Case scenario: perioperative administration of tocotrienols and green tea extract in a child with familial dysautonomia.

F AMILIAL dysautonomia (FD, also known as Riley-Day syndrome) is a rare (1:3,700 live births) autosomal recessive disorder, largely afflicting those of Ashkenazi Jewish descent. One of five hereditary sensory and autonomic neuropathies (HSAN, type III) influencing the development and survival of varied sensory, sympathetic, and parasympathetic neurons, FD is characterized by profound autonomic dysfunction resulting in sudden hypertension, orthostatic hypotension, vomiting, and/or abnormal sweating. Autonomic instability may result in significant morbidity and mortality in the perioperative period. Recent discoveries in disease genetics and pathophysiology have lead to improved treatments for FD patients, greatly reducing the incidence of dysautonomic crises and diminishing symptoms overall. Mutations in the gene for inhibitor of light polypeptide gene enhancer in B-cells, kinase complex-associated protein (IKBKAP), causing reductions in the cellular protein IKAP, have been linked to FD. In addition, tissue concentrations of monoamine oxidase A, an enzyme involved in the metabolic transformation of norepinephrine and dopamine, are low in FD. Both tocotrienols (vitamin E family) and epigallocatechin-3-gallate (a flavonoid found in green tea) increase its cellular concentration (fig. 1). Oral tocotrienols also increase monoamine oxidase A levels in FD patients. We describe the management of a child with FD scheduled for adenoidectomy and the impact of maintaining her long-term treatment with tocotrienols and green tea extract (GTE) on her perioperative course.