March 2017

Case of the Month


Author: Simon Nothling

Editor: Adrian Fung

 

Figure 1. Colour fundus photograph of the left eye shows a well-circumscribed dark patch at the fovea.

Figure 1. Colour fundus photograph of the left eye shows a well-circumscribed dark patch at the fovea.

 

A 21-year-old female was referred with a dark patch at her left macula.

 

Case History

A 21-year-old professional female rugby player presented with a 1-day history of  painless vision loss and central scotoma in her left eye.  She had no other neurological symptoms such as headache or limb paraesthesiae.  The day before she had been performing some intense scrummaging at training, as well as some heavy weight-lifting, which was slightly more than normal.  She denied any coughing or other straining recently.  She had no ophthalmic history and was well medically. She denied being pregnant.

On examination, her visual acuities were 6/5-1 right eye (OD) and 6/24-1 left eye (OS) unaided, with no improvement with pinhole.  Intraocular pressures were 15mmHg (OD) and 16mmHg (OS).  Anterior segment examination was normal in both eyes.  Blood pressure was 120/75. Colour fundus photographs reveal a left well-circumscribed, dome-shaped dark patch at the fovea (Figure 1). The right fundus was normal.

 

What is your diagnosis?

 

Differential diagnosis

The differential diagnosis of a dark patch at the macula includes:

  • Secondary choroidal neovascular membrane (CNV, e.g. secondary to choroidal reupture)
  • Myopic choroidal neovascular membrane
  • Valsava retinopathy
  • Ruptured macroaneurysm
  • Diabetic retinopathy
  • Traumatic Macula hole
  • Terson’s Syndrome
  • Posterior vitreous detachment

 

Additional examination and investigations

Optical coherence tomography (OCT) of left macula revealed a central, well-circumscribed sub-ILM hyper-reflective mass, thought to be haemorrhage (Figure 2).   The retinal pigment epithelium appeared normal. Bloods taken for diabetic and coagulopathy screening were negative.

 

Figure 2. A sub-ILM hyper-reflective mass is seen at the fovea.

Figure 2. A sub-ILM hyper-reflective mass is seen at the fovea.

Diagnosis

Valsalva Retinopathy.

 

Clinical Course

Therapeutic options for Valsalva retinopathy include conservative management, Nd:YAG laser membranotomy and surgery (vitrectomy). As the haemorrhage was small, and unilateral, conservative management was chosen. The patient was told to avoid strenuous exercise, straining and sexual activities for at least 2 weeks.  In particular, she was told to miss normal rugby training during this time. She was also told to avoid NSAIDs (such as Neurofen), which might increase bleeding. After 2 weeks she noticed her vision beginning to improve and was able to return to light training. By 4 weeks most of the haemorrhage had cleared and the scotoma was barely noticeable, so she returned to playing  contact rugby and competition.  At 6 weeks, her vision was 6/6, and her fundoscopy findings had returned to normal (Figures 3 and 4).

 

Figure 3. Six weeks later the macula haemorrhage had resolved with conservative management. There was no deep retinal or choroidal pathology visible once the blood had cleared.

Figure 3. Six weeks later the macula haemorrhage had resolved with conservative management. There was no deep retinal or choroidal pathology visible once the blood had cleared.

Figure 4. At 6 weeks, OCT of the left eye confirmed resolution of the haemorrhage, and normal recovery of the foveal and macula anatomy.

Figure 4. At 6 weeks, OCT of the left eye confirmed resolution of the haemorrhage, and normal recovery of the foveal and macula anatomy.

Discussion

Valsalva retinopathy was first described in 1972 by Thomas Duane, as a particular form of retinopathy, preretinal and haemorrhagic in nature, secondary to a sudden increase in intrathoracic pressure.1 Increasing intrathoracic pressure against a closed glottis may cause a rapid increase in venous pressure with spontaneous rupture of perifoveal retinal capillaries, leading to sudden painless loss of vision. The haemorrhage is usually located below the internal limiting membrane (ILM), but can occasionally break through to become a subhyaloid or intravitreal haemorrhage.2

Valsalva retinopathy often occurs in healthy young adults. Described causes include: heavy lifting, constipation (straining on the toilet), vomiting, coughing, sneezing, labours, automobile air-bag related trauma, vigorous sexual activity, vigorous dancing, bungee jumping, colonoscopies and continuos positive pressure ventilation.4 In this patient, the weight lifting at training was the likely causative action. Valsalva retinopathy usually occurs in otherwise healthy eyes, but occasionally it can be associated with acquired retinal conditions such as diabetic retinopathy or hypertensive retinopathy, or congenital conditons such as telangiectasias or retinal artery tortuosity.

The sub-ILM haemorrhage is usually a well-circumscribed, round or bilobed red elevation, causing haemorrhagic detachment of the ILM, as was seen in this patient.6 OCT scans can be used to differentiate between sub-ILM and subhyaloid haemorrhage.  The ILM on OCT has a higher reflective band compared with the lower reflectivity of the posterior hyaloid.5

Most cases improve with observation alone.  As in this case, patients are recommended to avoid strenuous activity and anti-platelet drugs (aspirin, NSAIDs, alcohol).  Most cases resolve within 1-3 months7 with full visual recovery.6 In more severe cases. Nd:YAG laser membranotomy or vitrectomy maybe considered , particularly in large haemorrhages over 3 disc diameters in size,4 or a haemorrhage in an only functioning eye.  Larger haemorrhages that take longer to clear can be toxic to the retina and result in permanent visual changes, thus intervention should be considered if the blood is persisting.

 

Take home points

  • Valsalva retinopathy should be considered as a cause of premacular haemorrhage in young, healthy patients.
  • Risk factors include excessive coughing, strenuous physical or sexual activity, vomiting and straining.
  • The haemorrhage is usually well circumscribed, centred on the macula and sub-ILM on OCT.
  • Most cases resolve fully with observation alone within 1-3 months. Laser hyaloidotomy and vitrectomy can be considered in severe cases or patients with only one functioning eye.

 

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References:

  1. Duane TD. Valsava hemorrhagic retinopathy. Trans Am Ophthalmol Soc. 1972. 70: 298-313.
  2. Ryan SJ, Schachat AP, Sadda S. 2013; Retina; 5th Edition; volume 2: 1569
  3. Khada et al. BMC Ophthalmology (2016) 16:41
  4. AhmedAbadi MN, Karkaneh R, Mirshani A. Premacular Hemorrhage in Valsava Retinopathy: A Study of 21 Cases. Iranian Journal of Ophthalmology 2009;21(3): 11-16
  5. Shukla D et al. Optical Coherence Tomography Findings in Valsalva Retinopathy. Am J Ophthalmol 2005. 140(1): 134-136
  6. Chapman-Davies A, Lazarevic A. Valsalva maculopathy. Clini Exp Optom 2002; 85(1): 42-45.
  7. Roberts KD, Mackay AK. Microhaemorrhagic maculopathy associated with aerobic exercise. J Am Optom Assoc 1987; 58: 415-418.