PP04.13 – 2780: The association of Moya Moya disease with pseudocoarctation: A case report

• Published in: European journal of paediatric neurology 2015-05, Vol.19, p.S45-S45
• Main Authors: Ünver, O, Kutlubay, B, Çetiner, N, Saylan, B, Baltacioğlu, F, Ekinci, G, Akalın, F, Türkdoğan, D
• Format: Article
• Language: English
• Subjects: Neurology; Pediatrics
• ISSN: 1090-3798; 1532-2130
• DOI: 10.1016/S1090-3798(15)30146-X

Objective Moya Moya disease is characterized by the progressive occlusion of the cerebral arteries at the circle of Willis, leading to formation of abnormal networks of small collateral vessels with a “puff of smoke” appearance on angiography. The classic pattern involves the distal internal carotid arteries bilaterally, but unilateral variants and involvement of additional anterior and posterior circulation vessels are well described. Coarctation of aorta is a rare association in Moya Moya disease. We present a case of Moya Moya disease and pseudocoarctation of aortic arch. Methods A 7-year-old girl was admitted with a brief loss of consciousness. History revealed clumsyness and frequent falls. All her developmental milestones were delayed comparing peers. Physical examination revealed slight left sided hemiparesis, dysmetria and dysdiadokinesia on the left side. Her EEG was normal. Brain magnetic resonance imaging (MRI) showed encephalomalacia in the right occipital lobe and conventional cerebral angiography revealed occlusion in several arteries including bilateral internal carotid arteries, bilateral anterior cerebral arteries, and bilateral posterior cerebral arteries and diffuse collateral vessels with a puff of smoke appearance. In echocardiographic study, aortic arch and isthmus was tortous, a gradient of 45 mmHg was measured between the proximal and distal parts of coarctation site by CW Doppler. Coarctation of aorta was suggested. Catheter angiography showed large dilated aortic arch kinking at before origin of left subclavian artery, without any pressure difference between the proximal and distal parts, pseudocoarctation was diagnosed. Aspirin was started for prevention of subsequent cerebral embolism. Results The diagnosis of Moya Moya disease was made based on conventional cerebral angiography findings. Conclusion The association of Moya Moya disease with aortic pseudocoarctation may be related to an extensive systemic involvement of blood vessels. Careful evaluation of children with Moya Moya disease is necessary to detect aortic coarctation.