Retinal hemorrhages are an important ophthalmic diagnostic sign for an underlying systemic vascular disorder. They range from the smallest dot and blot hemorrhage to massive sub-hyaloid hemorrhage. The location, size, and distribution of the hemorrhages provide clues to the etiology and uncover underlying systemic disorders such as vascular disease, hematologic disorders, and dyscrasias, infections, trauma, or hypoxia. Rarely it can also be seen idiopathically. Most require a detailed systemic work up to detect the underlying cause for the hemorrhages. Management consists of observation, treating the primary cause, and intraocular management to reduce the ischemic and neovascularization sequelae following the hemorrhages.
Following are the conditions which show retinal hemorrhages depending on the stage and severity of the disease:
Retinal hemorrhages in critically ill children with no history of trauma or abuse have a prevalence of 15%. Most cases are mild and are seen in children younger than two years and are associated with systemic infection, accidental brain trauma, and severe coagulopathy. Birth-related retinal hemorrhages are seen in 25% of newborns with normal delivery and 40% to 50% of newborns with instrumental deliveries. Retinal hemorrhages were seen in 30% of physically abused children, and most are less than six months of age. The prevalence of retinal hemorrhages in adults is most commonly seen after 40 years of age, as most systemic disorders are common in that age.
Retinal hemorrhages are classified according to the location in the retina: Retinal nerve fiber layer (RNFL), intraretinal, subretinal, subretinal pigment epithelium (RPE), subhyaloid/preretinal, and vitreous hemorrhage.
Retinal Nerve Fiber Layer (RNFL) Hemorrhages
RNFL hemorrhages are superficial and follow the direction, shape, and spread of the RNFL bundles. Three types of hemorrhages are seen at the level of RNFL: flame-shaped hemorrhages, splinter (disc) hemorrhages, and Roth spots.
These dot and blot hemorrhages are found within the inner nuclear and outer plexiform layers of the retina. These are dense, dark red, sharply outlined, and are seen in disorders that affect the pre-venular deep capillary layer. Common causes for such hemorrhages include diabetic retinopathy, retinal vein occlusions, ocular ischemic syndrome, sickle cell retinopathy, and juxta foveal telangiectasia. Purtsher retinopathy is an occlusive microvasculopathy associated with cranial or thoracic compressive trauma. Retinal findings are intraretinal whitening, cotton wool spots, and intraretinal hemorrhages. Purtscher-like retinopathy is seen in acute pancreatitis, renal failure, and autoimmune disease.
These hemorrhages occur between the photoreceptor layer and retinal pigment epithelium (RPE). The hemorrhages are deep red in color and broader in shape with diffuse margins. Such hemorrhages are commonly seen in ARMD, presumed ocular histoplasmosis, high myopia, PCV, retinal macroaneurysm, and trauma. Sub-macular hemorrhages are commonly seen in choroidal neovascular membranes secondary to ARMD.
These hemorrhages are located between RPE and Bruch membrane and appear dark red with well-defined sharp borders. They are commonly seen in choroidal neovascular membranes (CNVM), choroidal tumors, and choroidal rupture secondary to acute trauma.
Sub-hyaloid or Preretinal Hemorrhages
Preretinal hemorrhages are “boat” or ‘D’ shaped hemorrhages which collect between the posterior limiting membrane of the vitreous and internal limiting membrane (ILM) of the retina. Causes include Terson syndrome, Valsalva retinopathy, proliferative diabetic retinopathy, and proliferative retinopathy after retinal vein occlusions. Terson syndrome is intraocular hemorrhage associated with subarachnoid hemorrhage, intracerebral hemorrhage, or traumatic brain injury. Hemorrhage may be present in the vitreous, subhyaloid, or intraretinal /sub-internal limiting membrane. Valsalva retinopathy is a pre-retinal hemorrhage caused by a sudden increase in intra-thoracic or intra-abdominal pressure.
Vitreous Hemorrhage (VH)
Bleeding into the vitreous cavity is seen as fresh blood clots with sudden onset of floaters. Chronic hemorrhages appear as diffuse vitreous clouding with settled blood inferiorly. They are commonly seen due to the rupture of a vessel, as seen commonly in proliferative diabetic retinopathy, retinal arteriolar microaneurysm, or during posterior vitreous detachment. A subretinal hemorrhage can cause a breakthrough bleeding into the vitreous. Breakthrough hemorrhage into vitreous also happens in malignant choroidal melanoma, retinal vascular occlusions, and idiopathic polypoidal choroidal vasculopathy (IPCV).
