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Exophthalmos

Exophthalmic Condition

Exophthalmos is a condition characterized by bulging of the eyeball anteriorly out of the orbit. Such a type of condition can either be bilateral or unilateral in nature.


Presentation

Signs and symptoms of exophthalmos vary with the underlying condition. When thyroid diseases are the causative factor, then the following signs and symptoms are evident [6]:

In addition, to the above mentioned symptoms, individuals would also experience double vision. In severe cases, it almost gets impossible to close eyes during sleeping, which eventually leads to dryness of the cornea, making it susceptible to infections and development of ulcers.

Diplopia
  • New-onset diplopia was noted for 2.8% of patients after FROD.[ncbi.nlm.nih.gov]
  • BACKGROUND/PURPOSE: Postoperative diplopia remains a significant complication of orbital decompression in dysthyroid orbitopathy.[ncbi.nlm.nih.gov]
  • The onset of consecutive diplopia was not significantly different between the two groups (case n 2/15, controls n 5/15; P .203). Diplopia resolved spontaneously in all the patients of group 1, while all the patients of group 2 required surgery.[ncbi.nlm.nih.gov]
  • One patient developed postoperative diplopia and 5 complained of temporary diplopia. Appearance improved in 62 patients (85%).[ncbi.nlm.nih.gov]
  • Clinical signs and symptoms of orbital metastases may include exophthalmos, diplopia, pain, limited ocular motility, blurred vision, swollen eyelid, conjunctival hyperemia and edema, increased ocular pressure and papilledema.[ncbi.nlm.nih.gov]
Strabismus
  • Endocrine orbitopathy (EO) can have important consequences, such as exophthalmos and restrictive strabismus.[ncbi.nlm.nih.gov]
  • Strabismus surgery had to be performed in 6 patients due to unsatisfactory correction of double vision.[ncbi.nlm.nih.gov]
  • This combination of muscle impairment and forward displacement reduces eye movement, causing double vision and crossed eyes ( strabismus ).[encyclopedia.com]
  • PURPOSE: Bilateral convergent strabismus with exophthalmos (BCSE) is a widespread inherited eye defect in several cattle populations. Its progressive condition often leads to blindness in affected cattle and shortens their length of productive life.[ncbi.nlm.nih.gov]
  • Strabismus — Any deviation of the eyes from a common direction. Commonly called a turned eye.[medical-dictionary.thefreedictionary.com]
Lagophthalmos
  • A proptotic eye not adequately protected by the lids, as with lagophthalmos, can develop exposure punctuate keratopathy.[emedicine.medscape.com]
  • Sequelae of proptosis include blindness, strabismus, lagophthalmos, sensory deficit of the cornea, keratoconjunctivitis sicca, exposure keratitis, glaucoma, and phthisis bulbi. 4.[vin.com]
  • Examination: the orbit - look for lid swelling, engorged conjunctival and episcleral vessels and lagophthalmos/incomplete lid closure. Palpate the orbit for any tenderness or masses and examine the regional lymph nodes.[medigoo.com]
Bilateral Proptosis
  • A 52-year-old Chinese woman with progressively worsening bilateral proptosis presented to our hospital. Subsequently she was diagnosed as having primary bilateral macronodular adrenal hyperplasia and underwent bilateral laparoscopic adrenalectomy.[ncbi.nlm.nih.gov]
  • Inflammatory/Infection: Graves' ophthalmopathy due to Graves' disease, usually causes bilateral proptosis.[en.wikipedia.org]
  • The exam found bilateral proptosis with fat deposits behind the orbits. My proptosis also worsened during this last year.[realself.com]
  • Etiology The causes of proptosis are broad and include a wide range of mass lesions that originate within the cranium, sinuses, paranasal spaces, and orbit 3 : thyroid orbitopathy (the most common cause of uni/bilateral proptosis in adults) infection[radiopaedia.org]
  • Considering that the 78% patients in group 2 needed corticosteroid versus the 53% of patients of group 1, it could be argued that patients who progressed onto bilateral proptosis have worse disease in general, and this could be suggested by a trend even[bmcophthalmol.biomedcentral.com]
Unilateral Proptosis
  • Dacryoadenitis Erdheim-Chester Disease Mucormycosis Orbital pseudotumor – presents with acute, usually unilateral proptosis with severe pain.[en.wikipedia.org]
  • The leading symptoms are unilateral proptosis with ophthalmalgia, double vision or decreased acuity, a palpable mass in the superior medial aspect of the orbit or medial canthus with the fronto-ethmoidal mucoceles, and headaches. 2 On plain X-ray films[appliedradiology.com]
  • In our study, we found 13% of patients presenting unilateral TED with the appearance of unilateral proptosis. Bartley reported unilateral proptosis at the onset in 8,5% of patients in a cohort of cases with TED [ 24 ].[bmcophthalmol.biomedcentral.com]

