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 also may cause the globe to be retracted.[4, 5, 6] 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.[7, 8, 9, 10] Although less common, some cases of the sclerosing variant of idiopathic orbital inflammation (pseudotumor) have been reported to cause globe retraction.[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.
Blowout fractures typically occur when a large blunt object strikes eyelids and globe. Impact of 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.[4] 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.[8]
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.[11]
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Patient with metastatic breast carcinoma to the intraconal space of the right orbit resulting in mild globe retraction and enophthalmos.
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.[4, 5]
Metastatic tumors of the orbit account for approximately 1-13% of all orbital masses.[8, 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.
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Patient presented with persistent diplopia after an interpersonal altercation. Forced ductions revealed tight inferior and medial rectus muscles on ri....
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; exception being domestic violence and sexual assault where almost all cases of orbital fractures 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.
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
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
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 orbital decompression 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)
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.
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.
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[4, 5, 6]
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[7, 8, 13, 9]
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.[5] 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.
Clinical Context:
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.
Michael T Yen, MD, Professor of Ophthalmology, Division of Ophthalmic Plastic, Lacrimal, and Orbital Surgery, Cullen Eye Institute, Medical Director, Alkek Eye Center, Co-Director, BCM Aesthetics, Program Director, ASOPRS Fellowship, Baylor College of Medicine
Disclosure: Nothing to disclose.
Specialty Editors
Francisco Talavera, PharmD, PhD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference
Disclosure: Received salary from Medscape for employment. for: Medscape.
J James Rowsey, MD, Former Director of Corneal Services, St Luke's Cataract and Laser Institute
Disclosure: Nothing to disclose.
Chief Editor
Andrew G Lee, MD, Chair, Department of Ophthalmology, Blanton Eye Institute, Houston Methodist Hospital; Clinical Professor, Associate Program Director, Department of Ophthalmology and Visual Sciences, University of Texas Medical Branch School of Medicine; Clinical Professor, Department of Surgery, Division of Head and Neck Surgery, University of Texas MD Anderson Cancer Center; Professor of Ophthalmology, Neurology, and Neurological Surgery, Weill Medical College of Cornell University; Clinical Associate Professor, University of Buffalo, State University of New York School of Medicine
Disclosure: Received ownership interest from Credential Protection for other.
Additional Contributors
Gerhard W Cibis, MD, Clinical Professor, Director of Pediatric Ophthalmology Service, Department of Ophthalmology, University of Kansas School of Medicine
Patient with metastatic breast carcinoma to the intraconal space of the right orbit resulting in mild globe retraction and enophthalmos.
Patient presented with persistent diplopia after an interpersonal altercation. Forced ductions revealed tight inferior and medial rectus muscles on right side. CT scan revealed orbital floor and medial wall fractures in right orbit.
Patient presented with persistent diplopia after an interpersonal altercation. Forced ductions revealed tight inferior and medial rectus muscles on right side. CT scan revealed orbital floor and medial wall fractures in right orbit.
Patient with metastatic breast carcinoma to the intraconal space of the right orbit resulting in mild globe retraction and enophthalmos.
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