eMedicine Specialties > Ophthalmology > Extraocular Muscles

Globe Retraction

Michael T Yen, MD, Associate Professor of Ophthalmology, Department of Ophthalmology, Division of Ophthalmic Plastic, Lacrimal, and Orbital Surgery, Cullen Eye Institute, Baylor College of Medicine

Updated: Feb 13, 2009

Introduction

Background

Globe retraction occurs when the globe is displaced deeper within the orbit from its normal position. It may occur 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.^1,2,3

Enlargement of the orbital cavity after orbital blowout fractures also may cause the globe to be retracted.^4,5,6 It may be due to prolapse of orbital contents into adjacent 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 sclerosing orbital 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.

Pathophysiology

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 orbital pseudotumor. Immunohistologically, sclerosing orbital pseudotumor resembles idiopathic retroperitoneal and idiopathic mediastinal fibrosis; several authors have suggested common pathophysiology.¹¹

Patient with metastatic breast carcinoma to the intraconal space of the right orbit resulting in mild globe retraction and enophthalmos.

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.^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.

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.

• 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.

Clinical

History

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.

• 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
  • Diplopia - Strabismus, globe displacement, restriction of extraocular movements, traumatic nerve palsy
  • Narrowing of palpebral fissure - Ptosis (ie, mechanical, neurogenic, myogenic), narrowing during adduction, pseudoptosis (globe retraction/enophthalmos)
  • 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

Physical

• Complete ophthalmologic examination (defer if obvious globe rupture)
  • Vision and pupils - Amblyopia, ocular trauma, traumatic optic neuropathy
  • Extraocular motility and alignment - Traumatic 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
  • 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

Causes

• 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)
  • Obesity
  • History of previous radiation therapy to chest

Differential Diagnoses

Other Problems to Be Considered

Exophthalmos of contralateral globe
Orbital emphysema
Retrobulbar hemorrhage
Ruptured globe
Systemic malignancy
Traumatic optic neuropathy

Workup

Laboratory Studies

• 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

Imaging Studies

• CT scan of orbits - 3 mm cuts, axial and coronal views
  • Evaluate fractures.
  • Assess potential extraocular muscle entrapment.
  • Presence of orbital mass
• Chest x-ray
  • Lung nodules suggestive of lung carcinoma
  • May identify suspicious breast lesion
• CT scan of neck/thorax/abdomen - To evaluate for systemic malignancy
• B-scan ultrasonography - If any doubt of globe integrity

Procedures

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

Histologic Findings

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

Medical Care

• 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^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.

Surgical Care

• 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.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.

Consultations

• 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

Activity

• 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.

Medication

The goals of pharmacotherapy are to reduce morbidity and to prevent complications.⁵

Antibiotics

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.

Dosing

Adult

250-500 mg PO q6h

Pediatric

25-50 mg/kg/d PO in divided doses

Interactions

Coadministration with aminoglycosides increase nephrotoxic potential

Contraindications

Documented hypersensitivity

Precautions

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Adjust dose in severe renal insufficiency (high doses may cause CNS toxicity); superinfections, and promotion of non-susceptible organisms may occur with prolonged use or repeated therapy

Nasal decongestants

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.

Dosing

Adult

2 puffs in nostril not more often than q6h

Pediatric

<6 years: Not recommended
>6 years: Administer as in adults

Interactions

Hypotensive action of guanethidine may be reversed; concurrent administration with methyldopa may result in an increased vasopressor response; concurrent use of MAOIs and ephedrine may result in hypertensive crisis; pressor sensitivity to mixed-acting agents such as ephedrine may be increased; guanethidine potentiates effects of epinephrine and inhibits effects of ephedrine; phenothiazines may reverse action of nasal decongestants such as oxymetazoline; TCAs potentiate vasopressor response and may result in dysrhythmias

Contraindications

Documented hypersensitivity; MAO inhibitor therapy; heart disease; high blood pressure; thyroid disease; diabetes; prostate enlargement

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Caution in hyperthyroidism, coronary artery and ischemic heart disease, diabetes mellitus, increased intraocular pressure or prostatic hypertrophy; because of increase in vasoconstriction, hypertensive patients may experience change in blood pressure; do not use topical decongestants for longer than 3 to 5 d

Follow-up

Further Outpatient Care

• 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.
• 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 recurrences.

Deterrence/Prevention

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

Patient Education

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

Miscellaneous

Medicolegal Pitfalls

• Approximately 26% of patients with orbital metastases present with no known history of cancer. Misdiagnosis could lead to delay in appropriate treatment with claims of liability against the ophthalmologist.

Special Concerns

• Children with orbital floor fractures often present with entrapment of inferior rectus. They should undergo surgical repair within 1 week of initial trauma.

