Uneventful epidural labor analgesia and vaginal delivery in a parturient with Arnold-Chiari malformation type I and sickle cell disease.

Arnold-Chiari malformation is a disorder of the hindbrain which can lead to altered craniospinal pressures and abnormal flow of cerebrospinal fluid. The possibility of increased intracranial pressure imparts significant risk during labor and delivery, and has led to concern over the use of neuraxial anesthesia. Sickle cell disease is a disorder of abnormal hemoglobin that is prone to sickling under stressful conditions. The physiologic and metabolic changes associated with pregnancy and labor can precipitate sickling, which increases risks for both the mother and the fetus. Vaso-occlusive pain crisis in a parturient with sickle cell disease has been shown to improve with the initiation of neuraxial anesthesia. We present the first reported case of a parturient with both Arnold-Chiari malformation type I and sickle cell disease who presented to labor and delivery with acute pain crisis and who subsequently received epidural labor analgesia and underwent successful vaginal delivery. We include a discussion of the risks associated with pregnancy, labor, neuraxial anesthesia, and delivery in a patient with Arnold-Chiari malformation type I and sickle cell disease.