Retromastoid Craniotomy Research Articles

A Reliable Closure Technique for Retromastoid Craniotomy to Avoid Cerebrospinal Fluid Leaks and Meningitis

BACKGROUND AND OBJECTIVES: Postoperative cerebrospinal fluid (CSF) leaks and meningitis are well-known risks of retromastoid craniotomy. Use of abdominal fat grafts, collagen allografts, and rigid or semirigid buttresses have demonstrated efficacy in preventing CSF leaks and meningitis in endoscopic endonasal surgery. This study aims to determine the utility of a similar multilayered reconstruction technique for retromastoid craniotomy. METHODS: We retrospectively reviewed 212 consecutive patients who underwent retromastoid craniotomy for tumor removal or microvascular decompression from 2007 to 2022. Scalp incisions were linear or slightly curved, muscle and facia opening was performed sharply avoiding monopolar cautery; craniotomies had a maximum dimension of 3 cm. A primary water-tight dural closure was rarely achieved favoring collagen sponge overlay often augmented with autologous fat. Clinical factors including pathology, mastoid air cell entry, and reconstruction material were analyzed. Outcomes including postoperative CSF leakage and meningitis were assessed. RESULTS: Of 212 patients (mean age 56 ± 16 years; 60% female; 10% with prior surgery), 148 (70%) had tumor resection and 64 (30%) had microvascular decompression. Mastoid air cells were breached in 67%. Collagen sponge dural overlay was used in 201/212 (95%). A fat graft was placed in 116 (55%) cases: 69% with air cell entry, 27% without air cell entry; 158 (75%) patients had their bone flap replaced, 46 (21%) had titanium mesh cranioplasty, 8 (4%) had no bone flap or titanium mesh. There were no CSF leaks or meningitis. One patient had a lumbar drain placement preoperatively, none postoperatively. Median length of stay was 2 days. CONCLUSION: Retromastoid craniotomy multilayered reconstruction with liberal use of collagen sponge and abdominal fat grafts seems to reliably avoid postoperative CSF leaks and meningitis including in the setting of nonwatertight dural closure and mastoid cell entry. Use of shorter incisions, avoidance of monopolar cautery, and a relatively small craniotomy may contribute to the absence of CSF leaks in this series.

Atypical Presentation of Nocardia Brainstem Abscess: A Case Report (P1-10.006)

<h3>Objective:</h3> NA <h3>Background:</h3> Roughly 1–2% of cerebral abscesses are caused by <i>Nocardia spp.</i> and usually occur in immunocompromised patients. Mortality of <i>Nocardia</i> is around three times higher than other bacterial abscesses, making early diagnosis critical. Lesions are usually supratentorial and multiloculated due to daughter abscesses. <h3>Design/Methods:</h3> NA <h3>Results:</h3> A healthy 70-year-old male with history of diabetes mellitus and hypertension presented with new onset right upper extremity weakness, right-sided facial droop, and expressive aphasia. Brain MRI revealed an enhancing left-sided pontine mass with surrounding vasogenic edema. Lumbar puncture was obtained which was remarkable for leukocytosis, monocyte predominance (WBC 13thou/cmm). Cultures and PCR of cerebrospinal fluid were negative with no bacterial or fungal growth. He was started on intravenous steroids with improvement in his symptoms and discharged with close follow up. Three days later, he returned with worsening right upper arm weakness. Repeat MRI brain showed an expanding mass at the left aspect of the brainstem extending into the cerebral peduncle and medulla oblongata. MR spectroscopy favored abscess formation over metastasis or hematoma, and the patient was started on intravenous ampicillin empirically for presumed Neurolisteriosis. As the patient experienced only minimal neurologic and radiologic improvement after two weeks, the decision for surgical biopsy was pursued. Left retromastoid craniotomy was performed and cultures grew <i>Nocardia cyriacigeorgica</i>, and the patient was started on trimethoprim-sulfamethoxazole, imipenem and amikacin. <h3>Conclusions:</h3> While <i>Nocardia</i> CNS infections are typically supratentorial lesions, we present an atypical presentation of a patient who presented with progressive neurologic deficits from an expanding pontine mass. <i>Nocardia</i> abscesses are extremely rare and usually present in immunocompromised hosts. Despite being healthy with no significant prodrome prior to presentation, our patient had radiologic evidence of cirrhotic liver morphology which may have predisposed him to infection. This case highlights that <i>Nocardia</i> should be considered as an infectious agent in brainstem abscesses. <b>Disclosure:</b> Dr. Shastry has nothing to disclose. Mr. Sacks has nothing to disclose. Dr. Reyes has nothing to disclose. Dr. Su has nothing to disclose. Dr. Clauser has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Bigoen . The institution of Dr. Clauser has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Genzyme. Dr. Clauser has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Genentech . Dr. Clauser has received personal compensation in the range of $50,000-$99,999 for serving on a Speakers Bureau for Genzyme. Dr. Clauser has received personal compensation in the range of $50,000-$99,999 for serving on a Speakers Bureau for Biogen. Dr. Clauser has received personal compensation in the range of $50,000-$99,999 for serving on a Speakers Bureau for Novartid. Dr. Benjamin has nothing to disclose.

