PREOPERATIVE DIAGNOSES: 1. Intrinsic pontomedullary astrocytoma, evidence of progression following surgery, radiation therapy and chemotherapy x2. 2. Cerebellar and cranial nerve dysfunction secondary to diagnosis #1. POSTOPERATIVE DIAGNOSIS: Progressive intraaxial pontomedullary astrocytoma with evidence of solid tumor growth and multiple cyst formation extending from brainstem into cerebellum with worsening neurologic deficit. OPERATIVE PROCEDURE: 1. Image-guided CBYON posterior fossa craniotomy and aggressive total resection of intraaxial progressive low-grade brainstem astrocytoma with drainage of associated cerebellar cyst. 2. The use of the intraoperative microscope and microdissection. 3. Repair of posterior fossa with EnDura dural graft. 4. Application and removal of cranial fixation device. 5. Use of intraoperative somatosensory evoked potential, motor evoked potential and cranial nerve monitoring by Dr. Jaime Lopez. ATTENDING NEUROSURGEON: ASSISTANT: ANESTHESIA: Michael Edwards, M.D. Paul Jackson, M.D., Ph.D. General endotracheal anesthesia. ANESTHESIOLOGIST: Pediatric Anesthesia. PROCEDURE IN DETAIL: Prior to surgery, Xxxxx underwent a contrast-enhanced image-guided scan placed in the image-guided computer system. She was premedicated and brought to the operating where careful endotracheal anesthesia was established. Appropriate lines and monitors were placed, including an arterial line, Foley catheter, multiple IVs, and compression hose. Monitors and electrodes were placed for somatosensory evoked potentials, motor evoked potentials and cranial nerve monitoring. After all lines and monitors were placed, hyperventilation was begun. The patient was given dexamethasone 10 mg. She was carefully placed on Mayfield-Kees skull fixation and turned prone. Laminectomy rolls were also placed at her side. Her neck was moderately flexed. All bony prominences and peripheral nerves were padded. She was covered to maintain body warmth. The endotracheal tube was carefully suspended from the Mayfield-Kees skull fixation. Her hair was shaved in the occipitocervical area to extend as high as the ending in her prior incision. We then held her hair forward with a tie and then draped out the operative area with clean sterile drapes. The skin was scrubbed for 10 minutes with Betadine soap, painted with alcohol and DuraPrep. Sterile towels denied, band drape and sterile sheets were applied. The skin incision had been marked with plans to excise the scar. The skin was infiltrated with 8 cc of 0.5% local in 1:200,000 adrenaline. The old scar was then excised with a #15 blade and needlepoint Bovie scalpel. We dissected down through the prior scar and occipitocervical muscles, separating them off of the prior occipital craniotomy, which had healed, and separating the muscles and soft tissue off of the dura in the cervical area from C1, leaving the spinous process and arch of C2 intact. Deep retractors were placed. Hemostasis was established with a bipolar cautery on the muscle and soft tissue, and on the bone with bone wax. Using an angled curette, we freed the rim of the foramen magnum. Two small bur holes were made two-thirds of the distance up from the foramen magnum to the inion using the Midas Rex drill. We then used the high-speed pediatric Midas Rex Craniotome to perform an occipital craniotomy and the bone was preserved in normal saline. Hemostasis was established on the dura with the bipolar cautery and on the epidural space with FloSeal. We irrigated with warm saline. We draped beyond the operative area with clean sterile towels, cleansed our gloves, and placed moist Cottonoid patty at the edges of our craniotomy. The operating microscope was brought into the field. Using a sharp, the dura was opened in a V-shaped fashion. The midline sinus had already been occluded at the prior surgery. We carefully dissected the dura off of the cerebellum using the microirrigating bipolar cautery and retracted the dura superiorly with fine silk sutures. We then opened the dura inferiorly in the midline with a #15 blade and similarly elevated off the cerebellum. It was not attached at the region of the brainstem. We then retracted the dura with fine silk sutures and covered the dura with moist Telfa. The Greenberg retractor was attached to the head holder and the 1/2-inch blade was attached. Under higher magnification and using the fenestrated suctions and irrigating bipolar cautery, we first freed the cerebellar tonsils from the region of the brainstem and identified the tonsillar branches of the posterior and inferior cerebellar artery. No tumor was noted looking up towards the opening of the foramen of Magendie. However, based on the image-guided information, we identified that the tumor sat below the vermis in the midline, slightly above the region of the obex. Therefore, we used the irrigating bipolar cautery, fenestrated suction to dissect in the midline of the cerebellar vermis, at which time we entered a tan to grayish tumor, consistent with the exophytic component of the tumor coming up from the pons and extending into the cerebellum. On the right side we entered into the large cyst, which defined a good portion of the right side of the tumor. The cyst was filled with xanthochromic proteinaceous fluid and had some hemosiderin staining within the cystic cavity. We advanced Adaptic over the regions of the cerebellum and lightly retracted the cerebellum with the Greenberg retracting system. Using the microdissector and fenestrated suction with