Head and Neck 3 Flashcards
What is Orbital Apex syndrome?

- Affects CNs’
- II
- III
- IV
- V
- VI
- https://casereports.bmj.com/content/2017/bcr-2016-217382
- Orbital apex syndrome is a rare neuro-ophthalmic manifestation of herpes zoster virus infection. We report one such case with favourable outcome in an immunocompetent patient. A 60-year-old woman presented with rash in the dermatome of the left ophthalmic nerve (V1), followed by sudden loss of vision with complete left-sided external and internal ophthalmoplegia. MRI of brain and orbits with contrast revealed optic perineuritis and myositis without intracranial involvement confirming the diagnosis of orbital apex syndrome. Functional visual recovery was achieved after a course of intravenous and oral steroids under antiviral cover over a follow-up period of 3 months.
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What is the pathology behind this condition?

- Thyroid Ophthalmopathy
- Orbital pathology
- Cellular infiltration and deposition of Glycoproteins and mucopolysaccharides in the orbit
- Caused by autoantibodies targeting thyrotropin receptors found in the orbit (in addition to the thyroid)
What are the clinical signs of Thyroid ophthalmopathy?
- painless proptosis
- patients may be euthryoid, hypothyroid or hyperthyroid
Functional classification of Thyroid Ophthalmopathy
ie Clinical Severity and risk
Functional classification (clinical severity and risk):
- Mild:
- eyelid lag and retraction with
- proptosis
- in setting of active hyperthyroidism
- Moderate:
- soft tissue inflammation
- intermittent myopathy;
- stabilizes without major sequelae
- Severe:
- rapid and fulminant,
- greater mass effect,
- severe sequelae
- including optic nerve compromise
Treatment of Thyroid ophthalmopathy
- prednisolone
- radiation therapy
- surgical decompression
- surgery or 131 iodine for thyroid
Radiographic features of Thyroid Ophthalmopathy

- exophthalmos/proptosis
- >2/3 globe anterior to septal line
- CT
Assessment of proptosis on cross-sectional imaging is difficult and dependant on the study being acquired in the correct plane:
the plane of the study must be parallel to the head of the optic nerve and the lens
the patient must have their eyes open and be looking forward with no eye movement
The reference line for measurement of proptosis is the interzygomatic line (a line is drawn at the anterior portions of the zygomatic bones):
the distance from this line to the posterior sclera is normally 9.9 +/- 1.7 mm 2
the distance from this line to the anterior surface of the globe should be <23 mm 4
The thickness of the extraocular muscles can also be used 1.
- muscle involvement
- mnemonic for muscle involvement
- I’m SLow
- Inferior (most common)
- Medial
- Superior
- Lateral
- I’m SLow
- Enlargement is maximal in the middle of the muscle and tapers toward the end.
- infiltrative not inflammatory disease
- spares tendon insertions, although may be involved in the acute phase
- Often bilateral, symmetric
- Straightened or streatched optic nerve
- expansion or orbital fat
- Lacrimal gland enlargement

what is this?

- Idiopathic Orbital inflammation
- AKA orbital pseudotumour
- Inflammation of orbital soft tissues of unknown origin
- Clinically
- painful proptosis
- typically unilateral (25% bilateral)
- Steroid responsive
what are the variants of idiopathic orbital inflammation?

- Tolosa Hunt:
- extends into the cavernous sinus
- Pic 1
- Sclerosing orbital inflammatory pseudotumor
- chronic progressive fibrosis
- Pic 2
- In this case, computed tomographic (CT) scanning demonstrates a mass in the nasal orbit. The arrow demonstrates the optic nerve on stretch. The eye wall (sclera) is indented and the eye pushed out (proptosis).

Rad features of idiopathic orbital inflammation

- infiltrating intraconal or extraconal inflammation presenting as ill defined infiltration or less commonly as a mass
- Typically
- unilateral
- Myositic form involves extraoular muscles
- most common
- followed by lacrimal gland involvement
- followed by other areas of involvement
- any muscle can be affected
- superior complex and medial rectus are most frequent
- Muscle enlargement
- Tendons of themuscles are involved (unlike thyroid ophthalmopathy) because it is an inflammatory disease, not an infiltrative disease like thyroid ophthalmopathy.
- Stranding of orbital fat secondary to inflammation
- DWI may be helpful for distinguishing pseudotumour from lymphoma/malignant disease
- pseudotumour generally has higher values on ADC (ie less restricted)
Detection of Occular FB
Metal
Glass
Wood

