Bilateral pial synangiosis in a child with PHACE syndrome.

The acronym PHACE has been used to denote a constellation of abnormalities: posterior fossa anomalies, facial hemangiomas, arterial anomalies, cardiac anomalies, and eye abnormalities. Approximately 30% of patients with large facial hemangiomas have PHACE syndrome, with the vast majority having intracranial arteriopathy. Few reports characterize neurological deterioration from this intracranial arteriopathy, and even fewer report successful treatment thereof. The authors report on a case of a child with PHACE syndrome who presented with an ischemic stroke from a progressive intracranial arteriopathy and describe her successful treatment with bilateral pial synangiosis. An 8-month old girl diagnosed with PHACE syndrome was found to have bilateral internal carotid artery stenosis. Although initially asymptomatic, a few months after diagnosis she suffered a right frontal and parietal stroke. MRI and cerebral angiography investigations demonstrated progressive intracranial arterial stenosis and occlusion. The patient then underwent indirect cerebral revascularization surgery. At 2-year follow-up, she exhibited clinical improvement with persistent speech and motor developmental delay. Follow-up MRI and cerebral angiography showed no new ischemic events and robust extensive vascular collateralization from surgery. PHACE syndrome is an uncommon disease, and affected patients often have cerebral arteriopathy. Although the underlying natural history of cerebral arteriopathy in PHACE remains unclear, cerebral revascularization may represent a potential therapy for symptomatic patients.

Pediatric complicated pneumonia and pneumococcal serotype replacement: trends in hospitalized children pre and post introduction of routine vaccination with Pneumococcal Conjugate Vaccine (PCV7).

Recent studies have described an increase in the incidence of complicated pneumonia in children, primarily caused by Streptococcus pneumoniae. The objective of this study was to determine if the incidence of complicated pneumonias in total and due to different pneumococcal serotypes has changed following the introduction of routine immunization with heptavalent pneumococcal conjugate vaccine (PCV7). A retrospective review of patients admitted to the Stollery Children's Hospital in Edmonton, Alberta with complicated pneumonia between July 1, 1997 and June 30, 2007 (5 years before and after the introduction of PCV7) was completed. There were 34 children in the pre- and 68 in the post-PCV7 era (14.31 and 19.91 per 10,000 discharges, respectively, p = 0.114). Patient characteristics were not significantly different, and pneumococcus was the most common organism isolated (pre: 21% (7/34); post: 26% (18/68), p = 0.515). In patients where serotype data was available, non-vaccine pneumococcal serotypes accounted for 67% (12/18) cases in the post-PVC7 era versus 14% (1/7) in the pre-PCV7 era (p = 0.031). The incidence of non-vaccine serotypes was 0.42 and 3.51 per 10,000 discharges in the pre- and post-PCV7 eras, respectively (p = 0.020). There has been a non-significant trend towards an increase in the incidence of complicated pneumonia following the introduction of PCV7. S. pneumoniae remains the predominant organism identified with non-vaccine serotypes now accounting for almost all cases. Although it is not clear if this increase is attributable to the use of PCV7, expanding pneumococcal serotype coverage has the potential to prevent complicated pneumonia.