Oculoplastics 1 The UIC Eye Manual Produced by the

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Oculoplastics 1 The UIC Eye Manual Produced by the Powered By Docstoc
					                                                                                  1

                          The UIC Eye Manual
Produced by the faculty and residents at the Department of Ophthalmology and
Visual Sciences at the University of Illinois at Chicago

                           DISCLAIMER--PLEASE READ


While every attempt has been made to have dosages, medications, and treatment
recommendations as accurate and up to date as possible, this manual is no substitute
for clinical judgement and knowledge. UIC and the authors are not liable for any
action taken on the basis of this manual.

Oculoplastics

Editor Balaji Gupta MD
Revised by Noel Saks MD

I. Basic Science/Exam
Evaluation: Six P's
    Pain
inflam or infxn
orbital hemorrhage
malig lac gland tumors
nasophr ca
    Proptosis
    No Mass
cellulitis (most common cause in peds)
mass displaces globe away when present
inflammation: thyroid, pseudotumor, etc
    Axial Globe Displacement:
mass w/in muscle cone
cavernous hemangioma
glioma
meningioma
mets
avm
    Superior Globe Displ:
max sinus tumor
    Down + Medial Globe Displ:
dermoid cysts
lac gland tumor
    Down + Lateral Globe Displ:
frontoethmoidal mucocoele
abscess (subperiosteal)
osteoma
sinus ca
OU proptosis:
                                                                           2
thyroid (TAO) most common                       lymphoma
vasculitis                                      pseudotumor
mets                                            c-c fistula
leukemia                                        neuroblastoma
    Enophthalmos:
s/p blowout
sclerosing tumor i.e. breast ca mets (indents globe b/c it can’t move)
    Progression
days to weeks:
rapid thyroid eye disease                       cellulitis
hemorrhage                                      pseudotumor
thrombophlebitis                                rhabdomyosarcoma
neuroblastoma                                   granulocytic sarcoma.

months to years:
dermoids                                        benign mixed tumor
neurogenic tumor                                cav hemangioma
lymphoma                                        fibrous histiocytoma
osteoma                                         mets
     Periobita
classic signs:
salmon-colored mass in cul de sac : lymphoma
 lid lag and retraction & vasc injxn over rectus muscles : TAO
 corkscrew conj vessels : av fistula
 vasc anomaly over lid skin : lymphangioma, varix, or cap hemangioma
 S-shaped eyelid bag o’ worms : plexiform neurofibroma
 eczematous lid lesions : mycosis fungoides
 ecchymosis of lid skin : neuroblastoma, leukemia, amyloidosis
 prominent temple : sphenoid wing meningioma
 edematuous lower lid + optociliary shunt vessels : meningioma
 gaze-evoked amaurosis : optic n. sheath meningioma, orbital apex tumors
 frozen globe : mets or phycomycosis (mucor)
 black crusted lesions : phycomycosis
 ant uveitis: pseudotumor or sarcoid
 facial asymmetry: fibrous dysplasia, neurofibromatosis
     Pulsation
     w/o Bruits:
neurofibromatosis
meningoencephaloceles
post op ie s/p removal orb roof
     w/ Bruit:
 c-c fistula
dural av fistula
orbital av fistula
     Palpation
     Superonasal Mass:
mucocele                                        mucopyocele
                                                                                           3
encephalocele                                  neurofibromas
dermoids                                       lymphoma
    Superotemporal Mass:
dermoids
prolapsed lac gland
lac gl tumor
lymphoma
 pseudotumor
Exam (Orbit)
    Hertels
 normal whites 16-21 (some say <18 for F), blacks 18-24 (M>F), asymmetry = >2mm diff
    Palpation
 firm or soft
 anterior extent (palpation in the office makes lesions appear more anterior than at time
of surgery due to no anesthetic infiltration, displacement of fat with palpation, and upright
patient position)
 pulsations absent or present (see six P’s)
 lymph nodes
    CT (orbital)
 best for bones and acute trauma, bone destruction, calcification & esp blowout fx
must ask for axial and direct coronal cuts specifically or less useful reformatted coronal
images will be given; coronals also good for thyroid eye muscle eval; realize that pt’s
neck must be hyperextended for direct coronals & is unreasonable in some trauma, etc.
 modify cut size according to suspected pathology: 1 mm cuts often only nec for foreign
bodies and other distinct pathology, not most blowout fxs
contrast (iodine-containing) for well-vascularized tumors ie meningiomas; abscesses
    MRI
 best for soft tissues:
orbital apex                                     periorbital spaces
orbitocranial tumors                             demyelinating dz
vascular                                         hemorrhagic dz (fresh vs. old)
not bones
in general, pathology enhances w/ T2 images, T1 = best anatomy
 info in multiple planes w/o having to move pt.
 can’t use w/ metal in body, dental fillings ok but produce artifact
 IV gadolineum will brighten vascularized lesions to fat-density brightness, then need fat
suppression to visualize enhanced lesions
    MRA (Angiography)
 noninvasive visualization of arterial system, esp for aneurysms
 conventional arteriography used less frequently b/c of risk
    Ultrasound
useful in pre-op of orbital foreign bodies which may not show on CT, MRI
    Skull Plain Films
much less useful than CT, rarely indicated
in unilateral proptosis, abnormal in up to 40%
if optic foramen > 6.5 mm with asymmetry, consider glioma
    Labs
thyroid: T3 , T4, TSH 1st. If nl, antithyroglobulin abs and antimicrosomal abs, maybe
                                                                                               4
TRH. This is good enough in 60-70% of Graves pts
Wegener granulomatosis: pts w/orbital inflamm dz, sinus opacification, lung/kidney
probs: get ANCA (antinuclear cytoplasmic antibody) test
sarcoid: ACE, lysozyme in chronic orbital infl, esp w/ lac gland
Anatomy
     Orbit
     Dimensions
volume 30 cc, entrance height 35mm, entrance width 40mm, medial wall length 45 mm,
globe 25 x 25 mm.
     Bones
Frontal                                          Maxilla
Zygomatic                                        Lacrimal
Ethmoid                                          Palatine
Sphenoid
     Roof
frontal, lesser wing of sphenoid
features: trochlea 4mm posterior to margin, lac gland fossa, supraorbital notch/foramen
adj to ant cranial fossa and frontal sinus
     Medial
 maxillary, lacrimal, ethmoid, sphenoid
 adj to ethmoid and sphenoid sinuses, nasal cavity
 lacrimal gland fossa
 cribiform plate is at the level of the frontoethmoidal suture
 medial wall of optic canal is in the lateral wall of the spenoid sinus
 lamina papyracea is thinnest wall of orbit, covers ethmoid sinuses
     Floor
maxillary, zygomatic, palatine
infraorbital groove & canal
IO origin
slopes 20 degrees down
maxilla is thin in posteromedial aspect, subject to blowout fx where lamina papyracea
extends; in kids ethmoid sinusitis can extend thru l.p. to cause orbital cellulitis
forms roof of maxillary sinus
     Lateral
 zygomatic, greater wing of sphenoid
 lateral orbital tubercle of Whitnall below F-Z suture, = attachment site of lateral canthal
tendon
globe more vulnerable to trauma laterally since bone only protects posterior half
adj to middle cranial fossa, temporal fossa, temporalis fossa
     Orbital Foramina
         anterior/posterior ethmoidal foramina
medial wall along FE suture
ant and post ethmoidal blood vessels and nerve
provides route of entry for infections and tumors of the ethmoid sinus
         superior orbital fissure
separates greater and lesser wings of sphenoid
superior/inferior sections are separated by LR origin
CN III, IV, V1,VI, sympathetics
                                                                                              5
most orbital venous drainage exits via sup ophthalmic vein to cavernous sinus; look here
on CT for dilated vein in c-c fistula
        inferior orbital fissure
bounded by S, M, P, lies btw lateral orbital wall & orbital floor
V2, infraorbital nerve is branch of maxillary (V2); blowout fx of orb floor causes lower
lid, cheek, upper lip, teeth numbness

