Grand Rounds Conference Reema Syed, MBBS University of Louisville Department of Ophthalmology and Visual Sciences October 3, 2014

Subjective CC: “droopy right eyelid” HPI: 7 month old male referred to Oculoplastics clinic by Peds Ophthalmology for evaluation of right upper lid ptosis. Ptosis was present since birth, however, child was noted to have a fixation preference for OS at his pediatric ophthalmologist recently. Patching OS was started for 1 hour/day

History POH: ptosis OD PMH: full term, uncomplicated vaginal delivery, no birth trauma Eye Meds: None, patching OS 1hr/day Meds: none Allergies: None Family Hx: “lazy eye” (father)

Objective ODOS VA (sc):CS-UMCSM Pupils: 4 to 2 mm 4 to 2 mm No anisocoria or RAPD IOP:15 15 EOM: Full Full orthophoric in primary gaze Cycloplegic refraction: x x x x90

Objective PLE: ODOS Lidsupper lid position wnl does not change with feeding No palpable masses Eyelid creaseminimalnormal Palpebral fissure5 mm9 mm Marginal reflex1 mm4 mm distance - 1 Levator function2-4 mm10-12 mm

Objective Conjunctiva/ScleraNormal OU CorneaClear OU Anterior Chamberformed OU IrisNormal OU No heterochromia LensClear OU VitreousNormal OU ONc/d 0.2, sharp and pink OU MVPNormal OU

Assessment 7 month old M with upper lid ptosis and poor levator function OD, without any other ocular or systemic anomalies who has recently developed amblyopia OD 7 month old M with upper lid ptosis and poor levator function OD, without any other ocular or systemic anomalies who has recently developed amblyopia OD DDx: DDx: - Isolated unilateral congenital ptosis - Marcus Gunn jaw winking syndrome - Blepharophimosis syndrome - Congenital third nerve palsy - Congenital Horner's syndrome - Congenital fibrosis of extra-ocular muscles - Upper eyelid mass (neurofibroma, dermoid cyst) - Pseudo-ptosis

Congenital Ptosis Caused by maldevelopment of levator palpebrae superioris due to abnormal innervation Caused by maldevelopment of levator palpebrae superioris due to abnormal innervation normal muscle tissue replaced by fibrous and fatty tissue normal muscle tissue replaced by fibrous and fatty tissue Characterized by decreased levator function, lid lag, lagophthalmos Characterized by decreased levator function, lid lag, lagophthalmos

- May be sporadic or familial (gene unknown) - Strabismus 30%, anisometropia 12%, amblyopia 20% - 2/3 unilateral, 1/3 bilateral – may be asymmetric - Surgery usually delayed until 3-5 yrs of age unless: - Deprivational or anisometropic amblyopia - Ocular torticollis

Amount of upper lid excursionLevator function 11 mm or moreNormal 8-10 mmGood 5-7 mmFair 4 mm or lessPoor

Treatment Options - Levator resection: - used when levator function is good - amount of upper eyelid excursion used to determine the amount of muscle to be resected * Smith B, Della Rocca R, Nesi F. Ophthalmic Plastic and Reconstructive Surgery. Vol 1. Philadelphia: CV Mosby, 1987:Fig

-Frontalis suspension: -used when absent or poor levator function -most commonly used for congenital ptosis -eyelid suspended directly from the frontalis so that movement of the brow can be used to elevate the eyelid *Amato M, Monheit B and Shore J. Ptosis surgery. Volume 5, Chapter 78, fig 13

Choice of sling material AdvantagesDisadvantages Autogenous Fascia Lata- Gold standard - Excellent tensile strength - Best long-term results - Recurrence unlikely - Need for another surgical site - increased risk of infection - Minimum age 3 yrs Allogenic Fascia Lata- any age - Second donor site not required - Small chance of rejection - transmission of infection from donor to recipient Supramid- any age - Second donor site not required - High chance of recurrence due to degradation of sling material Silicone- Not associated with recurrence - Can be adjusted intra and post-operatively to adjust lid margin height over time

Complications Under/over correction Under/over correction Asymmetric eyelid contour Asymmetric eyelid contour Scarring Scarring Wound dehiscence Wound dehiscence Eyelid crease asymmetry Eyelid crease asymmetry Lagophthalmos with exposure keratitis Lagophthalmos with exposure keratitis

Back to our patient Underwent silicone rod frontalis suspension OD Underwent silicone rod frontalis suspension OD 1 week post-op

Retrospective chart review of 4 patients with ocular torticollis due to bilateral congenital or acquired ptosis who underwent surgical correction pre and post operative systemic symptoms in 1 adult and developmental milestones in 3 children are described Adult patient: 62 yr M with history of severe bilateral ptosis for 10 yrs presented to his PCP with debilitating upper back pain for 3 yrs. Pain persisted despite multiple referrals and therapies. Abnormal head posture due to ptosis found on ophthalmic exam. Pain began to resolve within a week of surgical correction of ptosis Children: 12m, 10m, 5m olds with bilateral ptosis and development of head-tilt, chin up position. No amblyopia but severe delays in gross motor development without delay in any other milestones. Caught-up with motor milestones within a few months of corrective surgery

References BCSC Orbit, Eyelids and Lacrimal System BCSC Orbit, Eyelids and Lacrimal System BCSC Pediatric Ophthalmology and Strabismus BCSC Pediatric Ophthalmology and Strabismus Smith B, Della Rocca R, Nesi F. Ophthalmic Plastic and Reconstructive Surgery Smith B, Della Rocca R, Nesi F. Ophthalmic Plastic and Reconstructive Surgery Amato M, Monheit B and Shore J. Ptosis surgery Amato M, Monheit B and Shore J. Ptosis surgery Loff HJ et al. transconjunctival frontalis suspension: a clinical evaluation. Ophthal Plast Reconstr Surg. 1999; 15(5): Loff HJ et al. transconjunctival frontalis suspension: a clinical evaluation. Ophthal Plast Reconstr Surg. 1999; 15(5): Baroody M et al. Advances in the diagnosis and treatment of ptosis. Curr Opin Ophthal. 2005; 16(6): Baroody M et al. Advances in the diagnosis and treatment of ptosis. Curr Opin Ophthal. 2005; 16(6): Lee M, et al. Frontalis Sling Operation using silicone rod compared with preserved fascia lata for cogenital ptosis. Ophthalmology 2009; 116(1) Lee M, et al. Frontalis Sling Operation using silicone rod compared with preserved fascia lata for cogenital ptosis. Ophthalmology 2009; 116(1)

Thank you