Moyamoya: to cut or not to cut is not the only question. A paediatric neurologist’s perspective

Abstract

The paediatric neurologist is usually the clinician who makes the diagnosis of moyamoya in children, yet most of the debate in the literature has focused on surgical management of the condition. Semantic confusion and variable use of the term among neuroradiologists continues to be unhelpful. Increasing recognition of moyamoya, for example in sickle cell disease, and the publication of clinical guidelines encouraging referral for surgical evaluation highlight the need to identify and engage with management of the condition. In practical terms, the most frequent management issues for the paediatric neurologist, other than when to refer for surgery, are headache, hypertension, and the concern of the family that other children might be affected. These issues are discussed in the context of the available literature, and areas in which there is a need for research and consensus are identified.

Headache in Caucasian patients with Moyamoya angiopathy – a systematic cohort study

“…Few hypotheses concerning the headache pathophysiology by MMA have been discussed so far (2,9). Dilated leptomeningeal collaterals stimulating dural nociceptors was suggested as a possible origin of headache (2).…”

Spontaneous occlusion of the circle of Willis in children: pediatric moyamoya summary with proposed evidence-based practice guidelines

“…Particular note should be made of the practice of using calcium channel blockers to ameliorate headache, because they can be very effective, but they can also potentially increase the risk of stroke through their propensity to induce hypotension. 16 Perioperative management of moyamoya in children requires coordinated care between the anesthesia, ICU, nursing, neurosurgery, and affiliated medical teams (such as hematology in patients with sickle cell disease). Evidence indicates that hyperventilation-as occurs in crying-can increase the risk of stroke in the perioperative period, supporting the use of measures to decrease pain (appropriate analgesia, use of absorbable sutures, sedation for potentially painful procedures) and reduce psychological stressors in children.…”

Diagnosis and management of uncommon and genetic cerebrovascular diseases

“…Safety and long-term benefit are as yet not established. Many other nonsurgical treatment dilemmas exist in this condition with little to no evidence on which to base decisions (89). Chronic and progressive problems with headache and hypertension are common.…”