inferior ophthalmic vein (drains to pterygoid plexus)
         supraorbital foramina
supraorbital nerve (V1), blood vessels
         infraorbital canal
 infraorbital nerve (V2)
         zygomatic foramina
ZF canal has ZF branch of zygomatic nerve, blood vessels thru lat wall to cheek
ZT “ “ “ “ ZT “ “ “ thru lat wall to temporalis fossa
         nasolacrimal canal
from lac sac fossa to inf meatus beneath inf turbinate in nose; thru this passes NLD
         optic canal
8-10 mm long, w/in lesser wing of S; separated from sup orb fissure by bony strut
CN II, ophthalmic artery, sympathetics
orbital end is optic foramen, nl = 6.5 mm dia, enlarged in optic n. glioma
if blunt trauma causes o.c. fx or apex hematoma, o.n. damage
     Optic Nerve
 intraorbital part = 30 mm long, 4 mm dia
 surrounded by pia, arachnoid, dura cont w/ brain
     Extraocular Muscles w/Nerves, Orbital Fat
all EOM’s except IO originate @ orbital apex
all 4 rectus m’s originate @ annulus of Zinn
levator m originates above annulus on lesser wing of S
SO originates medial to levator then heads ant to trochlea
IO originates from ant orb floor lateral to lac sac
in ant orbit, intermuscular septum connects recti, divides intraconal fat (central surgical
space) from extraconal fat (peripheral surgical space)
sup CN III to SR, levator; inf CN III to IR, MR, IO; LR6 SO4
CN IV is only CN to EOM which doesn’t pass directly into muscle cone entering orbit
     Orbit Vasculature
arterial mostly from ophthalmic a. from int carotid a., some from ext carotid
ophth a. to EOMs; central retinal via o.n.; post ciliary a.
superior ophthalmic vein drains most of orbit thru sup orb fissure to cav sinus: dilated in
cc fistula
     Lacrimal Gland
larger orbital lobe, smaller palpebral lobe
w/in lac gl fossa in frontal bone, superotemporal orbit
ducts from both lobes go thru palpebral lobe so don’t bx it
lac gl prolapse inferiorly out of fossa w/age
     Nerves
see neuro-ophthalmology
     Eyelids
usually more bleeding nasally due to larger caliber of vessels medially and prominant
                                                                                           6
loops
plica semilunaris-many goblet cells, remnant of nictitating membrane
     Skin
thinnest in body, no subcut fat
upper lid crease (fold) = levator apon. attachment to pretarsal orbicularis and skin; located
@ level of sup border of tarsus
upper punta is more medial
mucocutaneous border is post to meibomian gland level
grey line = muscle of Riolan (superficial orbicularis)
Zeis, sebaceous glands (holocrine) with cilia
Moll glands (only apocrine gl on lid) with skin
100 lashes on upper lid, 50 on lower
     Subcutaneous tissue
no fat, loose connective tissue holds fluid in preseptal > pretarsal area b/c less firmly
attached
     Orbicularis Muscle
main protractor
supplied by CN VII, narrows PF, helps lac pump
    orbital
voluntary sphincter (wink, blepharospasm)
origin @ medial canthal tendon and corrugator supercilius muscle
    palpebral (pretarsal & preseptal)
reflex blink and involuntary
pretarsal origin @ post lac crest (most important to keep lid apposed to globe to let
puncum lie in tear lake ) & ant limb of med canthal tendon; deep head of pretarsal m.
(Horner’s tensor tarsi) encircles canaliculi to facilitate tear drainage
upper & lower segs of pretarsal orb m. fuse to become lateral canthal tendon
pretarsal muscle firmly adherant
pretarsal muscle of Riolan = gray line = superficial orbicularis
     Septum
extension of periosteum
in non-Asians, upper lid septum fuses w/levator apo. 2-5 mm above sup tarsal border; in
lower lid it fuses w/capsulopalpebral fascia @ or just below inf tarsal border
passes medially in front of trochlea
barrier to heme & infxn btw lid and orbit
orb fat can herniate thru septum into lids causing bags
central orb fat pad lies behind septum, in front of lev apo.
     Lid Retractors
upper lid = lps w/ apo, Muller’s
lower lid = cpf, inferior tarsal m.
     Levator Palpebrae Superioris
starts just above annulus of Zinn, then 40 mm of muscle, then 14-20 mm of apo.
becomes vertical near Whitnall's (superotransverse) ligament (near transition m. to apo.)
which is a fulcrum for vert lid retraxn (lower lid analog is Lockwood’s ligament)
Whitnall's ligament is condensation of tissue around SR and LPS, helps suspend tissue
levator aponeurosis: attaches to lower 1/2 of ant tarsus; lat horn of apo divides lac gl into
orb and palp lobes, attaches to lat orb tubercle; medial horn attaches to post lac crest
lid crease is formed by attachment of ant portion of apo w/ septum btw the pretarsal
orbicular m.’s: here the pretarsal tissues are in close apposition to underying tarsus
                                                                                           7
    Tarsus
dense connective tissue, attach med & lat to periosteum
1 x 29 x 11 mm upper lids, 4 mm vert height in lower lids
meibomian glands are modified holocrine glands
in upper lid marginal arcade lies 2 mm sup to lid margin, ant to tarsus
peripheral art arcade is sup to tarsus, btw lev apo, Muller’s
    Superior Tarsal Muscle of Muller
posterior to LPS
sympathetically innervated; use neo gtt to test function in ptosis w/u: nl = 2 mm lift
origin from under LPS, attaches to upper tarsus, firm attachment to conj
    Capsulopalpebral Fascia
lower lid analog to lev apo
originates from attachments to IR; therefore do vertcal m. surgery before lid surgery
inserts onto lower tarsal border
inf tarsal m. is analog to Muller’s, runs post to cpf
    Conjunctiva
 mucin from goblet cells, aq from gl Krause & Wolfring
    Vasculature
extensive anastamosis btw supraorb, lacrimal branches of ophth a. (from int carotid) and
angular and temporal a. (from ext carotid)
venous drainage: pretarsal, poatarsal
NO lymphatics for the orbit except in conjunctiva
eyelid medial lymphatics drain to submandibular nodes and laterally to preauricular nodes
    Lacrimal Gland and System
    Lacrimal Gland
separated from orbit by fibroadipose tissue
divided by LPS aponeurosis, smaller palpebral portion visible, larger orbital half hidden
blood vessels, lymph, nerve, and excretory ducts pass from orbital part through palpebral
section, so don’t bx or remove palp sxn or can therefore significantly reduce tearing
ducts empty 5mm above superior tarsal border
reflex tear arc: afferent from V stims tear production from lac gl; efferent complicated (w/
parasymp’s from VII, sympathetics not understood)
exocrine gland, acinar and myoepithelial cells, lacrimal artery
    Accessory Glands of Krause and Wolfring
 no neural control, basal tear production (BST)
 located in sup fornix & above sup border of tarsus
    Canalicular System
puncta sit in tear lake, approx 6mm from canthus, then 2 mm ampulla, then canaliculi
extend medially 8-10 mm to common canaliculus (in 90% of population), then to lat wall
of tear sac
dilation prior to sac: sinus of Maier, enters sac superior and posterior
valve of Rosenmuller prevents reflux from sac into common canaliculus during tear pump
sac lies btw ant and post crura of med canthal tendon in lac sac fossa
puncta/lids move medially with lid closure
deep heads of preseptal orbicularis (Horner’s muscle) inserts on post lac crest, lateral half
of superior lacrimal sac, encircles canaliculi to help pump
    Bony System
interosseos direction of NLD = inf and sl. lateral, posterior
NLD is approx 12 mm long, intranasal ostium high up in inf turbinate, covered by valve
                                                                                           8
of Hasner, approx 2.5 cm post to naris on lat wall
lacrimal bone very thin, therefore aim posteriorly in DCR
ethmoid air cells are at superior and deeper parts of fossa, but may extend under entire
fossa
mucosa of ethmoid cells gray, thin, and friable
    Lacrimal Pump Model
orbicularis actively pumps tears from lake
Rosengren-Doane model: orb m contraction > + pressure in lac sac > tears forced into
nose > lids open, move laterally > - pressure in sac helped by closed valve of Hasner >
lids open fully and puncta pop open, w/ - pressure drawing tears into ampulla and
canaliculi
Jones model: closure--lateral move = neg pressure
Becker model: closure--upper half lateral move = lower pressure, lower half medial
move with higher pressure
fistulas develop inferior to medial canthal tendon b/c tendon itself is tough

II. Childhood Orbit
 hypertelorism is increased distance between medial orbital walls
 telecanthus is increased distance between medial canthi w/ nl interpupillary dist
 exorbitism is widely divergent lateral orbital walls (>90 deg)
see pediatric ophthalmology

III. Adult Orbit
Infections
    Dacryoadenitis
infection rare, most commonly mumps, gonococcus, HZO, trachoma, mononucleosis and
actinomyces
    Preseptal Cellulitis
 inflam and infxn confined to lids and periorbita anterior to septum
 usually 2o to trauma or skin infxn in kids and adults
 EOM’s, pupils, Va, Hertels, all OK
 teens and adults can be closely followed as outpt w/ PO abx (Augmentin) but if
worsening then CT and admit for IV abx
under 5 yo may have bacteremia, esp from H flu (violaceous erythema w/marked lid
swelling are classic) otitis media or pneumonia, more severe dz, need IV 3rd gen
cephalosporin abx after blood cx taken
surgical drainage of abscess may be necessary; do not violate septum and cause orb
cellulits
    Orbital Cellulitis
infxn posterior to orbital septum
90% from extension of acute or chronic bacterial sinusitis, reaminder s/p trauma or
surgery or 2o to extension from other orbital or periorbital infxn, or endogenous w/septic
embolization
fever, proptosis, restriction of EOM’s, pain on globe movement
decreased Va, APD, prolonged high IOP can be indications for aggressive management to
prevent orbital apex syndrome or cavernous sinus thrombosis
CT of orbit and sinuses to confirm sinus disease, r/o mass, r/o orbital foreign body if h/o
trauma (even remote), r/o orbital or subperiosteal abscess which will require surgical
drainage
                                                                                             9
blood culture then broad spectrum IV antibiotics to cover gram + cocci, H. influenzae
(although less prevalent in kids 2o to immunization), anaerobes, typically nafcillin and
3rd generation cephalosporin; ID consult if necessary; kids more often single organism
progression of infxn or no daily improvement on appropriate abx can mean abscess:
repeat CT prn and drain w/concomitant sinus drainage prn
cavernous sinus thrombosis: rapid progression of proptosis and neurologic signs of
intracranial dysfunction; may lead to meningitis; get neurosurgery consult
Inflammation
     Thyroid Eye Disease; Thyroid-Associated Orbitopathy (TAO)
also called Grave’s eye disease, etc.
most common cause of unilateral and bilateral proptosis in adults
typically F (>M by 4X ) between 25 to 50 years old, but more severe in older patients and
men
majority have history of thyroid problems with idiopathic hyperthyroidism the most
common; up to 15% of women and 30% of men have no thyroid abnormality
acute episodes may occur up to three times during the course of the disease and last from
3 months to one year, with residual cicatricial changes in EOM’s and orb fat leaving resid
porptosis and diplopia
     Signs
this is a clinical diagnosis
EOMs are targets with IR, MR, LR, and SR in decreasing order being affected
upper and lower lid retraction (increased with fixation) is very specific and common
more frequent signs:
lid lag (upper and lower)           exophthalmos               diplopia
lid edema                           chemosis                   conj injxn over recti
increased IOP with elevation        keratopathy

