Valsalva Retinopathy

Valsalva retinopathy was first described in 1972 by Thomas Duane as "a particular form of retinopathy, pre-retinal and hemorrhagic in nature, secondary to a sudden increase in intrathoracic pressure."[1] It was used to describe retinal hemorrhages in association with heavy lifting, coughing, straining at stool, or vomiting. The Valsalva maneuver was named after the Italian anatomist Antonio Maria Valsalva, who defined the Valsalva ligaments and anatomy related to the forcible exhalation effort against a closed glottis.

Valsalva retinopathy classically manifests as preretinal hemorrhage secondary to rupturing of superficial retinal vessels caused by physical exertion. The mechanism of a Valsalva maneuver is characterized by a sudden rise in intrathoracic or intraabdominal pressure against a closed glottis, which leads to a rapid rise of intravenous pressure within the eye, causing retinal capillaries to spontaneously rupture.

Prognosis usually is good, with spontaneous resolution occurring within months after onset, but depends on location of the hemorrhage and the layer of retina involved.

Initial presentation of a Valsalva retinopathy less than 24 hours following a Valsalva maneuver in an 18-year-old man. Note the large preretinal hemorrhage. Vision was finger counting at 5 feet.

At 4-month follow-up of the same patient as in the image above, most of the large preretinal hemorrhage had cleared with observation alone. Note the wrinkled internal limiting membrane temporal to the macula and the resolving hemorrhage at the edge of the demarcation line of the stretched internal limiting membrane inferiorly. Vision had returned to 20/20.

Increasing intrathoracic pressure against a closed glottis diminishes venous return to the heart, decreasing stroke volume and subsequently increasing the venous system pressure.[2, 3, 4, 5, 6]

The process occurs in 4 separate and distinct phases. First, a sudden increase in intrathoracic pressure decreases venous return to the right side of the heart. Second, diminished cardiac filling lowers the mean arterial pressure, slowing the pulse and leading to reflex tachycardia and peripheral vasoconstriction. Third, release of the strain causes a prompt reduction in the intrathoracic pressure, further lowering the blood pressure and simultaneously increasing the cardiac pressure. Finally, an abrupt increase in blood pressure occurs as venous blood surges back to the heart, inducing reflex bradycardia.

During a Valsalva maneuver, blood pressure in the peripheral portions of the body increases rapidly. As the sudden rise in intraocular venous pressure occurs, a spontaneous rupture of retinal capillaries ensues.

Frequency

The frequency of Valsalva retinopathy is difficult to ascertain considering the rare nature of the condition.

United States

The incidence of Valsalva retinopathy in the United States has not been reported.

International

The worldwide incidence of Valsalva retinopathy has not been reported.

Mortality/Morbidity

Decreased vision occurs in the affected eye or eyes, ranging from complaints of floaters to complete loss of central vision. Vision often improves over weeks to months, depending on the severity of the retinal findings.

Race

No racial predilection exists.

Sex

No sexual predilection exists.

Age

Persons of any age can be affected.[7]

Prognosis generally is good, as complete recovery of vision (with observation) usually occurs within weeks to months following onset. However, visual prognosis depends on the location of the hemorrhaging and specifically the layer of retina involved.

There is no definitive way to prevent Valsalva retinopathy, but refraining from "holding your breath" during lifting, sneezing, and coughing may reduce the risk. Furthermore, breathing freely and openly helps decrease the chance of a Valsalva maneuver.

While lifting heavy objects, patients should be advised not to hold their breath for extended periods of time and to take multiple breaths between bearing-down phases. Exhaling while lifting or straining prevents a Valsalva maneuver because one cannot exhale against a closed glottis. Straining during bowel movements should be avoided.

For excellent patient education resources, visit eMedicineHealth's Eye and Vision Center. Also, see eMedicineHealth's patient education article Subconjunctival Hemorrhage (Bleeding in Eye).

Valsalva retinopathy usually manifests as preretinal hemorrhage secondary to rupturing of superficial retinal vessels from physical exertion. The mechanism of Valsalva maneuver is characterized by a sudden rise in intrathoracic or intraabdominal pressure against a closed glottis, which leads to a rapid rise of intravenous pressure within the eye, causing retinal vessels to spontaneously rupture.

Patients with Valsalva retinopathy often present with loss of vision in one eye, but bilateral clinical findings are common. The degree of vision loss depends on the size and location of the preretinal (or, less commonly, subretinal) hemorrhage. Patients usually describe an antecedent Valsalva-like maneuver (eg, coughing, vomiting, heavy lifting, straining in the bathroom, vigorous sexual activity, labor and delivery). The severity of the Valsalva maneuver is not directly correlated with the severity of Valsalva retinopathy.

Some individuals with Valsalva retinopathy may experience spontaneous vision loss without a history of Valsalva maneuver. Without a definitive history of physical exertion, retinal vascular disease should be considered and other systemic conditions investigated. Furthermore, patients receiving anticoagulants may develop a similar clinical picture without a history of unusual exertion.

