BACKGROUND: Encephalo-duro-arterio-synangiosis (EDAS) in Moyamoya syndrome (MMS) treatment has been well described in the literature, however in MMS caused by sickle cell anaemia (SCA), EDAS use remains controversial with poor long-term follow-up. We present a case-series of SCA patients who have undergone EDAS for SCA-related MMS and describe their post-operative course as well as provide a literature review of the role of EDAS in the treatment of sickle cell anaemia. METHODS: A retrospective review of all the paediatric EDAS procedures conducted in our institution for SCA from 2007 to 2015. Two patients underwent behavioural screening. RESULTS: A total of eight patients with MMS secondary to SCA underwent EDAS. Unilateral EDAS was performed without complication in seven patients. One patient underwent bilateral EDAS but with a two-year gap in between procedures. Follow-up magnetic resonance angiography demonstrated no progression of Moyamoya collaterals or further ischaemic events with regression of collaterals clearly visible in one patient. All patients have demonstrated a return to normal school activities. CONCLUSIONS: EDAS is a well-tolerated revascularisation procedure for children with MMS. The prevention of further infarcts in our group with sickle cell disease has allowed these children to resume normal school activities.
BACKGROUND:Encephalo-duro-arterio-synangiosis (EDAS) in Moyamoya syndrome (MMS) treatment has been well described in the literature, however in MMS caused by sickle cell anaemia (SCA), EDAS use remains controversial with poor long-term follow-up. We present a case-series of SCA patients who have undergone EDAS for SCA-related MMS and describe their post-operative course as well as provide a literature review of the role of EDAS in the treatment of sickle cell anaemia. METHODS: A retrospective review of all the paediatric EDAS procedures conducted in our institution for SCA from 2007 to 2015. Two patients underwent behavioural screening. RESULTS: A total of eight patients with MMS secondary to SCA underwent EDAS. Unilateral EDAS was performed without complication in seven patients. One patient underwent bilateral EDAS but with a two-year gap in between procedures. Follow-up magnetic resonance angiography demonstrated no progression of Moyamoya collaterals or further ischaemic events with regression of collaterals clearly visible in one patient. All patients have demonstrated a return to normal school activities. CONCLUSIONS: EDAS is a well-tolerated revascularisation procedure for children with MMS. The prevention of further infarcts in our group with sickle cell disease has allowed these children to resume normal school activities.
Authors: Sciacca Francesca Luisa; Ambra Rizzo; Gloria Bedini; Fioravante Capone; Vincenzo Di Lazzaro; Sara Nava; Francesco Acerbi; Davide Sebastiano Rossi; Simona Binelli; Giuseppe Faragò; Andrea Gioppo; Marina Grisoli; Maria Grazia Bruzzone; Paolo Ferroli; Chiara Pantaleoni; Luigi Caputi; Jesus Vela Gomez; Eugenio Agostino Parati; Anna Bersano Journal: Int J Mol Sci Date: 2018-11-20 Impact factor: 5.923
Authors: M R DeBaun; L C Jordan; A A King; J Schatz; E Vichinsky; C K Fox; R C McKinstry; P Telfer; M A Kraut; L Daraz; F J Kirkham; M H Murad Journal: Blood Adv Date: 2020-04-28