less frequent signs
closed lid tremor        infrequent blinking                  difficult eversion upper lid
bruit over eye           decr forehead wrinkling with         increased hippus
                         upgaze
pigmented lids
     Optic Neuropathy Risk Factors
older age
less proptosis
increased MR size w/horizontal limitations
     Exam
hx, sx, thyroid meds and length of time on them
optic nerve function: Va, color vision, pupils for APD, optic nerve appearance,
HVF/tangent screen
lid, globe evaluation: ptosis workup, note retraction, measure sclera below, MRD2 (reflex
to lower lid margin), tear strips, SLE including K stain, IOP straight, in upgaze
orbit, EOM evaluation: diplopia fields, Hertel
check for goiter
     Labs
TSH, T4
consider TRH stimulation test
10% will be hypothyroid
                                                                                       10
if nl, consider repeat
     CT
to confirm dx,examine orbital apex, EOMs; coronals most helpful
muscles tendons are spared (vs pseudotumor)
multiple muscle enlargement w/ IR, MR esp affected virtually diagnostic but rarely
pseudotumor or amyloidosis can look similar
TAO associated with myasthenia gravis which can also simulate it, so consider chest CT
for thymoma
may need MRI for optimal orbital apex eval
     Orbitopathy Classification
    Type I
most often in women
minimal orbital and EOM inflammation
symmetric proptosis and lid retraction common
muscles are enlarged due to edema rather than myositis, less diplopia and chemosis
may have considerable keratopathy
    Type II
evenly divided among men and women
history of smoking
myositis, restrictive myopathy with diplopia, may be asymmetric, eye usually hypotropic,
esotropic
orbital inflammation and compressive neuropathy
chemosis and vasc engorgement over muscles frequent
unilateral or asymmetric
     Werner's Classification
NO SPECS, with each class in four grades 0-4, a, b, c: mild to severe
    0 No S/S
    1 Only signs (lid retraction)
    2 Soft tissue involved (chemosis, grit, etc)
    3 Proptosis (min <23, mod, marked >28)
    4 EOM involved
    5 Corneal involvement
    6 Sight loss
     Treatment
    local protective
treat exposure with ointments, drops
consider patching at night
    medical/ radiation
to decrease myositis, orbital edema, or optic nerve compression during acute phase,
consider 80-100 mg of oral prednisone for 6-8 weeks or IV methyprednisolone
if can’t wean off steroids after 6-8 weeks, consider radiation of 1500-2000 rads (may
complicate future surgery); cyclosporin
    surgical
wait for stability for at least 6 months before strabismus or lid surgery
STOP smoking which worsens lid retraction
correct strabismus (recession of a restricted rectus muscle, often on adjustable suture
preferable to resection of contralat muscle) before lid surgery, but after decompression
IR restriction can cause loss of lower lid retraction on downgaze or increased retraction
w/eye fixation
                                                                                        11
recession of eyelid retractors and lateral permanent tarsorrhaphy (if needed) useful in
cosmesis and to lessen exposure; one, two, or three wall orbital decompression for
cosmesis or optic nerve compression
    Hypothyroidism
myxedema (nonpitting) prominent in eyelids
loss of 1/3 of eyebrows, ?increased in COAG
treatment of thryoid abnl does not appear to affect orbitopathy for better or worse
    Pseudotumor; Orbital Inflammatory Syndrome
nonspecific, idiopathic, benign inflammatory process w/pleomorphic inflammatory
response, w/varying amounts of fibrosis
peak 40-50 yo, M=F, can affect kids and adults
multicentric inflammatory process w/variable but limited course:
sclerotenonitis                                  myositis
dacryoadenitis                                   perioptic n. inflammation
trochleitis              diffuse ant soft        sup orbital fissure & cavernous sinus
                         tissue inflammation syndrome (Tolosa-Hunt syndrome of
                                                 painful ophthalmoplegia)
in adults, bilateral involvement may mean systemic disease:
sarcoid                                          lymphoid tumor
metastatic disease                               vasculitis
syphilis                                         TB
Sjögrens syndrome
    Acute
mimics cellulitis with vision loss (if o.n. or post. sclera involved) so pts present with
acute eye pain, restricted movement, and proptosis, often conj injxn and chemosis
    Anterior Orbit Form
severe eye pain
may have posterior sceritis with greater ocular inflammation, pain to temples
   acute dacryoadenitis
pain superotemporally, swelling, redness, S shaped lid
palpate gland for tenderness
may involve LR
no proptosis or vision loss
   myositis
one or two muscle involvement only (thyroid much less likely if + myositis but no IR or
MR), with pain on movement, positive forced ductions
proptosis with resistance to ballotment
CT: muscle tendon involved & thickened (vs.TAO)
   posterior pattern
with signs/symptoms of orbital apex syndrome
    Subacute, Chronic
increased fibrous componant, less inflammation
insidious onset over weeks and months
PPLOV, diplopia, proptosis
chronic sclerosing variant usually with posterior orbital mass and slow onset of
symptoms, decompression won’t help b/c mass is non-maleable and won’t fill the
increased orbital volume
pain is variable, fibrosis common
                                                                                      12
     Pseudotumor in Children
1/3 bilateral
1/2 have HA, fever, vomiting, abdominal pain, lethargy
can have eosinophilia, high ESR, + ANA
     Tolosa Hunt
orbital inflammatory variant restricted to superior orbital fissure and cavernous sinus
     Diagnosis
clinical picture as above
may look like cellulitis w/o sinus involvement or h/o trauma
    CT:
ring sign of scleritis (tenonitis)
tendons inflamed
diffuse infiltrate with ragged, streaky edges
    U/S:
RD, choroidals or thickening of sclera, or edematous            Tenon’s
perineural and subTenon’s effusion causes the T sign at optic nerve junction
    FANG:
patchy choroidal filling, punctate areas of leakage
CME
     Path
pleomorphic cellular infiltrate w/ lymphs, plasma cells, eos w/ variable fibrosis
     Treatment
steroids 80mg qd, 5 mg/week taper; PF if w/anterior inflam; acute cases respond rapidly
if no response then IV steroids
if still unresponsive by 2 weeks, orbit bx, XRT 13000 cGys (rads)
cyclophosphomide 200 mg/day
rarely decompression needed if o.n. compression is severe
     Vasculitis
     Temporal Arteritis
jaw claudication, wt loss, tender temp art, fever, high ESR
CRAO or AION
see neuro-op
     Polyarteritis Nodosa
usually doesn’t cause orbital dz, more retinal and choroidal infarction
     Wegener's Granulomatosis
small vessel necrotizing granulomatous vaculitis of any organ system including orbit
lesions of upper and lower respiratory tract, necrotizing glomerulonephritis
+ANCA (anti-neutrophil cytoplasmic antibodies)
25% have scleritis
     Lethal Midline Granuloma
nebulous dz, relentless destruction of nose, paranasal sinuses, oral pharynx, palate
Orbital Trauma
    Blowout (Orbital Floor) Fracture
    Exam
CT w/ axial, direct coronal views most helpful to note EOM entrapment, size of fx, other
injury i.e. orbital heme (proptosis w/ high IOP), globe injury, CN II injury
 plain films OK for dx fx only
exam: complete eye exam plus:
                                                                                         13
Diplopia field: limit on up or downgaze: r/o        Hertel
IR entrapment vs hemorrhage/edema alone:
CT
hypoophthalmos (globe ptosis)                       orbit/lid emphysema 2nd to sinus wall fx
IOP str and upgaze (may inc w/IR entrapment) infraorbital anesthesia in floor fx
forced generation, forced duction                   lid measurements (PF and MRD1)
     Treatment
most do not require surgery
early surgery for marked muscle restriction confirmed on CT, forced duction
should observe 1-2 weeks, oral steroids (prednisone 1 mg/kg/day w/ taper) to decrease
swelling and fibrosis
antibiotics (Keflex) and nasal decongestants (Afrin), tell pt not to blow nose to decrease
orbital emphysema
     Surgery Indications
residual marked diplopia w/in 30o of 1o gaze 2o to        resrtiction (of IR)
large (50%) floor fx esp w/ large med wall fx b/c likely to get enoph
enophthalmos > 2 mm: usually there is initial proptosis 2o to orb infl/edema which
resolves; if + initial enoph or no prop, later surgery for enoph more likely
surgical repair easier within the first 2 weeks
surgical steps: usually inf fornix incision, elevate periorbita from orb floor, free tissues
from fx, implant (silastic or miniplate depending on size) over floor defect
     Medial Orbital Fracture
if indirect (blowout) extension of floor fx, no sx needed unless MR entrapped
lid/orbit emphysema common
direct naso-orbital fracture more serious, depressed nasal bridge; compl inlc
cerebral/ocular damage, ant ethmoid art. damage w/ severe epistaxis, CSF rhinorrhea,
traumatic telecanthus, needs miniplate stabilization
     Zygomatic Fracture
tripod fx often has 4 zygoma breaks @ lat & inf orb rim, zyg arch, lat wall of max sinus
can involve orb floor
if displaced, can have cosmetic deformity, trismus (2o to impingement on coronoid
process of mandible)
     Orbital Apex Fracture
often w/traumatic optic neuropathy (needs spinal cord dose IV steroids, maybe
decompression w/in 5 days, see neuro-op), other fractures
look for CSF rhinorrhea, CC fistula
     Orbital Roof Fracture
infrequent
may have intracranial lesions, CSF rhinorrhea, pneumocephalus
neurosurgery consult
     Orbital Emphysema
if severe can cause CRAO, etc if loculated ball valve type wound
usually smaller medial wall injuries
air usually located in area of wound
if decreased Va, high dose steroids
air decompression:
    CT for localization
retrobulbar needle into air pocket
fill syringe w/saline, take out plunger, watch for bubbles to appear
                                                                                                   14
look on CT for incranial air: needs neurosurgery consult
    Intraorbital Foreign Bodies
    Imaging
plain film or CT if radio-opaque
MRI may be better for some
u/s good option
    Removal Indications
esp if vegetable matter, or easy to get to in anterior orbit
may watch if inert, smooth edges, or in posterior orbit
    Blunt Forehaed Trauma
can cause visual loss from traumatic optic neuropathy 2o to direct (stretch,tear, contusion,
etc) or indirect (small nutrient vessels to nerve) injury to fixed canalicular or chiasmal
portion of o.n.
Treatment: spinal cord injury-dose I.V. steroids (see neuro-op sxn)
Tumors
(adult orbit discussed here; see Peds for hamartoma/choristoma, lipodermoid, teratoma, capillary
hemangioma, lymphangioma, optic n. glioma, neurofibroma, rhabdomyosarcoma, ped mets )
pertinent history:
prior cancer                                        neurofibromatosis
diabetes                                            sarcoid
TB                                                  proptosis
pain                                                double vision
inflammatory symptoms                               rate of progression
w/u includes all for thyroid, plus palpation, retropulsion (1-4), facial asymmetry,
transillumination, all need CT/MRI
observe most benign tumors (capillary and cavernous hemangioma, dermoid cyst) and
tumors not easily resected (lymphangioma, plexiform neurofibroma, optic nerve
meningioma, juvenile pilocytic astrocytoma)
benign tumors of orbit usually primary
S-100 staining for any neural crest cell derivative
    Primary Orbital Tumors
    Dermoid Cysts
if not noted until adulthood often not palpable b/c in post superotemporal orbit
choristoma: has tissue elements foreign to site
may displace globe, adnexa inferonasally
may erode/remodel bone, adhere to dura
keratin leak is inflammatory, can cause scarring
surgery difficult, try to keep wall intact
    Vascular Tumors
        Cavernous Hemangioma
 most common benign solitary lesion in adults
30-60 yo female is typical
slow axial proptosis over 3-5 yrs, averaging 5mm
retinal striae                                         hyperopia
strabismus                                             ON compression
rapid growth in pregnancy                              increased IOP
CT: smooth discrete lesion, fills with dye after 20 min; coronal cuts important to know
tumor position relative to o.n. for sugical plan
                                                                                         15
MRI: hypointense to fat on T1, hyperintense to fat on T2
U/S: high reflectivity (A-scan high amplitude internal echoes)
well encapsulated and tolerated
path shows large cavernous spaces w/rbcs
surgery for symptoms esp optic nerve compression
usually lateral orbitotomy with complete resection from intraconal position generally
feasible
       Hemangiopericytoma
uncommon, 30-60 yo
encapsulated, hypervascular and hypercellular
well defined with contrast CT
conjunctival prolapse, hyperemia of cul de sac conjuctiva
may restrict motility
looks blue at surgery
must be completely excized b/c can recur and be malignant
benign-appearing lesions may become cancerous, recur and metastasize, while
histologically “malignant” lesions may remain localized
       AVMs
developmentally abnl formed arteries and veins w/o intevening capillary bed
corkscrew episcleral vessels
treatment: first arteriography, then occlusion of feeder vessels before surgery; otherwise
exanguination possible
       AV Fistula
trauma or degeneration causes communication btw previously nl artery and vein
spontaneous/degenerative fistula often w/HTN, atherosclerosis
most commonly cavernous sinus-internal carotid artery (CC) fistula
h/o basal skull fracture most common cause         ischemic eye damage 2o to art. “steal”
atherosclerosis, HTN                               bruit
tortuous epibulbar vessels                         pulsating proptosis
pain                                               CN VI palsy 2o to high P in cav sinus