Patients with Valsalva retinopathy often present with the following symptoms:

• Floaters
• Red-tinged hue to the vision
• Cloudy or hazy vision
• Partial loss of vision
• Complete loss of vision

The clinical signs of suddenly increased systemic venous pressure often include the eye and skin. A subconjunctival hemorrhage may be evident, and skin petechia of the hand and neck may be present.

A comprehensive ocular examination is imperative and should include visual acuity testing, pupil testing, anterior segment examination, and a detailed posterior segment examination.

The "classic" clinical appearance of Valsalva retinopathy on dilated fundus examination is a well-circumscribed preretinal hemorrhage in either the subhyaloid or sub–internal limiting membrane (ILM) space. Interestingly, Valsalva retinopathy has a predilection for the macula region (both premacular and paramacular). Often, the ruptured vessels in the perifoveal vessels can cause a sudden and painless loss of central vision. Uncommonly, subretinal hemorrhage may occur.

Retinal edema in the macular region with associated edematous transudates and superficial intraretinal hemorrhages have been described.

Ocular findings and visual symptoms depend on the severity of the Valsalva force and the underlying status of the retina vasculature. Hemorrhages can vary in size, and, in many cases, a large preretinal hemorrhage encompassing several disc diameters may be observed.

Subhyaloid, sub–internal limiting membrane (ILM) hemorrhage in eye with Valsalva retinopathy. This image was originally published in the ASRS Retina Image Bank. Mitzy E. Torres Soriano, MD. Valsalva Retinopathy. Image Number 18218. © the American Society of Retina Specialists.

Subhyaloid, sub–internal limiting membrane (ILM) hemorrhage showing a fluid level and yellowish color as it settles over time. This image was originally published in the ASRS Retina Image Bank. Kathy Karsten, COT. Subhyaloid Hemorrhage, Right Eye. Number 23763. © the American Society of Retina Specialists.

The underlying etiology of the Valsalva maneuver most commonly is related to heavy lifting, vomiting, coughing, unusual physical exertion, and straining on the toilet. Other causes, such as sneezing, compressive trauma, labor, vigorous sexual activity, labor, and rollercoaster riding, have been described.

The pathophysiology of Valsalva retinopathy is related to a sudden increase in intraabdominal pressure in a closed glottis.

A large preretinal hemorrhage in a 42-year-old man following a Valsalva maneuver. This image was taken 2 days after he underwent heavy straining while lifting weights.

This 58-year-old man with uncontrolled diabetes presented with complaints of a spot in his vision following straining during a bowel movement. He had active proliferative diabetic retinopathy, and the hemorrhage shown in this image stems from a broken neovascularized blood vessel secondary to a Valsalva maneuver.

Patient with history of gastroenteritis and episodes of vomiting followed by loss of central vision.

Patient with sudden loss of vision during labor and seen soon after giving birth.

Physical examination of the skin may reveal petechiae. See Ocular Examination for clinical features of the eye examination.

Complications of Valsalva retinopathy may include the following:

• Permanent vision loss
• Hemorrhaging within and under the retina, destroying the cellular structure of the retina
• Slightly reduced visual acuity secondary to either incomplete blood resorption or mild retinal pigment epithelium changes in or around the macula
• Possible toxic damage to the retina due to prolonged contact of retina with hemoglobin and iron, causing irreversible visual impairment
• Bilateral choroidal detachment
• An Nd:YAG laser membranotomy producing epiretinal membrane formation with internal limiting membrane wrinkling as a late postoperative complication (although its frequency has not yet been identified)

The final visual outcome often depends on the location of the hemorrhage and the layer of retina involved (subretinal, intraretinal, preretinal). Specifically, subretinal hemorrhage in the macula most likely causes permanent vision loss.

Laboratory studies can be used to rule out predisposing risk factors, including diabetes, sickle cell disease, anemia, idiopathic thrombocytopenic purpura, and other blood dyscrasias. Important tests include the following:

• Complete blood count

• Fasting blood sugar, glucose tolerance test

• Prothrombin time, activated partial thromboplastin time

• Sickle-cell preparation, hemoglobin electrophoresis, antiphospholipid antibodies

• Urinalysis

Serial retinal photography can be used to monitor the progression and the resolution of retinal hemorrhages over time.

Retinal fluorescein angiography (FA) can be used to rule out choroidal or retinal neovascularization. Fluorescein angiography also can help identify retinal ischemia or other vasculopathic conditions not associated with Valsalva retinopathy. Finally, angiography can help localize the hemorrhage.

If blood in the vitreous is obstructing the view of the retina, B-scan ultrasonography can be used to rule out a retinal break, tumor, or retinal detachment as cause of the vitreous hemorrhage.

