Valsalva Retinopathy

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Valsalva Retinopathy


Disease Entity

Valsalva retinopathy

  • ICD-9-CM 362.10 Background retinopathy, unspecified

Disease

Valsalva retinopathy is a preretinal hemorrhage caused by a sudden increase in intrathoracic or intraabdominal pressure. It was first described by Duane in 1972 as hemorrhagic retinopathy of Valsalva.[1] Hemorrhage occurs due to spontaneous rupture of superficial retinal capillaries. It can be seen in an otherwise healthy eye and the hemorrhage spontaneously resolves.

Etiology

It occurs after an increase in intrathoracic or intraabdominal pressure due to activities such as

  • coughing,
  • vomiting,
  • lifting,
  • straining for a bowel movement,
  • strenuous exertion,
  • sexual intercourse,
  • labor,
  • blowing musical instruments, and
  • compression injuries.

Risk Factors

It usually occurs in otherwise healthy eyes but may be associated with retinal vascular abnormalities either acquired (diabetic or hypertensive retinopathy) or congenital (retinal telangiectasias and congenital retinal artery tortuosity). [2][3]

General Pathology

Rupture of small superficial capillaries in the macula leads to extravasation of blood into the retina, usually under the internal limiting membrane (ILM), but may also bleed to the vitreous cavity (due to a breakthrough bleed via a rupture in the ILM) or subhyaloid space.[4]

Pathophysiology

A sudden rise in intrathoracic or intraabdominal pressure against a closed glottis, the Valsalva’s maneuver reduces venous return to the heart and stroke volume, consequently increasing venous system pressure. The raise in venous pressure specially affects the upper part of the body, increasing intraocular venous pressure and subsequent rupture of small superficial capillaries of the macula. This results in unilateral or bilateral retinal hemorrhages.

Diagnosis

Diagnosis is primarily based on history and physical examination.

History

Sudden, painless visual loss or scotoma after a Valsalva’s maneuver. It is usually unilateral, although may rarely be bilateral.

Physical examination

Visual acuity is variable depending on the location, size and degree of the preretinal hemorrhage. Slit lamp examination of the anterior segment is usually normal, although subconjunctival hemorrhages could be present. Fundus examination reveals a preretinal hemorrhage typically located in the premacular area, below the internal limiting membrane, but may show break through to the sub hyaloid or intravitreal space. Hemorrhages vary in size. Blood located under the internal limiting membrane usually appears a well-circumscribed, round or dumbbell-shaped red elevation, causing a hemorrhagic detachment of the internal limiting membrane.[5] Dissection of the blood beneath the retina may occur if bleeding occurs in the foveal region. Choroidal hemorrhage, although rare, has been described. The blood is initially bright red, but turns yellow/white after several days to weeks. A fluid level may be seen. Serous detachment may persist up to weeks until complete resolution.[6] A double ring sign has been described which is characterized by the separate margins of sub hyaloid hemorrhage (outer ring) and sub-ILM hemorrhage (inner ring).[7]

Symptoms

Visual acuity is variably affected. It gradually improves with resolution of the hemorrhage, edema and detachment, at most often returns to baseline.

Diagnostic procedures

Optical coherence tomography may be used to determine the location of the hemorrhage (subhyaloid, sub-ILM, etc). [8] Retinal fluorescein angiography can be used to rule out retinal neovascularization or other active leakage, if suspected.

Laboratory test

Although usually not required, lab tests can be performed to rule out baseline pathology such as diabetes, sickle cell disease, or other blood dyscrasias.

Differential diagnosis

  • Posterior vitreous detachment
  • Diabetic retinopathy
  • Hypertensive retinopathy
  • Sickle cell retinopathy
  • Purtscher retinopathy
  • Terson’s syndrome
  • Ruptured macroaneurysm
  • Intraocular parasite

Management

General treatment

Conservative management is observation of spontaneous resolution, which occurs within weeks to months. Advise patients to avoid anticoagulant drugs and strenuous physical activity.

Medical therapy

No medical therapy has proven benefit.

Surgery

Neodymium:YAG laser or krypton laser membranotomy can be options for large hemorrhage especially if it occurs in the patient’s only normally functioning eye. Laser membranotomy disrupts the ILM or posterior hyaloid leading to drainage of the blood into the inferior vitreous cavity, producing a faster resolution. Complications associated with laser membranotomy include macular hole, retinal detachment, and epiretinal membrane formation.[9][10][11]

In rare cases of premacular hemorrhage, which do not resolve after reasonable duration of observation, pars plana vitrectomy with removal of ILM and the sub-ILM bleed is an option.

Prognosis

Good prognosis with complete recovery to baseline vision usually occurs after resolution occurring within weeks to months after onset.

References

  1. Duane TD. Valsalva hemorrhagic retinopathy. Trans Am Ophthalmol Soc 1972;70:298–313.
  2. Kassoff A, Catalano RA, Mehu M. Vitreous hemorrhage and the Valsalva maneuver in proliferative diabetic retinopathy. Retina.1988;8(3):174-6.
  3. de Crecchio G, Pacente L, Alfieri MC, Greco GM. Valsalva retinopathy associated with a congenital retinal macrovessel. Arch Ophthalmol. 2000 Jan;118(1):146-7.
  4. Celik Dulger S, Ozdal PC, Teke MY. Valsalva retinopathy: Long-term results and management strategies. Eur J Ophthalmol. 2021 Jul;31(4):1953-1960. doi: 10.1177/1120672120936175. Epub 2020 Jun 25. PMID: 32586109.
  5. Tildsley J, Srinivasan S. Valsalva retinopathy. Postgrad Med J 2009;85:110.
  6. Agarwall A. Gass’ Atlas of Macular Disease. 5th ed. Elsevier; 2012. Chapter 8, 730-731. 
  7. Sakamoto SI, Makino S, Tampo H. Double ring sign at the macula in a patient with Valsalva retinopathy. QJM. 2014;107(12):1045‐1046. doi:10.1093/qjmed/hcu102
  8. Shukla D, Naresh KB, Kim R. Optical coherence tomography findings in Valsalva retinopathy. Am J Ophthalmol 2005;140:134–6.
  9. Gabel VP, Birngruber R, Gunther-Koszka H, Puliafito CA. Nd:YAG laser photodisruption of hemorrhagic detachment of the internal limiting membrane. Am J Ophthalmol. 1989 Jan 15;107(1):33-7.
  10. Sahu DK, Namperumalsamy P, Kim R, Ravindran RD. Argon laser treatment for premacular hemorrhage. Retina. 1998;18(1):79-82.
  11. Leite J, Meireles A, Correia NA. Valsalva Retinopathy after a Vomiting Episode. Case Rep Ophthalmol. 2022 Sep 30;13(3):706-710.
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