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.
- Brain herniation
- Raised intracranial pressure