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If left untreated, exophthalmos can causes the eye lids to fail to close during sleep leading to [[cornea|corneal]] damage.  The process that is causing the displacement of the eye may also compress the [[optic nerve]] or [[ophthalmic artery]] leading to [[blindness]].
If left untreated, exophthalmos can causes the eye lids to fail to close during sleep leading to [[cornea|corneal]] damage.  The process that is causing the displacement of the eye may also compress the [[optic nerve]] or [[ophthalmic artery]] leading to [[blindness]].


== Epidemiology and Demographics ==


In adults, thyroid orbitopathy is the most common cause of unilateral and bilateral exophthalmos. Thyroid orbitopathy has a female preponderance with a female-to-male ratio of 5:1.
In caucasian males, the average distance of globe protrusion is 21 mm, and, in african american males, it is 23 mm. Females also show racial variation but a difference of more than 2 mm between the 2 eyes of any given patient is considered abnormal.
== Pathophysiology & Etiology==
The etiological basis of proptosis can be inflammatory, vascular, or infectious. In adults, thyroid orbitopathy is the most common cause of exophthalmos. In children, unilateral proptosis is often due to an orbital cellulitis, and, in bilateral cases, neuroblastoma and leukemia are more likely.
The etiology of the thyroid-related orbitopathy is an autoimmune-mediated inflammatory process of the orbital tissues, predominantly affecting the fat and the extraocular muscles.
== Genetics ==
See below regarding causes related to genetic diseases.
== Natural History and Complications==
Proptosis can compromise visual function and the integrity of the eye. A proptotic eye can develop exposure punctuate keratopathy. This will result in corneal compromise, epithelial death, ulceration, and possible corneal perforation. Proptosis secondary to a space-occupying process can result in a compressive optic neuropathy as well. Such manifestations as depression of visual and color acuities, pupillary dysfunction, and constriction of visual field can occur.


== Diagnosis ==  
== Diagnosis ==  


===Complete Differential Diagnosis of the Causes of Exophthalamus ===
===Complete Differential Diagnosis of the Causes of Exophthalmos: ===
(In alphabetical order)
(In alphabetical order)


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*Insulin receptor defect with insulin-resistant diabetes mellitus
*Insulin receptor defect with insulin-resistant diabetes mellitus
*Lowry-MacLean syndrome
*Lowry-MacLean syndrome
*[[Leukemia]]
*[[Lymphoma]]
*[[Meningioma]]
*[[Meningioma]]
*[[Mucormycosis]]
*[[Mucormycosis]]
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*Optic [[glioma]]
*Optic [[glioma]]
*Orbital [[cellulitis]]
*Orbital [[cellulitis]]
*Orbital emphysema
*Orbital inflammatory pseudotumor
*Orbital inflammatory pseudotumor
*Orbital mass or tumors
*Orbital mass or tumors
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*Trauma  
*Trauma  
*[[Von Recklinghausen's disease]] (Neurofibromatosis)
*[[Von Recklinghausen's disease]] (Neurofibromatosis)
*[[Varices]], orbital
*[[Wegener granulomatosis]]
*[[Wegener granulomatosis]]




 
===Complete Differential Diagnosis of the Causes of Exophthalmos:===
Make sure that each diagnosis is linked to a page.
 
===Complete Differential Diagnosis of the Causes of Exophthalamos:===
(By organ system)
(By organ system)
{|style="width:75%; height:100px" border="1"
{|style="width:75%; height:100px" border="1"
|style="height:100px"; style="width:25%" border="1" bgcolor="LightSteelBlue" | '''Cardiovascular'''
|style="height:100px"; style="width:25%" border="1" bgcolor="LightSteelBlue" | '''Cardiovascular'''
|style="height:100px"; style="width:75%" border="1" bgcolor="Beige" |Carotid-cavernous fistula
|style="height:100px"; style="width:75%" border="1" bgcolor="Beige" |[[carotid-cavernous fistula]], [[cavernous sinus thrombosis]], [[Tolosa-Hunt syndrome]], [[Hemangioma]], [[Varices]],
Cavernous sinus thrombosis, Tolosa-Hunt syndrome, [[Hemangioma]]
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
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|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Oncologic'''
| '''Oncologic'''
|bgcolor="Beige"| Histiocytosis X, nasal type natural killer/T-cell lymphoma, [[hemangioma]], [[meningioma]], [[neuroblastoma]], [[neurofibromatosis]], optic [[glioma]], [[retinoblastoma]], [[Von Recklinghausen's disease]]
|bgcolor="Beige"| Histiocytosis X, nasal type natural killer/T-cell lymphoma, [[hemangioma]], [[meningioma]], [[neuroblastoma]], [[neurofibromatosis]], optic [[glioma]], [[retinoblastoma]], [[Von Recklinghausen's disease]], [[Lymphoma]], [[Leukemia]]
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
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|-
|-
|}
|}
=== History and Symptoms ===  
=== History and Symptoms ===  
*Includes:
*Includes:
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=== Physical Examination ===  
=== Physical Examination ===  


