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Home , Ankyloblepharon, Blepharophimosis, Coloboma, Cryptophthalmos, Entropion, Gland of Zeis

Pediatric Disorders

Peter MacIntosh, MD Ophthalmic Plastic & Reconstructive February 8, 2018 Chalazia

• Chronic lipogranulomatous inflammation of the eyelid • Caused by plugging of • meibomian glands (internal chalazia)

• Zeis glands (external chalazia) www.drduerksen.com • sebaceous glands of ) • Treatment • Eyelid hygiene • Topical steroid ointment • Intralesional steroid injections • Incision and drainage

www.surgicalpathologyatlas.com Hordeola

• Acute infectious abscess of eyelid • Usually staphylococcal • Can affect • (external) • (internal) • Hordeola often evolve into chalazia • Treatment • Eyelid hygiene • Topical steroid/antibiotic ointment • Systemic Abx for cellulitis • Incision and drainage

en.wikipedia.org/wiki/Stye Milia

• Multiple tiny, white lesions • Common in infancy • Primary • on facial skin with hair follicles • Secondary • Pilosebaceous unit trauma • Pathology • Epidermoid cyst filled with keratin • Treatment • Observe • Incision with a sharp needle and expression of contents Molluscum Contangiosum • Pearly round papule • Central umbilicated • Follicular • Pathology • Pox viral infection • Epidermal hyperplasia • Henderson-Patterson bodies • Eosinophilic inclusions containing virus • Treatment • Observation • Excision • Cryotherapy • Curettage • Associations • In adults, consider AIDS

Cryptophthalmos ”Hidden ” • Sporadic Forehead skin extends over and onto cheek without forming • Syndromic • (AR) • Mutations in FRAS1 and FREM2 • Syndactyly • GU malformations • Orofacial clefting • MR • Musculoskeletal anomalies • Management • Full eyelid reconstruction • Flap/graft

6 • 0.7 / 10,000 live births • Isolated • Unilateral • Usually upper eyelid • +/- corneal palpebral adhesions • Mechanical forces e.g. amniotic membrane band • Embryological error in ectodermal or mesodermal migration

7 Coloboma • Syndromic • Fraser syndrome • Goldenhar syndrome • Treacher Collins syndrome • CHARGE

www.nature.com

Reviewofophthalmology.com

8 Coloboma • Management

• Early lubrication • <50% eyelid, close primarily • >50% need lid sharing technique • Caution for

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• Web of skin • Partial or complete eyelid fusion • Management • Clamp then cut with scissors

BCSC Epiblepharon Epiblepharon

• Lower eyelid pretarsal muscle and skin override lid margin • Management • Observation if no K issues • Often resolves with facial maturity • Small skin/muscle excision • Anterior lamellar everting BCSC sutures

Epiblepharon Epicanthus

• Medial canthal fold • Immature midfacial bones • Pseudoesotropia • Tarsalis • Upper eyelid • Inversus • Lower eyelid • Palpebralis

BCSC • Equal upper and lower eyelid Epicanthus Management

• Observation • Often resolves with facial maturity • Z plasty • Y-V plasty • ”Jumping man” plasty

Mustardé, 1963 Euryblepharon

• Horizontal widening of the • Inferior displacement of lateral canthal tendon • Antimongoloid slant • Management • Lateral tarsal strip

BCSC Congenital Syndrome

BCSC Congenital Blepharophimosis Syndrome

•

BCSC Congenital Blepharophimosis Syndrome

• Ptosis • Epicanthus inversus

BCSC Congenital Blepharophimosis Syndrome

• Ptosis • Epicanthus inversus • Telecanthus

BCSC Telecanthus versus

Telecanthus HypertelOrism (Telorbitism)

• Widened intercanthal distance • Widened interorbital distance and increased interpupillary distance

BCSC Congenital Blepharophimosis Syndrome

• Ptosis • Epicanthus inversus • Telecanthus • Autodomal Dominant

BCSC Congenital Blepharophimosis Syndrome

• Ptosis • Frontalis suspension • Hypertelorism and epicanthus inversus • Medial canthoplasty with Z- or Y-V-plasties • Transnasal wiring Tarsal Kink Syndrome

• Primary congenital is very rare • Usually involves medial lower eyelid • Tarsal kink may result in upper eyelid entropion • Related to • 90 degree angle in (kink) • Management • Horizontal incision though kink • Everting sutures

24 Congenital Ptosis

• Theories: • Disorder of muscle development • Disorder of muscle innervation • Pathology • Fibrosis • Decreased density of skeletal muscles fibers

25 Congenital Ptosis

• Characteristic findings: • Absent or poorly developed eyelid crease • Decreased levator function • Eyelid lag in down gaze

Visioncarecentre.blogspot.com Congenital Ptosis

• Characteristic findings: • Absent or poorly developed eyelid crease • Decreased levator function • Eyelid lag in down gaze Congenital Ptosis

• Characteristic findings: • Absent or poorly developed eyelid crease • Decreased levator function • Eyelid lag in down gaze Congenital Ptosis

• Management • Vision unaffected: observe • Head posturing/cosmetic • Surgery after 6 months if possible 30 Many patient will have poor LF and need frontalis suspension

31 Frontalis Suspension

Sling Materials • Silicone 74.3% • Autogenous fascia lata 34.9% • Cadaveric fascia lata 25.1% • Supramid 13.3%

Aakalu VK, Setabutr P. Current Ptosis Management: A National Survey of ASOPRS Members. Ophthal Plast Reconstr Surg. 2011 Feb 22. Ophthal Plast Reconstr Surg. 2011 Jul- Aug;27(4):270-6 Frontalis Suspension 34 Unilateral versus bilateral surgery for unilateral ptosis

• Controversial • Do unilateral frontalis surgery to avoid surgery on the normal eye • Do bilateral frontalis surgery without excision of levator muscle on normal side • Do bilateral frontalis surgery with excision of levator muscle on normal side

• Difficult to convince family to do surgery on normal side • Mostly do unilateral frontalis suspension

Conclusion

• Many pediatric eyelid lesions are benign • Manage corneal exposure in patients with coloboma • In congenital ptosis, remember to measure: • Lid crease • Levator function • Lag in down gaze

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