Efficacy of superficial temporal artery-middle cerebral artery double anastomoses in a patient with rapidly progressive moyamoya disease: case report

Abstract

Moyamoya disease can be associated with a rapidly progressive course in young patients. This report describes a patient with moyamoya disease who experienced rapid disease progression, resulting in cerebral infarction and a wide area of diminished cerebral perfusion. Double superficial temporal artery (STA)-middle cerebral artery (MCA) anastomoses were utilized to immediately increase cerebral perfusion in the affected area. This case involved a 5-year-old girl who had been diagnosed with moyamoya disease and had undergone STA-MCA anastomosis with indirect bypass in the right hemisphere at the age of 3. At the time of presentation, magnetic resonance (MR) imaging showed cerebral infarction at the left frontal lobe, and MR angiography showed rapidly progressive narrowing of the left MCA that had not been present 3 months prior. N-isopropyl-p-[I123] iodoamphetamine single-photon emission computed tomography (IMP-SPECT) showed markedly decreased uptake in the left hemisphere. She underwent emergent STA-MCA double anastomoses with indirect bypass on the left side. IMP-SPECT showed marked increase in uptake in the left hemisphere. The anterior cerebral artery (ACA) territory adjacent to the cerebral infarction also showed increased uptake on the SPECT. Postoperatively, there were no clinical or radiographic indications of ischemic or hemorrhagic complications. Double anastomoses are effective in quickly and significantly increasing blood flow. The postoperative course in this case was uneventful. Double anastomoses are a surgical option for patients with moyamoya disease who show rapid disease progression, even in those in the acute phase of cerebral infarction.

Fig. 1.

Magnetic resonance imaging study of the fluid-attenuated inversion recovery (upper) and T₂-weighted image (lower). A: Images on admission. High signal is present in the territory perfused by the anterior cerebral artery (ACA). B: Postoperative images obtained 7 days after surgery demonstrate disappearance of the high signal. No new ischemic signs are present. C: 3 months after surgery. Infarcted area in the ACA territory has transformed to atrophy.

Fig. 2.

Axial image of magnetic resonance angiogram. A: 3 months before surgery, the left middle cerebral artery (MCA) is patent. B: Preoperative image reveals immediate progression of the stenosis of the left M1 portion (arrow). C: Postoperative image obtained 7 days after surgery. Superficial temporal artery (STA)-MCA double bypass were patent, and STA did not show very high signal (arrowhead).

Fig. 3.

Cerebral hemodynamic studies with resting states of N-isopropyl-p-[I123] iodoamphetamine single-photon emission computed tomography images. A: 2 years before surgery. B: Preoperative images show poor perfusion in the right fronto-temporal lobe (arrow). C: Postoperative images obtained 3 days after surgery demonstrate a wide increase in the perfusion of the left hemisphere, with the exception of the area of infarction.

Fig. 4.

Operative photographs. A: View after the indirect bypass surgery. B: View of the superficial temporal artery-middle cerebral artery double anastomoses sites on the left cerebral convexity.