Craniofacial, inflammatory, and vasculopathic disorders of the orbit

cranial suture

1. fibrous joints btw the bones of the vault and face

2. important for cranial vault growth and brain growth (allows for expansion)

3. once they fully ossify, further expansion is impossible

what are the cranial sutures from anterior to posterior

1. mesopic suture

2. sagittal suture

3. lambdoid suture

craniosynostosis

1. the premature fusion of the cranial vault suture

2. results in abnormal skull and brain development

3. intracranial pressure can be elevated due to brain growht in a confined space

virchows law

1. the premature fusion of teh cranial vault suture inhibits normal skull growth PERPENDICULAR to the fused suture

2. a COMPENSATORY growth occurs at the open sutures

3. the general direction of growth after craniosynctosis is PARALLEL TO THE FUSED SUTURE

what are the ocular manifestations of craniosynostosis

1. proptosis

2. papilledema

   1. bilateral ONH swelling due to inc intracranial presure

3. optic nerve head atrophy

4. EOM paresis

what are the types of craniosynostosis

1. scaphelocephaly

2. trigonocephaly

3. brachycephaly

4. oxycephaly

5. positional plagiocephaly —- NOT a true craniosynotosis

scaphelocephaly cause

1. premature fusion of the saggital suture

scaphelocephaly appearance

1. elongation of the cranium anterior to posterior

2. resembles an inverted boat/ football

scaphelocephy tx

sx

trigonocephaly cause

premature fusion of the metopic suture

trigonocephaly appearance

1. high retreating forehead, V shaped

2. triangle

trigonocephaly tx

sx

brachycephaly caus e

1. premature fusion of BOTH coronal sutures

2. can have fusion of one coronal suture

brachycephaly appearance

shortened front to back diameter of the skull

head disproportionately wide

pumpkin?

oxycephaly cause

premature fusion of teh coronal and saggital AND/OR lamdoid sutures

oxycephaly appearance

tower skull or high head syndrome

positional plagiocephaly cause

1. all sutures are open

2. most common cause of misshapen head during infancy

3. back of head is often flatter on one side due to position in womb during pregnancy

   1. more common in twins, triplets, etc

treatment of positional plagiocephaly

1. ionce the infant has better control of their head

2. deformity decreases

3. SO NO TX (maybe a baby helmet)

orbital roof

1. lesser wing of the sphenoid bone

2. frontal

THE ROOF IS FRONT-LESS

orbital floor

1. maxillary

   1. could be involved in blow out fracture

2. palatine

3. zygomatic

MY PAL LEFT HIS Zs on the floor

medial wall of the orbit

1. maxillary

2. lacrimal

3. ethmoid

   1. the lamina papyracea of the ethmoid is the thinnest bone and can erode in ethmoid sinusitis infections

4. sphenoid

SLEM like Slime bc youre near your nose

lateral wall

1. greater wing of the sphenoid

2. zygomatic

Greater Z

syndromic craniosynostosis cause

1. inherited or genetic condition characterized by a collection distinct facial and body abnormalities

2. can be mild to severe

3. limb and hand abnormalities can be telling

4. often have related health and developmental issues

syndromic craniosynostosis diseases (5)

