Valsalva retinopathy is a specific form of retinopathy characterized by pre-retinal hemorrhages secondary to raised intrathoracic pressure. Thomas Duane first described it in 1972. It can occur in any person irrespective of health status. In almost all cases the hemorrhage resolves, and vision returns to normal depending on the location of the bleed in the retina.
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.
There is no specific age, sex, or racial predilection noted so far in the literature.
The Valsalva maneuver results from forcible expiration against a closed glottis and produces a sudden rise in venous blood pressure owing to 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.
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:
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 inner ring represents sub-ILM hemorrhage. 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. 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.
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 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
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.
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.
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. 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.
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.
If there is significant and long-standing pre-macular hemorrhage, or dense vitreous hemorrhage obscuring retinal evaluation pars plana, then vitrectomy can be attempted.
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.
There are very few ocular conditions in which multilayered hemorrhages can present, i.e., at pre retinal, intraretinal and subretinal level. 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. 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.
Prognosis is usually good with the spontaneous clearing of hemorrhage within weeks to months. However, visual recovery depends on the location of bleed in the fundus and layer of involvement of bleed. Subretinal hemorrhage at macula can cause adverse effects secondary to Iron within hemoglobin in blood, pressure effects or by fibrosis post clearing of bleed.
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.
Patients should receive counsel regarding relatively good prognosis in cases of Valsalva retinopathy. There is no way to predict which persons are at risk. However, one should avoid prolonged breath-holding during periods of exertion, such as lifting weights, sneezing, coughing, etc. Patients should be advised to take multiple breaths during activities of a substantial effort.
Any patient presenting with signs highly suggestive of Valsalva retinopathy should not only receive treatment by an ophthalmologist in isolation. It requires multispecialty evaluation to look for the cause of the bleed, which includes uncontrolled systemic hypertension, constipation, and unsupervised physical exertion.
The patient will most often present to the primary provider or nurse practitioner, and these professionals should be aware of the condition as it is treatable. Prompt referral to an ophthalmologist is necessary. These patients are then followed by their primary clinicians and should ensure compliance with treatment. The patients should be encouraged to maintain healthy body weight, exercise regularly, refrain from smoking, and avoid activities that lead to straining.
A dietician can guide the patient towards a diet rich in fiber, as it helps in avoiding constipation. Patients need to be counseled regarding the relatively good prognosis of the case as patients can get depressed secondary to sudden severe loss of vision.
Pharmacists can verify dosing on the medication management aspect of the condition, and report any issues with pharmaceutical therapy to the healthcare team. Nursing will be the first to see patients on followup and can assess treatment progress as well as evaluate compliance with both medication and lifestyle measures, and report any issues to the primary care physician. This collaborative, interprofessional approach to care can ensure optimal patient outcomes. [Level V]
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