Nursing issues in caring for children with moyamoya vasculopathy and their families.

Moyamoya vasculopathy is a rare, progressive neurovascular condition that may cause recurrent transient ischemic attacks, ischemic strokes, hemorrhagic strokes, or neurologic decline in children. Children with moyamoya disease have no clear contributing etiology, but children with moyamoya syndrome have contributory diagnoses such as Down syndrome or neurofibromatosis. The concerns and lived experiences of children affected by this disease and their families have not been well explored or addressed in the nursing literature. Nurses who understand this lived experience increase their own knowledge of the disease so they can manage the complex medical issues, educate families about the disease, and provide emotional support.

Pial synangiosis for treatment of Moyamoya syndrome in children.

Moyamoya syndrome is a chronic cerebrovascular disorder that is characterized by a progressive narrowing of the intracranial internal carotid arteries and their main branches and a compensatory dilation of smaller arteries (ie, moyamoya vessels) at the base of the brain. Common signs and symptoms in children include recurrent episodes of cerebral ischemia, transient hemiparesis, seizures, and cerebrovascular accidents (CVAs). The diagnosis is made primarily by cerebral angiography, and surgical treatment consists of pial synangiosis, whereby surgeons suture the superficial temporal artery to the pla mater under microscopic visualization. The goal of surgical intervention is to promote the development of collateral circulation to the brain and decrease the risk of CVAs, transient ischemic attacks, and seizures. Follow-up angiography demonstrates that pial synangiosis results in excellent postoperative collaterization of ischemic areas of the brain.