increased IOP                                      optic neuropathy
        direct
high flow and severe
dx often apparant
        dural
from extradural branch of ICA, ECA or both
low flow w/subtle sx: proptosis, mild pain
chronic red eye (2o to dilated episcl vessels)
asymmetric IOP (2o to incr episcl venous P)
CT w/ enlarged sup orb vein, diffuse enlargement all EOMs from venous engorgerment
small fistulas may spontaneously close
        Diagnosis
increased ocular pulse amplitude (differance of IOP systolic/diastolic BP) when compared
to fellow eye
can use pneumotonometer with paper strip for fifteen seconds; a difference of 1.5 mmHg
very suggestive of fistula
CT as above/MRI, orbital color doppler, selective arteriography
        Treatment
difficult
                                                                                      16
embolize vs ligation ICA
control IOP
may need orbital decompression
        Orbital Varicies
dilations of previously existing venous channels
proptosis w/dependent head position or post-Valsalva
CT w/contrast before & post-Valsalva shows enlargement of lesion 2o decreasing venous
return
treatment conservative; incomplete excision only when varix causes o.n. or globe damage
     Histiocytic Tumors
Litterer Siwe, eosinophilic granuloma, JXG
lytic skull lesions, proptosis
     Neural Tumors
        Meningioma
benign but invasive, usually arise intracranially (therefore these are secondary) from
arachnoid, extend thru bone, sup orb fissure, or optic canal into orbit
often w/ progressive vision loss, mild proptosis, female predominance
can be seen w/ NF but much less often than gliomas
    if near the sella
o.n. compression w/ early Va loss
o.n. shunt blood vessels, papilledema, o.n. atrophy
    if near the pterion (post end of spheno-parietal fissure @ lat portion sphenoid bone)
proptosis, temporal fossa mass
lower lid edema, chemosis
    primary orbital
typically arise from arachnoid of o.n. sheath
affect vision early, mean age @ presentation is lower, worse prognosis in children
can invade EOMs and globe
CT shows radiolucent center compared to o.n. glioma (denser center)
    diagnostic tests
skull film: hyperostosis associated w/Ca+2 of tumor, lytic lesions can be seen
CT as above, and hyperostosis w/ abnl Ca+2 of tumor
MRI to outline tumor extent
    Treatment
transcranial orbitotomy surgery on o.n. meningioma will cause blindness so only operate
if useful vision lost or if intracranial structures threatened
        Schwannomas (Neurilemomas)
young to middle aged
slow proptosis over yrs up to 4 mm
occur in 1% of pts with NF1
benign
choroidal striae with hyperopia, usually no diplopia
usually superior orbit
perineural spread and compression can cause pain
at surgery it is well encapsulated, yellow, and can be stripped away from nerves
can recur but low malignant potential
proliferations of Schwann cells encapsulated by perineurium
Antoni A (Verocay bodies, whorls and palisades) and B (myxoid with degeneration)
patterns, no axons present
                                                                                     17
S100 positive
        Neurofibromas
most common nerve sheath tumor
hamartoma of endoneural fibroblasts, Schwann cells, axons
   localized
present in early to middle age
like schwannomas, 10% have NF1
not encapsulated but cannot tell from CT/MRI
can be multiple
remove by superior orbitotomy, can undergo malignant transformation
   plexiform (diffuse)
seen in children (see Peds)
        Optic Nerve Glioma
primarily a tumor of childhood (see Peds)
malignant optic glioma (glioblastoma) is very rare; middle-aged males, rapid blindness
and death
    Lacrimal Gland Tumors
look for fullness of upper lid, asymmetry of superior sulcus, abnormal lid contour
majority lac gl masses are idiopathic inflammatory dacryoadenitis
CT very goood for differentiating inflam from tumor: inflam and lymphoid w/in gland
cause diffuse enlargement, elongated shape, contour around globe; neoplasms are
isolated, globular, displace & indent globe
especially S-shape, often palpable
check for mobility, smooth, rubbery or nodular
proptosis is evidence of posterior growth, otherwise globe is down and medial
        Epithelial Tumors:
50% benign mixed
50% carcinomas (50% of these are adenoid cystic, remainder: malignant mixed, 1o adeno
ca, mucoepidermoid ca, squamous ca)
        Benign Mixed Tumor (Pleomorphic Adenoma)
most common epithelial tumor
30-50 yo, M sl>F
palpable, painless, slow (hx often reveals sx > 1 year) growing w/globe displ down,
medial, axial proptosis
incites bony cortication, enlargement/expansion lac gl fossa, firm lobular mass
   CT
gland is oblong if inflammatory, globular if malignant
   pathology
metaplasia of epithelial cells to form stroma, cartilage
benign epithelial cell nests with loose mesenchymal connective tissue
variability of above = mixed tumor
microscopic extension into pseudocapsule causes recurrence if margins not adequate @
excision
   treatment
must excise it all w/ lateral orbitotomy with en bloc excision including pseudocapsule;
don’t biopsy b/c of 1/3 chance of recurence, sig risk of malignant degeneration
        Malignant Mixed Tumor
often arise from 1o benign mixed or from recurrent benign mixed if incomplete excision
path similar to benign mixed but w/malignant change
                                                                                                      18
least common epithelial tumor
    treatment
frequent exenteration, bone removal necessary
fatality rate of 50%
        Adenoid Cystic Carcinoma (Cylindroma)
most common (highly) malignant tumor of lac gl
PAIN from bone destruction, perineural invasion, rapid course diffs from benign mixed
    pathology
swiss cheese appearance, stain with mucicarmine, looks benign, infiltration of orbital
tissue, incl perineural invasion
basaloid pattern worst prognosis
    treatment
radical orbital exenteration (of roof, lat wall, floor, orb soft tissue, ant temporalis m.) , w/XRT
death from intracranial extension or systemic mets after multiple recurrences
        Nonepithelial Lacrimal Gland Tumors
        Inflammatory
1/2 of lacrimal tumors
orbital inflammatory syndrome: see pseudotumor
sarcoidosis: African or Scandinavian descent, systemic dz, non-caseating granuloma
w/monos, typical bilateral lac gl involvement, conj bx or lac gl bx or gallium scan may
establish dx, ACE, lysozyme, CXR for hilar adenopathy, anergy on skin test
benign lymphoproliferative lesions of lac gl: middle aged F, dry eye, if w/rheumatoid
arthritis, then classic Sjogren’s
     Fibro-osseous Tumors
uncommon, may involve orbit
        Fibrous Dysplasia
benign proliferative disorder
single bone or polyostotic (w/cutaneous pigmentation and precocious puberty in F =
Albright syndrome)
        Osteomas
benign, involve frontal sinuses
malignant chondro- or osteosarcomas destroy bone
     Lymphoid Neoplasms
almost exclusively in adults
continuum incl benign reactive lymphoid hyperplasia (pseudolymphoma) to atypical
lymphoid hyperplasia to low-grade then high grade malignant lymphoma; also
OIS/pseudotumorplasmacytoma (incl myeloma)
bimodal peak 30’s and 60’s
unilateral or bilateral
palpable rubbery mass fixed to rim
malig lymphoma & reactive lymphoid hyperplasia cause gradual (over a year or more)
progressive, painless proptosis (vs OIS), lacrimal enlargement
usually on conjunctiva, anterior orbit so palpable or visible
eyelid or bilateral orbital involvement suggests systemic disease
putty-like molding to undisplaced tissues so little Va or EOM loss; usually no bone
erosion or infiltration unless high-grade lymphoma
lymphoma in retrobubar fat is infiltrative
all pts w/ orbital lymphoid lesions need exam for systemic lymphoma (by oncology) w/
                                                                                     19
orb/abd/chest CT; CBC; check LN; bone marrow bx; CXR; bone/liver/spleen scan
cytological factors are more prognostic than mono/polyclonal; but most benign lesions
(reactive hyperplasia) are usually mostly T cells w/ polyclonal Bs; malignant lymphoma
usually more monoclonal B cells
both polyclonal and monoclonal varieties can develop systemic disease
open bx for path to give fresh tissue for touch preps; immunohistochemistry; flow
cytometry; and gene rearrangement studies; in formalin for micro; gluteraldehyde for EM
benign reactive hyperplasia: up to 25% have systemic lymphoma later on
atypical lymphoid hyperplasia: 40% get systemic within 5 yrs
    treatment
XRT for most orbital lymphoid lesions that are confined to orbit (50% of lymphomas)
chemorx for systemic, rx can be controversial
     Secondary Orbital Tumors
     Intraocular Origin
choroidal melanoma, retinoblastoma
     Eyelid Origin
sebaceous gl ca, squamous cell ca, basal cell ca
     Sinus Origin
  o
2 orb tumors most commonly from nose, paranasal sinuses
     Brain Origin
sphenoid wing meningioma, may compress optic n., supraorbital fissure, F>M
     Nonepithelal Tumors from Sinuses, Nose, Facial Bones
osteoma, fibrous dysplasia, sarcoma most common
     Mucocele/ Mucopyocele
progressive cystic enlargement of sinuses from ostia blockage
usually no clear hx of sinusitis, trauma, infection, or polyps
cystic, lined w/respiratory epithelium, filled w/thick mucoid secretions
invade orbit by expansion, erosion of orb wall bones, usually from frontal or ethmoid
CT diagnostic
        Frontal Sinus
most commonly w/inferior globe displacement and diplopia
can present as discharging lesion of upper lid
if infected (pyocele), can have fistula through upper lid
        Anterior Ethmoidal Sinus
medial canthal mass with telecanthal appearance
less diplopia
r/o encephalocele
        Sphenoid and Posterior Ethmoidal Sinuses
optic nerve compression, VF defects
cranial nerve palsies, pain
        Maxillary Sinus
sudden nontraumatic enophthalmos
        Treatment
surgical evacuation, reestablish drainage vs sinus obliteration
     Squamous Cell Carcinoma
most common epithelial tumor secondarily invading orbit
maxillary sinus, nasopharynx, oropharynx origin
nasal obstruction, epistaxis, epiphora
    treatment
                                                                                       20
sx excision w/XRT, exenteration if periorbital involvement
    Fibrous Histiocytoma
40 yo, upper nasal orbit benign tumor
path: histiocytes, fibroblasts, cartwheel (storiform) pattern
    Metastatic Tumors
breast mets in F, lung (bronchogenic) mets in M most common
pain, proptosis, inflammation, bone destruxn, early ophthalmoplegia
+ h/o known 1o tumor in 75%, orb met = presenting sign in 25%
EOMuscles often involved
CEA, FNA vs orbitotomy for bx
breast mets can cause fibrous response w/enophthalmos, motility restriction
fresh tissue for hormone receptor
prostate mets can cause pseudotumor picture
   treatment
palliative local radiation
wide excision for some isolated mets
Enucleation
entire globe removal for blind/painful, maligancy, cosmesis, severe trauma w/S.O. risk
     Orbital Implants
usually 20-22 mm for adults, <18 mm rarely indicated even for very young
     Inert Spheres
silicone, methyl methacrylate; inexpensive; relatively poor motility
     Hydoxyapatite
bioreactive: vascularization can take 6-12 months, +/- sew recti to original positions
can drill and place peg after 6-9 months (w/MRI or bone scan confirmation of
bioincorporation) for excellent direct prosthesis-shell motility
higher exposure rate
        Exposure
can occur w/in first 3-4 months, rarely after one year
10% when wrapped in banked sclera
can be difficult to treat
if < 3mm in diameter, can heal spontaneously
larger defects may need burring down of implant with drill and mild debridement of
conjunctiva and Tenon’s
may need a dermis fat graft to cover the central defect
     Porous Polyethylene (Medpore)
bioreactive petroleum-based implant
less expensive than hydroxyapatite, probably easier to work with, motility arguably equal
     Evisceration
globe contents removal; sclera, o.n. intact
     Advantages
less anatomy disruption
+/- good prosthesis motility
some prefer in endophthalmitis
simpler prcedure can even be done @ bedside in unstable pt
     Disadvantages
unsuspected intraoc tumor
sympathetic ophthalmia risk (should remove all uvea)
                                                                                         21
     Socket Complications
     GPC
esp w/prostheses
may need rx w/ mast cell stabilizers
good prosthesis hygiene is essential
     Deep Superior Sulcus
need to increase orbital volume by either larger secondary implant or a second implant
subperiosteally under the first
tighten lax lower lid if present +/- dermal fat graft
     Fornix Contracture
prevent by preserving conj, wearing conformer post-op
     Implant Exposure/Extrusion
    causes
anterior implant placement                       inadequate Tenon’s closure
post op infxn                                    poorly fit prosthesis or conformer
pressure points btw implant and prosthesis subject to infection so should be replaced
                                                 or covered with patch or dermal fat grafts
     Contracted Socket
fornices can’t hold a prostheic shell
causes:
radiation treatment                                 implant extrusion
severe initial injury: alkali, large lacs           poor surgery
multiple operations                                 removal of prosthesis for long time
    treatment:
incise/excise scarred tissue
full thickness mucous membrane grafts
     Misc Anophthalmic Problems
    ectropion
2o to prosthesis weight
Rx w/ tightening lateral (occ. medial) tendon
    entropion
2o to contracture/cicatrix
Rx w/marginal rotation vs. mucous memb graft
    ptosis
  o
2 to implant migration, upper fornix cicatrix, levator damage
Rx w/internal levator advancement (Mullerectomy) b/c predicable & makes higher crease
vs ext levator advancement w/intra-op adjustment
     Exenteration
removes orbital soft tissues, incl globe
for destructive tumors extending into orbit not responsive to XRT; intraoc malig
melanoma or RB w/extension outside globe, w/o mets; malignant epithelial tumors of lac
gl; sarcoma and other tumors that don’t respond to non-sx; phycomycosis
subtotal, total, extended

