Headache and papilledema in an adult with cyanotic congenital heart disease: the importance of fundoscopic evaluation rather than phlebotomy

Congenit Heart Dis. 2012 May-Jun;7(3):E14-7. doi: 10.1111/j.1747-0803.2011.00586.x. Epub 2011 Nov 9.

Abstract

Headaches and blurred vision in patients with cyanotic congenital heart disease and secondary erythrocytosis may be attributed to hyperviscosity and traditionally were treated with phlebotomy. In the current era, phlebotomy is rarely performed in these patients except in cases of hemoptysis or hyperviscosity symptoms. We report a case of a patient with a history of complex cyanotic congenital heart disease and secondary erythrocytosis who presented with headache and visual changes. He was found to have bilateral papilledema and increased intracranial pressure. Reduction of intracranial pressure with acetazolamide therapy led to alleviation of headache and visual changes. This demonstrates the need for formal ophthalmologic evaluation of these patients to assess other treatable causes of headache and visual changes before considering phlebotomy.

Publication types

  • Case Reports

MeSH terms

  • Acetazolamide / therapeutic use
  • Carbonic Anhydrase Inhibitors / therapeutic use
  • Cyanosis / etiology*
  • Headache / diagnosis*
  • Headache / drug therapy
  • Headache / etiology
  • Headache / physiopathology
  • Heart Defects, Congenital / complications*
  • Humans
  • Intracranial Hypertension / diagnosis*
  • Intracranial Hypertension / drug therapy
  • Intracranial Hypertension / etiology
  • Intracranial Hypertension / physiopathology
  • Intracranial Pressure / drug effects
  • Male
  • Ophthalmoscopes*
  • Ophthalmoscopy*
  • Papilledema / diagnosis*
  • Papilledema / drug therapy
  • Papilledema / etiology
  • Papilledema / physiopathology
  • Phlebotomy*
  • Polycythemia / etiology
  • Predictive Value of Tests
  • Treatment Outcome
  • Unnecessary Procedures
  • Young Adult

Substances

  • Carbonic Anhydrase Inhibitors
  • Acetazolamide