Globe Retraction

Globe retraction occurs when the globe is displaced deeper within the orbit from its normal position.

There are many causes of globe retraction. It may result from active co-contraction of the horizontal rectus muscles such as in Duane retraction syndrome. Patients with Duane syndrome have strabismus, upshooting or downshooting eye movements, narrowing of palpebral fissure, and retraction of the globe on adduction.[1, 2] Enlargement of the orbital cavity after orbital blowout fractures from trauma also may cause the globe to be retracted.[3, 4, 5] This may result from prolapse of orbital contents into the adjacent paranasal sinuses, atrophy of orbital fat, or contracture of necrotic extraocular muscles entrapped within the fracture. Globe retraction also can be seen in metastatic scirrhous breast carcinoma from cicatrization of orbital tissue.[6, 7, 8, 9] Although less common, some cases of the sclerosing variant of idiopathic orbital inflammation (pseudotumor) have been reported to cause globe retraction.[10, 11]

A thorough history and examination are required to determine appropriate management for patients with globe retraction.

Mechanism for globe retraction in Duane retraction syndrome is believed to be anomalous innervation of lateral rectus muscles from branches of oculomotor nerve (cranial nerve III).[2] Both electromyographic and autopsy studies in Duane syndrome patients have demonstrated this anomalous innervation. In attempted adduction, simultaneous contractions of lateral and medial rectus muscles cause the globe to retract.[12] Anomalous innervation between medial rectus and vertical rectus or oblique muscles also may explain upshoots and downshoots seen in adduction.

Traumatic blowout fractures typically occur when a large blunt object strikes eyelids and globe. The impact of the force causes retropulsion of orbital contents with an increase in intraorbital pressure. This results in fracture of the orbital floor and/or the medial wall.[3] Blowout fracture along with compression of air in the paranasal sinuses partially absorbs force of impact and prevents rupture of globe. Globe retraction results from either enlargement of orbital cavity after blowout fracture or prolapse of orbital tissue into adjacent sinus. Orbital fat atrophy or contraction of an entrapped extraocular muscle also can cause globe retraction after orbital blowout fractures.

Incidence of globe retraction in metastatic orbital tumors has been reported to be 10-25%.[13] Most common orbital metastasis to cause globe retraction is scirrhous breast carcinoma (82%), although it also has been reported with lung, gastrointestinal, and prostate carcinomas. The cause of globe retraction is cicatrization with contraction of myofibroblasts in orbital tissue.[7]

A similar mechanism can cause globe retraction in sclerosing idiopathic orbital inflammation (pseudotumor). Immunohistologically, sclerosing orbital pseudotumor resembles idiopathic retroperitoneal and idiopathic mediastinal fibrosis; several authors have suggested common pathophysiology.[10, 11]

Frequency

United States

In the general population, prevalence of Duane retraction syndrome is 0.1%.[1, 2] It accounts for approximately 1% of all strabismus cases. Commonly, left eye more often is involved in Duane syndrome (OS:OD is 3:1); 20% of cases are bilateral.[14]

Eye injuries account for approximately 100,000 visits to physicians annually. In the National Basketball Association (NBA) eye injury study, eye injuries accounted for 5.4% of all injuries and included orbital fractures. Orbital fractures commonly result from motor vehicle accidents, interpersonal violence, and sports-related injuries. Baseball, basketball, ice hockey, and racquet sports are considered high-risk sports.[3, 4]

Metastatic tumors of the orbit account for approximately 1-13% of all orbital masses.[7, 13] Metastasis of breast carcinoma to the orbit accounts for approximately 50% of orbital metastases. Prostate and lung carcinoma follow in frequency accounting for approximately 17% and 6%, respectively.

Mortality/Morbidity

Duane retraction syndrome: Incidence of amblyopia is similar to that in the general population. Binocularity often can be maintained with abnormal head position. Indications for intervention include cosmetically unacceptable strabismus in primary gaze, anomalous head position, retraction of globe, or large upshoot/downshoot eye movements. Duane syndrome has been reported to be associated with some systemic anomalies, including Goldenhar syndrome, Klippel-Feil syndrome, cervical spina bifida, and other facial and limb abnormalities.

Orbital blowout fracture: Diplopia immediately after suffering a blowout fracture is common; 20% of patients will have persistent diplopia if no surgical intervention is performed. Infraorbital nerve hyperesthesia can be present after blowout fractures of the globe, although symptoms typically improve with time. Enophthalmos greater than 3 mm occurs in approximately 20% patients.

Orbital metastases: Strabismus with diplopia is the most common finding in orbital metastases. Approximately 74% of patients present with a known primary tumor, in the remaining 26% no primary tumor is ever found despite thorough evaluation in 50% of cases. From the time of diagnosis of orbital metastasis, mean survival time is 13 months. Survival time after diagnosis is longer in breast carcinoma compared with prostate and lung carcinoma.

Sex

Duane retraction syndrome is slightly more common in females (54%) than in males (46%).[2]

Men are more than twice as likely to experience orbital trauma than women from most causes. The exceptions are domestic violence and sexual assault; almost all cases of orbital fractures in these situations occur in women.