IV. Lacrimal System
Adult Aquired Tearing
see Peds for congenital
    Etiology
                                                                                           22
eye surface irritation/reactive hypersecretion
outflow obstrutionn
1o idiopathic hypersecretion
     History
unilateral vs bilateral
nasal/sinus/facial fracture or       bloody tears/pain w/o inflam:       nasal polyps, sinusitis
surgery                              r/o tumor
hay fever                            external ocular irritation          Bells palsy
epinephrine                          phospholine iodide                  IDU
     Exam
check puncta for stenosis, position              conjunctivochalasis
conjunctival injection                           trichiasis
entropion                                        pseudoepiphora: tear eval (meniscus,
                                                 TBUT)
keratopathy                                      lid stiffness
lid laxity                                       pump fnxn/lagophthalmos
check VII nerve                                  look up nose
Schirmer 1                                       push on sac, look for d/c, +=NLDO
BST                                              DDT
     Dye Disappearance Test (DDT)
fluorescein to both fornices, look w/blue light for asymmetry after 5 min
confirms unilat epiphora
     Basic Tear Secretion (BST)
tear strips after anesthesia
     Primary Dye Test
fluorescein to eyes, blow nose, dye present or absent
     Irrigation
estimate flow through system
topical anesthesia
lower punctal dilation and irrigation, noting stenosis
drawing amount of flow 0-100%:
    reflux around canula or out superior punctum w/o lac sac distension = common
canaliculus block, if same + lac sac distension likely complete NLDO
    if no reflux but w/pain + lac sac distention = NLDO w/patent valve of Rosenmuller
    if reflux and drainage to nose = partial NLDO
     Secondary Dye Test = Informal Jones II
irrigate, dye present in nose = funtional NLDO, patent canalicular system, functional
pump; or absent
     Probing
diagnostic probing of adult upper system (puncta, canaliculi, lac sac) ok to find level of
obstrxn, not to probe NLD
     Dacryocystogram (DCG)
good for anatomy, not physiology eval
     CT
 if tumor suspected or post trauma
     Epiphora Causes
         Adequate Drainage
     Vth nerve stimulation: external/corneal dz/pseudoepiphora
                                                                                       23
dry eye syndrome                                conjunctivitis
blepharitis                                     uveitis
entropion                                       trichiasis
thyroid eye disease                             sinusitis
hay fever                                       dental prob
    VII nerve aberrant regeneration
post-Bell’s palsy; crocodile tears
may cause lac pump dysfnxn
    CNS
       Inadequate Drainage
    Stiff Lid
from burn, scar tissue, scleroderma
lac pump dysfnxn, punctal displacement
    Punctal Problems
       Agenesis
probe membranes, if none seen, do cut down (over probe)
can inject methylene blue inferior to medial canthal tendon
       Stenosis
rx with dilation, ampullotomy (one-snip), may need silicone intubation
       Eversion/Malposition
rx with internal vertical shortening, may need horizontal lid shortening for laxity
transconjunctival cautery inferior to puncta in bedridden pt
       Conjunctivochalasis
can occlude punctum
Rx: vasoconstrictor (Visine, etc) course then PF may be helpful
    Canalicular Problems
       Common Canaliculus Occlusion
trauma
meds: 5-FU, IDU, PI, eserine
viral
autoimmune (pemphigoid, Stevens-Johnson)
       Canaliculitis
mostly actinomyces israelii, gm+ filamentous rod, yellow concretions (sulfer), other bact
& fungi
Rx: compresses, antibx, curretage, canaliculotomy to remove concretions
       Functional Occlusion
may be total occlusion if poor pump fnxn
repeat probings
       NLD Obstruction
   Congenital see Pediatrics
   Acquired:
most commonly involutional                      F>M
trauma                                          FB
sinus dz                                        nasal polyps
sarcoid                                         Wegener's/other granulomatous dz
tumor                                           infxn (see below)
Lacrimal Sac
   Acute Dacryocystitis
                                                                                            24
many etiologies, all cause NLDO w/obstrxn of drainage from lac sac to nose
chronic tear stasis causes 2o infxn
edema, erythema below medial canthal tendon w/lac sac distention
+/- pain
r/o ethmoidal sinusitis, don't probe
     Treatment
warm compresses
oral/IV abx (Keflex, Augmentin), topical only limited value
I+D localized abscess
     Complications
mucocele, chronic conjunctivitis, orbital cellulitis
most need DCR after acute infxn subsides
     Chronic Dacryocystitis
distended lac sac w/min inflam, suppurative d/c from punctum c/w NLDO
probe/irrigate upper system only
dacryoliths from Actinomyces, Candida, topical meds, can cause lacrimal colic if
impacted in NLD
usually needs DCR for resolution
     Lacrimal Sac Tumors
rare, + mass above medial canthal tendon w/epiphora or chronic dacryocystitis
irrigation may pass to nose or blood may reflux from punctum
CT may show extent
may have skin ulceration, telangiectasia, + nodes
may originate from skin or nasal mucosal tumors
benign squamous papillomas most common 1o
squamous cell ca > adenoca most common malignant
Rx: dacryocystectomy; exenteration for malig ep tumor; XRT for lymphoid lesions, DCR
after 6 mo if no recurrence
Dacryocystorhinostomy (DCR)
for lower system block
goal: anastomosis btw lac sac and nasal cavity (middle meatus)
decreased success with prior nasal fractures, children, nasal mucosal disease, broad flat
nasal bridge, and reoperation
    Anesthesia
    General
b/c of pt anxiety during bony excision
    MAC with Local
with 2% lido/epinephrine with deep infiltration to bone
pack middle turbinate of nose w/ nasal neosynephrine/Afrin/Cocaine mixture
    Incision
avoid angular vessels, prevent bowstring contractures causing epicanthal folds
dissect skin from muscle with Tenzel periosteal elevators
spread orbicularis off periosteum w/ Steven’s or elevators just lateral to medial canthal
tendon (push to bone, then spread)
vertically incise periosteum 2 mm medial to ant lac crest, dissect away posteriorly, lac sac
reflected (supero)temporally
    Create Ostium
with hemostat, elevators, or drill, fracture posterior to ant lac crest thru lac bone/sac fossa
                                                                                         25
kerrison ronjeurs to make opening; be sure to get superior enough (under medial canthal
Tendon); smooth edges
inject local into nasal mucosa to blanch tissue and dissect from bone with muscle
hook(27 g 1.5 in needle)
incise mucosa over intranasal cotton swab with blade, make sure ant flap is large enough;
often no post flap
     Probe/Intubate
probe both canaliculi
cut into sac over probe, be sure to be completely thru sac (may need to pass wescotts to
make sure); make sure you have enough anteriorly
+/- relaxing incisions in flaps
suture posteromedial flap of lac sac to posterolateral lip of incised nasal mucosa if
possible using 5-0 PDS with P-2 needle
intubate and tie silastic tubes together at level of nasal osteomy w/ 6-0 nylon/silk to
prevent prolapse into eye
optional: bring balloon end of cut Foley catheter tube thru nose then thru ostium, fill
balloon then pack it back just inside nose to stent apart tissues, provide post-op
hemostasis, to be removed early post-op
suture anterolateral sac flap to anteromedial nasal mucosal lip using 2-prong hook for
exposure
close deep tissue w/ vicryl, then skin with suture of choice
tie ends of tubes together w/multiple throws, suture to lateral vestibule
Conjunctivodacryocystorhinostomy (CDCR)
use if >4mm upper and lower canalicular obstrxn, severe lac pump dysfnxn and canaliclar
stenosis
Jones (or Putterman-Gladstone) tube from caruncle thru osteotomy to middle meatus
tube needs daily post-op clearance by pt w/forced inspiration
Lacrimal System Trauma
canalicular lac 2o to sudden lateral traction
probe, check for medial canthal avulsion
can delay repair 24-48 hr; silicone intubation remains 3-12 mo
some consider not repairing single canalicular lac but 2o repair for epiphora harder

