OCULAR DISEASE DIAGNOSIS AND MANAGEMENT I LID DISEASES AND DISORDERS

1. OCULAR DISEASE DIAGNOSIS AND MANAGEMENT ILID DISEASES AND DISORDERS Judy Tong, O.D., F.A.A.O. Associate Professor Director of Residency Programs Southern California College of Optometry

2. Mite I hang on the Jungle Gym

3. Review Lid Anatomy Muscles Tissue Lashes Blood Supply Lymphatics Nerves Bacteria and other Congenital Ptosis Entropion Ectropion Lagophthalmos Blepharospasm Blepharoclonus Goal’s for Today

4. Dear Dr. Optometrist Prime: • We have a 2 year old beautiful baby boy who was born with the eyelid presentation as seen in the photograph below. • We would like a second opinion from you as to what ails our son (diagnosis)? • We are financially strapped and have no medical or eye insurance. • If we let it alone, will it get better on its own? • What would be the worse possible outcome? • If we opted to just obtain an evaluation from you today, what would this entail? • Signed: • Concerned Mother

5. REVIEW OF LID ANATOMY • Functions of Eyelids • Protect globe from injury or excess light • Form a mechanical barrier • Maintain optical clarity by resurfacingtears • Assist immune system • Part of “native immunity” when acting as a barrier • Remove pathogens in tears • Replenish immunological substances which inhibit bacterial growth • Lysozyme, lactoferrin, beta lysin • Immunoglobulins

6. Muscles • Levator (LPS) • Functions • Raises upper eyelid • Works with orbicularis in both involuntary blinking and forced lid closure • Innervated by cranial nerve III • Starts at superior part of orbital apex and attaches to the front of the superior tarsal plate

8. Dysfunction of levator causes ptosis • Mechanical ptosis: heavy lid, usually from lid mass (chalazion) • Myogenic “ptosis”: age-related or inherited levator deterioration • Neurogenic “ptosis”: due to CN III lesion causing poor levator function (diabetic stroke)

9. Mueller’s muscle • Functions • Maintains tonus of elevated upper lid • Maintains tonus of opened lower lid • Innervated by sympathetic nervous system • Originates on underside of levator and attaches to upper border of superior tarsal plate • Dysfunction of Mueller’s muscle > slight ptosis, inverse ptosis (1-3mm) Slide 3a

12. Orbicularis • Functions • Closes lids • Involuntary regular blinking • Forced voluntary blinking • Maintains apposition of lacrimal drainage apparatus against globe • Innervated by cranial nerve VII (which also innervates muscles of cheeks and mouth)

14. Muscles is elliptically shaped • Attached to nasal and temporal edges of orbit by medial and lateral palpebral ligaments • “Palpebral” portion in lid only; responsible for involuntary blinking • “Orbital” portion in forehead, temples, and cheeks; responsible for forced lid closure

15. Dysfunction of orbicularis • Lesion of CN VII causes paresis or paralysis of orbicularis and also some facial muscles • Bell’s palsy • Lagophthalmos • Loss of tonus of orbicularis and its ligaments allows puncta to fall away with resulting epiphora • Entropion resulting from forced lid closure; due to reduced tonus of lower orbicularis with inability to return lower lid margin to its normal position

16. Tissue • Skin • Thinnest skin in the body • 10 times more clinically sensitive to allergens than elsewhere in the body • Very loose, stretchy skin allows for considerable expansion of lid contents • Recurrent edema and aging can cause loss of elasticity of the skin • Areolar tissue • Connective tissue • Soft, loose, spongy • Easily distended

17. Eyelashes • Derived from hair follicles at lid margin • Highly sensitive • Glands located near lash follicle • Glands of Moll (sweat) • Glands of Zeis (sebaceous)

19. Tarsal Plate • Fibrous “skeleton” of lids • Give shape and firmness to eyelids • Meibomian glands located deep in tarsal plates • Provide oily top layer of tears • Secrete oil layer which acts as a barrier preventing spillover of tears onto lid

21. Blood Vessels • Arteries • Ophthalmic artery branches • Lacrimal artery branches • Empty into the veins of the forehead and temple

22. Lymphatics • Lympathics in posterior lid drain the conjunctiva and tarsal glands • Lympathics in anterior lid drain the skin and the associated skin structures • Lateral half of eyelids lympathics drain into the preauricular lymph node • Medial half of eyelids lymphatics drain into the submandibular lymph node

25. Nerves • Motor supply • Orbicularis supplied by CN VII • Levator supplied by CN III • Sensory supply (pg. 79) • From first two divisions of trigeminal (CN V) • Ophthalmic division of trigeminal (VI) • Maxillary division of trigeminal (V2) • Upper lid mainly supplied by supraorbital nerve, part of frontal division, one of three divisions of V1

