Exophthalmos

"Proptosis" redirects here. For the condition of drooping of the eyelid, see Ptosis (eyelid).

This article is about proptosis. For the weevils, see Exophthalmus.

Exophthalmos
Patient with left eye proptosis
Classification and external resources
Specialty
ICD-10	H05.2
ICD-9-CM	376.2-376.3
DiseasesDB	18612
MedlinePlus	003033
eMedicine	oph/616
Patient UK	Exophthalmos
MeSH	D005094

Exophthalmos (also called exophthalmus, exophthalmia, proptosis, or exorbitism) is a bulging of the eye anteriorly out of the orbit. Exophthalmos can be either bilateral (as is often seen in Graves' disease) or unilateral (as is often seen in an orbital tumor). Complete or partial dislocation from the orbit is also possible from trauma or swelling of surrounding tissue resulting from trauma.

In the case of Graves' disease, the displacement of the eye is due to abnormal connective tissue deposition in the orbit and extraocular muscles which can be visualized by CT or MRI.

If left untreated, exophthalmos can cause the eyelids to fail to close during sleep leading to corneal dryness and damage. Another possible complication would be a form of redness or irritation called "Superior limbic keratoconjunctivitis", where the area above the cornea becomes inflamed as a result of increased friction when blinking. The process that is causing the displacement of the eye may also compress the optic nerve or ophthalmic artery, leading to blindness.

Measurement

Measurement of the degree of exophthalmos is performed using an exophthalmometer.

Most sources define exophthalmos/proptosis as a protrusion of the globe greater than 18 mm.

The term exophthalmos is often used when describing proptosis associated with Graves' disease.

Anatomy

Proptosis is the anterior displacement of the eye from the orbit. Since the orbit is closed off posteriorly, medially and laterally, any enlargement of structures located within will cause the anterior displacement of the eye. Swelling or enlargement of the lacrimal gland causes inferior medial and anterior dislocation of the eye. This is because the lacrimal glands are located superiorly and laterally in the orbit.

Causes

Inflammatory/Infection:

• Graves' ophthalmopathy due to Graves' disease, usually causes bilateral proptosis.
• Orbital cellulitis - often with unilateral proptosis, severe redness, and moderate to severe pain, sinusitis and an elevated white blood cell count.
• Dacryoadenitis
• Erdheim-Chester Disease
• Mucormycosis
• Orbital pseudotumor - presents with acute, usually unilateral proptosis with severe pain.
• High altitude cerebral edema
• Granulomatosis with polyangiitis

Neoplastic:

• Leukemias
• Meningioma, (of sphenoid wing)
• Nasopharyngeal angiofibroma
• Hand–Schüller–Christian disease
• Hemangioma, cavernous

Cystic:

• Dermoid cyst

Vascular:

• Carotid-cavernous fistula
• Aortic insufficiency: manifests as a pulsatile pseudoproptosis, described by British cardiothoracic surgeon, Hutan Ashrafian in 2006

Others:

• Orbital fracture: apex, floor, medial wall, zygomatic
• Retrobulbar hemorrhage: trauma to the orbit can lead to bleeding behind the eye. The hemorrhage has nowhere to escape and the increased pressure pushes the eye out of the socket, leading to proptosis and can also cause blindness if not treated promptly.
• Cushing's syndrome (due to fat in the orbital cave)
• Pfeiffer syndrome

Other animals

Exophthalmos in a Pug

Exophthalmos is commonly found in dogs. It is seen in brachycephalic (short-nosed) dog breeds because of the shallow orbit. However, it can lead to keratitis secondary to exposure of the cornea. Exophthalmos is commonly seen in the Pug, Boston Terrier, Pekingese, and Shih Tzu.

It is a common result of head trauma and pressure exerted on the front of the neck too hard in dogs. Most commonly it occurs in brachycephalic (short nosed) breeds. In cats, eye proptosis is uncommon and is often accompanied by facial fractures.

About 40% of proptosed eyes retain vision after being replaced in the orbit, but in cats very few retain vision. Replacement of the eye requires general anesthesia. The eyelids are pulled outward, and the eye is gently pushed back into place. The eyelids are sewn together in a procedure known as tarsorrhaphy for about five days to keep the eye in place. Replaced eyes have a higher rate keratoconjunctivitis sicca and keratitis and often require lifelong treatment. If the damage is severe, the eye is removed in a relatively simple surgery known as enucleation of the eye.

The prognosis for a replaced eye is determined by the extent of damage to the cornea and sclera, the presence or absence of a pupillary light reflex, and the presence of ruptured rectus muscles. The rectus muscles normally help hold the eye in place and direct eye movement. Rupture of more than two rectus muscles usually requires the eye to be removed, because significant blood vessel and nerve damage also usually occurs. Compared to brachycephalic breeds, dochilocephalic (long-nosed) breeds usually have more trauma to the eye and its surrounding structures, so the prognosis is worse .

See also

• Boston's sign
• Enophthalmos
• Von Graefe's sign

External links

• exophthalmos at GPnotebook
• Can the human eyeball be knocked out of the head? at Straight Dope.
• What Is Exophthalmos? What Causes Exophthalmos? at Medical News Today. Accessed 2013-12-22.