V. Eyelids
w/u all pts Va, pupils, motility, PF, MRD1, LF, lag, stain; genetic counseling/w/u for
congenital hereditary dz
Congenital Disorders
     Ankyloblepharon
partial or complete fusion of lids by skin webs
AD; incomplete penetrance
partial > complete fusion with lateral cathus > medial canthus
pseudostrabismus
    surgery
at 3-4 y.o.; clamp then cut webs, appose skin & conj w/suture to prevent readhesion; may
need total reconstrxn
     Blepharophimosis Syndrome
AD with high penetrance
short PF
                                                                                            26
congenital tetrad syndrome w/ epicanthus inversus, telecanthus, severe blepharoptosis and
phimosis
hypoplastic tarsal plate w/ tight lower skin leading to ectropion
vertical brow hair crossing in midline, flat brow, can be infertile F
w/out family history, consider:
Waardenburg’s                                          Williams
FAS                                                    Trisomy 18
oculodigital syndrome
Treatment:
    1st fix telecanthus, epicanthus inversus w/ Z- or Y-V-plasty
    2nd bilateral frontalis sling for ptosis 3-4 mo later
     Coloboma
embryological cleft; usually full thickness isolated defect in upper lid at inner 1/3 junction
20% bilateral; most often isolated anomaly
true coloboma = lid margin defect
lower lid at lateral 1/3 often w/systemic syndromes, esp AD Treacher Collins, Goldenhar
lower lid often partial thickness w/adj margin deformities, i.e. trichiasis
surgery based on defect size, K status, w/lower lids tolerated well; avoid occlusion in kids
always check NLD
     Crytophthalmos
partial or complete absence of eyebrow, PF, lashes, and conj
fusion of adnexa to globe
reconstruction difficult; severe intraocular defects
     Distichiasis
extra row of lashes in place of meibomian glands; rare
if symptomatic or + corneal irritation, may try lubricate, use CL’s or pull, electrolyze,
cryoepilation double freeze thaw vs eyelid splitting/follicle removal/mucosal graft
     Entropion
congenital is rare, isolated by defn
worsens with time as cilia are curled in, aimed at globe
surgery based on corneal involvement; often doesn’t spont improve
     Congenital Horizontal Tarsal Kink
tarsal rotation to appose globe
needs early surgery due to infection and scarring
     Ectropion
2o to vertical shortage of ant lid lamellae
congenital usually with Down’s or blepharophimosis, rarely isolated
    Treatment:
mild: no rx
severe w/sx: tighten horiz, lengthen vert w/skin graft
     Total Bilateral Upper Lid Eversion
variant in newborns w/marked chemosis, conj prolapse; idiopathic or 2o to inclusion
conjunctivitis, Down’s;
Rx: lubricate, moisture chamber w/spontaneous inversion, may need temporary
tarsorrhaphy
     Epiblepharon
bilateral mild skin excess in lower lid w/vertical cilia 2o to pretarsal orbicularis & skin
riding above lid margin, most commonly Asians
r/o frank entropion
                                                                                             27
Rx: observe, tends to disappear w/facial maturation; lubricate
surgery for corneal problems (lashes often touch globe in downgaze but rarely cause
probs) w/simple excision of skin or Quickert sutures
     Epicanthus
medial canthal fold 2o to immature facial bones or fold of skin, subcut tissue
rare at birth but up to 20% by 2 yo, usually bilateral; pseudostrabismus (eso)
e. palpebralis: fold (simple) equally distrbuted upper & lower lids; most common,
e. inversus: fold most prom in lower lid; often w/blepharophimosis
e. tarsalis: fold most prom in upper lid; Asians
e. supraciliaris: fold from eyebrow to lac sac
     Treatment
most correct w/facial growth but not inversus
wait for facial maturity then fix w/linear Z- or Y-V-plasty
     Euryblepharon
uni- or bilateral horizontally widened PF w/anteroinferiorly placed lat canthus w/lat
ectropion
antimongaloid slant; exposure keratopathy 2o to poor blink & closure, lag
usually isolated finding, r/o buphthalmos and craniofacial anomaly
observe since usually improves; o/w tarsal strips +/- vert lid lengthening w/skin graft
     Capillary (Strawberry) Hemangioma
1-2% of newborns, 40% in head and neck
most not present @ birth, start @ 1-2 wks, grow over 9-12 months; regress after age 2
(1/2 completely by 4 y.o. and 3/4 by 7 y.o.)
lesions blanch, can be invasive
can involve upper lid, superonasal orbit w/proptosis
can cause thrombocytopenia with plt entrapment, high output CHF: check plts, airway
     Treatment:
to prevent amblyopia secondary to occlusion, strabismus, and anisometropia, esp
astigmatism
         steroids:
1.5 - 2.5 mg/kg prednisone over weeks
40 - 80 mg triamcinolone w/25 mg of methylprednisolone
inject slowly around base of lesion and not into substance
minimal response in 10%
risks: emboli, lid necrosis, fat atrophy, pigmentary changes, adrenal suppression, retinal
vascular occlusion
         laser
         surgery
lid crease or transconj incision, avoid entry into encapsulated lobular mass
cauterize feeder vessels
best for isolated orbital masses, not large areas of skin
risks: blood loss, scarring
     Waardenberg syndrome
AD                                               medial canthal dystopia
telecanthus                                      lateralized puncta
medial ankyoblepharon                            bulbous nose
heterochromia                                    white forelock
deafness                                         synophrys
                                                                                            28
Eyelid Inflammation
    Chalazion
Zeiss or meibomian gland obstruction causing sebum release into tarsus, muscles and
(chronic) lipogranulomatous inflammation
Rx: wc/lid scrubs, +/- topical anti-infl meds, +/- PO or topical abx (if 2o bact infxn); inc.
& curettage w/post approach often w/intralesional triamcinolone for best response/least
recurrence (can depigment skin); ant approach if ant inflammation
bx recurrent or suspicious lesions for sebacious gl ca, basal cell ca
    Hordeolum (Stye)
acute staph lid infxn
Rx: wc, topical abx (erythromycin)
    Miscellaneous Lid Edema
 insect bite, anasarca, angioneurotic edema, allergy to pillow (unilateral AM edema),
post-surgical 2o to lymphatic interruption
    Floppy Eyelid Syndrome
horizontally lax lids open easily during sleep, cause chronic papillary tarsal response,
nonspecific irritation
seen w/obesity, mechanical pressure, keratoconus
Rx: horizontal lid shortening
Eyelid Measurements
test      measurement                                        normal
PF        palpebral fissure vertical                         9 mm
PFd       palpebral fissure vertical in downgaze             2-4 mm
MRD1      light reflex to upper lid margin                   4-5 mm
MRD2      light reflex to lower lid margin                   4-5 mm
MRD3      margin to corneal light reflex in upgaze
BLF       upper lid margin from down gaze to upgaze          12-18 mm
MCD       on down gaze lid margin to crease                  7-10 mm
MFD       on primary gaze lid margin to crease               4-5 mm
MLD       margin to 6 oclock limbus in upgaze                9 mm
lag       lagophthalmos                                      0 mm
HOF (1-4) herniated orbital fat diagram
MLD used in ptosis + vertical strabismus
Ectropion
    Involutional
most common pres: w/lax lat > med canthal tendons, often lower lid 2ogravity, epiphora if
punctal malposition
untreated results in loss of tone; lid eversion & sag; conj hypertrophy, keratinization
    Treatment
horizontal eyelid shortening: plicate tendons, tarsal strip, lat lid wedge resection
reinsertion of lower eyelid retractors, esp for tarsal ectropion
medial canthal laxity difficult to repair
thermal cautery for punctal ectropion has high recurrance
    Paralytic
follows VIIth nerve palsy; corneal exposure problems
    Treatment:
lubrication
                                                                                          29
sx: temp or permanent lat tarsorraphy, tarsal strip, brow suspension, gold wts for upper,
inf retractor recession w/hard palate or ear cartiledge graft for inf exposure
    Cicatricial
ant lamellar contrxn from h/o trauma, burn, surgery, infxn, dermatitis, HZO, chronic infl
upper lid: lag, retraction;
lower lid: palpable scar, ectropion increased by opening mouth, decreased by lid elevation
on upgaze
typically have horizontal laxity as well
    Treatment
treat underlying dz
lower lid: relax scar; tarsal strip (shorten horiz); skin grafts from post- or pre-auricular
area, from upper lid if sig dermatochalasis (lengthen vertically)
    Mechanical
tumors, fluid, herniated fat, glasses; individualize rx
    Tarsal strip procedure
    Anesthesia:
 lidocaine 2% with epinephrine 2-3 cc in lateral canthal area
    Make Strip:
downward lat canthotomy following curvature of upper lid then cantholysis
make tarsal strip w/westcott scissors by splitting ant/post lamellae, cutting off few mm of
superior lashes, and scraping post epithelium away; for full strip, cut off inf protractors
    Tie Down
need to overcorrect at surgery; correct position is superoposterior
4-0 Prolene on P-2 needle to fashion superior/inferior sutures on strip edge
slipknot tie to check symmetry with patient sitting up
after passing through periosteum, pass superior suture into lateral edge of superior lid
prior to final tying to pull upper eyelid inward
preplace 6-0 silk margin sutures prior to tying tarsal strip
close w/deep 6-0 vicryl and skin sutures of choice
    Post Op
consider oral antibiotics (i.e. Keflex)
remove skin sutures in 5-7 d, margin sutures in 5-10 d
lubricants
    Punctal Rotation Procedure
subconjunctival lidocaine
excise 8 mm x 5 mm elipse of conjunctiva about 2 mm inferior to ampullae of punctum
close w/interrupted vicryl 6-0 taking a bite of deeper retractors inferiorly to invert puncta
and bury knot
Entropion
focal or diffuse
can be increased by having pt squeeze lids shut
check lower lid laxity, conjunctival scarring
think of cicatricial componant especially if pt has failed prior lid repairs
lower lid entropion (usually involutional) more common than upper lid entropion (usually
cicaticial)
    Involutional
usually lower lids; from horiz laxity (poor return from snap, scl show w/o retrxn); lid
retractor disinsertion/attenuation causing deep inf fornix and little/no lid movement on
                                                                                         30
downgaze, high-riding lower lid (lower lid ptosis); overriding preseptal orbicularis;
involutional enophthalmos
    Treatment
temporizing: Quickert sutures (high recurrence used alone)
horizontal lid tightening: tarsal strip
retractor repair: Weis full thickness marginal rotation makes scar btw ant/post lamellae
(upper or lower lids)
retractor reinsertion thru skin w/tarsal strip is common: infiltrate lower lid w/2%
lidow/epi; infraciliary blepharoplasty incision; blunt dissection underneath orbicularis to
expose septum; open septum and remove fat; find capsulopalpebral fascia and attach to
lower edge of tarsus w/5-0 vicryl; remove strip of skin and orbicularis; close wound after
tarsal strip is finished
    Spastic
baseline involutional changes can be exacerbated postop or w/other ocular irritation
orbicularis overrides as pt squeezes eyes; cycle perpetuated by corneal irritation
    Treatment
break irritation/entropion cycle w/taping of lids to evert, cautery, sutures, Botox, may
need more definitive surgery
    Cicatricial
basis is shortened post lamellae w/globe irritation from lashes; keratinization; inspect
conjunctiva
simple pressure on lower edge of tarsus will correct entropion if due to involutional
changes but not cicatrical causes; seen in:
Stevens Johnson (inflammatory)                       burn, trauma
cicatricial pemphigoid (autoimmune)                  allergic conjunctivitis
trachoma, HZO (infectious)                           prior surgery
    Treatment
depends on severity, underlying dz; goal: stop corneal irritation
temporizing measures until dz stabilizes: lubrication, symblepharon rings, cryo, Quickert
sutures alone will revert in 2 to 3 months, epilation
    Surgery:
slight inversion +/- trichiasis: recess/resect ant lamellae +/- mucous memb graft
inversion confined to margin: full thickness Weis transmarg rotation w/everting sutures
mod cic entrop w/intact tarsus: do ext margin rotation;
severe w/poor tarsus: tarsoconj grafts for upper, ear cartilage or hard palate grafts for
lower lid
can augment w/tarsal strip, retractor repair (see involutional)
Symblepharon
conj adhesions 2o to infl, trauma
Rx: w/conjunctival Z-plasty for localized; full thickness graft
Trichiasis
acquired lash misdirection
check for cicatricial entrop & rx as above
for local prob can epilate, electrolyze (both w/high recurrence), cryo w/thermocouple
better; surgical wedge resxn
(Blepharo)Ptosis
most common acquired: disinsertion/rarefaction of aponeurosis
                                                                                            31
most common congenital: poorly developed levator m
hx: variability (myasthenia, synkinetic)
     Exam
complete eye exam, with attention to:
PF                                       MRD1                                   lag, stain
PFdown *                                 BLF                                    MCD
Bells                                    Jaw wink                               MFD
K sensitivity                            tear film                              pupils (Horner’s)
head/chin position                       brow action in upgaze                  motility ^
FH                                       BST                                    Neo test
* PF in down: lag in congenital/scarring, ptotic in all dir esp in down in aponeurotic
^ cong oculomotor palsy, CPEO, myasthenia, oculopharyngeal dystrophy, oculomotor
palsy w/ aberrent regen
PF, LF, MRD1 crucial; photos, MCD often; see lid measurement table
     Pseudoptosis
brow ptosis                                        blepharospasm
hypotropia                                         apraxia of lid opening
enophthalmos                                       microphthalmia
phthisis                                           superior sulcus defect
contralateral retraction                           decreased orbital volume
     Aponeurotic
     Etiology:
levator disinsertion, etc s/p surgery, involutional, aging, chronic ocular irritation,
blepharochalasis, pregnancy
high crease, lid thinning superior to tarsal plate, normal LF
     Congenital (Myogenic)
lev dysgenesis w/less excursion, more lag          isolated
double elevator palsy                              Mobius syndrome
blepharophimosis                                   jaw wink
lid higher in downgaze                             anisometropic amblyopia
myasthenia mother                                  1st branchial arch syndrome
     Treatment
if > 4mm LF, do variable amt of levator advancement, w/less LF, most often frontalis
sling
     Supramid Sling
useful temporary in cong ptosis until old enough to have fascia lata sling
    incisions
two 2-3mm inc w/ #15 blade in upper lid, lat and med 2-3 mm above lid margin; make
three additional deep incisions down to periosteum above brow in triangle formation,
creating five total incisions in an elongated pentagon formation
    suture:
pass supramid from med to lat on upper lid and flip lid to check for unwanted full
thickness pass; pass both needles to sup brow incisions, incorporating septum; pass both
needles to top incision, tie; avoid incorporating orbicularis muscle; close with 6-0 plain
gut
     Myogenic (Aquired)
myasthenia, PEO, oculopharygeal or myotonic dystrophy, steroids, thyroid eye disease
Myo-neuro-GI encephalopathy syndrome: bilateral ptosis, poor ductions/saccades, optic nerve
                                                                                                   32
atrophy, RPE changes, CVA's with VF defects, mitochondrial DNA defect in cytochrome oxidase, MRI
w/white matter dz, proximal muscle weakness, GI motility disturbance
Rx: levator advancement vs frontalis sling to functional level but careful of exposure esp
in poor Bells
     Neurogenic
     Congenital
Horner's (ptosis, miosis, anhydrosis, ipsi iris hypopig), Mobius, CN III palsy,
synkinetic/Marcus Gun jaw-wink CN V/levator connection
     Acquired
myasthenia gravis, Guillain Barre, botulism, blepharospasm, CN III palsy, acquired
Horner’s; see neuro-op
     Traumatic
s/p blunt or sharp trauma to LPS or aponeurosis
usually observe x 6 mo, repair after function stabilizes
     Mechanical
hemangioma, tumors, conjunctival scarring, edema, occasionally heavy dermatochalasis,
consider hard contact lens as cause
     Treatment
levator apo advancement, either external or internal (Muller’s muscle/conj resxn =
MMCR)
levator resection
sling if poor LF (< 4 mm)
Neosynephrine test: 10% neo in one eye, if good MRD1 is achieved (4-5 mm), pt is
