AUTHOR AND EDITOR INFORMATION

Section 1 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

INTRODUCTION

Section 2 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Background

Enophthalmos is posterior displacement of the eye. The anterior projection of the eye is most commonly measured relative to the outer edge of the orbit, the orbital rim, but it also may be assessed relative to the frontal and maxillary prominences, or the contralateral eye.

Pathophysiology

Primary enophthalmos indicates a congenital etiology. Some degree of facial asymmetry is common, but congenital relative enophthalmos or ocular retrusion may occur with in utero maldevelopment (eg, plagiocephaly, microphthalmos).

Secondary enophthalmos is due to an acquired change in the volumetric relationship between the rigid bone cavity, the orbit, and its contents (predominantly the orbital fat and the eye). Expansion of the orbital cavity without change in the volume of the orbital contents (ie, a blow out fracture) leads to enophthalmos. Alternatively, scarring contracture of the orbital fat and extraocular muscles may decrease soft tissue volume, making the orbital cavity less full and causing enophthalmos.

Frequency

United States

The occurrence is frequent.

International

Same as in the United States.

Mortality/Morbidity

Enophthalmos greater than 2 mm relative to the contralateral eye creates an observable cosmetic deformity. Depending upon the etiology, other significant morbidity may be associated.

Age

This condition occurs in all ages.

CLINICAL

Section 3 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

History

Progressive sinking in of one or both eyes over any time frame (ranging from minutes to years).

Physical

Bilateral enophthalmos may be difficult to determine without radiographic studies or old photographs for comparison, but unilateral enophthalmos often is obvious comparing one eye with the other. Specific changes include the following:

• Narrowed vertical eyelid fissure (vertical fissure may be widened or normal if associated with downward displacement of the eye, also known as hypoglobus or globe ptosis)
• Superior sulcus deformity (deepened upper eyelid crease)
• Lost fullness of fat bulge in upper and lower eyelids
• Can be associated with hypoglobus (downward displaced eye), usually without significant associated diplopia (double vision)
• Other physical findings depending upon etiology
• • Skin and eyelids
  • • Thinned skin, muscle, fat, or even bone in a linear array may suggest Parry-Romberg syndrome or linear scleroderma.
    • Thickened indurated skin may suggest metastatic scirrhous carcinoma.
    • Blue boggy skin might indicate associated vascular malformation with varix.
  • Fifth nerve function
  • • Decreased function of second division may suggest nerve entrapment in fracture.
    • Decreased function of either of the first 2 divisions may suggest tumor infiltration or cavernous sinus involvement.
  • Exophthalmometry measurement - Important to establish progression or stability
  • Ocular motility - Dysmotility might suggest mass (orbital tumor) or restrictive process (fracture).

Causes

Causes of secondary enophthalmos include the following:

• Postnatal, inadequate, orbital cavity development
• • Bone growth arrest (eg, ionizing radiation for retinoblastoma)
  • Inadequate local tissue stimulation of orbital bone growth
  • • Intraorbital (eg, phthisis bulbi, anophthalmos, fat atrophy in childhood)
    • Extraorbital (eg, maxillary bone growth problems)
• Orbital cavity expansion
• • Outward fracture of orbital bones (frequency of fracture sites - floor > medial wall > lateral wall > roof)
  • Surgical expansion of the orbit (as in thyroid orbitopathy)
  • Silent sinus syndrome, ie, spontaneous, asymptomatic collapse of the maxillary sinus and orbital floor associated with negative sinus pressures
  • Orbital varix with presumed slow bone erosion when the varix fills during recumbent position
• Volumetric loss of orbital contents
• • Orbital fat atrophy
  • • Following concussive trauma
    • Following severe inflammation or infection
    • Following external beam irradiation
    • Associated with wasting disorders (eg, Parry-Romberg hemifacial atrophy, linear scleroderma)
  • Contraction of orbital fat - Scirrhous carcinomas (most commonly metastatic breast, but pulmonary, prostate, and gastrointestinal cancers may cause fat and globe retraction as well)
  • Following surgery (as in resection of a mass lesion associated with local fat atrophy)
  • Phthisis bulbi or prephthisis bulbi
• Pseudoenophthalmos
• • Unilateral blepharoptosis
  • Horner syndrome
  • Contralateral exophthalmos
  • Contralateral pseudoexophthalmos
  • Contralateral high myopia
  • Contralateral buphthalmos or megaloglobus
  • Contralateral eyelid retraction

DIFFERENTIALS

Section 4 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Other Problems to be Considered

Phthisis bulbi
Silent sinus syndrome
Orbital varix
Orbital trauma with fat atrophy
Metastatic scirrhous carcinomas
Contralateral exophthalmos

WORKUP

Section 5 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Lab Studies

• Neuroimaging is the most essential laboratory study. The remainder of the studies is guided by suspected etiology.

Imaging Studies

• CT scan of the orbits
• • Coronal images are best to evaluate blow out fractures and changes associated with the maxillary sinus.
  • Axial images best demonstrate orbital fat atrophy.
  • Rapid spin or spiral technology is useful for demonstrating varix during Valsalva maneuver.
• MRI with fat suppression, surface coils, and enhancement is best for characterizing neoplastic infiltrations.
• Bone scans are infrequently helpful to identify areas of bone inflammation seen with osteomyelitis or inflammatory wasting disorders.
• Old photographs can help to determine the rate of progression of the problem or an unrecognized congenital facial asymmetry.

Other Tests

• Relate to the specific suspected disease process (For example, for suspected contralateral orbital tumor, systemic evaluation for a primary malignancy or metastatic disease should be performed.)

Procedures

• Open biopsy may be indicated, but needle biopsy is rarely helpful in the diagnosis of enophthalmos.