Incidence of orbital metastasis from all tumor types is equal between men and women. Although men can develop breast carcinoma, there are no reports of orbital metastases of breast cancer in men.

Age

Duane retraction syndrome is a congenital condition. However, diagnosis often is delayed because of difficulty of eliciting full range of eye movements in infants.

Orbital trauma from almost all causes typically occurs in children and young adults.

Average age at the time of diagnosis of orbital metastases for breast and lung carcinoma is approximately 60 years.

Average age at the time of diagnosis of metastatic prostate carcinoma is 70 years.

Silent sinus syndrome can occur at any age.

The prognosis depends on the etiology.

Patients should be informed of the differential diagnoses and treatment plan.

Treatment should be directed at the underlying etiology.

Obtain a complete history, including age at onset of signs and symptoms, duration of symptoms, progression or improvement of symptoms, rapidity of progression, and review of systems for other medical conditions, as follows:

• Acute versus chronic

• Mechanism of injury - Fist, ball, motor vehicle accident, metallic foreign body

• Past ocular and medical history - Previous ocular or orbital trauma, previous eye surgery, other ocular conditions (eg, amblyopia, glaucoma, retinal detachment), family history of cancer, known primary tumor, previous surgeries (eg, biopsies or urogenital tract procedures), history of radiotherapy to orbit

Ophthalmic symptoms

Ophthalmic symptoms may include the following:

• Diplopia - Strabismus, globe displacement, restriction of extraocular movements, traumatic optic nerve or ocular motor nerve palsy, floor fracture, convergence retraction nystagmus

• Narrowing of palpebral fissure - Ptosis (ie, mechanical, neurogenic, myogenic), narrowing during adduction, pseudoptosis (globe retraction/enophthalmos), Duane syndrome

• Pain - Hemorrhage and edema, perineural invasion, or orbital tumor

• Decreased vision - Optic nerve compression, traumatic optic neuropathy, concurrent ocular injury, amblyopia

• Anomalous head position - Stereopsis, binocularity

Complete ophthalmologic examination (defer if obvious globe rupture)

A complete ophthalmologic examination includes the following:

• Vision and pupils - Amblyopia, ocular trauma, traumatic optic neuropathy, relative afferent pupillary defect, anisocoria

• Extraocular motility and alignment - Traumatic ocular motor cranial nerve palsy, entrapment of extraocular muscles, pain with extraocular movements, severity of strabismus, lack of abduction (Duane type I), lack of adduction (Duane type II), lack of both adduction and abduction (Duane type III), presence of upshoots or downshoots in adduction, multiple motility deficits (posterior traction and tethering of globe in scirrhous breast carcinoma)

• External examination - Infraorbital anesthesia, enophthalmos of globe (Hertel exophthalmopathy), globe ptosis, narrowing of palpebral fissure with adduction, blepharoptosis or pseudoptosis, deep superior sulcus, preauricular or submandibular adenopathy, restrictive enophthalmos (eg, scirrhous breast carcinoma), pseudoenophthalmos due to contralateral exophthalmos

• Intraocular pressure -Angle-recession glaucoma, secondary glaucoma from orbital mass

• Anterior segment - Evidence of previous trauma (eg, corneal scar, angle recession, corectopia, phacodonesis, iridodonesis, lens rupture, lens subluxation)

• Posterior segment - Retinal detachment, chorioretinal scarring, hemorrhage, optic nerve pallor, optic nerve avulsion

Duane retraction syndrome is a congenital condition that is believed to be due to aberrant innervation of extraocular muscles.

Abnormal synergistic innervation between medial and lateral rectus muscles causes co-contraction of 2 muscles resulting in globe retraction during attempted adduction.

Abnormal synergistic innervation between medial and vertical rectus muscles may explain upshooting and downshooting eye movements.

Blunt trauma is the most common cause of orbital blowout fractures.

Iatrogenic causes such as prior orbital decompression surgery or sinus surgery may cause enophthalmos.

Risk factors for breast cancer include the following:

• Increasing age

• Family history - Especially first- and second-degree relatives with premenopausal cancer (including paternal relatives)

• Early menstruation/late menopause

• Nulliparity

• Premalignant breast lesions or previous carcinoma in 1 breast (especially premenopausal)

• Obesity

• History of previous radiation therapy to chest

Physical examination should assess for the following:

• Evidence of trauma elsewhere
• Other cranial neuropathies
• Evidence of prior breast carcinoma or metastatic disease

Complications may result from the following:

• Delayed diagnosis
• Inadequate imaging
• Surgical complications

The following diagnostic tools may be used:

• Orbital imaging (CT scanning or MRI)
• Skull radiography may show the fracture, but CT scanning is superior.
• Orbital ultrasonography for tumor or pseudotumor of orbit
• Metastatic evaluation, if appropriate

Laboratory studies include the following:

• Complete blood count - If surgery is contemplated

• Electrolytes - If surgery is contemplated

• Liver function studies - For possible liver metastasis

• Carcinoembryonic antigen or CA-15-3 - If breast carcinoma is suspected

• Prostrate specific antigen - If prostate carcinoma is suspected

CT scanning or MRI of orbits consist of 3-mm cuts, axial and coronal views. CT is better for evaluating the bony structure. MRI is better for evaluating the orbital contents and soft tissues. Evaluate fractures. Assess potential extraocular muscle entrapment. These studies may reveal the presence of orbital mass.