candidate for MMCR. Take photo, then put drop in other eye.
Guide for LPS resection: in unilateral ptosis, MLD normal - MLD ptotic x 3 = mm of
LPS resection; in bilateral ptosis, (9 - MLD) x 3 = mm needed
     MMCR Technique
perform if 10% neo positive; standard rsxn = 8mm, but 6-9.5 depending on neo test
    anesthesia:
frontal block 2% lido w/epi 1.5cc lat to supraorbital notch about 1 inch deep on short
needle; may want to scratch mark bleph incision prior; inject for bridle suture as well
    mark:
evert lid w/Demarres, mark with 6-0 silk; loosen MMC w/forceps; clamping w/Putterman
MMCR clamp is most important: don’t incorporate tarsus and marking suture in clamp;
pull skin to assure no levator attachment
    resect:
5-0 double armed plain gut on S-14 needle with mattress suture temporal to medial; 1.5
mm beneath clamp; resect w/15 blade, run nasal suture back being careful not to cut
mattress suture; 5 throws & cut on knot, bury under conj;
    post-op:
heavy lubricants, check for K abrasion, po abx
Deramatochalasis
redundant skin often w/herniated orbital fat, indistinct or low crease +/- ptosis, vs
blepharochalsis: younger pts, F>M w/rare familial disorder, idiopathic episodes of infl lid
edema, thin wrinkled lid skin looks like dermatochalasis
pt c/o heavy feeling around eyes, brow ache, lashes in axis, decr sup field
always inquire about actinic exposure for mild cicatricial component to skin
evaluate brow ptosis
    Measurements
                                                                                            33
PF                 MRD1              lagophthalmos
PFd                BLF               MCD
MFD                tendon            BST
XS skin            LL laxity         HOF
brow               eye exam          Bell’s phenomenon
XS excess skin upper and lower graded 1-4; lower lid laxitiy graded 1-4; tendons: pull
medial and lateral and grade laxity, may need tarsal strips; brow: from lower brow to lid
margin, nl in women is 10 mm; if high MCD, address ptosis, if low, reform (see below)
     Treatment
blepharoplasty
coronal forehead lift (not for F w/high hairline, balding M, can use mid forehead lift)
brow lift
decisions incl upper or lower lids, brow lift (direct or indirect), tarsal strips, reformation
of upper lid crease (anchor lid skin via orb to levator)
     Blepharoplasty
    mark
lower incision on lid crease; nl crease = 10mm centrally, 9 mm temporally, 8mm nasally;
upper incision line marked w/dressing forceps by pinching skin until mild lash eversion;
if internal brow fixation, then hold brow in desired location while marking skin
    anesthesia
inject 3cc 2% lido w/epi
    resection
incise w/15 blade; remove skin, orbicularis en bloc; cauterize bleeding; identify and open
septum; clamp central and nasal fat pads; resect, inspect, and release; consider removing
temporal brow fat even if brow pexy is not performed b/c this alone may help elevate
brow some
    close
reform crease in women w/ 6-0 vicryl uniting pretarsal orbicularis w/supratarsal levator
fixation; close skin with 6-0 silk, nylon, or fast absorbing gut
    bleph complication:
LOV 2o retroorb hem: careful w/ASA, anticoags, HTN, check postop VA, warn re: XS
pain, proptosis; diplopia 2o to IO, IR, SR, SO damage; XS skin taken w/lag
     Internal Brow Fixation
    preparation
mark supraorbital notch, mid pupillary line; inject w/2% lido w/epi thru area; make
blepharoplasty wound
    dissection
dissect superiorly under orbicularis w/blunt and sharp techniques laterally approx 1-2 cm
above brows; resect lateral brow fat pad down to periosteum (half moon)
    fixation
pass 4-0 prolene suture as brow marker thru lower brow hairs near midline; engage
periosteum w/prolene 1 cm above orbital rim; adjust height based on sex, needs of
patient; pass needle back thru orbicularis where you originally marked lower brow; pull
marking suture into wound; tie gently, do not pucker the wound; place two to three of
these sutures
Lid Retraction
inf scleral show, upper lid stops > 2 mm above sup limbus
    Neurogenic
                                                                                         34
            rd
midbrain, III aberrent regeneration, volitional
Claude Bernard, contralateral ptosis
    Congenital
hydrocephalus of infancy (setting sun)
Marcus Gunn jaw wink
    Miscellaneous
thyroid-associated orbitopathy: most common; Rx: recess upper/lower lid retractors;
inserting spacers w/ear cartilage or hard palate graft, small lat tarsorrhaphy;
pseudotumor
overcorrected ptosis (Herring’s law); Rx: recess levator thru orig incision
s/p vert rectus EMS b/c of conxn btw IR/CPF, SR/LPS
loss of tissue (trauma, tumor resection, bleph overresxn); Rx: skin/muc memb graft, scar
release
sympathomimetic drops
prolonged doses of steroids
Blepharospasm
bilateral involuntary eyelid closure from orb m spasm, inactive in sleep
F > M , > 50 yo
majority are idiopathic, probably basal ganglia dysfunction
diagnosis is clinical, rare need for neuroimaging
CN exam; neuro hx; spasm test: repeat blinking, check for spasm; tear function tests
     Differential Diagnosis
     Facial Contracture
s/p Bell's palsy w/aberrent regeneration, spastic paretic
     Benign Myokymia
     Hemifacial Spasm
unilateral w/cheek, platysmal involvement w/ipsi facial n. weakness lasting seconds
persists in sleep
r/o facial tics and focal epilepsy
MRA to dx vascular compression of CN VII at CP angle, MRI if new onset to r/o tumor
(1%)
erhaptic transmission is lateral spread of impulses by damaged axons
Rx: neurosurgery (Jenetta procedure) vs carbemazepine, baclofen, and botulinum
     Facial Myokymia
pontine irritation, multiple sclerosis in adults and pontine glioma in children
     Neurological
Parkinson's, Huntington's, myotonic dystrophy, CVA, hypokalemic periodic paralysis,
Tourette's, tardive dyskinesia, Meige's syn (with oral facial dystonia)
     Vth n. Irritation
external ocular disease, dental, sinus, meningitis
     Treatment
     Botulinum A Toxin Injections
helps over 90% of patients; comes as freeze dried powder, must be kept frozen; molecule
is fragile, DO NOT SHAKE HARD when mixing
dilute 100 units with 4 ml of nl saline to get 2.5 units/0.1 ml; although unapproved, some
refreeze solution, use it later; initial dose of 2.5 unit in 0.1 ml per site just under skin
using 30g needle and increase by 2.5 units to titrate effects; typical sites incl med, lat
suprabrow, med, lat upper and lower lid, straight temporally, and top of maxilla on cheek;
                                                                                           35
60 units is average dose for patients in long term care;
side effects include ptosis, corneal exposure (lubricate), and diplopia
no patient receiving botox for blepharospasm, hemifacial spasm, or strabismus has
developed antibodies to it
     Surgical Myectomy
for severe refractory dz only; causes lag, etc
     Muscle Relaxants, Sedatives
little benefit
     Amantidine Trial
sometimes successful, eliminates need for Botox
Eyelid Trauma
w/u: hx, Va, complete ocular and orbital exam
     Eyelid Lacerations
 anesthesia: local or lid block; 2% lido w/epi
     Lacs Not Involving Lid Margin
skin, orbicularis only; 6-0 or 7-0 silk skin sutures, small bites, remove early
fat in wound = septum violation, look for intra-orb FB, levator eval and careful repair
     Marginal Lid Lacs
tarsal approximation most important to avoid notching
Rx: 1) three 6-0 silk double armed sutures thru lid margin from within lac 2 mm inf to
margin, thru tarsus and out thru meibomian gland plane 2mm fom each edge; similar
passes ant thru lash line and post thru gray line; pull taught to approx edges but don’t tie;
2) 5-0 or 6-0 Vicryl to approximate tarsus with external approach; 3) 6-0 silk interrupteds
to close skin; 4) tie the margin sutures, pull them anteriorly; incorporate ends of margin
suture under the knot of the most proximal skin suture
     Canalicular Lacerations:
see lacrimal system
     Canthal Angle Injuries
med tendon origin @ ant, post lacrimal crests; posterior limb critical for lid/punctum
position
in injuries w/med canthal angle rounding/displacement, telecanthus, suspect posterior
limb disruption
r/o fractures of nasal or lacrimal bones, eval lacrimal system, then reduce
Rx: transnasal wiring or wiring to post lacrimal crest after fracure reduction if present
     Burns
usually seen in major body burns
corneal protection w/lubrication, large tarsorrhaphies
Tumors
also see path sxn
    Color as Clue to Diagnosis
Pink or Red: increased vascularity; hamartomatous vessels in nevus flammeous,
capillary hemangioma; granulation tissue in pyogenic granuloma; inflammation in
chalazion, stye, cellulitis, etc.
Purple: stagnant or low blood flow; hemorrhage into cystic lesions (i.e. lymphangiomas),
low blood flow (deoxygenated blood) in cavernous hemangioma or varices; necrosis
w/hemorrhage in malignant tumors
Blue: Tyndall effect: melanin or hemosiderin in deep dermis: Nevus of Ota, blue nevus
Brown or Black: from melanin in epidermis/sup dermis: more black = more superficial:
                                                                                        36
junctional nevi more black than intradermal nevi; dry keratin i.e. seb keratosis and in
crater of keratoacanthoma
Gray or White: excess keratin; leukoplakia is descriptive only, not a disease
Yellow: lipid in dermis: xanthelasma, xanthogranuloma; elastosis (i.e. pseudoxanthoma
elasticum); syringoma
     Benign
     Epithelial
squamous papillomaskin tag, frondlike, most common benign lid tumor; excise, cryo
verruca vulgaris (wart): epidermal papovavirus infxn; cryo w/mask on to prevent spread
seborrheic keratosis: common in elderly, stuckon, oily, crusty; shave bx, no malig trans
inverted follicular keratosis: sk w/inflammation; wartlike keratotic nodule; cutaneous
horn; recurs
pseudoepitheliomatous hyperplasia (PEH): rapid growth near areas of chronic infl: burns,
fungal infxn, basal cell ca, lymphoma, looks like sq cell ca on bx but grows faster
keratoacanthoma: form of PEH; rapid gr, central ulcer crater w/keratin, spont resolve in
4-6 mo but can destroy tissues so excise; path may call squ cell ca, so give hx, large bx
epidermal inclusion cyst: solitary, slow gr, from hair follicle, excise or currette lining
     Adnexal
ductal cysts/sweat gland origin:
   sudoriferous cysts, hidrocystomas: tranlsucent cysts, +/- transillumination defect since
watery center; lid margin or lateral canthus; Moll (apocrine) and eccrine glands (worse in
warm weather)
    syringoma: most common adnexal tumor of lid, multiple, eccrine sweat structure,
young F
    eccrine acrospiroma: single, deep, painful nodule w/erythema, mimics keratoacanthoma
acquired sebaceous hyperplasia: multiple small yellow, well-circumscribed nodules, in
elderly, seen in chronic dermatitis; incr incidence of visceral ca (Muir-Torre syndrome)
hair follicle tumors:
    trichoepithelioma: papules w/telangiectasia, can look like basal cell ca;
    trichofolliculoma
    trichilemomma: resembles verrucas
     pilomatrixoma: young adults, reddish subcut mass; excision curative
     Nevi
melanocytic skin lesions
junctional (deep dermis)
compound (in junctional zone, dermis)
intradermal (most common, benign)
in kids, usually juncxl; nevus moves deeper w/age, becomes compound
blue nevus: flat, blue, deep dermal melanocytes; nevus of Ota is congenital oculodermal
melanocytosis w/mel’s in higher #s in dermis, uvea, sclera, episclera
     Misc Benign
molluscum contagiosum: viral, seen in AIDS, central umbilication; excise, curette, cryo
xanthelasma: yellow plaques of lipid-laden macs sometimes from high cholesterol; recurs
     Benign Vascular
 hemagiomas, lymphangiomas
 pyogenic granuloma: granulation tissue that has no pus and is not granulomatous
     Malignant
predisposing factors include hx of:
skin cancer                                        sun exposure
                                                                                                          37
radiation therapy                                          red hair, fair skin
English, Irish, Scottish, Scandinavian                     blue eyes
lesion hx of:
growth                                                     intermittant drainage or bleeding
crusting                                                   waxing and waning size
color changes
 exam clues include:
destruction of lid margin, lash loss                       + nodes
ulceration                                                 pearly translucent margins, central
                                                           umbilication
loss of fine wrinkles                                      thickening of skin beyond margin of lesion
    Actinic Keratosis
chronic inflam, hard, cutaneous horn, erythematous, flat, papillary, or white flaky surface
cellular atypia; 12-25% develop squamous cell CA
cryo superficial lesions
    Basal Cell Carcinoma
90% of malignant eyelid tumors; most common; see predisp factors above
rare in mucosa
lower lid > upper; medial > lateral
nodular: most common; firm, raised, pearly nodule, telangiectasia, central ulceration,
peripheral palisading
nodular ulcerative
fibrosing (morpheaform): firm, sl elevated, ulcerated, indistinct borders, thin cords on
path, more aggresive, may simulate inflam, madarosis
cystic: w/central necrosis; looks like blue lid cyst
   Treatment
incisional bx for suspicious
excisional bx for small, grossly malig tumor away from margin; mark borders; can use 1-
2 mm wide excision w/frozen sxn or Mohs’ micrographic surgery esp for morpheaform,
deep infiltrative tumors for max retention of nl tissue; cryo only for pts unable to tolerate
sugery; see reconstruction below
careful of medial canthal/vertical midface, partially treated, morpheaform & neglected
tumors b/c can have deep extension: need to know borders so don’t use cryo or XRT, pts
w/the above are majority of the 3% who die of ocular adnexal basal cell ca; may need
exenteration
   Basal Cell Nevus (Gorlin) Syndrome: AD; congenital or childhood; multiple basal cell
CA; mental retardation, calcification of falx, tentorium; pitted skin of soles/palms; skeletal anomalies:
odontogenic cysts of jaw; spina bifida occulta; cleft lip; 1/3 w/eye involvement w/early multiple lid tumors
extending to orbit or brain; hypertelorism, colobomas, cataract, glaucoma, epicanthal fold
    Xeroderma Pigmentosum: see below
    Squamous Cell Carcinoma
40X less common than basal cell but more aggressive, mets thru blood, lymph, perineural
arise from actinic keratosis, sun injury, worse w/immunodeficiency, HPV
    Treatment:
wide margin excision b/c of aggressiveness, tumor-free borders critical; recurrence may
need exenteration
    Sebaceous Adenocarcinoma
highly malignant, potentially lethal
from meib or Zeiss gl of tarsal plate, lids, caruncle, face, even eyebrow
                                                                                          38
F > M, > 50, younger pts w/h/o facial RT
upper lid 2x > lower lid
mimics chalazia, blepharitis, basal cell or squ cell ca, OCP, SLK, inclusion conjunctivitis
effaces meib gl orifices, madarosis; yellow due to lipid
spread to local LNs
poorer prognosis with >10mm diam or symptoms >6mo
if multiple sebaceous tumors (adenoma or CA) may have GI cancers
    Treatment:
full-thickness, MAP bx; path may confuse it with CIN so check closely for foamy
cytoplasm, fat stains; wide excision, possbile exenteration; Mohs’ not as good
    Miscellaneous Malignant Eyelid Lesions
mucinous adenocarcinoma (sweat gland)
tricolemmal ca (resembles keratoacanthoma, no mets, local excision)
metastasis
rhabdomyosarcoma can arise in eyelid
Kaposi sarcoma: aggressive, reddish dermal mass, AIDS, conj lesions may look like FB
granuloma or cavernous hemangioma; rx w/cryo, excision, radiation, intralesional chemo
cutaneous melanoma: < 1% of all lid tumors, better prognosis on lid than other parts of
body; lentigo maligna melanoma = 90% of all head/neck melanoma but only 10% of lid
melanoma; nodular melanoma = most common lid melanoma; wide excision; node
dissection if vasc/lymph involvement; met w/u for > 1.5 mm thick
    Lid Reconstruction
upper lid:
small (<1/3 lid): direct closure +/- cutting sup crus of lat canthal tendon for 3-5 extra mm
of give
moderate (1/3-1/2): advance lat segment by cutting lat canthal tendon, semicirc skin flap
from below lat eyebrow, may also try full-thickness composite graft from opposite lid
large (>50%): advance adjacent tissue w/full thickness lower lid flap, free tarsoconj graft
from opposite upper lid w/skin-muscle flap, or adjacent fornix-based tarsoconj flap
transposed w/full thickness skin placed anteriorly over it
lower lid:
small (<1/3): direct closure +/- cut inf crus of lat canthal tendon
moderate (1/3-1/2): semicirc advancement flap, tarsoconjunctival flap/graft w/skin graft
large (>1/2): tarsoconj graft from upper to lower lid for post lamellae w/ant skin graft
most often from behind ear (Hughes); possibly rotating cheek (Mustarde) flap