the irrigating bipolar, we began defining a margin between normal brain and tumor. We obtained multiple biopsies, which were reviewed by neuropathology and consistent with a lowgrade astrocytoma, possibly juvenile pilocytic in origin. By using a combination of the image-guided system and throughout the procedure performing sensory and motor-evoked potentials, we carefully checked function as we began essentially debulking tumor with the Cavitron ultrasonic aspirator. This allowed us the luxury of defining the lateral margins. As we were able to work around the lateral margins, we eventually were able to define an interval between tumor and its extent directly into the pons. Using very low settings on the bipolar cautery and by debulking the tumor and then carefully elevating it with small tumor forceps, we were able to define a plane around the tumor; first laterally and then superiorly. We slowly defined this plane using microdissection or the microirrigating bipolar cautery and carefully maintained this plane with Cottonoid patties. By working from inferior to superior, we were able to begin elevating the tumor out of the central portion of the pons. Again multiple testing was done, at one point there was some activity in the 11th cranial nerve. We did not get any activity in the facial nerve. The tumor extended directly within the brainstem, but there appeared to be a margin between tumor and surrounding brain, probably enhanced by the fact that the patient had prior radiation and chemotherapy. Therefore, we were able to work in this interval and slowly elevated tumor from the brainstem, removing multiple specimens for permanent pathology. By continuing to debulk this rather extensive tumor, we were then able to find the lateral and inferior margins and eventually accomplish what we thought to be a gross total removal of the tumor extending into the pons. The tumor was relatively avascular and any bleeding was easily controlled with the bipolar cautery or application of a small piece of brain cotton for a matter of minutes. We then inspected into the cavity inferiorly and noted that there was evidence of tumor coming from the cerebellar tonsils and extending directly into the area of the medulla at the junction of the obex. We again were able to use the Rhoton microdissector, as well as the irrigating bipolar cautery, to define a margin. We again centrally debulked tumor and rolled this margin into our view, carefully defining between tumor and brainstem. At the completion of this rather prolonged and tedious dissection, which ran many hours, we were able to resect, would what appear to our view, all of the tumor visible. Throughout the procedure, warm saline was used to maintain normal neural transmission. Dexamethasone was given at the initiation of the procedure and six hours into the procedure. We carefully inspected along the cerebellar hemispheres. The primary large cyst and two small secondary cysts had been adequately drained. We could visualize through the aqueduct of Sylvius and had an excellent view directly into the pons and the pontomedullary junction. No further tumor could be identified. There were some areas of hemosiderin staining due to the cyst, but no definitive tumor was visible to our view by either angling the patient from side to side or by movement of the microscope superiorly, inferiorly or laterally. We again ran multiple evoked potentials, which remained intake. We irrigated the operative site copiously with warm saline. No bleeding was noted from the operative site or cerebellum. No hemostatic material was left in place. We then used a triangular piece of the EnDura, which we anchored in place with interrupted 4-0 Nurolon suture and sutured to the dura with running 4-0 Nurolon; performing a watertight closure, before placing our last sutures filling the posterior fossa with warm saline. No bleeding was noted from the posterior fossa. We obtained a watertight closure. We then irrigated the dura and muscles with warm saline and bacitracin solution. We placed a large piece of DuraGen over the suture line, followed by fibrin glue. We then closed the muscle and fascia in multiple layers with interrupted #0 Vicryl suture; second, a more superficial layer with interrupted 2-0 and 3-0 Vicryl suture, and the skin with running 4-0 Monocryl. Xeroform gauze, Telfa and Tegaderm dressings were applied. The drapes were removed. The patient was turned supine and taken out of the head holder. The recording equipment was removed. The patient was noted to be breathing adequately and a decision was made to extubate the patient in the operating room and transfer to the Pediatric Intensive Care Unit extubated. The patient was extubated and was noted to be ventilating without difficulty. She was therefore transferred to the Pediatric Intensive Care Unit in stable condition. At no time during the procedure was there any significant hemodynamic instability. On some occasions there were some slight changes in either heart rate or blood pressure; all of which were very transient and responded to our stopping dissection or irrigating with warm saline solution. At the end of the procedure, it would appear that a gross total resection of tumor was obtained. The patient tolerated the procedure well. There were no intraoperative complications. Needle, sponge, and Cottonoid count was correct. Specimens to pathology consisted of intramedullary brainstem tumor. I was the primary surgery with operative assistance of our chief resident.