Associated with ultraviolet light exposure
Differential DiagnosesConjunctival/corneal squamous cell carcinoma
◦ Leukoplakic: Discrete thickening of conjunctiva with overlying white hyperkeratonic plaque (top picture)
◦ Papillomatous: Extremely vascularized mass (bottom right)◦ Gelatinous: Translucent thickening of conjunctiva (bottom left)◦ All typically present with prominent afferent and efferent blood vessels◦ Associated with older age, UV light exposure, and squamous cell carcinoma
of the skin
ManagementAlmost always benign
Can monitor in clinic
Reasons to refer for removal◦ Induced as gma sm → refrac ve amblyopia ◦ Blocking visual axis → depriva on amblyopia◦ Chronic irritation from hairs or dellen
formation◦ Cosmesis (especially for children)
Amblyogenic Refractive Errors
Refractive Error Isometropia Anisometropia
Myopia > 8.00 D > 3.00 D
Hyperopia > 5.00 D > 1.00 D
Astigmatism > 2.50 D > 1.50 D
ManagementVarious surgical excision methods
◦ Simple excision◦ Lamellar dissection/excision with penetrating sclerokeratoplasty
Repair after excision◦ Lamellar corneoscleral graft◦ Intrastromal lenticule graft from SMILE◦ Amniotic membrane transplantation◦ Reconstruction with anterior corneal button from donor DSAEK tissue
Method/procedure depends on the ◦ Grade of limbal dermoid◦ Surgeon preference◦ Resource availability (donor corneas)
Removal ConclusionLimbal dermoids are typically a benign finding of the anterior segment
Need to be monitored carefully in infants and young children to prevent amblyopia
Presence of a limbal dermoid may also warrant a systemic evaluation to help screen for conditions such as Goldenhar syndrome, along with potential genetic testing
Important to understand various differentials to ensure correct diagnosis
In adults, it is important to monitor limbal dermoids to ensure no significant changes or signs of malignancy occur
AcknowledgmentMann-Grandstaff VA Medical Center and staff
QuestionsFeel free to email me at: [email protected]
ReferencesBagheri N, Wajda B, Calvo C, Durrani A. The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease. Lippincott Williams & Wilkins; 2016.
Spalton DJ, Hitchings RA, Hunter P. Atlas of Clinical Ophthalmology. Elsevier Health Sciences; 2013.
Cho W-H, Sung M-T, Lin P-W, Yu H-J. Progressive large pediatric corneal limbal dermoid management with tissue glue-assisted monolayer amniotic membrane transplantation: A case report. Medicine. 2018;97(46).
Yanoff M, Duker J. Ophthalmology. In. 4th ed: Elsevier; 2014.
Kasturi N, Rajkumar J. Double limbal dermoid with Tessier's paramedian facial cleft. Journal of American Association for Pediatric Ophthalmology and Strabismus {JAAPOS}. 2018;22(5):395-397.
Bowling B. Kanski's Clinical Ophthalmology: a systematic approach. Saunders Ltd; 2015.
Singh M, Kaur M, Grewal AM, et al. Ophthalmic features and management outcomes of 30 children having Goldenhar syndrome. Int Ophthalmol. 2019:1-9.
Kaiser PK, Friedman NJ, Roberto Pineda I. The Massachusetts Eye and Ear Infirmary Illustrated Manual of Ophthalmology. Elsevier Health Sciences; 2014.
Yangzes S, Gupta PC, Ram J. Limbal Dermoid in a Teenage Boy. JAMA Ophthalmology. 2017;135(10):e173268-e173268.
Wan Q, Tang J, Han Y, Ye H. Surgical treatment of corneal dermoid by using intrastromal lenticule obtained from small-incision lenticule extraction. Int Ophthalmol. 2019:1-7.
Lam J, Dohil MA, Eichenfield LF, Cunningham BB. SCALP syndrome: sebaceous nevus syndrome, CNS malformations, aplasia cutis congenita, limbal dermoid, and pigmented nevus (giant congenital melanocytic nevus) with neurocutaneous melanosis: a distinct syndromic entity. J Am Acad Dermatol. 2008;58(5):884-888.
Kuroda Y, Ohashi I, Enomoto Y, et al. A postzygotic NRAS mutation in a patient with Schimmelpenning syndrome. Am J Med Genet A. 2015;167(9):2223.
Nakamura N, Akiyama K, Shigeyasu C, Yamada M. Surgical repair of orbital fat prolapse by conjunctival fixation to the sclera. Clinical Ophthalmology (Auckland, NZ). 2015;9:1741.
