para>One of the few true emergencies in ophthalmology
EPIDEMIOLOGY
Incidence
- Incidence after facial trauma is ~2 " 3%.
- Incidence with blepharoplasty is 0.055% (3).
ETIOLOGY AND PATHOPHYSIOLOGY
- Eye
- Decreased visual acuity due to optic nerve and vascular compression
- Decreased visual movements of the globe and diplopia due to restriction of extraocular muscles
- Neurologic
- Expansion of tissue (Graves) or fluid (blood, edema) within a closed space. The orbit is bounded on four sides by bone and anteriorly by the orbital septum, which is fused to the orbital rim and lids.
- Orbital hemorrhage, with accumulation of blood (3)
- Posttraumatic retrobulbar hemorrhage is the most common cause overall.
- Precipitating trauma does not have to be severe enough to cause fracture and can be "indirect, " from uncontrolled sneezing, coughing, Valsalva maneuver, labor, or barotraumas.
- Retrobulbar hemorrhage can also occur following eyelid/periocular surgery, particularly when the septum is breached during preaponeurotic fat excision, when there is traction on periorbital fat, or when the effects of epinephrine have worn off, resulting in reflex vasodilation. May also occur with anesthetic injections into the orbit, such as a retrobulbar block.
- Nonophthalmic surgeries can also be responsible (sinus, facial trauma surgery, orthognathic, neurosurgery).
- Hematologic disorders may be predisposing.
- Proptosis (e.g., as in Graves disease), with limitation of forward movement by the eyelids
- Eyelid burns
- Nose blowing, which can force air into the orbit, in patients with orbital fracture
- Orbital edema
- Inappropriate packing during orbital/sinus surgery
- Excess pressure on eye during periocular surgery
- Intracranial surgery increases risk
- Intraorbital abscess
- Orbital cellulitis (infectious or chemical)
- Rapid growth of a neoplasm
- Prolonged hypoxemia with capillary leak
- Intraorbital foreign body
- Excessive fluid resuscitation after burn injury or blood loss (2)
RISK FACTORS
- Trauma (including trauma that does not result in facial fracture, indirect trauma)
- Coagulopathy (including blood dyscrasias, hepatitis)
- Use of NSAIDs, antiplatelet medications, anticoagulants, thrombolytics, certain herbal medications, and corticosteroids
- Graves disease (subacute compartment syndrome)
- Excessive IV fluids or blood products (2)
GENERAL PREVENTION
- Encourage patient to avoid facial trauma (e.g., bar fights, car accidents, contact sports) and wear protective head gear when possible (e.g., helmets).
- Treat coagulopathies.
- Limit patient 's use of NSAIDs, antiplatelet and anticoagulants, thrombolytics, certain herbal medications, and corticosteroids.
- Monitor Graves ophthalmopathy closely.
- Avoid excess IV fluids, when possible.
- Ensure close contact of patient with orbital surgeon or emergency ophthalmologic care.
- Counsel patient to avoid blowing nose after facial trauma, particularly orbital fracture (1).
COMMONLY ASSOCIATED CONDITIONS
- Graves disease
- Coagulopathies
- Intraorbital abscesses
- Orbital cellulitis
- Surgery with significant blood loss (requiring IV fluids or blood products) (2)
DIAGNOSIS
Mostly made on a clinical basis
HISTORY
Ask about the following:
- Acute onset of pain
- Painful periorbital edema
- Decreased vision, double vision (due to extraocular muscle restriction)
- Trauma (including trauma not significant enough to cause facial fracture)
- Uncontrolled sneezing, coughing, Valsalva maneuver, labor, and barotrauma
- Presence/history of orbital fracture
- Bleeding disorders
- Hepatitis
- Antiphospholipid antibody syndrome
- Chronic sinusitis and other head, ears, eyes, nose, throat (HEENT) infections
- Recent history of ocular, sinus, facial trauma, orthognathic, or neurologic surgery
- Presence/history of cancer
- Graves disease
- Use of antiplatelet, anticoagulant, or thrombolytic medications
- Use of NSAIDs, cold and flu medications, and herbal supplements (Ginkgo biloba, ginseng, garlic)
- Chronic corticosteroid use
- Spider bites near the orbit
PHYSICAL EXAM
- General appearance: Patient may appear uncomfortable.
