Proptosis
Proptosis is the protrusion or forward displacementof the eye.
Principal Causes of Proptosis
- Trauma
- Infection/inflammation
- Orbitalcellulitis/abscess
- Orbital pseudotumor
- Craniofacial disorders
- Coronalcraniosynostosis
- Craniofacial dysostosis (Crouzon syndrome)
- Thyroid eye disease
- Non-neoplastic masses
- Mucocele
- Craniomeningocele and encephalocele
- Neoplasm
- Histiocytoses
- Vascular lesions
- Orbitalvarices
- Orbital aneurysm
- Carotid cavernous fistula
- Cavernous sinus thrombosis
- Bone disorders
- Fibrousdysplasia
Clinical Features and Diagnosis
Trauma
Contusion or hematoma can cause proptosis.CT is procedure of choice to detect fractures and bony defects.
Infection/Inflammation
Orbital Cellulitis/Abscess
Usuallyarises from extension of sinusitis.Swollen painful eye, proptosis, andimpaired extraocular movements are usual findings.Most common pathogens are S. aureusand group A Streptococcus. H. influenzae type b is much less commonsince the advent of H. influenzae type b vaccine to prevent thisinfection.CT can distinguish orbital cellulitisfrom orbital abscess. Orbital Pseudotumor
Nonspecificinflammatory process of unknown cause that can infiltrate soft tissuesof the orbit. Usually unilateral but can be bilateral. Eye is painfuland proptotic.CT and MRI can define location andextent of lesion.Biopsy distinguishes this lesion frominfiltrative malignancy. Craniofacial Disorders
Coronal Craniosynostosis
Is prematureclosure of 1 or more sutures of the skull. Closure of coronal sutureproduces short wide skull with flattening of frontal area, shalloworbits, and proptosis.Skull radiographs confirm diagnosis.This type of craniosynostosis occursin Apert syndrome (see Chap.63, Stertor, Stridor, and Airway Obstruction). Craniofacial Dysostosis (Crouzon Syndrome)
Autosomal-dominantdisorder with variable expression. Gene locus has been mapped tochromosome 10q26.Characteristic findings include craniosynostosis,which produces shallow orbits and proptosis; hypertelorism; beak-shapednose; and hypoplasia of maxilla. Thyroid Eye Disease
1 manifestation of Graves disease is proptosis(exophthalmos). Other eye signs include retraction of upper lidsand lid lag. Optic neuropathy sometimes complicates thyroid eyedisease. Clinical manifestations and diagnosis of Graves diseaseare discussed in Chap. 42, NeckMasses.
Non-Neoplastic Masses
Mucocele
Cystic swellingthat arises from paranasal sinuses, usually frontal or ethmoid,and less commonly the maxillary. Expansion into orbit can causepain and proptosis.CT shows location and extent of lesion. Craniomeningocele and Encephalocele
Both canbe found within orbit, displacing its contents.CT and MRI locate and define extentof lesions. Neoplasm
CT and MRI can locate and define extent oftumor mass. With exception of hemangioma that has superficial components,histologic diagnosis is mandatory, especially with any suspectedmalignancy.
Benign Tumors
Hemangioma
Superficialhemangioma that involves upper lid is usually confined to preseptal space.Another form of hemangioma may occurin postseptal area and expands to fill bony orbit, causing proptosis.CT and MRI can usually distinguishhemangioma from other masses by distinct enhancement pattern. Lymphangioma
Often arises in superior aspect of orbitbehind globe and may infiltrate tissues of lid and orbit to produceproptosis.
Dermoid
Usual location is along upper lateral marginof the orbit; however, some dermoids lie within orbit and produceproptosis as they enlarge.
Teratoma
This rare orbital tumor, which is composedof all 3 germ layers, is typically present at birth but may notbecome evident until later in childhood.
Neurofibroma
Peripheral nerve tumor that may involve intraorbitalnerves and lids, causing proptosis or ptosis.
Optic Nerve Glioma
This optic nerve tumor produces proptosisand visual loss. May be associated with neurofibromatosis.
Meningioma
Primary orbital meningioma may involve opticnerve sheath or any part of the orbit. Also may invade orbit throughbone or along optic canal from intracranial site.
Osteoma
Some eye displacement may be seen with thisbenign bone-forming tumor, which usually arises from paranasal sinuses.Radiographs show dense well-defined lesion.
Ossifying Fibroma
This benign expanding lesion may involveorbit or paranasal sinuses.