Optical coherence tomography (OCT) can be used to better localize the perimacular or premacular hemorrhage (eg, subhyaloid, sub–internal limiting membrane).[8]

Blood pressure measurement is an essential ancillary test to rule out hypertension as a predisposing risk factor.

Preretinal hemorrhages often are located just under the internal limiting membrane and on the surface of the nerve fiber layer. Vitreous hemorrhage and subhyaloid hemorrhage can be seen in this condition. The hemorrhage tends to arise from the superficial capillary bed. As the hemorrhage resolves over time, the blood typically settles at the inferior aspect of the internal limiting membrane in a D-shaped pattern. Very specific color changes are associated with resolution: red to yellow and yellow to white. Upon complete resolution of the hemorrhage, retinal function typically is unaffected.

Valsalva retinopathy has a predilection for the macula. The perifoveal capillary bed presumably is targeted because of its detailed structural architecture.

Conservative medical treatment is observation. Preretinal hemorrhage (sub–internal limiting membrane and subhyaloid) due to Valsalva retinopathy usually resolves within a few weeks or months. Vitreous hemorrhage may take longer to resolve, depending on the density of the hemorrhage, possibly up to 6 months.[9]

Patients should be instructed to avoid anticoagulant medications and strenuous activities to prevent a rebleed.

Patients should be instructed to sleep in a sitting position to promote blood settling, which may improve visual acuity. However, this effect may be transient upon resumption of physical activities.

Stool softeners may need to be considered for those with constipation.

Although there is no widely accepted treatment modality other than observation, Nd:YAG laser membranotomy and Krypton laser membranotomy have been described for the treatment of large (>3 disc diameters in size) macular subhyaloid hemorrhages of less than 3 weeks' duration. The membranotomy causes immediate drainage of the hemorrhage into the vitreous cavity, which causes the blood to settle into the inferior vitreous and out of the visual axis, prompting a rapid return of central visual acuity. Pulsed Nd:YAG lasers, krypton lasers, argon lasers, Q-switched Nd:YAG lasers, and frequency doubled Nd:YAG lasers all have been used for disruption of the posterior hyaloid or the internal limiting membrane.[10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21]

The location of the membranotomy should be chosen away from the fovea and major blood vessels, at the inferior edge of the hemorrhage, in an area with sufficient underlying hemorrhage present to protect the retina from laser-induced damage. Complications of such maneuvers include the following: retinal tears; hemorrhaging into the choroidal, subretinal and vitreous spaces; retinal detachment; and permanent visual loss. Pressure applied to the eye (with a contact lens, with a Honan balloon, or digitally) may promote clotting in laser-induced hemorrhaging.

If there is a concomitant or underlying retinal disease that requires immediate attention, a membranectomy can be considered to allow thorough evaluation of the underlying retina.

A membranotomy is a particularly useful procedure in those individuals with poor vision in their fellow eye or in patients who require rapid restoration of their vision to continue work.

If a dense vitreous hemorrhage or significant premacular hemorrhaging is present (and no improvement with initial observation), pars plana vitrectomy may be required. 

A consultation with a retinal specialist is recommended to better diagnose and treat the underlying problem and to evaluate any other concomitant retinal pathology contributing to the disease process.

Diet restrictions are not essential in the management of Valsalva retinopathy. A diet rich in fiber is advisable for those patients with constipation in order to prevent further Valsalva maneuvers that possibly could cause a rebleed.

To reduce the risk of a rebleed, physical activity should be limited immediately after the clinical diagnosis and until the retina has sufficiently healed.

Individuals with known proliferative diabetic retinopathy (or other retinal vascular diseases) are at increased risk for the development of a vitreous hemorrhage secondary to a Valsalva maneuver; therefore, they always should try to limit activities that cause sudden increases in intrathoracic pressure against a closed glottis.

Patients should be advised to sleep in an upright sitting position to promote settling of the blood inferiorly out of the visual axis.

Avoid Valsalva-like maneuvers, if possible. 

To prevent a rebleed, physical activity should be limited immediately following detection until the retina has healed.

A medical workup, as suggested by the individual's history and physical examination, to look for predisposing factors, may be helpful in detecting underlying diseases or contributing causes that are preventable or treatable.

Depending on the magnitude of the retinopathy, various follow-up schedules may be used accordingly.

Typically, for those patients who are being observed, follow-up care is at 1 week, 1 month, and 3 months following the initial incident. Wide variations in the timing and the frequency of follow-up care, depending on the location, the severity, and the underlying cause of the hemorrhage, are not uncommon.

For those patients who have undergone a laser membranotomy, follow-up care usually is arranged at 24 hours, 1 week, 1 month, 3 months, 6 months, 12 months, and 18 months. This schedule may vary depending on individual circumstances.

If the patient requires a pars plana vitrectomy, standard postoperative care is instituted.

Inpatient care may be required if indicated by the medical workup.[22]

No proven medical therapy is available for Valsalva retinopathy. The underlying risk factors contributing to the disease development should be treated.