Shaken Baby Syndrome
This comes under abusive head trauma (AHT), is seen in young children caused by repeated acceleration-deceleration injury. Characteristic clinical features include extensive bilateral multilayered retinal hemorrhages, intracranial injury (hemorrhage/hypoxic-ischemic injury), and occult fractures (ribs and long bone metaphysis).
A detailed history about social conditions, medication use, abuse or trauma, recent Valsalva maneuvers or chest compression injury, flashes or floaters with a sudden transient dimming of vision, are all important to come to a diagnosis. All patients need a slit lamp examination of the anterior segment, dilated fundus examination, and intraocular pressure measurement. Optical coherence tomography (OCT), fundus fluorescein angiography (FFA), and fundus photography are all helpful to determine the location and depth of the hemorrhage. One should suspect abusive head trauma if a child less than three years shows retinal hemorrhages with an intracranial injury.
Blood pressure, body mass index, and blood sugar are mandatory before evaluating further. Also, the following workup would help in diagnosing the underlying systemic disorder leading to the retinal hemorrhages:
A solitary retinal hemorrhage can be observed and followed up for progression in size and number. Hemorrhages that do not obscure or threaten vision do not warrant immediate treatment, and the primary systemic disorder causing the hemorrhages needs to be diagnosed and addressed. Direct intervention for retinal hemorrhages is indicated in submacular, subhyaloid, and vitreous hemorrhages because of their potential to damage vision irreversibly.
Most dot and blot, splinter, and flame-shaped hemorrhages are not vision-threatening and are usually found in the posterior pole sparing the fovea and can be observed. Hemorrhages seen in retinal vein occlusions are not treated per se, but the retinal edema or the neovascularization, which follows as a sequela, needs to be treated. Roth spots are generally asymptomatic and typically resolve with treatment of the underlying disease, especially subacute bacterial endocarditis.
Subhyaloid hemorrhage is seen typically in Terson syndrome and Valsalva retinopathy. Besides, bilateral hemorrhages in the vitreous, sub-hyaloid, or intra-retinal hemorrhages are also seen. The first approach for a subhyaloid hemorrhage is always conservative observation. If no signs of resolution are seen within 1 to 3 months or for patients with high professional visual demand, Nd: YAG laser membranotomy is done to rupture the posterior hyaloid or the internal limiting membrane to drain the blood into vitreous. The blood settles into the inferior vitreous and out of the visual axis, prompting a rapid return of central visual acuity. Unresolved vitreous hemorrhage obstructing vision needs pars plana vitrectomy (PPV).
Submacular hemorrhage (SMH) is a sight-threatening complication that can occur in a CNVM secondary to exudative ARMD, PCV, high myopia, angioid streaks, and others. CNVM appears as a dirty grey-green color lesion, deep to the retina. The grey-green appearance is believed to be due to the hyperplastic response of RPE. It may be accompanied by subretinal hemorrhage and/or lipid, serous or hemorrhagic PED, neurosensory exudate detachment, macular edema, and cystoid macular changes. Signs of choroidal neovascularization include RPE elevation, exudate, or subretinal fluid.
Patients prone to develop CNVM are advised to self-check their retinal status weekly through Amsler grid charts. Any sudden onset of distortion of lines is suspicious of the development of CNVM. When left untreated, SMH causes irreversible vision loss by damaging the photoreceptors. The treatment strategies for SMH include:
Vitreous hemorrhage is commonly seen in vascular conditions such as diabetic retinopathy and retinal vein occlusions. The treatment strategies depending on the severity of hemorrhage and vision loss are as follows:
Since retinal hemorrhages can be found in both children and adults, therefore the differentials also vary according to the age group.
Neonatal: hemorrhages from birth trauma, including spontaneous vaginal delivery, vacuum extraction, and double instrument deliveries (vacuum and forceps).
Infants and Children:
Diabetes, hypertension, leukemia, blood dyscrasias, hypoxia/ anoxia/high altitudinal syndromes, high myopia, posterior vitreous detachment, retinal tear, and detachment.
Most neonatal and infant retinal hemorrhages secondary to trauma have a good prognosis and resolve in 2 to 4 weeks. Retinal hemorrhages secondary to metabolic syndromes and venous occlusions resolve with control of the underlying conditions. Submacular and subretinal hemorrhages have a very poor prognosis and can irreversibly affect the vision due to damage to the photoreceptor layer. Subhyaloid hemorrhages, if ruptured by ND-YAG laser at an appropriate time, have a good prognosis. Most vitreous hemorrhages resolve within three months, and fine traces of blood clot can still be seen settled inferiorly. Unresolved hemorrhages or hemorrhages which cause secondary glaucoma carry a poor prognosis.
Following are the complications that can arise secondary to retinal hemorrhages and underlying disorders:
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