History and Examination for Orbital Pathology

A comprehensive history and examination are particularly important in the context of ordering imaging studies to accurately decide on, for example, the modality, need for contrast, and frequency of reimaging.

History

Chief complaint

• Decreased vision
• Double vision
• Pain/anesthesia/paresthesia
• Pain is typically associated with infection or inflammation
• Dysesthesia, paresthesia or hypesthesia can be associated with specific tumor types, suggestive of sensory nerve involvement
• Inflammatory disorders
• Idiopathic orbital inflammation
• Pain with extraocular muscle movement
• Inflammatory response in muscle and tendon
• Tolosa Hunt syndrome
• “Painful ophthalmoplegia”
• Inflammation surrounding the orbital apex
• Granulomatosis with polyangiitis
• Chronic acute and subacute pain — an important clue to the diagnosis
• Necrotizing granulomatous vasculitis
• Optic neuritis: Pain with EOM movements, marked decrease in visual acuity
• Ruptured dermoid: Acute or chronic pain associated with inflammation
• Infection
• Orbital abscess, cellulitis, cavernous sinus thrombosis — usually systemically ill with malaise, fever and leukocytosis
• Herpes zoster — varicella zoster virus
• History of chicken pox
• Pain prodrome along ophthalmic branch of CN V
• Orbital hemorrhage
• Soft tissue injury
• Orbital fracture
• Associated facial and systemic injury
• Vascular lesion
• Lymphangiomas — pain associated with hemorrhage
• Malignancy
• Adenoid cystic carcinoma of the lacrimal gland causing pain or paresthesia of several months duration
• Metastatic carcinoma can be associated with rapid onset of inflammatory conditions
• Rhabdomyosarcoma can mimic an inflammatory or infectious condition, but generally does not cause severe pain
• Squamous cell carcinoma — pain or numbness at forehead associated with supraorbital nerve involvement

History of present illness

• Age and sex of patient
• Onset, quality and duration of symptoms
• Immediate: minutes to hours
• Varix (upon Valsalva) — worse with head-down position
• Hemorrhage
• Lymphangioma — exacerbated by upper respiratory infection or other states of high blood flow
• Rapid — days to weeks
• Children
• Capillary hemangioma
• Rhabdomyosarcoma
• Retinoblastoma
• Neuroblastoma
• Leukemia
• Inflammatory disease
• Idiopathic orbital inflammatory disease
• Thyroid eye disease (thyroid orbitopathy)
• Ruptured, inflamed dermoid
• Infection
• Orbital cellulitis
• Abscess
• Cavernous sinus thrombosis
• Trauma
• Postsurgical hemorrhage
• Orbital hemorrhage
• Malignancy
• Rhabdomyosarcoma
• Metastatic tumors
• Adenoid cystic carcinoma
• Vascular
• Carotid-cavernous (C-C) fistula
• Lymphangioma
• Months to years
• Dermoid cysts
• Benign mixed tumors
• Neurogenic tumors
• Cavernous venous malformation
• Lymphoma
• Carotid-cavernous fistula
• Causes for exacerbation or improvement
• Associated changes in vision — gaze-evoked amaurosis
• Pain and progression

Past medical history (PMH)

• General medical diseases, diabetes, hypertension, thyroid abnormalities, cancers, trauma
• Eye diseases

Current medications/allergies

• Anticoagulant management perioperatively

Family history

• Systemic diseases
• Craniofacial anomalies
• Genetic diseases

Review of systems (ROS)