Chest radiography is performed if lung nodules suggest lung carcinoma. It may identify any suspicious breast lesions.

CT scan of neck/thorax/abdomen may be used to evaluate for systemic malignancy.

B-scan ultrasonography may be performed upon any doubt of globe integrity.

Orbital biopsy is indicated when orbital mass is present and no known systemic malignancy is identified.

Often in metastatic scirrhous tumors, needle biopsy can result in minimal or no tissue retrieval. If biopsy is required for identification of an orbital mass, open biopsy with microscopical and histochemical evaluation should be performed. This also allows for identification of specific hormonal receptors that may alter ultimate chemotherapeutic regimen.

Treatment should be directed at the underlying etiology.

Duane retraction syndrome[1, 2, 14, 15]

Correct refractive errors and treat amblyopia if present.

Patient can be kept under observation if there is no significant strabismus in primary gaze, anomalous head position, cosmetically unacceptable upshoots/downshoots, or globe retraction.

Orbital blowout fracture[3, 4, 5]

Patients should avoid nose blowing.

Oral antibiotics and nasal decongestants should be prescribed.

Cold compresses can be used to reduce eyelid edema to facilitate follow-up examinations.

Metastatic carcinoma[6, 7, 13, 8]

Patients should undergo appropriate systemic screening to identify primary tumor.

Orbital radiotherapy and/or systemic chemotherapy should be considered.

Goals for surgery in Duane retraction syndrome include correction of ocular alignment in primary gaze, elimination of any abnormal head position, reduction of magnitude of upshoots/downshoots, and globe retraction, as follows[1] :

• Recession of ipsilateral medial rectus in type I when esotropia is less than 30 prism diopters

• Bilateral medial rectus recession in type I when esotropia is greater than 30 prism diopters

• Recession of ipsilateral lateral rectus muscle in type II

• Never perform rectus resection because this is likely to increase globe retraction.

• Recession of both horizontal recti to reduce globe retraction

• Splitting of lateral rectus in a Y configuration or using posterior fixation sutures on the lateral rectus may reduce upshoots.

Orbital blowout fractures should be repaired if there is obvious entrapment of an extraocular muscle, no improvement in diplopia, or unacceptable enophthalmos.[4] Autogenous or allogenic bone, demineralized bone, hydroxyapatite, and synthetic implants (eg, Teflon, Silastic, Supramid, porous polyethylene) may be used to repair the fracture. Surgical management of orbital and periorbital trauma may require cooperative efforts from otolaryngology and neurosurgery.

Consultations include the following:

• Strabismus surgeon for extraocular muscle surgery in Duane retraction syndrome

• Orbital surgeon for repair of blowout fractures

• Oncologist and radiation oncologist for management of metastatic carcinoma

Patients with orbital blowout fractures should have limited activity.

Avoidance of Valsalva maneuver and blowing of nose is crucial to reduce probability of developing orbital emphysema.

The goals of pharmacotherapy are to reduce morbidity and to prevent complications.[4]

Class Summary

To prevent development of orbital cellulitis after blowout fractures.

Cephalexin (Keflex, Biocef, Keftab)

First-generation cephalosporin that arrests bacterial growth by inhibiting bacterial cell wall synthesis.

Class Summary

Promote nasal airflow and reduce likelihood of orbital emphysema.

Oxymetazoline (Dristan, Afrin, Chlorphed, Neo-synephrine)

Applied directly to mucous membranes where stimulates alpha-adrenergic receptors and cause vasoconstriction. Decongestion occurs without drastic changes in blood pressure, vascular redistribution or cardiac stimulation.

Patients with Duane syndrome should be observed for development/worsening of strabismus, anomalous head position, or amblyopia.

Patients with orbital blowout fractures should be observed for persistent diplopia and development of enophthalmos. Some patients may benefit from medical therapy in the acute setting (antibiotics, nasal decongestants, steroids). Patients with severe enophthalmos may require reconstructive orbital surgery.

Patients with metastatic tumors of orbit treated with radiation therapy should be observed for development of keratitis, dry eye, cataract, or radiation retinopathy. All patients with orbital metastases should be monitored to assess for evidence of recurrence and evaluated and treated systemically by an oncologist (eg, chemotherapy, radiation therapy, surgery).

Silent sinus syndrome may respond to medical therapy or may require surgery.

Protective eyewear should be worn during participation in high-risk activities.

For excellent patient education resources, visit eMedicineHealth's Eye and Vision Center. Also, see eMedicineHealth's patient education article Eye Injuries.