Patient: Female, 18-month-oldFinal Diagnosis: Moyamoya diseaseSymptoms: SeizuresMedication: —Clinical Procedure: —Specialty: NeurosurgeryObjective:Rare co-existance of disease or pathologyBackground:Cerebral hyperperfusion syndrome is a rare complication of indirect revascularization due to moyamoya disease, but has not been reported previously in the pediatric population. We present a case of an 18-month-old girl with moyamoya disease that was treated with bilateral pial synangiosis and had complications consistent with cerebral hyperperfusion syndrome. This case report discusses the pathophysiological mechanisms involved in cerebral hyperperfusion in moyamoya syndrome.Case Report:An 18-month-old female Caucasian presented with seizures and weakness of the left side. Angiography confirmed bilateral cerebral moyamoya disease that was worse on the right side. Indirect revascularization with pial synangiosis was first performed on the right side to allow for healing. Five months later, pial synangiosis was then performed on the left side. Postoperatively, the patient experienced increased intracranial pressure (ICP), suggesting cerebral hyperperfusion syndrome. She was treated with a repeat lumbar puncture, a lumbar drain, and a lumbar shunt.Conclusions:This report demonstrates a case of cerebral hyperperfusion syndrome as a complication of moyamoya disease in a pediatric patient. Although the patient progressed well after placement of a lumbar shunt, this case demonstrates the occurrence of cerebral hyperperfusion syndrome as a complication of revascularization in pediatric patients and highlights the need for further research in this area.

In 43 patients who underwent aortic valve replacement for aortic stenosis with or without regurgitation the accuracy of preoperative left ventricular angiography, parasternal long axis cross sectional echocardiography of left ventricular outflow tract and proximal ascending aorta, and M mode echocardiography of aortic root in predicting aortic root size and thereby prosthesis size was compared. Cross sectional echocardiographic measurements and angiographic measurements of aortic root correlated well with prosthesis size, with over two thirds of the indirect measurements being within 2 mm of prosthesis diameter. M mode echocardiography did not yield useful predictive information. Non-invasive preoperative evaluation of patients likely to require aortic valve replacement may be usefully extended to include aortic root dimensions measured by cross sectional echocardiography.

No abstract