• Neurological
• Cardiovascular
• Pulmonary
• Renal
• Connective tissue

Physical examination

General medical examination

Eye examination

• Visual acuity, uncorrected and corrected — induced hyperopia might suggest retrobulbar mass
• Pupils
• Afferent pupillary defect
• Optic Neuropathy
• Compression
• Ischemia
• Extraocular muscle motility
• Ophthalmoplegia
• Forced duction testing may be performed to distinguish restrictive vs. paretic type
• Causes
• Strabismus
• Noncomitant motility disturbance
• Orbital apex syndrome
• Paralysis of cranial nerves (CN) III, IV, and VI
• Injury
• Tumor
• Orbital fracture
• Hemorrhage
• Infection
• External soft tissue examination
• Periorbital changes
• Erythema
• Edema
• Ecchymosis
• Lid ptosis, retraction
• Craniofacial defects
• Exorbitism
• Hypertelorism
• Telecanthus
• Sulcus defect
• Enophthalmos, microphthalmos
• Fat atrophy
• Trauma volume loss (blowout orbital floor fracture)
• Silent sinus syndrome
• Age-related ptosis
• Silent brain syndrome
• Nasal and sinus tenderness, discharge, mass
• Valsalva — increase in proptosis, varix
• CN palsy
• Acoustic neuroma
• Mass
• Trauma
• Eyelid malformations
• Capillary hemangioma
• Neurofibroma/S-shaped lid deformity
• Vascular findings
• Lymphangioma
• Capillary hemangioma
• Hemorrhage
• Ecchymosis (neuroblastoma)
• Varix
• Auscultation for bruits
• Skin changes
• Café au lait spots
• Juvenile xanthogranuloma (JXG)
• Xanthelasma
• Pigment changes (melanoma nevus)
• Hutchinson sign (Herpes Zoster)
• Involvement of nasociliary nerve seen by lesion on nasal tip
• Suggests corneal involvement
• Exophthalmometry
• Normal distance between corneal apex and lateral orbital rim is less than 20 mm
• Identify asymmetry between eyes — a difference of more than 2 mm between eyes is abnormal
• Normal Hertel measurements are higher for African Americans and lower for Asian Americans
• Palpation of lesion characteristics
• Firm, soft, rubbery
• Irregular borders, well-circumscribed
• Tender, painless
• Fixed to deep tissues/bone, mobile
• Compressible, fluctuant
• Expands with Valsalva
• Resistance to retropulsion
• Visual fields
• Central scotoma
• Constriction
• Altitudinal defects
• Slit-lamp examination
• Exposure keratopathy from lagophthalmos, proptosis, or paralysis
• Chemosis, injection, discharge
• Hemorrhage, dilated vessels
• Consider gonioscopy to look for blood in Schlemm’s canal
• Intraocular pressure
• Increased
• Higher in upgaze in thyroid eye disease
• Fundus
• Optociliary shunt vessels
• Optic nerve swelling/pallor
• Choroidal folds suggestive of posterior orbit mass

6 Ps of orbital history and physical examination

• Pain
• Proptosis
• Progression
• Palpation
• Pulsation
• Periocular Changes

Differential diagnosis

Diagnostic laboratory tests and imaging

See magnetic resonance imaging and computed tomography.

If indicated, biopsy or complete excision

Medical or surgical treatment appropriate to diagnosis

Type of proptosis

• Axial displacement
• Retrobulbar intraconal mass
• Tumors
• Symmetric enlargement of extraocular muscles in thyroid eye disease
• Hemorrhage
• Nonaxial displacement: extraconal lesions
• Superior displacement
• Maxillary sinus tumor
• Hemorrhage
• Inferomedial displacement
• Dermoid, epidermoid cyst of orbit
• Lacrimal gland tumor
• Lymphoma
• Cholesterol granuloma
• Inferolateral displacement
• Frontoethmoidal mucoceles
• Encephalocele
• Frontal sinus abscess
• Osteoma
• Frontal sinus carcinoma
• Lateral displacement — subperiosteal abscess or tumor associated with ethmoid sinus
• Unilateral
• Most commonly associated with thyroid eye disease
• Common presentation for most orbital neoplasms
• Abscess, hemorrhage, inflammation, or autoimmune disease
• Bilateral
• Most commonly associated with thyroid eye disease
• Can be seen in
• Pseudotumor
• Metastatic tumors
• Carotid-cavernous (C-C) fistula
• C-C thrombosis
• Systemic conditions, e.g.,
• Leukemia
• Neuroblastoma
• Granulomatosis with Polyangiitis
• Displacement with Valsalva: Suspect varix.
• Mastication proptosis: Dermoid cyst protrudes into temporalis fossa.
• Inferior displacement
  • Schwannoma
  • Solitary Fibrous Tumor

Enophthalmos

• Secondary to
• Sclerosing tumors
• Metastatic breast carcinoma
• Orbital trauma/fractures
• Silent sinus syndrome, silent brain syndrome (hydrocephalus)

Pseudoproptosis

• Large globe (axial myopia)
• Facial asymmetry
• Contralateral enophthalmos
• Eyelid retraction

Clinical correlation of proptosis

Thyroid eye disease is the most common cause of unilateral or bilateral proptosis.

Primary orbital neoplasms are usually unilateral.

Bilateral proptosis is seen in inflammatory conditions, immune processes, or systemic diseases.

