BMJ Case Reports 2015; doi:10.1136/bcr-2015-211573

Fluctuating nature of an orbital venous-lymphatic anomaly in association with intracranial vascular malformations: a classical presentation

  1. Monica Smith Pearl3,4
  1. 1Department of Ophthalmology, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
  2. 2Johns Hopkins Hospital, Baltimore, Maryland, USA
  3. 3Department of Radiology, Johns Hopkins Hospital, Baltimore, Maryland, USA
  4. 4Department of Radiology, Children's National Medical Center, Washington DC, USA
  1. Correspondence to Dr Monica Smith Pearl, msmit135{at}
  • Accepted 16 September 2015
  • Published 5 October 2015


Venous-lymphatic anomalies (VLA) are rare and benign congenital lesions of the lymphatic system, composed of endothelial-lined lymphatic cysts. They are most frequently located in the region of the head and neck, and represent 4% of all orbital masses. In those patients with extensive orbital VLAs, a strong association with intracranial vascular anomalies has been reported. Factors known to suddenly increase the size of these lesions include upper respiratory tract infections or intralesional haemorrhage; however, complete spontaneous regression is rare. We report on the classic presentation of a patient with a fluctuating right orbital VLA in association with an intracranial cavernous malformation and intracranial developmental venous anomaly.

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