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| | '''For patient information click [[{{PAGENAME}} (patient information)|here]]''' |
| {{DiseaseDisorder infobox | | | {{DiseaseDisorder infobox | |
| Name = Exophthalmos | | | Name = Exophthalmos | |
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| DiseasesDB = 18612 | | | DiseasesDB = 18612 | |
| }} | | }} |
| {{SI}} | | {{Exophthalmos}} |
| {{CMG}}; {{JFS}} | | {{CMG}}; {{JFS}} |
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| ==Overview== | | {{SK}} Proptosis ; Bulging eyes |
| '''Exophthalmos''' is a bulging of the [[eye]] anteriorly out of the [[orbit (anatomy)|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<ref>Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:77 ISBN 1591032016</ref> <ref>Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:68 ISBN 140510368X</ref>.
| | ==[[Exophthalmos overview|Overview]]== |
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| Exophthalmos can be either bilateral (as is often seen in [[Graves-Basedow disease|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.
| | ==[[Exophthalmos historical perspective|Historical Perspective]]== |
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| 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 [[Computed axial tomography|CT]] or [[MRI]].<ref>{{cite book | author = Owen Epstein, David Perkin, John Cookson, David P de Bono | title = Clinical examination | edition=3rd edition | publisher = Mosby | location = St. Louis | year = 2003 | month=April | id = ISBN 0-7234-3229-5 }}</ref>
| | ==[[Exophthalmos pathophysiology|Pathophysiology]]== |
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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]].
| | ==[[Exophthalmos causes|Causes]]== |
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| == Epidemiology and Demographics == | | ==[[Exophthalmos differential diagnosis|Differentiating Exophthalmos from other Diseases]]== |
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| 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.
| | ==[[Exophthalmos epidemiology and demographics|Epidemiology and Demographics]]== |
| 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.
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| == Pathophysiology & Etiology== | | ==[[Exophthalmos risk factors|Risk Factors]]== |
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| 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.
| | ==[[Exophthalmos screening|Screening]]== |
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| 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.
| | ==[[Exophthalmos natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
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| == Genetics == | | == Diagnosis == |
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| See below regarding causes related to genetic diseases.
| | [[Exophthalmos history and symptoms| History and Symptoms]] | [[Exophthalmos physical examination | Physical Examination]] | [[Exophthalmos laboratory findings | Laboratory Findings]] | [[Exophthalmos CT|CT]] | [[Exophthalmos MRI|MRI]] | [[Exophthalmos ultrasound|Ultrasound]] | [[Exophthalmos other diagnostic studies|Other Diagnostic Studies]] |
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| == Natural History and Complications==
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| 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.
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| == Diagnosis ==
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| ===Complete Differential Diagnosis of the Causes of Exophthalmos: ===
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| (In alphabetical order)
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| *[[Arteriorvenous malformation]]
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| *[[Aspergillosis]] (invasive, orbital)
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| *[[Basedow syndrome]] (combination of hyperthyroidism, goitre, and exophthalmos )
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| *[[Carotid-cavernous fistula]]
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| *[[Cavernous Sinus Thrombosis]]
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| *[[Churg-Strauss syndrome]]
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| *Cloverleaf skull syndrome
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| *Cole-[[Carpenter syndrome]]
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| *[[Corneal ulcer]]
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| *Crouzon craniofacial dysostosis (Crouzon syndrome)
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| *[[Dalrymple's sign]]
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| *[[Dermoid cyst]]
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| *[[Endophthalmitis]]
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| *[[Erdheim-Chester disease]]
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| *[[Graves' disease]]
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| *[[Hemangioma]]
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| *[[Histiocytosis X]]
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| *[[Hyperthyroidism]]
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| *[[Infiltrative ophthalmopathy]] (hyperthyroidism)
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| *Insulin receptor defect with insulin-resistant diabetes mellitus
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| *Lowry-MacLean syndrome
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| *[[Leukemia]]
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| *[[Lymphoma]]
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| *[[Meningioma]]
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| *[[Mucormycosis]]
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| *Nasal type natural killer/T-cell [[lymphoma]]
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| *Neu-Laxova syndrome
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| *[[Neuroblastoma]]
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| *[[Neurofibromatosis]]
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| *Optic [[glioma]]
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| *Orbital [[cellulitis]]
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| *Orbital emphysema: from fracture of a sinus or sneezing.
