Choroidal ruptures are breaks in the choroid, the Bruch membrane, and the retinal pigment epithelium (RPE) that result from blunt ocular trauma (the most common eye injury).[1]
Choroidal rupture can be secondary to indirect or direct trauma. Cases secondary to direct trauma tend to be located more anteriorly and at the site of impact and parallel to the ora, whereas those secondary to indirect trauma occur posteriorly. These ruptures have a crescent shape and are concentric to the optic disc. Indirect choroidal ruptures are almost 4 times more common than direct ruptures. See the image below.
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A 23-year-old man was in a motor vehicle accident 2 months before his presentation. His visual acuity is 20/400, and an afferent pupillary defect is p....
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Mid-phase fluorescein angiogram in a 23-year-old man who was in a motor vehicle accident 2 months before his presentation. (Courtesy of Jorge Gutierre....
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Late-phase fluorescein angiogram in a 23-year-old who man was in a motor vehicle accident 2 months before his presentation. (Courtesy of Jorge Gutierr....
After blunt trauma, the ocular globe undergoes mechanical compression and then sudden hyperextension. Because of its tensile strength, the sclera can resist this insult; the retina is also protected because of its elasticity. The Bruch membrane does not have enough elasticity or tensile strength; therefore, it breaks.
Concurrently, the small capillaries in the choriocapillaris are damaged, leading to subretinal or sub-RPE hemorrhage. Hemorrhage in conjunction with retinal edema may obscure the choroidal rupture during the acute phases. The deep choroidal vessels are usually spared. As the blood clears, a white, curvilinear, crescent-shaped streak concentric to the optic nerve is seen.
During the healing phase, choroidal neovascularization (CNV) occurs. Vascular endothelial growth factor (VEGF) has been shown to be a key molecular player in the pathogenesis of CNV. In most cases, it involutes spontaneously.
In 15-30% of patients, CNV may arise again and lead to a hemorrhagic or serous macular detachment with concomitant visual loss. This usually occurs during the first year but can also occur decades later. If the rupture does not involve the fovea, good vision is expected.
Older age and macular rupture, the length of the rupture, and the distance of the rupture to the center of the fovea may be risk factors for CNV.
Blunt ocular trauma is the most common type of eye injury. Approximately 5-10% of patients with such injury develop a choroidal rupture. Most eyes have a single rupture, but up to 25% of eyes have multiple ruptures. About 80% of ruptures occur temporal to the disc, and 66% involve the macula.
Mortality/Morbidity
Vision loss depends on whether the choroidal rupture involves the fovea and whether and where CNV occurs.
Sex
Men appear to be more prone to ocular trauma than women.
A male-to-female ratio of 5:1 is reported for choroidal ruptures.[2, 3]
Age
In most series, this condition occurs in patients aged 20-40 years.[2, 3]
Because choroidal ruptures occur as a consequence of blunt ocular trauma, the ocular examination must be thorough to rule out orbital fractures or globe ruptures.
CT scanning/MRI
Consider CT scanning and MRI of the eye and orbit under appropriate circumstances.
Fluorescein angiography
Fluorescein angiography may be a useful adjunct to detect CNV.
If CNV is absent, hypofluorescence occurs during the early phase of the angiogram due to disruption of the choriocapillaris. During later stages, hyperfluorescence occurs from the adjacent healthy choriocapillaris.
If CNV is present, early hyperfluorescence followed by late leakage is present on the angiogram.
Angiography
Indocyanine green (ICG) angiography may be useful if subretinal blood blocks or hides CNV detection on a fluorescein angiogram.
Optical coherence tomography
With the advent of anti-VEGF therapy, optical coherence tomography (OCT) plays a major role in the management of CNV. Most clinicians consider the presence of fluid on the OCT scan as an indication of CNV activity and the need for further treatment.
Direct choroidal ruptures are characterized by a complete absence of choroid and RPE. The overlying retina is intact but atrophic.
In indirect choroidal ruptures, CNV is a common finding during the early healing phases. Most CNV is in the subretinal space (Gass type 2). With time, most CNV involutes spontaneously. In a small number of cases, a disciform scar or fibrous tissue may grow into the retina and vitreous cavity.
During the healing phase of virtually all choroidal ruptures, CNV is present. CNV may be thought of as part of the wound healing response. Most cases of CNV involute spontaneously. In up to 30% of patients, CNV may arise again and cause visual loss.
Prior to the advent of anti-VEGF therapy, good management options for subfoveal CNV were not really available; therefore, a conservative approach was recommended for most choroidal ruptures.
In the current era of anti-VEGF therapy, the extraordinary results obtained in CNV secondary to age-related macular degeneration have been extrapolated to other causes of CNV with apparent good results.[4, 5]
Currently available anti-VEGF agents include bevacizumab, ranibizumab, pegaptanib sodium, and aflibercept (see Medication).[6, 7]
If CNV is extrafoveal, it may be treated successfully with laser photocoagulation. Recurrences seem few.[8]
Prior to the advent of anti-VEGF therapy, pars plana vitrectomy with membrane extraction was considered for subfoveal or juxtafoveal CNV.[9]
The role of photodynamic therapy with verteporfin is unclear; however, several case reports and case series using this treatment have shown encouraging results in these patients.
ICG-guided photocoagulation transiently closes feeder vessels of subfoveal CNV, but, eventually, these vessels become reperfused.
