Anterior Segment 1 - Lecture 1

What kind of joints are cranial sutures?

Fibrous Joints

What is the purpose of the cranial sutures?

Allows for expansion of the brain

What are the four cranial sutures?

Metopic, sagittal, coronal, lambdoid

What is craniosynostosis?

Premature fusion of the cranial sutures

What is the result of craniosynostosis?

Abnormal skull and brain development

What is Virchow's Law?

In cases of craniosynostosis, restricted growth of the skull occurs perpendicular to the prematurely fused suture.

According to Virchow's law, what happens to skull growth parallel to a fused suture in craniosynostosis?

Compensatory overgrowth occurs parallel to the fused suture.

What finding may be elevated due to brain growth within a confined space?

Intracranial pressure

What are the ocular manifestations of increased intracranial pressure? (3)

1. Proptosis

2. Papilledema

3. Paresis of EOMs

What are the five non-syndromic craniosynostosis?

1. Brachycephaly

2. Scaphocephaly

3. Trigonocephaly

4. Oxycephaly

5. Positional Plagiocephaly

B...STOP

What is brachycephaly?

Premature fusion of coronal sutures

Head shape: Brachycephaly

Skull will grow parallel to the prematurely fused suture, so head will be wide and shortened front to back according to Virchow's law

Treatment: Brachycephaly

Surgery

What is scaphocephaly?

Premature fusion of the sagittal suture

Head shape: Scaphocephaly

Head will grow parallel to prematurely fused suture, so elongated anterior to posterior

An inverted boat or football head shape is seen with which non-syndromic craniosynostosis?

Scaphocephaly

Treatment: Scaphocephaly

Surgery

What is trigonocephaly?

Premature fusion of the metopic suture

Head shape: Trigonocephaly

Head will grow parallel to the fused suture so V-shaped head with high retreating forehead

Treatment: Trigonocephaly

Surgery

What condition is characterized by premature fusion of the coronal and sagittal and/or lambdoid sutures?

Oxycephaly

What condition presents with a tower skull or "high-head" syndrome?

Oxycephaly

Treatment: Oxycephaly

Surgery

What is the most common cause of misshapen head during infancy?

Positional plagiocephaly

Which sutures prematurely fuse in positional plagiocephaly?

None; all sutures remain open

What results in the flatter head in positional plagiocephaly?

Due to position in the womb during pregnancy

In which condition is positional plagiocephaly more common?

In multiple pregnancy (twins or triplets)

What is the treatment for positional plagiocephaly?

No treatment needed

What two bones make up the orbital roof?

1. Frontal

2. Lesser wing of the sphenoid

(Roof is Front-Less)

What is found in the superotemporal aspect of the orbital roof?

Lacrimal gland

What 3 bones make up the orbital floor?

1. Maxillary

2. Palatine

3. Zygomatic

(My pal left his Z's on the floor)

Which part of the orbital floor is susceptible to orbital fracture/blow out fracture?

Posteromedial portion of the maxillary bone

What makes up the roof of the maxillary sinus?

Orbital floor

What four bones make up the medial wall of the orbit?

1. Maxillary

2. Lacrimal

3. Ethmoid

4. Sphenoid

SMEL (medial near the nose)

What is the thinnest bone within the orbit and can erode in ethmoid sinusitis infections?

Lamina papyracea of the ethmoid

What two bones make up the lateral wall of the orbit?

1. Greater wing of the sphenoid

2. Zygomatic

The lateral wall of the orbit protects the (anterior/posterior) half of the globe.

Posterior (anterior half is susceptible to trauma)

What are syndromic craniosynostosis?

Inherited/genetic conditions characterized by a collection of distinct facial and body abnormalities

What identifying feature can differentiate between various syndromic craniosynostosis?

Presence of limb and hand abnormalities

What are the five syndromic craniosynostosis?

1. Crouzon syndrome

2. Apert syndrome

3. Muenke syndrome

4. Pfeiffer syndrome

5. Saethre-Chotzen syndrome

CAMPS

Inheritance pattern for syndromic craniosynostosis?

Autosomal dominant

Acrocephalosyndactyly

Apert syndrome

Which craniosynostosis is present in Apert syndrome?

Brachycephaly and/or oxycephaly

Proptosis can be seen in which syndromic craniosynostosis?

Apert syndrome

T/F Fusion of the digits is present with Apert syndrome

True

Which craniosynostosis is present with Crouzon syndrome?

Brachycephaly

T/F Crouzon syndrome does not present with any hand/feet abnormalities

True

Which syndromic craniosynostosis presents with wide set proptotic eyes, underdeveloped jaw and potential dental issues?

Crouzon syndrome

Which craniosynostosis is present with Muenke syndrome?

Brachycephaly

Which syndromic craniosynostosis is associated with flattened cheek bones and mild hand and feet abnormalities?