26. Medial side of the upper lid assisted by: • Supratrochlear, part of the frontal division (one of three divisions of V1) • Infratrochlear, part of the nasociliary division (one of three divisions of V1) • Lateral side of the upper lid assisted by lacrimal nerve, one of three divisions of V1 • Lower lid supplied by infraorbital, one of the two divisions of V2

27. Indigenous Flora of the Eyelids • Bacteria • Cultured from normal eyes • Species %of patients harboring S. epidermidis 37 S. aureus 17 Diphtheroids 1 Combination of above 35 Misc. 9 Total 99

28. Most common anaerobe=propionibacterium acnes • Non-bacterial flora • Pityosporon ovale (orbiculare) • Yeast • No definitive pathogenic effect is known • Demodex folliculorum and D. Brevis (arthropods) • D. folliculorum in eyelash follicles • D. brevis in sebaceous glands

29. Herpes simplex virus periodically excreted in tears without being associated with any disease process

30. Skip! • Self study pages 5 thru 10

31. Congenital ptosis • Onset at birth • Watch for other congenital anomalies • In patient beyond 1 or 2 years of age a ptosis will generally be acquired, however, congenital ptosis should be considered and ruled out • History • Superior lid sulcus (absent) • Old photos…“FAT scan”

33. Differential diagnosis is very important since the congenital form is not usually accompanied by ongoing neurological disease (exception is neonatal myasthenia gravis) • Significance • Occlusion amblyopia • If significant degree of ptosis, get surgical consult to prevent occlusion amblyopia (pediatric ophthalmology consult)

34. Types • Autosomal dominant • Approx. 70% are autosomal dominant • Check family members • Also look for other congenital anomalies • If obstructing pupil significantly > pediatric ophthalmology consult for surgical correction of ptosis

35. SR palsy with LPS palsy • Isolated SR palsy congenital is very, rare • Look for vertical tropia in primary position with ptosis

36. Paradoxical CN III innervation • Congenital > Marcus Gunn jaw-winking = ptosis accompanied by retraction during stimulation of ipsilateral pterygoid muscle. Amblyopia secondary to ptosis in 20% of these cases • Management: surgery for ptosis • Acquired > pseudo von Graefe sign

38. Congenital Horner’s • Look for heterochromia irides (suggests onset prior to age 2) • Birth trauma • Not common at all • Neonatal myasthenia gravis

39. Tips in diagnosing congenital ptosis • History (may be reliable) • Old untouched photos (most parents have lots of baby pictures) • Superior lid sulcus - absent • Lid lag (slight) in downgaze due to inelasticity of the lid

40. Management aspects • If significant obstruction of pupil and/or apparent reduced acuity or dislike for fixation with the eye then consult with pediatric ophthalmologist is strongly advised. Ptosis must be eliminated to prevent occlusion amblyopia • Pediatric neurology (or ophthalmology) consult if congenital Horner’s or suggestion of neonatal myasthenia gravis

41. Ankyloblepharon • Adhesions between upper and lower eyelids along the lid margin • Results from significant mucocutaneous disease, i.e., ocular pemphigoid or as a congenital anomaly • Usually at outer canthus (external ankyloblepharon). Can be inner canthus (internal) • Causes pseudostrabismus

43. Symblepharon • Adhesion of the tarsal conjunctiva to the bulbar conjunctiva resulting generally from significant inflammatory disease, Stevens-Johnson syndrome, pemphigoid, alkali burn • Occurs as tarsal conjunctiva and bulbar conjunctiva heal • Prevention is easier than treatment > BID to QID breaking of adhesions with glass rod • Tx with cyclosporin…Restasis

44. Vitiligo • Patchy depigmentation of the skin • In ocular disease skin of eyelids affected • Usually accompanied by depigmentation of lashes > poliosis • Alopecia > along with poliosis, vitiligo • R/O Cocaine use

46. Entropion • Condition where the eyelid margin turns inward toward the globe - more common in lower lid • Significance • Can result in trichiasis, but not necessarily

47. Types • Congenital entropion • Usually other developmental anomalies • Cause is usually deformity in tarsal plate development causing entropion • Management is surgical reformation of tarsal plate • Epiblepharon-horizontal lid skin fold physically pushing lashes toward globe (trichiasis). Management: surgical excision of excess skin

48. Cicatricial entropion • Cicatrix = scar • Cicatricial entropion caused by scar on tarsal conjunctiva with contraction of scar tissue resulting in tractional entropion • Usual cause is inflammation of tarsal conjunctiva such as trachoma • Other causes…chemical injuries, lacerations, surgical procedures

50. Spastic entropion • Caused by excessive contraction of palpebral portion of orbicularis (or very rarely atrophy of the lid retractors) • Most common cause is blepharospasm due to ocular irritation, injury, inflammation (conjunctivitis, keratitis, etc.)