=== Appearance of the Patient ===
==== Appearance of the Patient ====


=== Eyes ===
==== Eyes ====
*full optomologic exam
*full optomologic exam
*[[Boston's sign]]: spasmodic lowering of the upper eyelid on downward rotation of the eye, indicating exophthalmic goiter.
*[[Boston's sign]]: spasmodic lowering of the upper eyelid on downward rotation of the eye, indicating exophthalmic goiter.
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*Von Graefe's sign: immobility or lagging of the upper eyelid on downward rotation of the eye, indicating exophthalmic goiter.
*Von Graefe's sign: immobility or lagging of the upper eyelid on downward rotation of the eye, indicating exophthalmic goiter.


 
==== Ear Nose and Throat ====
 
=== Ear Nose and Throat ===
*full otolaryngologic exam
*full otolaryngologic exam


=== Neurologic ===
==== Neurologic ====
*full neurologic exam
*full neurologic exam


== Laboratory Findings ==  
=== Laboratory Findings ===  
*Labs include
*Labs include
:*[[thyroid function tests]]
:*[[thyroid function tests]]
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:*[[Blood culture]]
:*[[Blood culture]]


=== MRI and CT ===  
==== MRI and CT ====  
*[[CT]] of orbits
*[[CT]] of orbits


=== Echocardiography or Ultrasound ===  
==== Echocardiography or Ultrasound ====  
*[[Ultrasound]] if [[arteriovenous malformation]] is suspected
*[[Ultrasound]] if [[arteriovenous malformation]] is suspected


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*Systemic steroids -> if non infectious
*Systemic steroids -> if non infectious


== Surgery and Device Based Therapy ==  
=== Surgery and Device Based Therapy ===  


=== Indications for Surgery ===  
==== Indications for Surgery ====  
*Surgical decompression
*Surgical decompression


Revision as of 19:39, 11 June 2009

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Associate Editor-In-Chief: John Fani Srour, M.D.


Please Take Over This Page and Apply to be Editor-In-Chief for this topic: There can be one or more than one Editor-In-Chief. You may also apply to be an Associate Editor-In-Chief of one of the subtopics below. Please mail us [2] to indicate your interest in serving either as an Editor-In-Chief of the entire topic or as an Associate Editor-In-Chief for a subtopic. Please be sure to attach your CV and or biographical sketch.

Overview

Exophthalmos is a bulging of the eye anteriorly out of the orbit. Some sources define exophthalmos as a protrusion of the globe greater than 18 mm and proptosis as a protusion equal to or less than 18 mm. (Epstein et al, 2003). Others define "exophthalmos" as protusion secondary to endocrine dysfunction and "proptosis" as any non-endocrine-mediated protusion[1] [2].

Exophthalmos can be either bilateral (as is often seen in Grave's Disease) or unilateral (as is often seen in an orbital tumor). Measurement of the degree of exophthalmos is performed using an exophthalmometer. 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.[3]

If left untreated, exophthalmos can causes the eye lids to fail to close during sleep leading to corneal damage. The process that is causing the displacement of the eye may also compress the optic nerve or ophthalmic artery leading to blindness.

Epidemiology and Demographics

In adults, thyroid orbitopathy is the most common cause of unilateral and bilateral exophthalmos. Thyroid orbitopathy has a female preponderance with a female-to-male ratio of 5:1. In caucasian males, the average distance of globe protrusion is 21 mm, and, in african american males, it is 23 mm. Females also show racial variation but a difference of more than 2 mm between the 2 eyes of any given patient is considered abnormal.

Pathophysiology & Etiology

The etiological basis of proptosis can be inflammatory, vascular, or infectious. In adults, thyroid orbitopathy is the most common cause of exophthalmos. In children, unilateral proptosis is often due to an orbital cellulitis, and, in bilateral cases, neuroblastoma and leukemia are more likely.

