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When is Ptosis not “just” Ptosis?

Cat Burkat, MD, FACS Associate Professor

Oculoplastic, Facial Cosmetic, & Orbital Surgery

University of Wisconsin- Madison

March 7, 2015

Typical Ptosis

•  Complains of drooping –  Heavy lid, fatigue, has to lift lid

manually

•  MRD low, approaches pupil •  Droopy lid triad:

1.  Skin excess 2.  Levator muscle dehiscence

(Involutional ptosis) 3.  Brow/forehead ptosis

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So…we all sag as we age

When can’t we blame it on age?

•  52 yo M with progressive RUL ptosis over 1 year - Hard to put contacts in OD

•  Motility, pupils, SLE nl •  MRD 0.5 mm OD, 4 mm OS •  LVF 11 mm OD, 16 mm OS •  Recommend internal ptosis repair

Case

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•  Intraoperative findings: –  Eversion –  Thick lid

H&E. 40x

H&E. 10x Case

•  Diagnosis: RUL ptosis-- yes… but –  MRD 0.5 mm OD, 4 mm OS –  LVF 11 mm OD, 16 mm OS

•  Levator dehiscence alone (which can occur with CL use) should =normal levator function!

Patient:

Ptosis with poor levator function was due to infiltration of levator muscle from lymphoma

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•  Levator function: –  Normal ≥ 15mm –  Must negate forehead

movement

•  43 yo M, never seen MD for 30 yrs, with eyelid drooping and “lumps” OU.

–  puffy lids

•  Pupils, motility, LVF nl •  Proptosis

Case

•  S-shaped eyelid contour:

Ø  Suggests lacrimal gland involvement

Ø  Palpate!

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•  Palpate – Firm large smooth masses – Proptosis – Ptosis

•  Think LG etiologies •  Concurrent pneumonia?

Case

•  Patient: BUL ptosis yes… but with S-shaped eyelid contour

–  Lacrimal gland enlargement causes mechanical ptosis effect

Case

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Case

•  48 yo F with several mos drooping RUL •  VA, pupils, motility, SLE nl •  No proptosis

•  No erythema, tenderness •  ?Just aging fat?

Case •  48 yo F with several mos drooping RUL •  VA, pupils, motility, SLE nl •  No proptosis •  No erythema, tenderness •  ?Just aging fat?

•  Dx: Lymphoma

TOUCH the lids! Patient: RUL ptosis…yes, but firm smooth oval subQ discrete mass

- normal fat prolapse = nondiscrete and without a border

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Lymphoma

•  #1 most common malignant orbital tumor in adults –  Age 50-70

•  Insidious onset - painless proptosis, ptosis, diplopia, lid edema •  Lacrimal gland is most common orbital site involved

–  Up to 50% arise in lacrimal fossa •  Bilateral occurrence common (17%)

•  Orbital presentation –  Anterior orbit –  Subconjunctival salmon patch –  “Putty-like molding” around structures rather

than invasion •  usually vision / EOM’s remain intact

•  Predilection for superior/anterior orbit → downward globe displacement

MANAGEMENT •  INCISIONAL BIOPSY: fresh specimen for immunocytochemistry +

formalin specimen •  Labs: Chem panel, LFT, Alk phos, CT abd/pelvis/chest, bone marrow bx •  Chemotherapy •  Radiotherapy

–  Localized orbital adnexal disease (Achieves local control and may prevent spread)

Lymphoma

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Lymphoma •  Only 20-30% have h/o previous or concomitant systemic disease •  20% of pt with lymphoid tumors of conjunctiva,

35% of pt with orbital tumors, & 67% with eyelid disease will eventually develop systemic

lymphoma PROGNOSIS •  Visual prognosis good unless radiation retinopathy •  All with localized lesions need LIFELONG follow-up for

development of systemic disease

Case •  6 yo F “bumped heads

with student”, shortly after had RUL swelling. Eye pressure and headaches.