Currently, anti-VEGF therapy appears to have the most success.[5, 4]
Clinical Context:
Recombinant humanized IgG1-kappa isotype monoclonal antibody fragment designed for intraocular use. Indicated for neovascular (wet) age-related macular degeneration (ARMD). In clinical trials, about one third of patients had improved vision at 12 mo that was maintained by monthly injections. Binds to VEGF-A, including biologically active, cleaved form (ie, (VEGF110). VEGF-A has been shown to cause neovascularization and leakage in ocular angiogenesis models and is thought to contribute to ARMD disease progression. Binding VEGF-A prevents interaction with its receptors (ie, VEGFR1, VEGFR2) on surface of endothelial cells, thereby reducing endothelial cell proliferation, vascular leakage, and new blood vessel formation.
Clinical Context:
Selective vascular endothelial growth factor (VEGF) antagonist that promotes vision stability and reduces visual-acuity loss and progression to legal blindness. VEGF causes angiogenesis and increases vascular permeability and inflammation, all which contribute to neovascularization in age-related wet macular degeneration.
Clinical Context:
Murine derived monoclonal antibody that inhibits angiogenesis by targeting and inhibiting vascular endothelial growth factor (VEGF). Used investigationally for ARMD secondary to choroidal neovascularization.
Clinical Context:
Fusion protein of key domains from human VEGF receptors 1 (VEGFR1) and 2 (VEGFR2) with human IgGFc designed for intraocular use. Indicated for neovascular (wet) age-related macular degeneration (ARMD) and macular edema secondary to central retinal vein occlusion. Binds to VEGF-A, including biologically active, cleaved form (ie, (VEGF110) and placental growth factor. VEGF-A has been shown to cause neovascularization and leakage in ocular angiogenesis models and is thought to contribute to ARMD disease progression. Binding VEGF-A prevents interaction with its receptors (ie, VEGFR1, VEGFR2) on surface of endothelial cells, thereby reducing endothelial cell proliferation, vascular leakage, and new blood vessel formation.
Vascular endothelial growth factor (VEGF) is essential for angiogenesis. Inhibitors of VEGF that bind to the receptor of VEGF-A isoforms prevent its interaction with Flt-1 and KDR on the endothelial cell surface, and therefore decreases cell proliferation and new blood vessel formation.
Most patients with choroidal ruptures do not reach a final visual acuity of 20/40 or better. Poor visual acuity is associated with macular rupture and poor baseline visual acuity.[12]
If the rupture does not involve the fovea, good vision is expected.
A hemorrhagic or serous macular detachment secondary to CNV may threaten visual function.
If CNV is extrafoveal, it may respond well to laser photocoagulation. Few recurrences are reported after laser photocoagulation.
Bevacizumab is a humanized recombinant monoclonal IgG antibody that binds and inhibits all VEGF isoforms. A few series have reported beneficial results following intravitreal bevacizumab.[4, 5]
If CNV is juxtafoveal or subfoveal, consider pars plana vitrectomy with membrane extraction. Gross et al reported good visual acuities in a small case series.[9]
Lihteh Wu, MD, Ophthalmologist, Costa Rica Vitreo and Retina Macular Associates
Disclosure: Received income in an amount equal to or greater than $250 from: Bayer Health; Quantel Medical.
Coauthor(s)
Dhariana Acón, MD, Ophthalmologist, Caja Costarricense Seguro Social, Hospital de Guapiles, Costa Rica
Disclosure: Nothing to disclose.
Specialty Editors
Simon K Law, MD, PharmD, Clinical Professor of Health Sciences, Department of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine
Disclosure: Nothing to disclose.
Steve Charles, MD, Founder and CEO of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine
Disclosure: Received royalty and consulting fees for: Alcon Laboratories.
Chief Editor
Hampton Roy, Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Disclosure: Nothing to disclose.
Additional Contributors
Russell P Jayne, MD, Vitreoretinal Surgeon, The Retina Center at Las Vegas
Disclosure: Nothing to disclose.
Acknowledgements
Teodoro Evans, MD Consulting Surgeon, Vitreo-Retinal Section, Clinica de Ojos, Costa Rica
A 23-year-old man was in a motor vehicle accident 2 months before his presentation. His visual acuity is 20/400, and an afferent pupillary defect is present. Traumatic optic neuropathy and choroidal rupture are observed. This is a red-free photograph. (Courtesy of Jorge Gutierrez, MD.)
Mid-phase fluorescein angiogram in a 23-year-old man who was in a motor vehicle accident 2 months before his presentation. (Courtesy of Jorge Gutierrez, MD.)
Late-phase fluorescein angiogram in a 23-year-old who man was in a motor vehicle accident 2 months before his presentation. (Courtesy of Jorge Gutierrez, MD.)
A 23-year-old man was in a motor vehicle accident 2 months before his presentation. His visual acuity is 20/400, and an afferent pupillary defect is present. Traumatic optic neuropathy and choroidal rupture are observed. This is a red-free photograph. (Courtesy of Jorge Gutierrez, MD.)
Mid-phase fluorescein angiogram in a 23-year-old man who was in a motor vehicle accident 2 months before his presentation. (Courtesy of Jorge Gutierrez, MD.)
Late-phase fluorescein angiogram in a 23-year-old who man was in a motor vehicle accident 2 months before his presentation. (Courtesy of Jorge Gutierrez, MD.)
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