- Eye: Assess for the following:
- Decreased visual acuity
- Diplopia
- Observe for the following:
- Classic signs: tense eyelids with ecchymosis confined by the orbital rim
- Decreased ocular movement
- Proptosis, with a tight orbit
- Difficulty opening eyelids
- Subconjunctival emphysema (air bubbles within the conjunctiva) or subconjunctival hemorrhage
- Vertical globe dystopia
- Afferent pupillary defect
- Palpate for the following:
- Periorbital crepitus
- Tender periorbital edema
- Resistance to retropulsion of globe
- Funduscopic evaluation for the following:
- Retinal venous congestions
- Central retinal artery pulsations
- Retinal edema
- Optic disc swelling
DIFFERENTIAL DIAGNOSIS
- Idiopathic/autoimmune orbital inflammation
- Thyroid ophthalmopathy
- Ruptured dermoid cyst
- Progressively enlarging masses
DIAGNOSTIC TESTS & INTERPRETATION
Consider coagulation studies, including bleeding time for spontaneous orbital hemorrhage, to investigate a potential coagulopathy:
- If abnormal, consider further laboratory workup, including testing for severe anemia, von Willebrand disease, hemophilia, leukemia, sickle cell disease, hepatitis, and scurvy.
- This is a clinical diagnosis, and waiting for imaging is often not advised, given the need to treat immediately.
- Imaging can be considered in pediatric patients, when it may be difficult to elicit a history, or if the condition does not respond to surgical treatment, when imaging may help with diagnosis of another treatable condition (e.g., subperiosteal hematoma) and help identify the location and source of orbital tension (3)[C].
- Imaging can also be considered following surgery.
- The following guidelines about choosing imaging are outlined for those purposes:
- Imaging modalities for the orbit: CT/MRI (3)[C]
- CT more immediately available and, therefore, more commonly used
- MRI useful for identifying different stages of hemorrhage
- MRI contraindicated if there is a metal foreign body
- Findings to look for on CT:
- Posterior globe tenting, development of a conical shape (may also be seen in other conditions, including severe proptosis/globe subluxation in thyroid ophthalmopathy) (3)[C]
- Proptosis
- Increased stretch angle and distance from globe to orbital apex (better predictors of prognosis than proptosis) (4)[C]
- Modalities to better visualize vascular lesions causing hemorrhage and OCS (e.g., intraorbital venous malformations, lymphangiomas):
- Angiography and venography are the gold standard (3)[C].
- Magnetic resonance angiography (MRA) or magnetic resonance venography (MRV) (3)[C]
TREATMENT
ALERT
In acute OCS, immediate lateral canthotomy and cantholysis are required to prevent permanent blindness.
GENERAL MEASURES
- Patients should avoid blowing the nose, Valsalva maneuver, or coughing.
- Patients should avoid platelet inhibitors and blood thinners.
- Patients should elevate the head of the bed to at least 45 degrees.
- Patients should use ice packs to reduce blood flow and edema to the orbit.
- Other surgeries, including for facial trauma fracture, should ideally be delayed until after treatment and optic nerve recovery.
MEDICATION
- Give patients with orbital cellulitis/inflammation antibiotics or anti-inflammatory agents as indicated, which may help control orbital pressure (3)[C].
- Avoid the use of steroids, especially in patients with severe head trauma given link to increased mortality and possible increase in axonal loss (5)[A].
- Consider osmotic agents, carbonic anhydrase inhibitors, or aqueous suppressants (3)[C], (5)[A].
- Treat coagulopathies prior to surgery (3)[C].
- Consider cough suppressants, antiemetics, and stool softeners to suppress exacerbating factors (3)[C].
- May consider giving antibiotics prophylactically, following surgery (3)[C].
SURGERY/OTHER PROCEDURES
- The gold standard treatment of acute OCS is immediate lateral canthotomy and inferior cantholysis (3)[C]. May need to aspirate hematoma.
- Surgical intervention should occur within 2 hours of trauma (5)[A].
- Inferior orbital septum release is equivalent in efficacy to lateral canthotomy and cantholysis, according to one study. Performing both procedures in conjunction may also have additive effects (6)[B].
- Subacute OCS should be treated urgently, but not necessarily emergently, with planned decompression (3)[C].
- The eyelid should be able to evert easily, and the globe should be able to sublux forward following treatment, resulting in a decrease in intraocular intraorbital pressure (3)[C].