•  Rash, fatigue, fevers – ER dx: URI

•  VAcc: 20/20 OU •  Pupils: equal, reactive,

no RAPD •  Motility: limited upgaze,

hypoglobus

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Case •  69 yo WF awoke one AM

with left eyelid drooping. •  No h/o trauma,

discharge, pain. •  VAcc: 20/30 OU •  Pupils: equal, reactive,

no RAPD •  Motility: full OU •  SLE, IOP: nl

•  PO antibiotics by PCP

Case

•  H(93.5): 16 OD, 15 OS •  Resistance to retropulsion OS •  Brawny thickened LLL

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• CT orbits: dx: “pre- and post-septal cellulitis” –  Ill-defined LLL diffuse enhancement –  Inferior rectus –  No bony involvement –  Bilateral maxillary sinus disease

Case

PMH on review •  PMH:

–  HTN –  GERD –  Breast CA-dx 1980

•  S/p mastectomy 1984, 1993 •  No h/o metastases

Patient: LUL ptosis yes… but also lower lid elevation (or reverse ptosis), eyelid thickening, enophthalmos

- If you don’t ask about the remote history, they often won’t tell

- Ptosis in this pt due to enophthalmos

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Metastasis to the Orbit •  Accounts for ≈ 10% of all orbital neoplasms. (5% hematogenous,

5% from adjacent structures)

•  What is the most common malignancy to spread to orbit? –  Breast Cancer (42%) –  Lung Carcinoma (11%) –  Unknown Primary Cancer (11%) –  Prostate (8%) –  Melanoma (5%) Average survival after dx is 9 months

•  25% of metastatic tumors to the orbit are the initial presentation

–  Metastatic neuroblastoma –  Leukemia, lymphoma

•  In pediatric population, metastatic disease is far less common

Enophthalmos

•  10% of metastatic cases –  80% breast cancer –  Contraction of fibroblasts in the scirrhous tumor →

posterior traction on globe

•  Breast > GI > prostate, lung

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Case

•  64 yo M with droopy RUL x 3 wks after picked up new glasses. Also complains of intermittent diplopia before then.

•  No dysarthria, systemic weakness

•  VA: 20/40 OD, 20/20 OS

•  Pupils, motility nl. Orthotropic

•  MRD minus 2mm OD, +1mm OS

•  LVF 12mm OU

•  Diagnosis: RUL ptosis-- yes… but –  MRD minus 2mm OD, +1mm OS –  Acute onset –  Intermittent diplopia preceding

•  Levator dehiscence alone should = slowly progressive ptosis

Patient: Variable measurements or true fluctuating symptoms should warrant additional evaluation

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Ice Pack test •  Cheap, safe, quick bedside test •  Apply ice to closed lids for 2–5 minutes

–  Positive if improvement of diplopia or elevation of MRD by 2 mm –  Physiological theory: by cooling skeletal muscle fibers (below 28˚C), the activity of acetylcholinesterases is inhibited

Previous pt: MRD minus 2mm OD, +1mm OS After ice test: MRD +1.5mm OD, +1mm OS

Another Case of MG

•  Ice test: – Much over 3 minutes, reduction of muscle fiber temperature below 22˚C may reduce contractile force of the muscle potential false-negative result

•  Acetylcholine receptor antibody test

potential false-negative? Patient:

Ptosis due to neuromuscular junction abnormality resulting in decreased muscle contraction

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Case

•  28 yr old F with ptosis “since she was little” worsening over past yr, resulting in neck pain

–  Adopted chin-up position –  Severe ptosis BUL –  Levator function 4-5mm –  Motility nl –  No lagophthalmos –  Bell’s phenomenon

decreased

•  Severe ptosis BUL –  MRD minus 2mm OU

•  Levator function 4-5 mm •  Bell’s phenomenon

decreased

•  3 siblings all have similar appearances

Blepharophimosis-Ptosis- Epicanthus Inversus Syndrome:

Short vertical & horizontal palpebral fissure

Epicanthus inversus lower lid fold Telecanthus

Flat nasal bridge High arched brows

Ectropion Nystagmus, strabismus

Case

Dx: congenital ptosis?