1. apert syndrome

2. crouzon syndrome

3. muenke syndrome

4. pfeiffer syndrome

5. saethre - chotzen syndrome

apert syndrome is also called

1. acrocephalosyndactyly

apert syndrome inheritance

autosomal dominant

apert syndrome appearance

1. flat elongated forehead

2. proptosis

3. underdeveloped midface

4. fusion of the digits

brachycephaly and/or oxycephaly

crouzon syndrome inheritance

autosomal dom

appearance of crouzon syndrome

1. wide set proptotic eyes

2. beaked nose

3. underdeveloped jaw

4. dnetal issues - possible cleft lip and palate

5. no associated hand and feet anomalies

brachycephaly

muenke syndrome INHERITANCE

autosomal dom

muenke syndrome appearance

1. abnormally shpaed head

2. wide set eyes

3. flattened cheek bones

4. mild hand/feet abnormalities

5. MOST HAVE NORMAL INTELLECT

brachycephaly

pfeiffer syndrome inheritance

autosomal dom

pfeiffer syndrome appearance

1. abnormally shaped head

2. wide set eyes

3. beaked nose

4. underdeveloped upper jaw

5. thumbs and big toes point away from other digits

   1. may hae webbing or fusion

6. hearing issues D

saethre-chotzen syndrome inheritance

autosomal dom

saethre chotzen syndrome appearance

1. abnormally shaped head

2. low frontal ahirline

3. ptosis

4. wide spaced eyes

5. broad nasal bridge

6. facial asymmetry

7. small ears

8. fusion of 2nd and 3rd fingers

9. normal intellect

what do we do for craniosynostosis?

1. recognize it

2. refer to pediatric craniofacial subspecialist

3. most have proptosis

   1. artificial tears

      1. preservative free if doing more than 4x a day

   2. gel or ointment (ung) at bedtime (QHS)

   3. possible lid taping QHS

what are the eyelid tissues from superficial to deep

1. eyelid skin

2. subcutaneous areolar layer

3. orbicularis oculi

   1. orbital and palpebral portion

   2. CN 7

4. orbital septum

5. posterior muscular system

   1. levator and mueller muscle

      1. levator - CN 3

      2. mueller - sympathetics

6. tarsal plate

whatas the arterial supply and venous drainage of the eyelids

1. lateral palpebral artery (branch of lacrimal artery) + 2. medial palpebral a (banch of ophthalmic a) ==anastomose==> 3. palpebral arcades

whats above the tendinous annulus

1. lacrimal nerve

2. frontal nerve

3. superior ophthalmic vein

4. trochlear nerve

LFSTUNALI - Large Fluffy Squirrels Take Unexpected Naps Among Lush Ivy!

whats thru the tendinous annulus annulus

1. Upper division of CN 3

   1. SR

   2. levator

2. nasociliary nerve

3. abducens nerve

4. lower division of CN 3

Large Fluffy Squirrels Take Unexpected Naps Among Lush Ivy!

whats below the tendinous annulus

1. inferior opthalmic vein

Large Fluffy Squirrels Take Unexpected Naps Among Lush Ivy!

orbital cellulitis presentation

1. eyelid edema

2. erthema - swelling

3. warmth

4. tenderness

5. conjunctival infection, chemosis (swelling of conj)

6. proptosis

7. febrile and malaise

8. restricted EOM/pain w eye movement

orbital cellulitis symptoms

1. red eye

2. pain

3. blurred vision

4. double vision

5. eyelid swelling

6. nasal congestion/discharge

7. sinus headache, congestion, pressure

8. tooth pain

9. infra/suprorbital pain

orbital cellulitis cause

1. • Direct extension from a paranasal sinus infection (especially ethmoiditis)

2. • Focal periorbital infection

   1. • Dacryoadenitis

   2. • Dacryocystitis

3. • Dental infections

4. • Sequela of orbital trauma

5. • Sequela of orbital surgery or paranasal sinus surgery

6. • Vascular extension (seeding from a systemic bacteremia)

7. • Secondary to orbital venous stasis and inflammation from a septic cavernous sinus thrombosis

8. • Infection by Staphylococcus (adults), Streptococcus (adults), H. influenzae (children)

9. • Fungal infection in immunocompromised patients (diabetics, HIV, chemotherapy

   1. • Mucormycosis/zygomycosis

   2. • Aspergillus

orbital cellulitis clinical exam

1. Ask about trauma, surgeries, ear/nose/throat infections, tooth pain/abscess, stiff neck or mental changes

2. • Palpation of the affected lid assessing for warmth and tenderness

3. • May need to open eye to assess for:

   1. • Visual acuity

      1. • Reduced in affected eye

   2. • (+) APD

      1. • Indicates compressive optic neuropathy

   3. • Extraocular motilities/restrictions

   4. • Proptosis

      1. • Resistance to retropulsion

   5. • Conjunctival congestion/chemosis

   6. • Dilated fundus exam

      1. • Swollen optic nerve head

   7. • Check for febrile status

what does the ER do for orbital cellulitis

1. preform a Ct scan w contrast of the orbits and paranasal sinuses to confirm diagnosis

2. give IV antibiotics stat

3. CONCERN FOR INFECTION OF THE CNS AND MENINGITIS

   1. REFER IMMEDIATELY

what are the 4 types of idiopathic orbital inflammation

1. dacryoadenitis

2. myositis

3. dacryoadenitis + myositis

4. orbital apex syndrome

how does idiopathic orbital inflammation (IOI) present

• Usually unilateral, although bilateral more common in children

• Marked tenderness of the involved region

• Lid edema, erythema

• Lacrimal gland enlargement (dacryoadenitis)

• Limitation of and pain with EOM (myositis)

• Proptosis

• Decreased orbital retropulsion

• Conjunctival chemosis

• Reduced corneal sensation

• Increased IOP

what are the symptoms of IOI

• Acute onset of orbital pain

• Decreased vision

• Binocular diplopia •

Red eye

• Headaches

• Fever, nausea, vomiting

whats teh cause of IOI

• Idiopathic acute or chronic inflammatory disorder of the orbital tissue.

• This condition is a diagnosis of exclusion.

what are the differentials for IOI

• Thyroid related ophthalmopathy

• Orbital cellulitis

• Orbital tumor •

Lacrimal gland tumor

• Orbital vasculitis

• Trauma

• Cavernous sinus thrombosis

• Cranial nerve palsy

• Herpes Zoster Ophthalmicus

what do we do in a clinical exam for IOI

1. • Ask about trauma, surgeries, ear/nose/throat infections, tooth pain/abscess, stiff neck or mental changes •

2. Palpation of the affected lid assessing for warmth and tenderness •

3. May need to open eye to assess for:

   1. • Visual acuity

      1. • Reduced in affected eye

   2. • (+) APD •

      1. Indicates compressive optic neuropathy

   3. • Extraocular motilities/restrictions •

   4. Proptosis

      1. • Resistance to retropulsion

   5. • Conjunctival congestion/chemosis

   6. • Dilated fundus exam

      1. • Swollen optic nerve head

4. • Check for febrile status

what does the muscle look like in IOI vs thyroid ophthalmopathy

1. On CT scan, the tendons & extraocular muscles are enlarged in idiopathic orbital inflammation. In thyroid ophthalmopathy, only the muscle belly is enlarged (coke bottle)

which is Dacryoadenitis + Myositis and which is Thyroid ophthalmopathy

how do we treat IOI

1. • Admit/refer to ED/hospital for orbital CT scan STAT

   1. • ED to initiate systemic steroids once all other conditions have been ruled out

   2. • ED will run hematology to rule out presence of vasculitis in adults

      1. • (i.e. Wegner’s granulomatosis, Polyarteritis nodosa)

orbital apex syndrome presentation

1. • Unilateral vision loss with ophthalmoplegia involving multiple cranial nerves

   1. • Vision loss is due to CN II being affected. Optic atrophy can occur weeks to months after initial presentation if no remedied •

   2. Ophthalmoplegia due to involvement of • CN III, IV, VI •

2. Periorbital facial pain and forehead hypoesthesia due to CNV1 involvement

   1. • Decreased corneal sensitivity

3. • Mydriasis and ptosis •

   1. Due to CN III involvement

• Proptosis

• Injection/chemosis

orbital apex syndrome symptoms

1. • Poor vision in affected eye

2. • Binocular diplopia

3. • Orbital pain

cause of orbital apex syndrome

1. • Inflammatory

2. • Infectious

3. • Neoplastic

4. • Iatrogenic/Traumatic

5. • Vasculopathic

whats the diff diagnosis for orbital apex syndrome

1. cav sinus syndromes - NO ON involvement

what do we do in a clincial exam for orbital apex syndrom

1. • Ask about trauma, surgeries, ear/nose/throat infections, tooth pain/abscess, stiff neck or mental changes