- CT > xray
- Metal
- CT 100% sensitive when object is >0.06mm
- Glass
- depends on type of glass
- Overall high sensitivity of CT for denser glass types > 1.5mm
- Wood
- difficult to detect bc wood density is similar to soft tissue
- On CT wood appears as air density with geographic margin
- Dry wood lower attenuation than fresh wood
What is the molecular pathophysiology of NF 2?
https://disorders.eyes.arizona.edu/disorders/neurofibromatosis-type-ii

what is Erdeim Chester disease

- Lipid granulomatosis
- aka non langerhans histiocytosis
- retro-orbital deposition or mass,
- xanthelasma of eyelids
- skeletal manifesations
- medullary sclerosis
- cortical thickening
- cardiopulmonary manifesations
- result of cholesterol emboli
- rare
- diffuse infiltrative mass most commonly involving intraconal space or other area in orbit

What are 6 Occular Manifestations of NF 1?

- Lisch nodules
- Optic pathway glioma
- Optic nerve sheath ectasia
- Sphenoid bone dysplasia
- Choroidal hamartoma
- Plexiform neurofibroma
Patterson, Brittany & Barton, Christopher & Lakhotia, Arpita. (2019). Unequaled overgrowth inside and out—an exceptional example of hypertrichosis overlying plexiform neurofibroma. JAAD Case Reports. 5. 670-671. 10.1016/j.jdcr.2019.04.018.

Ocular manifestations of NF 2
- Juvenile Cataracts 80%
- Epiretinal Membranes12-40%
- retinal hamartomas
- Optic nerve damage from schwannoma
- CT scans reveal calcification along the optic nerve in a “tram-track” configuration which occurs in 20-30% of patients with NF type II.
Clinical Characteristics
Ocular Features:
It is not uncommon for children to present with visual complaints secondary to cataracts, retinal hamartomas, or optic nerve damage from a schwannoma. The most common eye findings are juvenile cataracts (up to 80% of patients), followed by epiretinal membranes in 12-40%, and retinal tumors in 6-22%. Lens opacities may be located in the posterior subcapsular region or as cortical wedge-shaped opacities in the periphery where they are easily missed if the pupil is not fully dilated. Translucent, grayish epiretinal membranes with white edges are present in a significant number of patients, including children. Elevated retinal hemartomas in the macula are often associated with pigmentary changes. Lisch nodules characteristic of type I neurofibromatosis (162200) are generally not found in type II. Corneal damage may occur as a complication of hypesthesia resulting from damage to the fifth cranial nerve.
CT scans reveal calcification along the optic nerve in a “tram-track” configuration which occurs in 20-30% of patients with NF type II.
Systemic Features:
Type II neurofibromatosis often presents in the third or fourth decade of life as hearing loss accompanied by tinnitus and dizziness.
A significant proportion of children (30%) present with the same symptoms although they are more likely to complain of visual disturbances.
Type II accounts for about 10% of neurofibromatosis cases.
Acoustic neurinomas, usually bilateral, are far more common in type II (95%) and are considered diagnostically distinctive by some.
Such schwannomas also occur in other cranial and peripheral nerves.
Neurofibromas are uncommon but meningiomas, ependymomas, and astrocytomas are seen frequently.
Schwannomas can form anywhere along peripheral nerves and at least a third of patients require surgical excision of one or more of these lesions.
These account for the majority of skin plaques and lumps and are found in more than half of patients.
Café-au-lait spots are uncommon or even absent in many patients with type II.
Patients with type II neurofibromatosis do not have the cognitive problems sometimes seen in those with type I.
Longevity overall is reduced.
The average patient lives about 15 years after diagnosis and the average age of death is 36 years.
Genetics
Type II neurofibromatosis is an autosomal dominant disorder caused by mutations in the NF2 gene (22q12.2) which encodes neurofibromin-2, sometimes called merlin or schwannomin. This protein product, like neurofibromin in type I (162200), functions as a tumor suppressor. New mutations are responsible for approximately half of cases.
Cognitive deficits and Lisch nodules on the iris are more commonly found in neurofibromatosis type I (162200) but acoustic neuromas are less common. Type I results from mutations in NF1.
Treatment
Early surgical treatment of small acoustic and facial schwannomas can preserve nerve function in many cases. This is a progressive disease requiring lifelong monitoring for disease progression. The peripheral lens opacities usually do not progress and therefore cause little visual morbidity.
https://disorders.eyes.arizona.edu/disorders/neurofibromatosis-type-ii

Ocular Manifestations of Sturg-Weber
aka encephalotrigeminal angiomatosis
- choroidal haemangioma
- Buphthalmos
- Glaucoma
- Abnormal occular enhancement on MRI, thickest of the the posterior portion of the globe
Sturge-Weber syndrome, or encephalotrigeminal angiomatosis, is a phakomatosis characterised by facial port wine stains and pial angiomas.
Approximately a third of patients have choroidal or scleral angiomatous involvement, which may be complicated with retinal detachment, buphthalmos or glaucoma 1.
Sturge-Weber syndrome is a rare syndrome, with an incidence estimated at 1 case in 20,000-50,000 persons 11.


