Craniocephalic disproportion with increased intracranial pressure and brain herniation: A new clinical syndrome in anemic patients: Report of two cases

Setti S. Rengachary, Jeffrey Blount, Deborah Heros, Susan Bowers, Charles Truwit

Research output: Contribution to journalArticlepeer-review

14 Scopus citations

Abstract

OBJECTIVE AND IMPORTANCE: We describe a new clinical syndrome in two patients with chronic anemia. The major manifestation of the syndrome is herniation of the brain resulting in death caused by longstanding craniocephalic disproportion. The disproportion was caused by extreme thickening of the cranium because of erythroid hyperplasia. CLINICAL PRESENTATION: Two patients with known chronic anemia presented with chronic increase in intracranial pressure with acute deterioration resulting in brain herniation. INTERVENTION: Despite maximum medical therapy, both patients died as a result of uncontrollable increase in intracranial pressure. CONCLUSION: Patients with chronic anemia presenting with progressive headaches should be monitored for this newly described clinical phenomenon.

Original languageEnglish (US)
Pages (from-to)297-304
Number of pages8
JournalNeurosurgery
Volume41
Issue number1
DOIs
StatePublished - Jul 1 1997

Keywords

  • Anemia
  • Brain herniation
  • Raised intracranial pressure
  • Thalassemia

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