PREOPERATIVE DIAGNOSES: 1. Intraventricular tumor, etiology undetermined. 2. Associated ventricular cyst, probably secondary to tumor. 3. Status post diagnosis and biopsy of nasopharyngeal carcinoma involving the sphenoid sinus, superior clivus, with metastases to right cervical lymph nodes. POSTOPERATIVE DIAGNOSES: 1. Intraventricular tumor, etiology undetermined (frozen section consistent with very low-grade tumor, probably subependymoma). 2. Associated ventricular cyst, probably secondary to tumor. 3. Status post diagnosis and biopsy of nasopharyngeal carcinoma involving the sphenoid sinus, superior clivus, with metastases to right cervical lymph nodes. OPERATIONS PERFORMED: 1. Left frontal bur hole. 2. Image-guided endoscopic biopsy, partial resection of intraventricular tumor with fenestration and drainage of associated tumor cyst. 3. Use of the endoscope and guidance using MRI-assisted endoscopy. 4. External ventricular drain. SURGEON: Michael Edwards, M.D. ASSISTANT: ANESTHESIA: Paul Jackson, M.D., Ph.D. General endotracheal anesthesia, pediatric anesthesia. PROCEDURE IN DETAIL: Xxxxxxxxx, with great difficulty, underwent a stereotactic MR scan. Because of his claustrophobia, sedation was necessary. He was then brought to the operating room, where careful general endotracheal anesthesia was established, appropriate lines and monitors placed. An arterial line and Foley catheter were inserted. He was given Rocephin 2 G. Hyperventilation was begun and he was given dexamethasone 10 mg. He was carefully positioned supine and placed in the Mayfield-Kees skeletal fixation. After he was carefully positioned and all bony prominence and peripheral nerves were padded, the fiducials on his forehead were correlated with the image-guided system. We then determined the trajectory at the midpupillary line overlying the coronal suture that would allow for a post to his tumor into the ventricle that was smaller than normal necessitating the use of image guidance. The location for the bur hole was made. We also plotted out an area to make a craniotomy flap should it be necessary to convert this from an endoscopic procedure to a transcortical procedure. After marking the skin and removing the fiducials, we shaved hair in the left frontal area. We left a marker at the glabella and then we draped out the skin with Steri-Drapes. The skin was then scrubbed for 10 minutes with Betadine solution, painted with alcohol and DuraPrep. The skin incision was re-marked. Sterile towels and Ioban drape were applied. The skin was infiltrated with 5 cc of 0.5% local. The image-guided system was covered with a sterile drape and a Steri-Drape placed over the operative site. We again checked using our image-guided system. We then opened the skin with a #15 blade and a needlepoint Bovie scalpel and placed a self-retaining retractor. We again located our entry point on the skull and using the Midas-Rex drill made a bur hole large enough to receive the endoscope along with the 30K scope. The edges of bone were bone waxed. The dura was coagulated. The dura was opened in a cruciate fashion with an 11-blade and coagulated. The pial surface was coagulated with the irrigating bipolar cautery, and similarly incised with a #11 blade. The endoscope was set up, white balanced, and irrigation attached. Using the image-guided system and calculating the distance from the cortex to the ventricle, we used a #7 Ellsberg cannula in a pass along the same trajectory and entered into the ventricle. When CSF was obtained, we removed the Ellsberg cannula. We carefully advanced the endoscopic sheath down to the ventricular wall and anchored the sheath to the surrounding drapes with a skin staple. The internal stylet was removed. The endoscope was slowly advanced through the protective sheath and we immediately entered into the ventricle. On inspection of the ventricle, we were able to determine that we were in the left lateral ventricle. We identified the choroid plexus running posteriorly. As we looked more anteriorly we identified the choroid plexus in the left foramen of Monro. The left foramen of Monro was opened, and sitting above it anterior was a cystic structure with a veil overlying a mass anterior to the foramen of Monro in the frontal horn of the lateral ventricle. We opened the veil of tissue and removed the slightly proteinaceous fluid. Biopsies of the wall were obtained with an endoscopic biopsy forceps and this was reviewed by neuropathology. There was no specific tumor diagnosis that could be made on the cyst wall but the cyst wall was widely fenestrated. Using image guidance and the endoscope, we then obtained multiple biopsies of the mass, which was white, soft, and relatively nonvascular. No significant bleeding was noted. The specimens were reviewed with neuropathology. It was felt that there were signs of calcification. The lesion appeared to be very low grade, uniform, long-standing, and the preliminary diagnosis was that of a subependymoma. There were no signs that this was a malignancy or evidence of metastases from his nasopharyngeal carcinoma. We realized that the tumor was a portion of the ventricle. It appeared to be lying under the ependyma and, therefore, the diagnosis most consistent with a subependymoma. Given that diagnosis, we decided not to perform a craniotomy at this time, given the child's overriding nasopharyngeal carcinoma diagnosis and need for treatment, as well as for the fact that this is a slow-growing lesion and if necessary could be addressed at a later date, as it was not causing a specific problem at this time. The key reason for surgery was to rule out this lesion as a metastasis or secondary malignancy so that appropriate treatment could be planned in one sitting along with treatment of his nasopharyngeal carcinoma and neck metastases. We then obtained multiple biopsies of the tumor, which was submitted for permanent pathology. Adequate tissue was obtained from multiple areas. We copiously irrigated within the ventricle with warm Ringer's. We made sure there was no evidence of any bleeding from our biopsy sites. Because of a concern of the possibility of swelling and the possible closure of the foramen of Monro, a 35 cm external ventricular drainage catheter was inserted through the endoscopic sheath and advanced into the body of the left lateral ventricle. The catheter sheath was removed. The external drain was rather beneath the skin and brought out through a separate stab wound posteriorly. We irrigated copiously with saline solution. We sealed the cortical area with Gelfoam. A piece of Gelfoam was placed over the bur hole. We irrigated the bone and skin with bacitracin solution. We closed the galea with inverted interrupted 3-0 Vicryl, the skin with running 4-0 Monocryl. The external drain was anchored to the skin with a 2-0 silk suture. It was attached to the connector with a 2-0 silk and a blind connector to prevent any loss of CSF. Before closing the drain, the ventricle was refilled with warm saline. We then dressed the wound with Xeroform gauze, Telfa, and Tegaderm, including the external drain to prevent migration of the drainage system. Throughout the procedure the use of image guidance was helpful in identifying our location within the tumor and within the ventricular system. The probe was used to identify the structures within the ventricle and correlate with the MRI. At the end of the procedure the drapes were removed, the child was taken out of the Mayfield-Kees skeletal fixation. He was awoken, extubated, and returned to the pediatric intensive care unit in stable condition. The estimated blood loss was less than 10 cc. Blood transfusions none. Xxxxxxxxx tolerated the procedure well. Needle, sponge, and Cottonoid count was correct. SPECIMENS TO PATHOLOGY: Consisted of intraventricular tumor. The inpatient attending was Dr. Michael Edwards.