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| *Orbital inflammatory pseudotumor: The term is used to describe any idiopathic inflammatory lesion that simulates a neoplasm within the orbit. It can be subdivided into different types: granulomatous, sclerosing, vasculitic and eosinophilic, depending upon the histological characteristics. There are strong similarities between OIP and the Tolosa-Hunt syndrome of painful ophthalmoplegia.
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| *Orbital mass or tumors
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| *Osteodysplasty (Melnick-Needles)
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| *[[Polyarteritis nodosa]]
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| *Pseudo[[proptosis]] (buphthalmos, contralateral enophthalmos
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| ipsilateral lid retraction, axial myopia, contralateral blepharoptosis)
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| *[[Raine syndrome]]
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| *[[Relapsing polychondritis]]
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| *[[Retinoblastoma]]
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| *Schinzel-Giedion midface-retraction syndrome
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| *[[Sinusitis]]
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| *Sturge-Weber syndrome: a neurological disorder indicated at birth by seizures accompanied by a large port-wine stain birthmark on the forehead and upper eyelid of one side of the face.
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| *TAO (Thyroid associated orbitopathy)
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| *[[Tolosa-Hunt syndrome]]
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| *Trauma
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| *[[Von Recklinghausen's disease]] (Neurofibromatosis)
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| *[[Varices]], orbital
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| *[[Wegener granulomatosis]]
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| ===Complete Differential Diagnosis of the Causes of Exophthalmos:===
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| (By organ system)
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| {|style="width:75%; height:100px" border="1"
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| |style="height:100px"; style="width:25%" border="1" bgcolor="LightSteelBlue" | '''Cardiovascular'''
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| |style="height:100px"; style="width:75%" border="1" bgcolor="Beige" |[[carotid-cavernous fistula]], [[cavernous sinus thrombosis]], [[Tolosa-Hunt syndrome]], [[Hemangioma]], [[Varices]],
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Chemical / poisoning'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Dermatologic'''
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| |bgcolor="Beige"| Sturge-Weber syndrome
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Drug Side Effect'''
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| |bgcolor="Beige"| No underlying causes | |
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| |-bgcolor="LightSteelBlue"
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| | '''Ear Nose Throat'''
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| |bgcolor="Beige"| Orbital inflammatory pseudotumor, Orbital mass, [[Sinusitis]]
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| |- | |
| |-bgcolor="LightSteelBlue"
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| | '''Endocrine'''
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| |bgcolor="Beige"| [[Basedow syndrome]], [[Graves' disease]], [[Hyperthyroidism]]
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Environmental'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Gastroenterologic'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Genetic'''
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| |bgcolor="Beige"| Cloverleaf skull syndrome, [[dermoid cyst]], Lowry-MacLean syndrome
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| [[Sturge-Weber syndrome]], Raine syndrome, Osteodysplasty (Melnick-Needles), Crouzon craniofacial dysostosis, Insulin receptor defect with insulin-resistant diabetes mellitus,
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| Neu-Laxova syndrome, Schinzel-Giedion midface-retraction syndrome, [[Neuroblastoma]]
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| [[Neurofibromatosis]], [[Retinoblastoma]], [[Von Recklinghausen's disease]]
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| |- | |
| |-bgcolor="LightSteelBlue"
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| | '''Hematologic'''
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| |bgcolor="Beige"| [[Lymphoma]], [[Leukemia]]
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| |-
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| |-bgcolor="LightSteelBlue" | |
| | '''Iatrogenic'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Infectious Disease'''
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| |bgcolor="Beige"| orbital [[cellulitis]], [[endophthalmitis]], [[sinusitis]], [[aspergillosis]], [[mucormycosis]]
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| |- | |
| |-bgcolor="LightSteelBlue"
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| | '''Musculoskeletal / Ortho'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Neurologic'''
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| |bgcolor="Beige"|[[Meningioma]], Sturge-Weber syndrome
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Nutritional / Metabolic'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Obstetric/Gynecologic'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Oncologic'''
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| |bgcolor="Beige"| [[histiocytosis X]], nasal type natural killer/T-cell [[lymphoma]], [[hemangioma]], [[meningioma]], [[neuroblastoma]], [[neurofibromatosis]], optic [[glioma]], [[retinoblastoma]], [[Von Recklinghausen's disease]], [[Lymphoma]], [[Leukemia]]
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| |- | |
| |-bgcolor="LightSteelBlue"
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| | '''Opthalmologic'''
