Craniocervical arterial dissection in children: clinical and radiographic presentation and outcome

Craniocervical arterial dissection is a recognized cause of arterial ischemic stroke in children. Whether children with craniocervical arterial dissection have dissection characteristics different from those of adults is unclear. A retrospective review of children, 1 month to 18 years of age, with dissection from two Canadian pediatric ischemic stroke registry centers was conducted. From 213 patients with arterial ischemic stroke, 16 (7.5%) were identified with dissection, 37.5% had warning symptoms, and 50% had a history of head or neck trauma. The clinical presentation included headache (44%), altered consciousness (25%), seizures (12.5%), and focal deficits (87.5%). Dissection involved extracranial vessels in 75% and anterior circulation in 56%. Follow-up included complete recovery in 43%, mild to moderate deficits in 44%, and severe deficits in 13%. Fourteen (87.5%) children received antithrombotic treatment. Follow-up angiography showed resolution of abnormalities in 60% of vessels. Total occlusion had the worst outcome for recanalization. In conclusion, the etiology of arterial dissection in the majority of children appears to be either trauma or idiopathic. Long-term angiography shows variable outcomes, depending on the initial findings. The relationship of angiographic outcomes with recurrent strokes requires further study in pediatric dissection. (J Child Neurol 2006;21:8-16).     

Hypomelanosis of Ito and Moyamoya disease

Moyamoya disease is a cerebrovascular disease characterized by stenosis and occlusion of the arteries of the circle of Willis, with abnormal telangiectatic collateral circulation at the base of the brain. An association between moyamoya disease and neurofibromatosis 1, a neurocutanoeus disorder, is well established in the literature. However, its association with other neurocutaneous syndromes is infrequently reported. Hypomelanosis of Ito, another neurocutaneous syndrome, is characterized by macular hypopigmented skin whorls and variable neurologic involvement. Only one case study of an association between hypomelanosis of Ito and moyamoya disease has been reported in the English literature. We report a 17-year-old girl with both hypomelanosis of Ito and angiographic moyamoya disease. She presented with intractable seizures, progressive left hemiparesis, and skin manifestations of hypomelanosis of Ito. Although one might consider a coincidental association, this second case points to an association between the two disorders. Detailed neuroimaging, in particular angiography, should be considered in children with hypomelanosis of Ito and abnormal neurologic findings.     

Intra-arterial tissue plasminogen activator for thrombosis complicating cerebral angiography in a 17-year-old girl

Few reports describe the use of intraarterial recombinant tissue plasminogen activator to treat intracranial thrombosis in children. A 17-year-old girl with a history of prior venous thrombosis developed a left middle cerebral artery thrombus during diagnostic cerebral angiogram. Therapy with intra-arterial tissue plasminogen activator was initiated. An immediate follow-up angiogram demonstrated recanalization, and diffusion-weighted magnetic resonance imaging 9 hours later showed no evidence of infarction. Following the angiogram, femoral artery thrombosis developed. Treatment with supratherapeutic levels of heparin, localized delivery of intra-arterial tissue plasminogen activator, embolectomy, danaparoid, and dipyridamole failed to re-establish perfusion to the lower leg, and below the knee amputation was required. Neurologic examination remained normal 1 year later. Cerebral damage was avoided with the use of emergency intra-arterial tissue plasminogen activator for cerebral artery thrombosis in this child.     

Peripheral ivory osteoma of the mandible in a young female patient

Osteoma is an uncommon benign neoplasm composed of mature bone. Growth is slow and continuous and located principally in the cranio‐maxillo‐facial region, and can be central (endosteal) or peripheral (periosteal). Osteomas can be solitary or multiple masses, and they are generally asymptomatic. We discuss a case of ivory osteoma of the mandible in a 35‐year‐old female, which was present at the left body of the mandible since she was 10 years old, and was gradually increasing in size. The osteoma was removed surgically through an intraoral approach, and no recurrence was observed.     

The Pediatric Stroke Recurrence and Recovery Questionnaire: Validation in a prospective cohort

OBJECTIVE: We developed the Recurrence and Recovery Questionnaire (RRQ) by converting the Pediatric Stroke Outcome Measure (PSOM) to a questionnaire for telephone interview and sought to validate the RRQ in a large cohort. METHOD: We analyzed parents' RRQ responses and same-day PSOM assessments for 232 children who had arterial ischemic stroke, cerebral sinovenous thrombosis, or presumed perinatal ischemic stroke. We assessed the agreement and consistency of the PSOM and RRQ, and we identified conditions that contributed to differences between the 2 measures. We tested selected factors as predictors of differences between the total PSOM and total RRQ (tPSOM and tRRQ) scores. RESULTS: Median PSOM score was 1.5 and median RRQ score was 1.5. There was good agreement between tPSOM and tRRQ, and RRQ was a reliable estimator of PSOM at the total and component level. Preexisting neurologic deficits or chronic illnesses increased the difference between the tPSOM and tRRQ; the chronic illness effect was confirmed with univariate analysis. CONCLUSIONS: The RRQ can characterize poststroke function when a child cannot return for examination. While the RRQ is not identical to the PSOM, the 2 measures likely assess closely related aspects of recovery. The RRQ is particularly useful when assessing outcomes of large cohorts, and will be useful in performing long-term follow-up studies of pediatric stroke.