Ss. Rengachary et al., CRANIOCEPHALIC DISPROPORTION WITH INCREASED INTRACRANIAL-PRESSURE ANDBRAIN HERNIATION - A NEW CLINICAL SYNDROME IN ANEMIC PATIENTS - REPORT OF 2 CASES, Neurosurgery, 41(1), 1997, pp. 297-303
OBJECTIVE AND IMPORTANCE: We describe a new clinical syndrome in two p
atients with chronic anemia. The major manifestation of the syndrome i
s herniation of the brain resulting in death caused by longstanding cr
aniocephalic disproportion. The disproportion was caused by extreme th
ickening of the cranium because of erythroid hyperplasia. CLINICAL PRE
SENTATION: Two patients with known chronic anemia presented with chron
ic increase in intracranial pressure with acute deterioration resultin
g in brain herniation. INTERVENTION: Despite maximum medical therapy,
both patients died as a result of uncontrollable increase in intracran
ial pressure. CONCLUSION: Patients with chronic anemia presenting with
progressive headaches should be monitored for this newly described cl
inical phenomenon.