Valsalva Retinopathy

Etiology

This condition occurs secondary to various day-to-day activities where Valsalva maneuver occurs includes weight lifting, vomiting, coughing, sneezing, aerobic exercises, constipation, blowing musical instruments, straining, physical activities, sexual intercourse, after cardiopulmonary resuscitation, and compression injuries.[2]

Reported associations include[3][4][3]:

• Proliferative diabetic retinopathy (PDR) - There is a report of a patient with PDR who developed bilateral vitreous hemorrhage resulting from 'an extended episode of vomiting' after a fluorescein angiogram.
• Congenital retinal macrovessel
• Hypertensive retinal angiopathy
• Congenital changes in retinal vessels including retinal arterial tortuosity and retinal telangiectasis

Epidemiology

There is no specific age, sex, or racial predilection noted so far in the literature.

Pathophysiology

The Valsalva maneuver results from forcible expiration against a closed glottis and produces a sudden rise in venous blood pressure owing to a rise in intrathoracic or intra-abdominal pressure.  Incompetent or absent valves in the venous system of the head and neck allow transmission of thoracic and abdominal pressure into the eye. Thus, this sudden rise of blood pressure leads to a sudden increase of intraocular venous pressure leads to rupture of perifoveal superficial retinal capillaries causing a hemorrhagic detachment of the internal limiting membrane (ILM) which acts as a barrier preventing its spread to the subhyaloid space.[1][5]

History and Physical

Symptoms: Patients with Valsalva retinopathy often present with sudden onset of painless unilateral (rarely bilateral) loss of vision preceded by a history of Valsalva-like maneuvers (e.g., coughing, vomiting, violent sneezing, weight lifting, blowing musical instruments, end-stage labor). Patients describe the symptoms as seeing a black spot (scotoma) in front of the eye, floaters, reddish hue of vision, blurring of vision to loss of vision depending on the severity and location of the bleed.

Signs: The typical clinical appearance of Valsalva retinopathy includes[6]:

• Well-circumscribed
• Round, oval, or dumb-bell shaped
• Usually more than one disc area
• Pre-retinal hemorrhage
• At or around the fovea; either sub ILM (internal limiting membrane) or subhyaloid space
• Sub-ILM bleed usually appears as a smooth mound with some glistening at the surface.
• There may be some ILM striae over the hemorrhage.
• In the acute stage, the pre-retinal blood is bright red and elevated with a convex surface towards the vitreous with some movement if the eye moves. There may be a fluid level with gravitated red blood corpuscles inferiorly with a horizontal upper level.

If blood gets trapped in both sub-ILM and subhyaloid spaces, it presents with a ‘double ring sign’; the outer ring represents sub hyaloid, and the inner ring represents sub-ILM hemorrhage.[7] Though there is a predilection for macular involvement, there can be multiple lesions some away from the macula.

Sometimes, the pre-retinal hemorrhage may be less than one disc area in size with a strawberry-like appearance due to yellowish-white spots. 

Other ocular signs include subconjunctival hemorrhages, petechial hemorrhages of eyelids, superficial intraretinal hemorrhages, subretinal hemorrhages at the fovea due to the dissection of blood beneath the retina, retinal transudation, and breakthrough vitreous hemorrhage. Moderate myopes may rarely have choroidal hemorrhage after excessive vomiting.

Once the hemorrhage clears, the only sign seen in old or resolved Valsalva retinopathy includes cavity formation at the level of the bleed such as a sub-ILM cavity or serous detachment of the ILM. This appearance can be clinically confused as a neurosensory detachment.[8] A clinical clue to previous sub-ILM bleed is the presence of brown pigments (presumably blood products) at the margin of serous ILM detachment. This detached ILM usually reattaches with time.

With time the sub-ILM hemorrhage may dehemoglobinize giving a yellow and later white color to the hemorrhage. 

Evaluation

Systemic examination to look for signs of Valsalva – bradycardia, increased peripheral blood pressure, petechial hemorrhages of skin.