VI. Systemic
Necrobiotic Xanthogranuloma
xanthomatous firm lid nodules that ulcerate and progress
associated w/conjunctival hyperemia, uveitis, episcleral plaques
infiltrative orbital masses w/restrictive motility
80% show paraproteinemia, usually multiple myeloma IgG monoclonal gammopathy,
hyperlipidemia, neutropenia
Dx: bx w/granulomas in all layers of skin with Touton/FB giant cells
Rx: excision, local steroid inj, phasmapheresis, immunosuppressives
Acanthosis Nigricans
benign pigmented papillomas of epithelium
brown velvety hyperkeratotic plaques, symmetric and rarely generalized
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most commonly in axilla, neck, genitalia, but can be hands, mouth, and eyes
no increase in melanin
benign: AD, childhood, puberty, without CA
pseudo form: w/obesity, endocrine abnl incl insulin resistance, polycystic ovaries
malignant form: w/adenoca of GI, >20 yo, eye involvement demands systemic w/u,
usually lid plaques that can block meibomian glands, even NLDO; no local therapy
Xeroderma Pigmentosa
rare, AR, defective DNA repair mech, UV damage
cutaneous problems by 2 yo w/freckling, dry skin, telang, actinic keratoses
50% w/cancer by 15 yo w/95% involving head/neck
most commonly progressive LL atrophy starting at lid margin w/ectropion, exposure, loss
of lashes, photophobia, conjunctival injection, exposure keratitis
frequency of ocular neoplasms increased about 2000x esp limbal squamous cell CA
Porphyria
congenital (no hypertension, neurologic dysfunction, overproduction), variegate
(neurological, hepatic)
cicatricial ectropion, loss of lashes/brows, bilateral exophth
chorioretinitis, ON atrophy, conjunctivitis with shrinkage, scleromalacia, corneal pannus
no specific ocular therapy
Ichthyosis
thickened scaly skin
vulgaris (AD), epidermolytic hyperkeratosis (AD), and sex linked ichthyosis associated
w/dot shaped deep corneal opacities not affecting Va
lamellar (AR) w/cicatricial lower lid ectropion
Ehlors Danlos
AD elastic skin w/hyperextensibility, gaping wounds after mild trauma w/irregular scars
classic, varicose, arterial, scoliosis forms
epicanthal folds most commonly, angiod streaks, strabismus, blue sclera, keratoconus,
microcornea, ectopia lentis, familial RD
Dego's syndrome
malignant atrophic papulosis is rare vasculitis in young M>F
multiple red papules 2-10 mm progressing to porcelain white atrophic depressed center
w/surround telang on trunk/limbs
eyelid lesions, conjunctival telang/MA, conjunctival plaques, focal retinal vasculitis
fatal from peritonitis
no RX, bowel resection is temporizing
Amyloidosis
orbital/adnexal involvement, usually painless, more often 1o amyloidosis w/systemic
signs
most typical is ptosis followed by ophthamoplegia by restrictive infiltration
proptosis from orbital mass or fatty infiltration
lacrimal involvement w/either mass or sicca
optic nerve sheath or other cranial nerve involvement
lid involvement (hemorrhagic waxy papules) has high likelihood of systemic involvement
can be nodular or diffuse, unilateral or bilateral