Workup

A preliminary examination of the eye would be carried out to evaluate the disorder. This would be followed by examination with exophthalmometer, which would measure the extent of protrusion of the eyes. In addition, imaging studies such as CT scan and MRI would also be useful. The abnormal deposition of connective tissues in the eyes for people with Graves disease can be detected through CT scan or MRI examination [7].

Blood tests would also be required to determine the underlying disease condition that is causing exophthalmos. Individuals, who have developed exophthalmos as a result of orbital cellulitis, should get complete blood count, nasal cultures, blood culture tests and sinus imaging studies as part of diagnosis evaluation [8].

Treatment

Correcting the underlying disease condition that is causing exophthalmos forms the basis of the treatment regime. The following methods are employed for treating the eye condition:

  • Medications: Medications such as corticosteroids are helpful to reduce the inflammation and pain in the eye. Such medicines can either be taken orally or through the intravenous route [9].
  • Radiotherapy: This is particularly required when the corticosteroids do not bring about desired effects. In this method, high beam radiation is imparted to the affected eye in order to destroy the affected cells [10].
  • Surgery: Surgery is often the last resort or considered when the symptoms are severe and cannot be managed effectively through medications or other forms of treatment [11]. Three types of surgery are considered for correcting exophthalmos, which include orbital decompression surgery, eyelid surgery and eye muscle surgery.

Prognosis

Prognosis of the condition is pretty favorable, if treatment is initiated at the right time. However, failure to do so can cause severe complications to set in. Untreated exophthalmos can cause corneal dryness and superior limbic keratoconjunctivitis. In many instances, the optic nerve in the affected eye can also get compressed causing blindness. Many individuals also suffer from long term vision problems such as development of double vision.

Etiology

Several factors are responsible for causation of exophthalmos. Various infections or inflammatory disease conditions such as Graves disease, mucormycosis, orbital cellulitis, Erdheim-Chester disease, orbital pseudotumor, Eegner’s granulomatosis, and high altitude cerebral edema play major role in development of exophthalmos. In addition to these, disease conditions that cause exophthalmos include dermoid cyst, leukemia, hemangioma, meningioma, Hand-Schuler-Christian disease and nasopharyngeal angiofibroma.

Individuals suffering from orbital fracture, Cushing syndrome, retrobulbar hemorrhage and Pfeiffer syndrome are at an increased risk of developing exophthalmos [2].

Epidemiology

It has been estimated that, every year exophthalmos occurs in about 2.9 cases per 100,000 populations in men and affects about 16 women per 100,000 populations. The condition can affect adults and children at any age. Women in the age group of 30 to 50 years are at increased risk of falling prey to thyroid orbitopathy [3].

Sex distribution
Age distribution

Pathophysiology

Exophthalmos is also sometimes referred to as protopsis. Inflammatory response, infections, neoplasia, and several traumatic factors favor the development of protopsis.

Individuals with Graves disease are more prone to contract such a type of eye condition with increased chances of developing the bilateral form [4]. In Graves disease, bulging of the eyes occurs due to abnormal deposition of connective tissue in the extraocular muscles as well as the orbit. Such a type of deposition causes displacement of the eye, which presents as if the eyes are protruded from their original position.

Unilateral exophthalmos occurs in patients with orbital tumor. There have been cases, when there is complete dislocation from the orbit; this primarily occurs due to trauma which in turn induces swelling in the tissues surrounding the orbit.