- If orbital tension does not decrease and perfusion to the optic nerve and retina is not restored after canthotomy and cantholysis (response normally within a few minutes), more extensive incision of the orbital septum and even orbital bony decompression may be required. The orbital septum should be separated from the orbital rims (3)[C].
- May consider repair of the lateral canthus 1 week following canthotomy and cantholysis treatments. However, the lateral canthus will often heal without any secondary interventions (3)[C].
- Other surgeries that have been used include inferolateral anterior orbitotomy, direct needle aspiration of orbital emphysema (risk of globe perforation), and fracturing the orbital floor (risk of damaging infraorbital neurovascular bundle). At least one case reported recurrent orbital air collection successfully managed by orbital punctures (1)[C].
- If possible, avoid prone positioning of patient (3)[C].
- Consider regular intraoperative monitoring for signs of increased orbital pressure if prone positioning is required (3)[C].
ONGOING CARE
PATIENT EDUCATION
Patients should immediately seek medical assistance if they have increased pain, proptosis, or blurry vision, which may signal worsening of disease or disease recurrence.
PROGNOSIS
Full visual recovery is possible. Better prognosis if
- Patient receives immediate surgical management.
- Posterior tenting of globe is less severe on CT scan.
- Patients with increased stretch angle and distance from globe to orbital apex are treated more aggressively (4)[C].
COMPLICATIONS
Irreversible visual loss or blindness
REFERENCES
11 Ahnood D, Toft PB. Recurrent orbital compartment syndrome caused by a blow-out fracture and accumulation of air; management by orbital punctures. Acta Ophthalmol. 2012;90(2):199 " 200.22 Brodt J, Gologorsky D, Walter S, et al. Orbital compartment syndrome following extracorporeal support. J Card Surg. 2013;28(5):522 " 524.33 Lima V, Burt B, Leibovitch I, et al. Orbital compartment syndrome: the ophthalmic surgical emergency. Surv Ophthalmol. 2009;54(4):441 " 449.44 Oester AEJr, Sahu P, Fowler B, et al. Radiographic predictors of visual outcome in orbital compartment syndrome. Ophthal Plast Reconstr Surg. 2012;28(1):7 " 10.55 Soare S, Foletti JM, Gallucci A, et al. Update on orbital decompression as emergency treatment of traumatic blindness. J Craniomaxillofac Surg. 2015;43(7):1000 " 1003.66 Oester AEJr, Fowler BT, Fleming JC. Inferior orbital septum release compared with lateral canthotomy and cantholysis in the management of orbital compartment syndrome. Ophthal Plast Reconstr Surg. 2012;28(1):40 " 43.
ADDITIONAL READING
- Bernardino CR. OCS: one of our few true emergencies. Rev Ophthalmol. 2009;16:78 " 80.
- Hubbard JJ, James LP. Complications and outcomes of brown recluse spider bites in children. Clin Pediatr (Phila). 2011;50(3):252 " 258.
- Johnson D, Winterborn A, Kratky V. Efficacy of intravenous mannitol in the management of orbital compartment syndrome: a nonhuman primate model [published online ahead of print April 3, 2015]. Ophthal Plast Reconstr Surg.
- Rabinowitz MP, Goldstein SM. Diesel fuel injury to the orbit. Ophthal Plast Reconstr Surg. 2013;29(1):e31 " e33.
CODES
ICD10
- H40.059 Ocular hypertension, unspecified eye
- H05.20 Unspecified exophthalmos
- H05.239 Hemorrhage of unspecified orbit
- H05.269 Pulsating exophthalmos, unspecified eye
- H40.052 Ocular hypertension, left eye
- H40.053 Ocular hypertension, bilateral
- H05.229 Edema of unspecified orbit
- H40.051 Ocular hypertension, right eye
ICD9
- 365.04 Ocular hypertension
- 376.30 Exophthalmos, unspecified
- 376.32 Orbital hemorrhage
- 376.33 Orbital edema or congestion
- 376.35 Pulsating exophthalmos
SNOMED
- Raised intraocular pressure (finding)
- Exophthalmos (disorder)
- Exophthalmos due to orbital hemorrhage
- Exophthalmos due to orbital edema or congestion (disorder)
- Orbital hemorrhage
CLINICAL PEARLS
- OCS is primarily a clinical diagnosis, although sometimes labs and imaging may be useful in determining severity and for risk stratification.
- OCS is an ophthalmologic emergency, requiring immediate surgical treatment.
- Delayed treatment may result in permanent blindness.