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Blepharophimosis-Ptosis- Epicanthus Inversus Syndrome

•  4-6% of congenital ptosis •  Autosomal dominant inheritance

–  FOXL2 gene mutation •  May be associated with female infertility

or congenital heart disease

•  Surgical options: –  None –  Limited elevation due to high risk

lagophthalmos –  Frontalis sling –  Stepwise approach- telecanthus, epicanthal

fold, ectropion, ptosis last

•  Diagnosis: BUL ptosis-- yes… but –  Minus MRD –  LVF 4-5 mm OU –  high arched brows –  “small eye openings”

•  Ask for family history or photos

Patient:

Ptosis with poor levator function due to hypoplasia of levator muscle (myogenic)

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Developmental defects in muscle structure

•  Histopathology: levator muscle and aponeurosis are dystrophic, infiltrated or replaced by fat and fibrous tissue –  In severe cases,

may be little or no striated muscle

•  Higher risk of: –  Reoperation –  Lagophthalmos

Case •  73 yo M with BUL droopy lids x 25 yrs. Previous lid repair.

–  No diplopia, dysarthria –  Mild dysphagia –  VA, pupils, SLE nl

•  Extreme forehead flexion and chin-up position •  MRD 0mm OD, minus 2mm OS •  LVF 7mm OU •  Motility limited in all gazes, except fair in downgaze

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CPEO Chronic progressive external ophthalmoplegia

•  Slowly progressive paralysis of EOM and eyelid muscles

–  Bilateral symmetric severe ptosis followed by ophthalmoparesis later

•  Often do not notice diplopia due to symmetric nature

•  Mitochondrial myopathy •  Rule out Kearns-Sayre syndrome if young pt:

–  CPEO –  Onset prior to 20 –  Pigmentary “salt & pepper” retinopathy –  Cardiac conduction defects, seizures, death

•  Regular cardiac exams

Case

•  52 yo F with sudden RUL drooping. No trauma. No pain, decreased vision, diplopia. Not presentable for her public job. •  PMH unremarkable •  VA, SLE, motility nl

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Dx: RUL ptosis but…

•  Ptosis •  Reverse ptosis •  Miosis •  Other findings: anhidrosis, anisocoria worse in dark

Patient: Ptosis due to Horner’s Syndrome. Don’t forget the exam basics!

Sympathetically-innervated Muller’s muscle and inferior retractors

Case

•  58 yo F with OS drooping, FBS and redness x 2 mos •  Hx of multiple nonhealing corneal ulcers •  PMH bipolar disorder with psychosis •  VA: HM OS •  Corneal scar and pannus •  Loss of lashes

Definitely not just ptosis!

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•  Differential Dx: –  Conjunctival intraepithelial neoplasia –  Sebaceous cell carcinoma –  Lymphoproliferative process –  Severe follicular conjunctivitis

• Pseudotrachoma

•  Histopathology: –  conjunctival lichen simplex chronicus

•  Referred to as the “scratch-itch-scratch” lichenification cycle

–  pruritus is dominant symptom

Patient: Loss of lashes at uneven lengths suggests self-induced behaviors causing ptosis

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Case •  Unilateral ptosis over several months

•  Pseudoptosis due to retraction of opposite side

– Thyroid disease is most common cause of unilateral or bilateral proptosis/retraction

Atypical Ptosis “Clues”

Symptoms: •  Diplopia •  Pain •  Decreased vision •  Lump •  Swelling, redness •  Rapid onset

Exam findings: •  Levator function •  Motility •  Pupils •  Proptosis •  Mass •  S-shaped lid contour •  Disc edema, retinal changes

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Summary

•  Not all ptosis is “just ptosis” from levator dehiscence

•  Often will present with other clues •  Get close •  Knowledge and exam skills •  Diagnosis of these diseases can often be the initial

presentation of disease, and can be life-saving (malignancy, MG, Kearns-

Sayre, OPD, Horner’s)