1 - Pediatric Neurosurgery Unit, Hospital Dona Estefânia, Hospitalar Universitário de Lisboa Central
2 - Neurosurgery Department, Hospitalar Universitário de Lisboa Central
3 - Pediatric Neurology Department, Hospital Dona Estefânia, Hospitalar Universitário de Lisboa Central
4 - Interventional Neuroradiology Unit, Neuroradiology Department, Hospitalar Universitário de Lisboa Central
5 - Pediatric Neuroradiology Unit, Hospital Dona Estefânia, Hospitalar Universitário de Lisboa Central
- 26th biennal congress of the European Society for Pediatric Neurosurgery
Resumo:
Introduction Moyamoya is a rare disease in Western countries, mainly in Europe, with an incidence that ranges from 0.5 in East Asia to 0.08 per 100.000 population. Characterized by progressive bilateral distal ICA stenosis and the formation of abnormal vascular network, it can be divided in two incidence peaks: at 5 to 15 and at 30 to 40 years of age, been females twice affected. Presentation in children is typically related to ischemic events, 40% as TIA and 40% as stroke. There are few reports of its presentation at infant stage as a result of fast aggressive evolution. Material and Methods The authors describe a case of a six months white male with aggressive moyamoya disease diagnosed after stroke and review the literature. Results A six months white male was admitted to our emergency department after seizure and new onset of hemipharesis. There were no previous history of either infection disease or fever. There were no haematological problems, neurocutaneous stigmata or familial history, Down´s syndrome, congenital heart disease or perinatal adverse events. CT scan, MRI/MRA, diffusion-perfusion MRI and cerebral angiography revealed bilateral carotid stenosis, moyamoya vessels, bilateral cerebral hypoperfusion and ischemic changes at distal cortical áreas. The authors initiated antiplatelet therapy and performed a bilateral multiple burr hole indirect revascularization procedure. The postoperative period went uneventful. Six months after surgery images showed significant bilateral increase in cerebral perfusion with multiple ECA tributaries entering cranial volt at burr holes site. From neurological point of view he has a normal development and had a progressive recovery from hemipharesis. The authors show dynamic angiography and diffusion-perfusion MRI images pre- and postoperative, and surgical procedure. Discussion There are few reports of moyamoya disease diagnosed at first year of life. From the author´s point of view the best surgical technique in this scenario is multiple burr holes indirect revascularization because it enables an easy technical procedure, bilateral approach, multiple anastomosis points, optimal revascularization and low complication rate.
Palavras Chave: moyamoya, infant, multiple burr holes