Indirect Bypass With Pericranial Transposition for Moyamoya Syndrome in an Infant

Abstract

Moyamoya syndrome (MMS) is a progressive disease that can result in debilitating strokes. Surgical revascularization is the mainstay of treatment. Selection of the proper bypass technique depends on the vascular anatomy and location of the hypoperfused cerebral territory. We describe here a case of successful indirect bypass utilizing a pericranial flap as well as dural inversion. A seven-month-old female was transferred from an outside facility to our institution for further evaluation and surgical treatment of MMS. She presented with bilateral brain infarcts worse on the left, with right-sided body weakness. After medical stabilization and hyperhydration, she was taken to the operating room for a left-sided indirect bypass. The superficial temporal artery (STA) was traced utilizing doppler but was determined to be too diminutive for transposition, so the decision was made to proceed with encephalo-duro-pericranio-synangiosis (EDPS). A pericranial graft was successfully affixed to the cortical surface in the hypoperfused middle cerebral artery (MCA) territory, and the dura was inverted. Postoperatively, the patient developed a pseudomeningocele, so a revision surgery was performed. She was discharged shortly after this and returned for encephalo-duro-arterio-synangiosis (EDAS) of the contralateral side. She followed up three months after her initial bypass surgery at age 10 months and was crawling without any focal deficits. She was lost to follow-up thereafter. EDPS is a safe technique for infants with MMS whose STA is too diminutive to be used for bypass surgery. This may be an effective method for indirect bypass in these patients.