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| |bgcolor="Beige"| orbital inflammatory pseudotumor, orbital [[mass]], [[corneal ulcer]], Pseudo[[proptosis]], orbital [[Varices]], orbital emphysema,
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Overdose / Toxicity'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Psychiatric'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Pulmonary'''
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| |bgcolor="Beige"| [[Churg-Strauss syndrome]], [[Wegener granulomatosis]]
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Renal / Electrolyte'''
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| |bgcolor="Beige"|[[Wegener granulomatosis]]
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Rheum / Immune / Allergy'''
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| |bgcolor="Beige"| [[graves' disease]], [[polyarteritis nodosa]], [[wegener granulomatosis]], [[churg-Strauss syndrome]], [[Relapsing polychondritis]],
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Sexual'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Trauma'''
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| |bgcolor="Beige"| Orbital emphysema, [[Corneal ulcer]]
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Urologic'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Miscellaneous'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |} | |
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| === History and Symptoms ===
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| *Includes:
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| :*age
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| :*rate of onset
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| :*[[pain]]
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| :*[[fever]]
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| :*[[diplopia]]
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| :*[[thyroid disease]]
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| :*[[sinusitis]]
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| :*[[diabetes]]
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| :*[[cancer]]
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| === Physical Examination ===
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| ==== Appearance of the Patient ====
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| ==== Eyes ====
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| *full optomologic exam
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| *[[Boston's sign]]: spasmodic lowering of the upper eyelid on downward rotation of the eye, indicating exophthalmic goiter.
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| *Stellwag's sign: infrequent or incomplete blinking associated with exophthalmos or Graves orbitopathy.
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| *Von Graefe's sign: immobility or lagging of the upper eyelid on downward rotation of the eye, indicating exophthalmic goiter.
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| ==== Ear Nose and Throat ====
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| *full otolaryngologic exam
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| ==== Neurologic ====
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| *full neurologic exam
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| === Laboratory Findings ===
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| *Labs include
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| :*[[thyroid function tests]]
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| :*[[ESR]]
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| :*[[CRP]]
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| :*[[CBC]]
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| :*[[ANCA]]
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| :*[[ANA]]
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| :*[[Blood culture]]
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| ==== MRI and CT ====
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| *[[CT]] of orbits
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| ==== Echocardiography or Ultrasound ====
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| *[[Ultrasound]] if [[arteriovenous malformation]] is suspected
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| == Treatment ==
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| === Acute Pharmacotherapies ===
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| *Direct IV antibiotics -> if infectious
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| *Systemic steroids -> if non infectious
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| === Surgery and Device Based Therapy ===
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| ==== Indications for Surgery ====
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| *Surgical decompression
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| == Primary Prevention ==
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| *Prevent eye injury
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| *Artificial tears
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| *Eye protection (sunglasses)
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| | ==Treatment== |
| | [[Exophthalmos medical therapy|Medical Therapy]] | [[Exophthalmos surgery|Surgery]] | [[Exophthalmos primary prevention|Primary Prevention]] | [[Exophthalmos secondary prevention|Secondary Prevention]] | [[Exophthalmos cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Exophthalmos future or investigational therapies|Future or Investigational Therapies]] |
| | ==Case Studies== |
| | [[Exophthalmos case study one|Case #1]] |
| ==Related Chapters== | | ==Related Chapters== |
| *[[Boston's sign]] | | *[[Boston's sign]] |
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| *[[Graves ophthalmopathy]] | | *[[Graves ophthalmopathy]] |
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| ==References==
| | [[Category:Medical signs]] |
| {{Reflist|2}}
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| {{Eye pathology}}
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| [[Category:Signs and symptoms]] | | [[Category:Signs and symptoms]] |
| [[Category:Physical Examination]] | | [[Category:Physical Examination]] |
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| [[Category:Endocrinology]] | | [[Category:Endocrinology]] |
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