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A rare but important differential diagnosis in transient monocular blindness
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  1. Raman Mehrzadl,
  2. Rishi Bajaj2
  1. 1Department of Medicine, Steward Carney Hospital, Tufts University School of Medicine, Boston, Massachusetts, USA
  2. 2Department of Cardiology, Saint Vincent Hospital, University of Massachusetts Medical School, Worcester, Massachusetts, USA
  1. Correspondence to Dr Raman Mehrzad, raman_m1{at}hotmail.com

https://doi.org/10.1136/bcr-2014-206812

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    Description

    A 45-year-old man presented with transient episodes of monocular blindness. Physical examination, including full neurology and funduscopy examination was unremarkable. Head CT was negative for intracranial bleed. Carotid ultrasound with Doppler was unremarkable. A two-dimensional echocardiogram was performed and showed a mobile mass attached to the aortic valve. To further evaluate the mass, a transesophageal echocardiography (TEE) was performed which showed a multilobulated, mobile, pedunculated mass attached to the aortic surface of the non-coronary aortic cusp, measuring 1.5 cm×0.4 cm without any valvular involvement (figure 1 and video 1). Via thoracotomy, the mass was surgically excised. Aortic valve competence was confirmed intraoperatively before the aortotomy was closed. Gross examination showed a pedunculated mass with frond-like projections and filamentous endings, appearing like a ‘sea anemone’ (figure 2). Histopathology revealed branching avascular papillae with single layer of endothelium, confirming the diagnosis of papillary fibroelastoma (PFE; figure 3). No symptomatic recurrence or evidence of regurgitation was seen on follow-up at 6 months.

    Figure 1
    Figure 1

    Transesophageal echocardiographic image of aortic valve.

    Figure 2
    Figure 2

    Gross examination showing pedunculated mass with frond like projections and filamentous endings appearing as ‘sea anemone’.

    Figure 3
    Figure 3

    Histopathological examination showing avascular branching papillae covered by single layer of endothelium in a mucopolysaccharide matrix.

    Video 1

    TEE showing a multilobulated, mobile, pedunculated mass attached to the aortic surface of the noncoronary aortic cusp.

    PFEs are rare cardiac tumours, but should be differentiated from thrombus, vegetation, myxoma and Lambl's excrescence.1 Although patients are typically asymptomatic, PFEs can potentially lead to severe complications, primarily due to their embolic potential. A TEE most often helps in making a presumptive diagnosis.2 Surgical excision is curative and is indicated in symptomatic patients, lesions larger than 1 cm, and in patients with left-sided lesions.3 Our case underscores the importance of PFE in the differential diagnosis of transient blindness, especially in young patients without significant medical histories.

    Learning points

    • Papillary fibroelastoma are rare cardiac tumours, typically found on the heart valves, that has the embolic potential.

    • A transesophageal echocardiography will help diagnose them and they should be differentiated from thrombus, vegetation, myxoma and Lambl's excrescence.

    • Surgical excision is curative and is indicated in symptomatic patients, lesions larger than 1 cm, and in patients with left-sided lesions.

    References

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      1. Aryal MR,
      2. Badal M,
      3. Mainali NR, et al
      . Papillary fibroelastoma of the aortic valve: an unusual cause of angina. World J Cardiol 2013;5:102–5.
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    2. ↵
      1. Fine NM,
      2. Foley DA,
      3. Breen JF, et al
      . Multimodality imaging of a giant aortic valve papillary fibroelastoma. Case Rep Med 2013;2013:705101.
      OpenUrlPubMed
    3. ↵
      1. Zhang M,
      2. Liu X,
      3. Song Z, et al
      . Cardiac papillary fibroelastoma: a retrospect of four cases. J Cardiothorac Surg 2013;8:65.
      OpenUrlCrossRefPubMed
    View Abstract

    Footnotes

    • Contributors Both authors reviewed the scientific literature, interpreted the data, and wrote the manuscript.

    • Competing interests None.

    • Patient consent Obtained.

    • Provenance and peer review Not commissioned; externally peer reviewed.