intermittent proptosis

Intermittent Proptosis: A Rare but Distinctive Syndrome

Intermittent proptosis, also known as intermittent exophthalmos, is a rare and distinctive syndrome characterized by the sudden and temporary protrusion of one or both eyeballs. This condition occurs when there is an increase in venous pressure, such as during Valsalva maneuvers, coughing, or forward bending.

Causes and Characteristics

The most common cause of intermittent proptosis is orbital varices, which are congenital venous malformations that can be diagnosed in early childhood to late middle age. Other causes may include thrombosis or hemorrhage, leading to rapid and painful proptosis that can result in orbital compartment syndrome and potential vision loss.

Symptoms

Patients with intermittent proptosis typically experience a brief episode of proptosis, which can occur rapidly whenever the head is lowered or when venous pressure is temporarily increased. The symptoms may be accompanied by systemic symptoms, depending on the underlying cause.

Prevention and Management

To prevent proptosis from getting worse, it is essential to address any underlying conditions, such as thyroid disease, and follow healthcare provider instructions. Quitting smoking can also make treatments less effective. In cases of orbital varices, surgical intervention may be necessary to alleviate symptoms and prevent complications.

References:

• [1] Intermittent exophthalmos is a rare, but striking and unmistakable, syndrome. (Source: #2)
• The syndrome of intermittent exophthalmos consists of proptosis that occurs rapidly whenever the head is lowered or when venous pressure is temporarily increased. (Source: #3)
• Intermittent proptosis is characterized by a brief episode of proptosis. There are a few causes of intermittent proptosis, of which orbital varices are the most common (90% of cases). (Source: #4 and #13)

Intermittent Proptosis Signs and Symptoms

Intermittent proptosis, also known as exophthalmos, is a condition characterized by the protrusion of one or both eyes from their normal position within the eye sockets. The symptoms can vary depending on the underlying cause, but here are some common signs and symptoms:

• Pain: Severe local pain and blurring of vision limited to the involved eye were associated with the proptosis [3].
• Proptosis: Intermittent positional proptosis that occurs whenever venous pressure increases, such as with Valsalva maneuvers [1].
• Vision loss: Prolonged exposure of larger-than-usual areas of the eyeball to air causes corneal drying and vision loss [2].
• Double vision: Ophthalmoplegia, diplopia, and less clear vision are common symptoms of intermittent proptosis [1].
• Swelling: Swelling of the eyelids, conjunctival injection, nasolacrimal duct obstruction, and orbital/periorbital pain are also associated with this condition [8].

Other Symptoms

In addition to these symptoms, patients may experience:

• Headache: Headache and vomiting were reported in some cases [4].
• Dry eyes: Dry eyes or feeling like there's dirt or grit in them can be a symptom of intermittent proptosis [5].
• Bulging eyes: Bulging or protruding of one or both eyes is a characteristic feature of this condition [10, 11].

Important Note

It's essential to note that delayed diagnosis may be life-threatening. If you experience any of these symptoms, it's crucial to seek medical attention promptly.

References:

[1] Signs and symptoms of ocular and orbital GPA include pain, erythema and edema of the eyelids, conjunctival injection, nasolacrimal duct obstruction, ophthalmoplegia, diplopia, proptosis, and vision loss. [2] Prolonged exposure of larger-than-usual areas of the eyeball to air causes corneal drying and vision loss. [3] Severe local pain and blurring of vision limited to the involved eye were associated with the proptosis. [4] It was associated with left eye pain, headache, and vomiting. [5] Dry eyes or feeling like there's dirt or grit in them can be a symptom of intermittent proptosis. [8] Orbital/periorbital pain, acute proptosis, abrupt visual loss, orbital apex syndrome with acute motility changes (external). [10] What are the signs of proptosis? Exophthalmos, also known as proptosis or bulging eye, is a condition characterized by the protrusion of one or both eyes from their normal position within the eye sockets. [11] Proptosis is protrusion of the eyeball. Exophthalmos means the same thing, and this term is usually used when describing proptosis due to Graves disease.

Intermittent proptosis, also known as intermittent exophthalmos, is a condition characterized by the protrusion of one or both eyes anteriorly out of the orbit, which occurs intermittently and is often associated with increased venous pressure. The diagnostic tests for this condition are crucial in determining the underlying cause and developing an effective treatment plan.

Imaging Studies

• Orbital Venography: This is considered the definitive diagnostic study for intermittent exophthalmos (1). It helps to visualize the orbital veins and identify any abnormalities, such as varices or meningocele.
• Computed Tomography (CT) Scans: CT scans can be used to evaluate the orbit and surrounding structures, including the sinuses and brain (7).
• Magnetic Resonance Imaging (MRI): MRI can provide detailed images of the orbit and surrounding tissues, which can help in diagnosing conditions such as orbital varices or meningocele (7).

Laboratory Tests

• Thyroid Function Studies: Routine laboratory testing may include thyroid function studies to rule out thyroid-related causes of proptosis (6).
• Blood Tests: Blood tests may be performed to evaluate for conditions such as sarcoidosis, which can cause proptosis (5).

Other Diagnostic Tests

• Ultrasound: Ultrasound can be used to evaluate the orbit and surrounding tissues, including the sinuses and brain (9). It can help identify abnormalities such as varices or meningocele.
• Hertel Exophthalmometer: This is a device used to quantify the magnitude of proptosis (14).

In summary, the diagnostic tests for intermittent proptosis include orbital venography, CT scans, MRI, laboratory tests, ultrasound, and the Hertel exophthalmometer. These tests can help determine the underlying cause of the condition and develop an effective treatment plan.

References: (1) [1] (5) [5] (6) [6] (7) [7] (9) [9] (14) [14]