PREOPERATIVE DIAGNOSIS: Moyamoya disease with bilateral supraclinoid internal carotid artery occlusion status post bilateral TIAs. POSTOPERATIVE DIAGNOSIS: Moyamoya disease with bilateral supraclinoid internal carotid artery occlusion status post bilateral TIAs. OPERATION: Right frontal temporal craniotomy with microscopic extracranial-to-intracranial revascularization anastomosis under mild hypothermia using intraoperative electrophysiological monitoring. SURGEONS: ANESTHESIA: Gary Steinberg, M.D., Justin Massengale, M.D. General endotracheal anesthesia. ANESTHESIOLOGIST: Rebecca Claure, M.D. ELECTROPHYSIOLOGIST: SURGICAL NURSES: EEG TECHNOLOGIST: Jaime Lopez, M.D. Sarah Yahn, S.T., Paul Dunning, S.T., Patsy Tew, R.N. Dru Sigmon. INDICATIONS: This 8-year-old female first presented with headaches in 08/2005 that were associated with nausea and vomiting. These were diagnosed as possible migraines. In 02/006 she had a period of confusion followed by the migraine, and in 03/2006 and 11/2006 had 5 episodes of migraine headache. Then, in November 2006, she had recurrent confusion with associated headaches and recurrent episodes of unsteadiness on her feet as well as intermittent dilated pupils. She also experienced a tingling in her left and right upper extremities, sometimes associated with difficulty expressing words. MR head scan disclosed bilateral watershed infarcts, worse on the right side, and an angiogram demonstrated moyamoya disease with occlusion of the bilateral supraclinoid internal carotid arteries and extensive moyamoya vessels. First SPECT cerebral blood flow study was reported to show impaired hemodynamic reserve on the right side. Prior to surgery the patient was neuro-electrically normal. FINDINGS: A 1.0-mm-diameter parietal branch of the superficial temporal artery was found to be donor. A 1.3-mm-diameter in fore branch of the middle cerebral artery emergent from the sylvian fissure on the frontal side was found as the recipient. PROCEDURE: The patient was brought to the operating room with an IV in place. She was intubated. General endotracheal anesthesia was instituted. An arterial line, central venous line and Foley catheter were placed. She was positioned supine on the operating room table and her head placed into 3-point pin fixation. The head was elevated above the heart and turned to the left side, bringing the right frontal temporal region uppermost in the field. Stimulating recording electrodes were placed for monitoring the bilateral somatosensory evoked potentials and bilateral EEG. A cooling blanket was used to the patient to a core body temperature of 34.1 degrees Centigrade. The right frontal temporal region was shaved and the course of the superficial temporal artery parietal branch mapped out with the Doppler. This region was prepped and draped sterilely. An incision was made anterior to the right ear and the superficial temporal artery dissected free. The operative microscope was positioned and used for harvesting of the superficial temporal artery. This was essential in order to protect the artery during dissection. A length of approximately 7 cm of superficial temporal artery parietal branch was harvested with a generous cuff of soft tissue to protect it. However, at the underlying temporalis fascia and muscle were split and dissected from the frontal temporal bone. Burr holes were placed and a craniotomy bone flap removed overlying the sylvian fissure on the right side. The dura was tacked up and hemostasis obtained. Then, the superficial temporal artery was dissected of soft tissue proximally and distally. It measured 1.0 mm in its distal extent. The dura was opened in a cruciate manner. The underlying brain was relaxed. The operating microscope was positioned and used for the remainder of the case and subdural closure. The operating microscope was absolutely essential for this case in order to perform the microscopic anastomosis between the superficial temporal artery parietal branch and the middle cerebral artery and fore branch using 10-0 suture. A 1.3-mm-diameter and fore branch of the middle cerebral artery emergent from the sylvian fissure on the frontal side was identified. The arachnoid overlying a 7-mm segment of this vessel was microscopically opened. Two tiny branches originating from this segment were coagulated and divided. A high-visibility background was placed under the M4 segment. Then, the temporal artery was temporarily occluded proximally and distally. It was truncated distally. The distal superficial temporal artery stump was coagulated and the temporary clip removed. Release of the proximal superficial temporal artery clip revealed good flow. The artery was irrigated with heparinized saline, truncated to the proper length for anastomosis and fish-mouthed. The patient was administered IV thiopental to induce slowing in the EEG. The mean arterial pressure was raised up to 80-90, and the M4 middle cerebral artery branch was temporarily occluded with temporary Sugita aneurysm clips. An arteriotomy was made of the M4 branch, removing an elliptical portion of the superior wall. It was irrigated with heparinized saline, then an end-to-side anastomosis between the superficial temporal artery parietal branch and the middle cerebral artery and fore branch was performed using 10-0 interrupted suture. An excellent anastomosis was achieved. The temporary clips were removed from the M4 branch, restoring flow. Total occlusion time was 17 minutes. There were no leaks in the anastomosis. The clip on the proximal superficial temporal artery was removed, establishing flow from this vessel into the M4 branch. Good flow was confirmed with the microvascular Doppler. Surgicel was placed around the anastomosis site. The brain remained nicely relaxed. The superficial temporal artery graft was laid on the cortical surface to induce an indirect bypass as well as the direct one. The dura was closed with 4-0 suture followed by DuraGen, leaving an opening for the graft to enter without compromise. The bone was replaced using the Synthes plating system, leaving a hole for the graft to enter unimpeded. The temporalis muscle and scalp were closed __________ in the usual fashion. A sterile dressing was applied. The patient was rewarmed, extubated and taken to the pediatric intensive care unit in satisfactory condition. There were no complications of the procedure and no changes in the electrophysiological monitoring parameters during part of the case with the exception of temporary slowing in the EEG after thiopental was given. I was present and directly participated in the key portion of the procedure, which included final dissection of the superficial temporal artery in the scalp, microscopic exposure of the M4 branch, placement of temporary clips, microscopic arteriotomy of the M4 branch, microscopic anastomosis between the superficial temporal artery and middle cerebral artery branch, removal of temporary clips, confirming good blood flow in all vessels, obtaining hemostasis. During the non-key portions of the case I was either present and directly participating or immediately available to return to the operating room if necessary.