2. • Palpation of the affected lid assessing for warmth and tenderness

3. • May need to open eye to assess for:

   1. • Visual acuity

      1. • Reduced in affected eye

   2. • (+) APD

      1. • Indicates CN II involvement

   3. • Extraocular ophthalmoplegia

   4. • Proptosis

      1. • Resistance to retropulsion

   5. • Conjunctival congestion/chemosis

   6. • Dilated fundus exam

      1. • Swollen optic nerve head

   7. • Check for febrile status

how do we treat orbital apex syndrome

1. • Admit/refer to ED/hospital for orbital CT and MRI scan STAT

   1. • ED to initiate systemic steroids once all other conditions have been ruled out

whats the blood supply to the retina

1. ophthalmic artery —> 2. CRA —> 3. Retina

whats the blood flow out of the eye

1. choroid

   1. vortex vein

2. retina

   1. central ertinal vein

3. Sup/

4. anteiror ciliary vein —> Inf ophthalmic vein

5. cav Sinus

whats in the cav sinus

1. 3

2. 4

3. 6

4. V1

5. V2

6. sympathetics

7. ICA

orbital varix presentation

1. intermittent proptosis of one or both eyes which is non pulsatile and not associated w a brut (rhythmic pattern)

2. proptosis is precipitated by valsava manuever or compression of jugular veins

   1. inc venous pressure

symptoms of orbital varix

1. intermittent bulging of eyes on command or w valsalva maneuver

differential diagnosis for orbital varix

1. carotid cavernous fistula

diagnosis, treatment, management of orbital varix

1. xray of orbit to rule out mass

2. pt education

carotid cavernous fistula presentation

1. • Typically, unilateral presentation

2. • Red eye

   1. • Chemosis and injection from backup of venous blood

3. • Pulsatile proptosis

4. • Orbital bruit

5. • Decreased visual acuity

carotid cavernous fistula symptoms

1. • Pulsations of the globe

2. • Irritated red eye

3. • Eyelid edema

carotid cavernous fistula diff diagnosis

orbital varix

what does carotid cavernous fistula appearance

1. pulsatile proptosis

   1. proptosis is beating at the rhythm of the heart

what are the 2 types of carotid cavernous fistulas

1. high flow or direct fistula

2. low flow or dural fistula

high flow/ direct fistula

• Characterized by direct connection of internal carotid artery to the cavernous sinus

• 80% of lesions

• Often secondary to closed head trauma

low flow / dural fistula

1. • Characterized by a connection of the sinus with any of the meningeal branches of the internal carotid

2. • Often associated with systemic disease (i.e. HTN, connective tissue disorders, atherosclerosis)

diagnosis, treatment, and management of carotid cavernous fistula

1. • Orbital CT or MRI: enlargement of the superior ophthalmic vein

2. • Arteriography usually required to identify fistula

3. • High-flow fistula are closed by surgical repair of the lesion, if possible or embolization with balloon occlusion

4. • Low-flow fistulas are closed by endovascular balloon occlusion, if possible. May have conservative monitoring with low-flow fistula as some spontaneously resolve.

palliative care (optometrists) for carotid cavernous fistula

1. we don’t treat the underlying issue

2. we refer

3. pt will need to be referred for emergent imaging and initiation of systemic antibiotics/steroids/immunomodulators

proptosis

1. gel based artificial tears (preservative free) q1hr - PRN

2. ointments w lid taping QHS

elevated IOP

1. topical anti hypertensive agent

2. Alphagan 1 gtt TID in affected eye