The etiology of the thyroid-related orbitopathy is an autoimmune-mediated inflammatory process of the orbital tissues, predominantly affecting the fat and the extraocular muscles.

Genetics

See below regarding causes related to genetic diseases.

Natural History and Complications

Proptosis can compromise visual function and the integrity of the eye. A proptotic eye can develop exposure punctuate keratopathy. This will result in corneal compromise, epithelial death, ulceration, and possible corneal perforation. Proptosis secondary to a space-occupying process can result in a compressive optic neuropathy as well. Such manifestations as depression of visual and color acuities, pupillary dysfunction, and constriction of visual field can occur.

Diagnosis

Complete Differential Diagnosis of the Causes of Exophthalmos:

(In alphabetical order)

ipsilateral lid retraction, axial myopia, contralateral blepharoptosis)


Complete Differential Diagnosis of the Causes of Exophthalmos:

(By organ system)

Cardiovascular carotid-cavernous fistula, cavernous sinus thrombosis, Tolosa-Hunt syndrome, Hemangioma, Varices,
Chemical / poisoning No underlying causes
Dermatologic No underlying causes
Drug Side Effect No underlying causes
Ear Nose Throat Orbital inflammatory pseudotumor, Orbital mass, Sinusitis
Endocrine Basedow syndrome, Graves' disease, Hyperthyroidism
Environmental No underlying causes
Gastroenterologic No underlying causes
Genetic Cloverleaf skull syndrome, dermoid cyst, Lowry-MacLean syndrome

Sturge-Weber syndrome, Raine syndrome, Osteodysplasty (Melnick-Needles), Crouzon craniofacial dysostosis, Insulin receptor defect with insulin-resistant diabetes mellitus, Neu-Laxova syndrome, Schinzel-Giedion midface-retraction syndrome, Neuroblastoma Neurofibromatosis, Retinoblastoma, Von Recklinghausen's disease

Hematologic No underlying causes
Iatrogenic No underlying causes
Infectious Disease orbital cellulitis, endophthalmitis, sinusitis, aspergillosis, mucormycosis
Musculoskeletal / Ortho No underlying causes
Neurologic No underlying causes
Nutritional / Metabolic No underlying causes
Obstetric/Gynecologic No underlying causes
Oncologic Histiocytosis X, nasal type natural killer/T-cell lymphoma, hemangioma, meningioma, neuroblastoma, neurofibromatosis, optic glioma, retinoblastoma, Von Recklinghausen's disease, Lymphoma, Leukemia
Opthalmologic orbital inflammatory pseudotumor, orbital mass, corneal ulcer, Pseudoproptosis
Overdose / Toxicity No underlying causes
Psychiatric No underlying causes
Pulmonary Churg-Strauss syndrome, Wegener granulomatosis
Renal / Electrolyte Wegener granulomatosis
Rheum / Immune / Allergy graves' disease, polyarteritis nodosa, wegener granulomatosis, *churg-Strauss syndrome, Relapsing polychondritis, Wegener granulomatosis
Sexual No underlying causes
Trauma No underlying causes
Urologic No underlying causes
Miscellaneous No underlying causes


History and Symptoms

  • Includes:

Physical Examination

Appearance of the Patient

Eyes

  • full optomologic exam
  • Boston's sign: spasmodic lowering of the upper eyelid on downward rotation of the eye, indicating exophthalmic goiter.
  • Stellwag's sign: infrequent or incomplete blinking associated with exophthalmos or Graves orbitopathy.
  • Von Graefe's sign: immobility or lagging of the upper eyelid on downward rotation of the eye, indicating exophthalmic goiter.

Ear Nose and Throat

  • full otolaryngologic exam

Neurologic

  • full neurologic exam

Laboratory Findings

  • Labs include

MRI and CT

  • CT of orbits

Echocardiography or Ultrasound

Treatment

Acute Pharmacotherapies

  • Direct IV antibiotics -> if infectious
  • Systemic steroids -> if non infectious

Surgery and Device Based Therapy

Indications for Surgery

  • Surgical decompression

Primary Prevention

  • Prevent eye injury
  • Artificial tears
  • Eye protection (sunglasses)

See also

References

  1. ↑ Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:77 ISBN 1591032016
  2. ↑ Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:68 ISBN 140510368X
  3. ↑ Owen Epstein, David Perkin, John Cookson, David P de Bono (2003). Clinical examination (3rd edition ed.). St. Louis: Mosby. ISBN 0-7234-3229-5. Unknown parameter |month= ignored (help)


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