Anesthetic challenges in pediatric moyamoya disease: A report of two cases.

Moyamoya disease (MMD), a rare cause of pediatric stroke, is a cerebrovascular occlusive disorder resulting from progressive stenosis of the distal intracranial carotid arteries and their proximal branches. In response to brain ischemia, there is the development of basal collateral vessels, which gives rise to the characteristic angiographic appearance of moyamoya (puff of smoke). If left untreated, the disease can result in overwhelming permanent neurological and cognitive deficits. Whereas MMD refers to the idiopathic form, moyamoya syndrome refers to the condition in which children with moyamoya also have a recognized clinical disorder. The classic pediatric presentation in moyamoya is recurrent transient ischemic attacks (TIAs) and/or completed/repeated ischemic strokes. Surgical revascularization, including direct and indirect techniques, remains the mainstay of treatment and has been shown to improve long-term outcome in children with MMD. Various risk factors identified for perioperative complications are as follows: history of TIAs, severity of disease, intraoperative hypotension, hypercapnia and hypovolemia, and substantial reduction in hematocrit intraoperatively. Thus, providing perianesthetic care to pediatric patients undergoing revascularization procedure for MMD is like walking a tightrope, and we present two such cases handled successfully.