Inflammatory disorders

• Thyroid disease
• Most common cause of proptosis
• Extraocular tendon-sparing muscle enlargement
• Idiopathic orbital inflammation (orbital pseudotumor)
• Granulomatosis with Polyangiitis

Infection

• Orbital abscess
• Cellulitis

Vascular

• Orbital hemorrhage
• Lymphangioma (sudden)
• Carotid-cavernous fistula, dural-sinus fistula
• Orbital varices-proptosis with Valsalva

Tumor

• Benign
• Cavernous venous malformation
• Lymphangioma
• Malignant
• Adenoid cystic carcinoma
• Lymphoma
• Glioma
• Contiguous
• Sinus
• Intracranial nasopharynx
• Skin
• Systemic
• Lymphoma
• Leukemia
• Metastatic
• Breast — can cause enophthalmos
• Lung
• Prostate
• Neuroblastoma (sudden eyelid ecchymosis, elevated urinary catecholamines)
• Rhabdomyosarcoma — sudden or rapid onset of proptosis in a child

Clinical correlation of location of a palpable mass

Superonasal quadrant

• Mucocele, mucopyocele, encephalocele
• Neurofibroma
• Dermoid
• Lymphoma
• Orbital abscess
• Rhabdomyosarcoma

Superotemporal quadrant

• Dermoid, epidermoid cyst
• Prolapsed lacrimal gland
• Lacrimal gland tumor
• Lymphoma
• Idiopathic orbital inflammation
• Orbital abscess
• Sphenoid wing meningioma

Inferonasal quadrant

• Dacryocystitis
• Lacrimal sac tumor
• Invasive sinus tumor
• Orbital abscess
• Granulomatosis with Polyangiitis
• Lymphoma

Inferotemporal quadrant

• Invasive sinus tumor
• Hemorrhage
• Displaced zygomatic fracture
• Lymphoma

Intraconal masses

• Typically not palpable if behind globe
• Globe can be firm to retropulsion

Clinical correlation of pulsation in orbit

With bruits/thrills

• Carotid-cavernous fistula
• Orbital arteriovenous fistula
• Dural arteriovenous (AV) fistula

Without bruits

• Meningoencephaloceles
• Neurofibromatosis
• Orbital roof defect

• For the most educated initial diagnosis, consider all aspects of
• History
• Examination
• Patient symptoms
• Ancillary tests
• For follow-up and treatment planning
• Appropriate follow-up depending on presumed diagnosis
• If suspect systemic or metastatic disease: palpation of orbit, thyroid, regional lymph nodes, and abdomen
• If indicated, medical or surgical management; see individual topics for detailed management.
• If indicated for optimal patient care, enlist a multidisciplinary team.
• If clinical behavior appears to be atypical, reassess diagnosis, imaging, and management.

The original description in 1905 by Emil Hertel of a device to measure proptosis had only two mirrors; it was actually never made because it was difficult to see the scales which measure the position of the cornea. (Strabismus 2008; 16:45)

Two additional mirrors were added by the manufacturer, Carl Zeiss, to help superimpose the image of the rulers, which are at the sides of the frames, onto the image of the cornea.

The position of this second set of mirrors dictates whether the patient needs to fixate with the eye being measured. If the second set of mirrors are at the sides of the frame the image of the cornea and the image of the ruler are in the same plane.

Hertel’s device was designed to replace the Birch-Hirschfeld exophthalmometer, described in 1900, which was more expensive and more difficult to manipulate, which in turn replaced the Weiss exophthalmometer, described in the 1890s, which was bulky and cumbersome.

Descriptions of various exophthalmometers date back at least to the mid-1800s.

The Naugle exophthalmometer was introduced in the early 1990’s as an alternative to the Hertel exophthalmometer (Ophthalmic Surg 1992; 23:836).

Naugle’s device is similar in design but it uses the superior and inferior orbital rim as a baseline

There are numerous circumstances, including trauma, tumor and surgery, in which the lateral orbital rim has been displaced and can no longer be used as a reference point.

1. AAO, Basic and Clinical Science Course. Section 7: Orbit, Eyelids, and Lacrimal System, 2013-2014.
2. Rootman, J. Diseases of the Orbit: A Multidisciplinary Approach, Second Edition. Lippincott Williams & Wilkins, Philadelphia PA. 2003; 85-103.
3. Naugle TC, Couvillion JT: A superior and inferior orbital rim-based exophthalmometer. Ophthalmic Surg 1992; 23:836.
4. Simonsz HJ: Historical perspective: the description of Emil Hertel’s exophthalmometer. Strabismus 2008; 16;45.
5. Hertel E: Ein einfaches exophthalmometer. Graefes Arch Ophthalmol 1905; 50:71.
6. Birch-Hirschfeld A. Ein neuer Exophthalmometer. Klin Mbl Augenheilk. 1900; 38:721.
7. Cohn H. Messungen der Prominenz der Augen mittels eines neuen Instru- ments, des Exophthalmometers. Klin Mbl Augenheilk. 1867; 5:339.