Ocular Manifesations of Sturg-Weber
https://www.researchgate.net/figure/Bilateral-Sturge-Weber-syndrome-Postcontrast-axial-MR-image-A-shows-gross_fig1_14472655
Bilateral Sturge-Weber syndrome. Postcontrast axial MR image ( A ) shows gross leptomeningeal thickening and enhancement related to the right hemisphere and less extensive abnormality of the left occipital region. Unenhanced axial T1-weighted image ( B ) shows thickening of the posterior globes bilaterally; after administration of contrast material ( C ), marked enhancement is seen in both globes. Abnormal high signal is present in this region on the proton density–weighted image ( D ). Bilateral, diffuse choroidal hemangiomas were found at ophthalmoscopy (not shown).
Ocular manifestations of Tuberous Sclerosis
- Retinal hamartomas
- Punched out areas of retinal depigmentation
- Angiofibromas of the eyelids
- Colobomas

What is the Ocular manifestation of Von Hipple Lindau?
Retinal Hemangioblastomas

Lymph node level 1a

superiorly:
inferiorly:
anteriorly:
posteriorly:
There are two sublevels:
Submental LNs
superiorly: mylohyoid muscle and mandible
inferiorly: inferior border of the hyoid bone
anteriorly: platysma muscle
posteriorly: posterior border of the submandibular gland
There are two sublevels:
level Ia (submental nodes): anteromedial between the anterior bellies of both digastric muscles
level Ib (submandibular nodes): posterolateral to the anterior belly of the digastric muscles

Level II lymph nodes
Boundaries
superiorly:
inferiorly:
anteriorly:
posterolaterally:
medially:
There are two sublevels:
Level II: upper internal jugular (deep cervical) chain
superiorly: base of the skull at the jugular fossa
inferiorly: inferior border of the hyoid bone
anteriorly: posterior border of the submandibular gland
posterolaterally: posterior border of the sternocleidomastoid muscle
medially: medial border of the internal carotid artery
There are two sublevels:
level IIa: inseparable from or anterior to the posterior edge of the internal jugular vein; includes jugulodigastric nodal group
level IIb: posterior to and separable by a fat plane from the internal jugular vein

Level III lymph nodes
Boundaries
superiorly:
inferiorly:
anteriorly:
posterolaterally:
medially:
There are two sublevels:

Level IV lymph nodes Boundaries
superiorly:
inferiorly:
anteriorly:
posterolaterally:
medially
Level IV: lower internal jugular (deep cervical) chain
superiorly: inferior border of the cricoid cartilage
inferiorly: level of the clavicle/manubrium
anteriorly: anterior border of the sternocleidomastoid muscle
posterolaterally: oblique line drawn through the posterolateral edge of the sternocleidomastoid muscle and the lateral edge of the anterior scalene muscle 2
medially: medial border of the common carotid artery
includes medial supraclavicular nodes including Virchow node 1

Level V lymph nodes
Boundaries
superiorly:
inferiorly:
anteriorly:
posterolaterally:
medially
There are two sublevels:
Level V: posterior triangle

superiorly: skull base at the apex of the convergence of sternocleidomastoid and trapezius muscles
inferiorly: level of the clavicle
anteromedially: posterior border of the sternocleidomastoid muscle
posterolaterally: anterior border of the trapezius muscle
There are two sublevels:
level Va: superior half, superior to inferior border of the cricoid cartilage (posterior to levels II and III); includes spinal accessory nodes
level Vb: inferior half, inferior to inferior border of the cricoid cartilage (posterior to level IV); includes lateral supraclavicular nodes 1
Level VI lymph nodes
Boundaries
- superiorly:
- inferiorly:
- anteriorly:
- posterolaterally:
- medially
Level VI lymph nodes
Boundaries
Level VI: central (anterior) compartment
superiorly: inferior border of hyoid bone
inferiorly: superior border of manubrium (suprasternal notch)
anteriorly: platysma muscle 8
posteriorly: trachea (medially) and prevertebral space (laterally) 8
laterally: medial borders of both common carotid arteries (medial to levels III and IV)
includes anterior jugular, pretracheal, paratracheal, prelaryngeal/precricoid (Delphian), and perithyroidal nodes





























