Rule out predisposing factors such as anemia, sickle cell disease, idiopathic thrombocytopenic purpura, diabetes, and hypertension. The workup may include a hemogram with platelet count, peripheral blood smear, fasting blood sugar, blood pressure, prothrombin time (PT), activated partial thromboplastin time (APTT), hemoglobin electrophoresis to rule out sickle cell anemia

Ocular examination:

• Best-corrected visual acuity – serves to monitor patient, to plan intervention, intraocular pressure, pupillary reaction, and comprehensive ocular examination
• Subconjunctival hemorrhage
• Optical coherence tomography – to look for the location of bleed (sub-ILM/subhyaloid)[9] and plan laser photocoagulation or surgery
• Fundus fluorescein angiography to look for neovascularization and other retinal pathologies
• Fundus picture – to monitor the progression of the disease
• B scan ultrasonogram – in cases with breakthrough vitreous hemorrhage

Treatment / Management

Once the diagnosis of Valsalva retinopathy is confirmed, management depends on the location of the bleed. Observation is the standard treatment. However, cases of massive bleed at the macula (particularly subretinal) require early intervention.

Medical management

Treat inciting agents such as stool softeners for constipation, avoid anticoagulants, and avoid strenuous exercises.

Patients should receive instruction to adopt a propped up position so that blood settles inferiorly.

It may take as many as 6 months for vitreous hemorrhage to resolve in some cases.[10]

Laser treatment

For large subhyaloid hemorrhage/sub Internal limiting membrane hemorrhage obscuring macula of fewer than three weeks duration – Nd YAG (pulsed/ Q switched/ frequency-doubled), krypton laser membranotomy can be used to make an opening in the posterior hyaloid or ILM, so that blood escapes into the vitreous cavity and settles inferiorly.[11][12] This action clears the visual axis and helps in improving vision. Most studies have used Nd-YAG laser (1064 nm) with a power of 2.2 to 9.7mJ, and the central part of the Goldmann 3-mirror lens was used to focus the laser beam.[2][13](B2)

Site of membranotomy – Away from large blood vessels, away from the fovea, at the inferior margin of the hemorrhage, at the site of maximum underlying hemorrhage

Complications of laser therapy include iatrogenic retinal tear, hemorrhage into subretinal and choroidal space, epiretinal membrane formation, retinal detachment, and permanent vision loss.[14](B3)

 Surgical treatment

If there is significant and long-standing pre-macular hemorrhage, or dense vitreous hemorrhage obscuring retinal evaluation pars plana, then vitrectomy can be attempted.

Differential Diagnosis

In atypical cases with no antecedent history of Valsalva maneuver, various conditions which can mimic Valsalva retinopathy include:

Proliferative vascular retinopathies – secondary to diabetes, retinal vein occlusion, and other diseases. It is necessary to rule out retinal new vessels of new vessels at the optic disc before making a diagnosis of Valsalva retinopathy.

• Hypertensive retinopathy
• Sickle cell anemia
• Battered baby syndrome
• Terson syndrome[15]
• Ruptured macroaneurysm
• Anemic retinopathy
• An acute posterior vitreous detachment can also cause hemorrhages, and it is necessary to rule out peripheral retinal tears.

There are very few ocular conditions in which multilayered hemorrhages can present, i.e., at pre retinal, intraretinal, and subretinal levels. Conditions causing multilayered hemorrhages include Valsalva retinopathy, anemic/leukemic retinopathy, and trauma (battered baby syndrome).

Sometimes, pre-retinal hemorrhage identical to Valsalva retinopathy occurs in apparently normal patients without any history of Valsalva or exertion.[16][17][13] In such cases, there might be some retinal angiopathy (hypertension or diabetes), impaired platelet aggregation, or patients might be on anticoagulant therapy. Some patients with recurrent pre-retinal hemorrhage may have a family history, and it may be an autosomal dominant disease.[18]

Prognosis

Prognosis is usually good with the spontaneous clearing of hemorrhage within weeks to months. However, visual recovery depends on the location of the bleed in the fundus and the layer of involvement of the bleed. Subretinal hemorrhage at macula can cause adverse effects secondary to Iron within hemoglobin in the blood, pressure effects, or fibrosis post clearing of bleed.

Complications

Permanent visual loss in cases of subretinal hemorrhage at the macula, ERG changes in cases of long-standing hemorrhage wherein ferritin from dehemoglobinized blood gets deposited within Retinal pigment epithelial cells.