In individuals with thyroid disease, the phenomenon of exophthalmos primarily occurs due to inflammatory reactions of the orbital tissues, which triggers the deposition of glycoaminoglycans in the orbit. With such sequence of events, fibrosis can set in gradually [5].

Prevention

There has been certain evidence suggesting a strong link between cigarette smoking and development of Graves disease. Therefore, avoiding smoking can go a long way in prevention of the disease. In addition, seeking prompt treatment for underlying disease condition can also prevent the onset of exophthalmos.

Summary

Bilateral exophthalmos is more commonly seen in individuals suffering from Graves disease [1]. If the condition is left untreated, then it can lead to various complications due to improper shutting of eyes during sleep. Such a type of event can gradually cause corneal dryness and subsequently its damage. In addition to these conditions, untreated exophthalmos can also lead to development of superior limbic keratoconjunctivitis. In more severe cases, the condition can also lead to blindness.

Patient Information

Definition: Exophthalmos is a condition, wherein the eyes protrude due to certain disease conditions or inflammatory response to various factors. Such a type of eye disease can either be bilateral or unilateral in nature. The condition should be promptly treated to avoid onset of debilitating complications.

Cause: Graves disease and thyroid disease significantly increase the risk of developing exophthalmos. Other causes include orbital cellulitis, leukemia and neuroblastoma that cause the eyes to protrude out of the orbit.

Symptoms: Symptoms of exophthalmos include redness and inflammation of the affected eye, frequent blinking of the eyes, development of photophobia, and eyes turn dry and gritty.

Diagnosis: Diagnosis of exophthalmos involves physical examination of the eye to study the extent of eye movements. Thereafter, with the help of exophthalmometer, the extent of protrusion of eye is studied. In addition, blood tests to evaluate underlying disease conditions and imaging studies are done for conducting a detailed examination of the eye socket.

Treatment: Treatment of exophthalmos involves managing the underlying disease condition to reduce the severity of the symptoms. Medications such as corticosteroids are administered either orally or intravenously. Surgery is employed only when medications do not yield any positive outcome.

References

Article

  1. Tsai CC, Kau HC, Kao SC, Hsu WM. Exophthalmos of patients with Graves' disease in Chinese of Taiwan.Eye (Lond). May 2006;20(5):569-73.
  2. Tellez M, Cooper J, Edmonds C. Graves' ophthalmopathy in relation to cigarette smoking and ethnic origin.Clin Endocrinol (Oxf). Mar 1992;36(3):291-4
  3. Ahmadi H, Shams PN, Davies NP, Joshi N, Kelly MH. Age-related changes in the normal sagittal relationship between globe and orbit. J Plast Reconstr Aesthet Surg. 2007;60(3):246-50.
  4. Burch HB, Wartofsky L. Graves' ophthalmopathy: current concepts regarding pathogenesis and management. Endocr Rev 1993; 14:747.
  5. Maheshwari R, Weis E. Thyroid associated orbitopathy. Indian J Ophthalmol. Mar-Apr 2012;60(2):87-93.
  6. Henderson JW. Orbital Tumors. 3rd ed. New York: Raven Press; 1994.
  7. Dijkstal JM, Bothun ED, Harrison AR, Lee MS. Normal exophthalmometry measurements in a United States pediatric population. Ophthal Plast Reconstr Surg. Jan 2012;28(1):54-6
  8. Nageswaran S, Woods CR, Benjamin DK Jr, et al. Orbital cellulitis in children. Pediatr Infect Dis J 2006; 25:695.
  9. Bartalena L, Marcocci C, Bogazzi F, et al. Use of corticosteroids to prevent progression of Graves' ophthalmopathy after radioiodine therapy for hyperthyroidism. N Engl J Med 1989; 321:1349.
  10. Mourits MP, van Kempen-Harteveld ML, García MB, et al. Radiotherapy for Graves' orbitopathy: randomised placebo-controlled study. Lancet 2000; 355:1505.
  11. Wakelkamp IM, Baldeschi L, Saeed P, et al. Surgical or medical decompression as a first-line treatment of optic neuropathy in Graves' ophthalmopathy? A randomized controlled trial. Clin Endocrinol (Oxf) 2005; 63:323.

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Last updated: 2019-07-11 22:23