Muenke syndrome

T/F Muenke syndrome has no associated intellectual disabilities

True

Which craniosynostosis is associated with wide set eyes, underdeveloped upper jaw, hearing issues, and thumb/big toe pointing away from other digits?

Pfeiffer syndrome

Which craniosynostosis is present with Saethre-Chotzen syndrome?

Brachycephaly

Which craniosynostosis may present with ptosis?

Saethre-Chotzen syndrome

Which craniosynostosis presents with wide spaced eyes, ptosis, small ears, facial asymmetry, fusion of the 2nd and 3rd fingers, and broad nasal bridge?

Saethre-Chotzen syndrome

T/F Saethre-Chotzen does not affect intellect

True

What are the ocular risks of proptosis?

Exposure keratopathy

Who to refer patients with craniosynostosis?

Pediatric craniofacial specialist

Treatment for exposure keratopathy secondary to proptosis

Artificial tears (QID), gel/ointment qHS, possible lid taping qHS

What innervates the eyelid skin?

Upper: CN V1

Lower: CN V2

What innervates the orbicularis oculi?

CN VII

What CN provides taste to the anterior 2/3 of the tongue?

CN VII

Which CN aids in lacrimation?

CN VII

Levator is innervated by which CN?

CN III

Muscle of Mueller is innervated by what?

Sympathetics

What artery gives rise to the lateral palpebral artery?

Lacrimal

What artery gives rise to the medial palpebral artery?

Ophthalmic

Where do all recti muscles originate?

Common tendinous ring (tendinous annulus)

Which condition presents with eyelid edema, erythema, warmth, tenderness, conjunctival injection and chemosis, febrile state with fatigue and proptosis?

Orbital cellulitis

T/F Orbital cellulitis is a life threatening condition

True

Motility with orbital cellulitis

Restricted extraocular motiltiy and pain with eye movements

What is the cause of orbital cellulitis?

1. Bacterial infection in vicinity that spread to the orbit

2. Orbital trauma or surgery

3. Systemic bacteremia

4. Orbital venous stasis or cavernous sinus thrombosis

Which bacteria are responsible for orbital cellulitis in adults?

Staphylococcus or streptococcus

Which bacteria is responsible for orbital cellulitis in children typically?

H. influenzae

A fungal infection from which species could result in orbital cellulitis?

Mucormycosis/Zygomycosis or aspergillus

What does the presence of an APD in orbital cellulitis suggest?

Compressive optic neuropathy

What is the workup for orbital cellulitis in the ED?

CT scan of the orbit/paranasal sinuses with contrast to confirm diagnosis

What is the treatment for orbital cellulitis?

Hospitalization with IV antibiotics

What are the four types of idiopathic orbital inflammation conditions?

Dacryoadenitis, Myositis, Dacryoadenitis + Myositis, Orbital apex syndrome

What is the cause of IOI?

Idiopathic acute or chronic inflammatory disorder of orbital tissue

Myositits

Limitation of EOM and pain eith EOM movement

Which condition typically presents unilaterally in adults with dacryoadenitis, myositis, proptosis, decreased orbital retropulsion, conjunctival chemosis, increased IOP

IOI

T/F Idiopathic orbital inflammation is a diagnosis of exclusion

True

Dacryoadenitis

Inflammation of the lacrimal gland

How to differentiate between IOI and thyroid ophthalmopathy on CT scan?

In IOI both tendon and muscle is enlarged, in Thyroid ophthalmopathy the muscle belly is enlarged

Treatment of IOI

Hospitalization for CT scan, systemic steroids once all other conditions have been ruled out

Hemotology is run in cases of IOI to rule out which condition?

Vasculitis (Wegner's granulomatosis, Polyarteritis nodosa)

Which condition involves unilateral vision loss with ophthalmoplegia involving multiple cranial nerves?

Orbital apex syndrome

Periorbital facial pain and forehead hypoesthesia is caused by what in orbital apex syndrome?

Involvement of CN V1

Vision loss in orbital apex syndrome is caused by what?

CN II affected

Ophthalmoplegia in orbital apex syndrome is caused by what?

Involvement of CN III, IV, VI

Mydriasis and Ptosis are seen with which condition?

Orbital apex syndrome due to CN III involvement

How to differentiate between orbital apex syndrome and cavernous sinus syndromes?

Cavernous sinus syndromes do not involve CN II, thus no vision loss should be expected

Treatment of orbital apex syndrome

Hospitalization for CT and MRI and initiate systemic steroids once other conditions have been ruled out

Which nerves travel within the cavenous sinus?

CN III, IV, VI, V1, V2, and sympathetics

Orbital varix is what type of disorder of the orbit?

Vasculopathic

Carotid cavernous fistula is what type of disorder of the orbit?

Vasculopathic

What condition is characterized by intermittent proptosis that is non-pulsatile and not associated with a bruit?

Orbital varix

What causes proptosis in orbital varix?

Valsalva manuever (on command) or compression of jugular veins

How to diagnose orbital varix?

Xray of orbit to rule out mass