Malignant Tumors
Rhabdomyosarcoma
Most common malignant tumor arising in theorbit in childhood. Rapid enlargement of mass is often accompaniedby downward displacement of globe.
Retinoblastoma
Can extend from eye into orbit, producingproptosis. Detailed ophthalmoscopic exam is essential. See furtherdiscussion in Chap. 74, VisionDisturbances.
Lymphoma/Leukemia
Lymphoma or leukemic infiltrates may resultin orbital mass and proptosis.
Metastatic Neuroblastoma
Involvement of orbits is secondary to metastaticspread from primary tumor in abdomen, mediastinum, or neck. Continuedgrowth of orbital mass produces proptosis that may be accompaniedby periorbital ecchymoses.
Histiocytoses
Histiocytic infiltration of orbit can occur.Soft tissue mass associated with smooth margin lytic bone defectsuggests this diagnosis, which is confirmed by biopsy.
Vascular Lesions
Orbital Varices
Dilatationsof ≥1 orbital veins.History of visible varices or markedincrease in proptosis with coughing, crying, or lying in Trendelenburgposition is presumptive evidence of orbital varices.CT is diagnostic. Orbital Aneurysm
Producespulsating mass lesion within the orbit, which can cause proptosis.Although magnetic resonance imaging(MRI)/magnetic resonance angiography (MRA) may be diagnostic,conventional angiography is definitive. Carotid Cavernous Fistula
Orbitaltrauma or basilar skull fracture with rupture of carotid arteryinto cavernous sinus may produce carotid cavernous fistula.Proptosis and pulsation within theorbit are usual findings. Roaring intracranial noise and tortuousenlarged conjunctival vessels are also common. Digital compressionof ipsilateral carotid artery reduces noise.MRI/MRA may be diagnostic,but conventional angiography is definitive. Cavernous Sinus Thrombosis
Risk factorsinclude head trauma, dehydration, septicemia, head and neck infections, cardiacdisease, prothrombotic disorders, malignancy, and procoagulant drugs.Clinical manifestations include proptosis,eyelid edema, impaired movement of extraocular muscles, and headache.CT and MRI are diagnostic, but specificcause must be investigated. Bone Disorders
Fibrous Dysplasia
Normal medullarybone is replaced by fibrous tissue with varying amounts of mineralization.Involvement of bones surrounding orbit may produce proptosis.Although plain radiographs may be diagnostic,CT is usually performed to define extent of lesion. Diagnostic Approach
Proptosisis usually obvious clinically. When presence of proptosis is uncertain,distance from lateral orbital margin to cornea should be measuredwith special instrument called exophthalmometer. Distance is usually <20mm in normal individuals. Difference of >2 mm between eacheye is significant.History and physical exam are diagnosticof trauma and orbital cellulitis. In any child with orbital cellulitis,CT should be performed to determine if orbital abscess is present.Skull radiographs are useful in diagnosisof craniosynostosis and fibrous dysplasia.Plain radiographs of orbit, skull,and optic foramina; CT; and MRI are useful in locating and definingextent of mass lesions. If mass is palpable and nonpulsating, tissuemust be obtained for definitive diagnosis, except perhaps in thecase of visible hemangioma.MRI/MRA can diagnose orbitalaneurysm or carotid cavernous fistula, but conventional angiographyis definitive. References
- Ball WS Jr, ed. Pediatric neuroradiology.Philadelphia: Lippincott-Raven, 1997.
- Nelson LB, ed. Harley's pediatric ophthalmology,4th ed. Philadelphia: WB Saunders, 1998.
- Rudolph AM, ed. Rudolph's pediatrics, 20thed. Stamford, CT: Appleton & Lange, 1996.
- Sindhu K, et al. Aetiology of childhood proptosis.J Paediatr Child Health 1998;34:374–376.
- Swaiman KF, Ashwal S, eds. Pediatric neurology: principlesand practice, 3rd ed. St. Louis: CV Mosby, 1999.
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Book Source Details
- Book Title: The Diagnostic Approach to Symptoms and Signs in Pediatrics
- Author(s): Paul S. Bellet
- Year of Publication: 2006
- Copyright Details: The Diagnostic Approach to Symptoms and Signs in Pediatrics, Copyright © 2006 Lippincott Williams & Wilkins.
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Copyright Details: The Diagnostic Approach to Symptoms and Signs in Pediatrics, Copyright © 2008 Williams & Wilkins.
More About Causes of Bulging eyes
» Next page: Exophthalmos [Proptosis] (Nursing: Interpreting Signs and Symptoms)
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