Histologic Findings

The wide range of causes for enophthalmos provides a wide range of histopathologic findings. One of the most curious findings may be silent sinus syndrome in which spontaneous enophthalmos and hypoglobus occur over days to years without any associated trauma and the histopathology shows only mild, chronic mucosal inflammation and bone reparative changes.

TREATMENT

Section 6 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Medical Care

Medical treatments are directed at specific diseases such as chemotherapy or ionizing radiation for metastatic disease or immunosuppressive treatments for inflammatory disorders. Once the disease process is stabilized, nonsurgical management may include camouflage glasses (hyperopic [magnifying] correction over myopic contact lens).

Surgical Care

Once medical or surgical treatment of the underlying process is achieved, correction of enophthalmos begins with approximating normal orbital bone positions before addressing soft tissue volume loss.

• Orbital fracture repair
• • Maintain the convexity of the posterior, medial orbital floor.
  • Stabilize floor implants posteriorly on intact floor ledge.
  • Release any major adhesions or scar bands to allow mobilization of soft tissues. In late posttraumatic cases, sharp rather than blunt dissection often is required.
  • If using bone grafts rather than synthetic materials, allow for 15-30% resorption.
  • Overcorrect to obtain 1-2 mm of exophthalmos intraoperatively.
  • Perform forced duction testing of the globe prior to closure.
• In replacing lost orbital soft tissue volume, perform the following:
• • A forward traction test on the globe to determine the amount of correction possible
  • Augment from the orbital walls inward with bone or synthetic materials. For pure enophthalmos correction without hypoglobus, inferolateral and retrobulbar mass effect is desirable.

Consultations

Consultation with oculoplastic surgery or neuro-ophthalmology may be useful in defining the cause of enophthalmos.

Activity

Activity restrictions depend upon the etiology.

MEDICATION

Section 7 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Medication depends upon the etiology.

FOLLOW-UP

Section 8 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Further Inpatient Care

• Dependent upon etiology

Further Outpatient Care

• Dependent upon etiology

Complications

• Long-standing enophthalmos, especially associated with very extensive orbital trauma, may be associated with severe orbital scarring, and correction can be very difficult or impossible.

Prognosis

• Most causes of enophthalmos are treatable, and surgical correction is most frequently excellent following outpatient surgery.

Patient Education

• Perhaps the single most important advice to give a patient until the workup of suspected new-onset enophthalmos has been completed is not to blow their nose and to sneeze with their mouth open.
• • One of the most common causes of enophthalmos is orbital fracture. Barometric changes in the nasopharynx (wind velocity may exceed 200 mph during a sneeze) can force air into the orbit. The loose orbital fat may then fall back into place covering the bone defect, acting as a ball valve, trapping the air, and creating an acute orbital compartment syndrome with blinding potential.
  • Other causes of bone loss between the orbit and the sinuses (most notably varix and silent sinus syndrome) also may be affected by dramatic barometric pressure changes.

MISCELLANEOUS

Section 9 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Medical/Legal Pitfalls

• Missing the primary diagnosis
• • If the suspected affected side seems normal based on radiographic imaging findings, consider contralateral exophthalmos.
  • If clinical photographs support a change in appearance, but the MRI findings are interpreted as normal, ensure that the MRI was performed with fat saturation or suppression technique. Consider CT scan as well.
  • If clinical photographs demonstrate a change in appearance, but the radiologist's interpretation and/or the physician's interpretation of the orbit and contents are normal, look again at the sphenoid wing. Relatively small volumetric disease in the sphenoid wing (eg, meningioma, fibrous dysplasia, osteoblastic metastatic disease) can make a significant change in axial globe position and may be missed on first examination of the radiographic studies.
• Missed associated disease
• • Orbital fractures frequently are associated with posttraumatic optic neuropathy, especially with blows to the mid glabella and lateral brow.
  • Prompt recognition is essential. Red light desaturation, bright light desaturation, or the presence of a relative afferent pupillary defect is suggestive of the diagnosis in the setting of trauma.

REFERENCES

Section 10 of 10

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

• Cline RA, Rootman J. Enophthalmos: a clinical review. Ophthalmology. Mar 1984;91(3):229-37. [Medline].
• Cory RC, Clayman DA, Faillace WJ, et al. Clinical and radiologic findings in progressive facial hemiatrophy (Parry-Romberg syndrome). AJNR Am J Neuroradiol. Apr 1997;18(4):751-7. [Medline].
• Davidson JK, Soparkar CN, Williams JB, et al. Negative sinus pressure and normal predisease imaging in silent sinus syndrome. Arch Ophthalmol. Dec 1999;117(12):1653-4. [Medline].
• Eubanks LE, McBurney EI, Galen W, et al. Linear scleroderma in children. Int J Dermatol. May 1996;35(5):330-6. [Medline].
• Manson PN, Clifford CM, Su CT, et al. Mechanisms of global support and posttraumatic enophthalmos: I. The anatomy of the ligament sling and its relation to intramuscular cone orbital fat. Plast Reconstr Surg. Feb 1986;77(2):193-202. [Medline].
• Numa WA, Desai U, Gold DR. Silent sinus syndrome: a case presentation and comprehensive review of all 84 reported cases. Ann Otol Rhinol Laryngol. Sep 2005;114(9):688-94. [Medline].
• Soparkar CN, Patrinely JR, Cuaycong MJ, et al. The silent sinus syndrome. A cause of spontaneous enophthalmos. Ophthalmology. Apr 1994;101(4):772-8. [Medline].
• Soparkar CN, Patrinely JR, Davidson JK. Silent sinus syndrome-new perspectives?. Ophthalmology. Feb 2004;111(2):414-5; author reply 415-6. [Medline].

Article Last Updated: Feb 13, 2007