Discovery of asymptomatic moyamoya arteriopathy in pediatric syndromic populations: radiographic and clinical progression

Abstract

<jats:sec> <jats:title>Object</jats:title> <jats:p>Limited data exist to guide management of incidentally discovered pediatric moyamoya. Best exemplified in the setting of unilateral moyamoya, in which the unaffected side is monitored, this phenomenon also occurs in populations undergoing routine surveillance of the cerebral vasculature for other conditions, such as sickle cell disease (SCD) or neurofibromatosis Type 1 (NF1). The authors present their experience with specific syndromic moyamoya populations to better characterize the natural history of radiographic and clinical progression in patients with asymptomatic moyamoya.</jats:p></jats:sec> <jats:sec> <jats:title>Methods</jats:title> <jats:p>The authors performed a retrospective review of the clinical database of the neurosurgery department at Children's Hospital Boston, including both nonoperative referrals and a consecutive series of 418 patients who underwent surgical revascularization for moyamoya disease between 1988 and 2010.</jats:p></jats:sec> <jats:sec> <jats:title>Results</jats:title> <jats:p>Within the period of time studied, 83 patients were asymptomatic at the time of radiographic diagnosis of moyamoya, while also having either unilateral moyamoya or moyamoya in association with either SCD or NF1. The mean age at presentation was 9.1 years (range 1–21 years), and there were 49 female (59%) and 34 male (41%) patients. The mean follow-up duration was 5.4 ± 3.8 years (mean ± SD), with 45 patients (54%) demonstrating radiographic progression and 37 (45%) becoming symptomatic within this period. Patients with SCD had the highest incidence of both radiographic (15 patients [75%]) and clinical (13 patients [65%]) progression, followed by NF1 (20 patients [59%] with radiographic progression and 15 patients [44%] with clinical progression) and patients with unilateral moyamoya (10 patients [35%] with radiographic progression and 9 patients [31%] with clinical progression).</jats:p></jats:sec> <jats:sec> <jats:title>Conclusions</jats:title> <jats:p>Radiographic progression occurred in the majority of asymptomatic patients and generally heralded subsequent clinical symptoms. These data demonstrate that moyamoya is a progressive disorder, even in asymptomatic populations, and support the rationale of early surgical intervention to minimize morbidity from stroke.</jats:p></jats:sec>

Moyamoya disease with mesial temporal sclerosis

“…There is abundant evidence supporting the role for surgical revascularization of both symptomatic and asymptomatic moyamoya in children with operations such as pial synangiosis. [5][6][7][8] Regarding the MTS, the American Academy of Neurology practice parameter recommends surgery as the treatment of choice for medically intractable temporal lobe epilepsy. 4 The patient's seizures in this case were controlled with medication at the time of diagnosis of moyamoya, but the natural history of MTS suggests that a minority of children will continue to attain seizure control on medication alone, whereas nearly two-thirds of children can experience long-term seizure control after selective temporal lobectomy (as studied in a Class I randomized, controlled trial).…”

“…In addition, there are data to support a role for surgical revascularization of progressive moyamoya in children (even if they are asymptomatic). 6 Coexistence of these 2 distinct pathologies is both interesting and has implications for planning long-term treatment strategies.…”

A Rare Case of Neurofibromatosis Type 1 Associated with Vertebral Arteriovenous Fistula and Moyamoya Syndrome

“…4) Although there are increased risks for subsequent clinical and radiologic worsening, most cases are initially asymptomatic as shown in this present case. 10) The initial symptoms are often ischemic events, such as transient ischemic attacks and completed ischemic strokes. 11) As regard the arterial occlusive lesions in NF-1, it has been suggested that the obliteration of the large-sized arteries is caused by the growth of the neurofibroma or ganglioneuroma in the tunica adventitia, and that of the small-sized arteries including cerebral arteries is caused by the proliferation of the smooth muscle cells.…”

The Maya Behind Moyamoya--The Two Extremes of the Disease : Correspondence

“…In pediatric population, there are multitude of symptoms associated with moyamoya disease, including transient ischemic attack, ischemic strokes, intracranial hemorrhages, seizures, headaches, choreiform movements, and cognitive deficits. Recently researchers have also reported asymptomatic cases of moyamoya and approximately half of them remained clinically asymptomatic over a mean follow up period of 5.4 y [2]. Thus, it is not unusual to see these variable presentations of moyamoya.…”