Multimedia

Media file 1: 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.

Media file 2: Patient with metastatic breast carcinoma to the intraconal space of the right orbit resulting in mild globe retraction and enophthalmos.

References

1. Britt MT, Velez FG, Thacker N, Alcorn D, Foster RS, Rosenbaum AL. Surgical management of severe cocontraction, globe retraction, and pseudo-ptosis in Duane syndrome. J AAPOS. 2004;8:362-367. [Medline].

2. Chua B, Johnson K, Donaldson C, Martin F. Management of Duane retraction syndrome. J Pediatr Ophthalmol Strabismus. 2005;42:13-17. [Medline].

3. Athanasiov PA, Prabhakaran VC, Selva D. Non-traumatic enophthalmos: a review. Acta Ophthalmol. Jun 2008;86(4):356-64. [Medline].

4. Cepela MA, George CE. Orbital trauma. Curr Opin Ophthalmol. Oct 1997;8(5):64-9. [Medline].

5. Putterman AL, Smith BC, Lisman RD. Blowout fractures. In: Nesi FA, et al, eds. Smith's Ophthalmic Plastic and Reconstructive Surgery. 2nd ed. 1998:209-23.

6. Remulla HD, Bilyk JR, Rubin PA. Pseudo-entrapment of extraocular muscles in patients with orbital fractures. J Craniomaxillofac Trauma. 1995;1:16-29. [Medline].

7. Goldberg RA, Rootman J. Clinical characteristics of metastatic orbital tumors. Ophthalmology. May 1990;97(5):620-4. [Medline].

8. Rootman J, Ragaz J, Cline R, et al. Tumors: Orbital metastasis. In: Rootman J, ed. Diseases of the Orbit. 1988:405-26.

9. Tijl J, Koornneef L, Eijpe A, et al. Metastatic tumors to the orbit--management and prognosis. Graefes Arch Clin Exp Ophthalmol. 1992;230(6):527-30. [Medline].

10. Alsuhaibani AH, Carter KD, Nerad JA, Lee AG. Prostate carcinoma metastasis to extraocular muscles. Ophthal Plast Reconstr Surg. May-Jun 2008;24(3):233-5. [Medline].

11. Mombaerts I, Goldschmeding R, Schlingemann RO, Koornneef L. What is orbital pseudotumor?. Surv Ophthalmol. Jul-Aug 1996;41(1):66-78. [Medline].

12. Strachan IM, Brown BH. Electromyography of extraocular muscles in Duane''s syndrome. Br J Ophthalmol. Aug 1972;56(8):594-9. [Medline].

13. Shields CL, Shields JA. Metastatic tumors to the orbit. Int Ophthalmol Clin. Summer 1993;33(3):189-202. [Medline].

14. Khan AO, Aldamesh M. Bilateral Duane syndrome and bilateral aniridia. J AAPOS. Jun 2006;10(3):273-4. [Medline].

15. Oohira A, Masuzawa K. A case of congenital oblique retraction syndrome with upshoot in adduction. Strabismus. 2002;10:39-44. [Medline].

Keywords

globe retraction, enophthalmos, diplopia, orbital blowout fracture, orbital fracture, orbital trauma, ocular trauma, eye trauma, eye injury, sports injury, retracted globe, Duane retraction syndrome, Duane syndrome, amblyopia, orbital tumor, orbital metastasis, breast cancer, breast carcinoma, lung cancer, lung carcinoma, prostate cancer, prostate carcinoma, metastatic carcinoma

Contributor Information and Disclosures

Author

Michael T Yen, MD, Associate Professor of Ophthalmology, Department of Ophthalmology, Division of Ophthalmic Plastic, Lacrimal, and Orbital Surgery, Cullen Eye Institute, Baylor College of Medicine
Michael T Yen, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Cataract and Refractive Surgery, American Society of Ophthalmic Plastic and Reconstructive Surgery, and Association for Research in Vision and Ophthalmology
Disclosure: Nothing to disclose.

Medical Editor

Gerhard W Cibis, MD, Clinical Professor, Director of Pediatric Ophthalmology Service, Department of Ophthalmology, University of Kansas, Kansas City
Gerhard W Cibis, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, and American Ophthalmological Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

J James Rowsey, MD, Former Director of Corneal Services, St Luke's Cataract and Laser Institute, Florida
J James Rowsey, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for the Advancement of Science, American Medical Association, Association for Research in Vision and Ophthalmology, Florida Medical Association, Pan-American Association of Ophthalmology, Sigma Xi, and Southern Medical Association
Disclosure: Nothing to disclose.

CME Editor

Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri
Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

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