Retromastoid Craniotomy: An Adaptable and Panoramic Approach to the Cerebellopontine Angle

There are many approaches to the cerebellopontine angle, all of which involve different degrees of disruption of the normal anatomy. The retromastoid craniotomy has stood the test of time for the efficiency, panoramic exposure, and flexibility it provides while allowing excellent control over the vital cerebrovascular structures including the brainstem. This approach allows the surgeon not to get distracted by how to get there but instead to focus on what to do when he or she is there. Herein, I discuss the nuances of this simple approach with its "extended" variations.

RESECCIÓN MICROQUIRURGICA DE UN CAVERNOMA PONTINO MEDIANTE UN ABORDAJE SUPRACEREBELOSO INFRATENTORIAL EXTREMO LATERAL

Introduction: Cavernomas on the posterolateral pontomesencephalic surface can be approached from an extreme lateral supracerebellar infratentorial corridor, although the theory is scarce. The brainstem has a dense concentration of nuclei and fibers that are responsible for a high rate of morbidity when treating brainstem lesions. The objective of this work is to demonstrate the safe microsurgical resection technique for the complete removal of a pontine cavernoma. Clinical Case: 27-year-old woman with a 5-month history of disease; characterized by headache, left facial paresis and right half body paresis. Magnetic resonance imaging (MRI) showed a hemorrhage in the middle cerebellar peduncle compatible with a ruptured cavernoma, initially receiving medical treatment. Subsequently, due to an increase in the motor deficit and the presence of keratopathy in the left eye, surgery was decided. A retromastoid craniotomy and an extreme lateral supracerebellar infratentorial approach were performed. A safe entry zone was identified and the cavernoma was completely excised. In the postoperative period, she did not present additional neurological deficit, being extubated at 24 hours, with a score on the Glasgow scale of 15 points. Postoperative brain tomography (CT) showed the absence of the cavernoma. Conclusion: The far lateral supracerebellar infratentorial approach is safe for the excision of cavernomas with a middle cerebellar peduncle. When combined with the significant reverse Trendelenburg position it results in minimal cerebellar retraction as the cerebellum descends with gravity. Keywords: Hemangioma, Cavernous, Pons, Middle Cerebellar Peduncle, Craniotomy (source: MeSH NLM)

MICROCIRUGÍA ASISTIDA CON ENDOSCOPÍA EN LA RESECCIÓN DE TUMOR EPIDERMOIDE DEL ÁNGULO PONTOCEREBELOSO

Introduction: Intracranial epidermoid tumors are rare, slow-growing, and histologically benign congenital neoplasms. The microsurgical approach with endoscopic assistance is described as a minimally invasive technique that facilitates the work of the neurosurgeon in the complete resection of an epidermoid tumor located in the cerebellopontine angle. Clinical Case: 62-year-old woman, with a 3-year disease characterized by pain on the right side of the face. Symptoms increase in the following 2 years, becoming disabling and only partially improving with pregabalin and oxcarbazepine. Brain MRI showed a hypointense lesion with a cystic appearance at the level of the prepontine cistern with expansion to the right cerebellopontine angle that compressed the right trigeminal nerve (V). Retromastoid craniotomy and a right cerebellopontine angle approach were performed. Under the microscopic vision, the trigeminal nerve was identified which was pulled by the tumor, the cranial nerve complex VII-VIII, and vessels such as the superior petrous vein and the anteroinferior cerebellar artery. With the support of the endoscope, the tumor was better visualized in inaccessible areas, the prepontine cistern was accessed and total resection of the tumor was achieved. The patient evolved favorably with remission of pain on the right side. Conclusion: The microsurgical technique assisted by endoscopy allows safe removal of the tumor, and it is immensely helpful in the resection of tumors from regions not visible under the microscope. Keywords: Brain Neoplasms, Trigeminal Nerve, Endoscopy, Cerebellopontine Angle. (source: MeSH NLM

Hearing Outcomes after Microvascular Decompression for Hemifacial Spasm: An Institutional Experience.

Introduction:Hearing loss following microvascular decompression (MVD) for hemifacial spasm is one of the most dreaded complications. Several factors such as stretching of VIII cranial nerve, vasospasm of labyrinthine artery, and acoustic trauma due to drill noise may be considered in its causation. We evaluated the incidence and severity of hearing loss following MVD in hemifacial spasm and the factors which might be responsible for this complication.Methods:A retrospective analysis of 30 patients operated for hemifacial spasm between January 1, 2014, and December 31, 2018, with at least 3 months of follow-up were included in the study. Retromastoid craniotomy was made, and Teflon was placed between involved vessel and VII nerve.Results:Freedom from hemifacial spasm was noted in 27 of 30 patients. Moderate spasm persisted in one patient, which was controlled with medications. The recurrence was noted in 3 patients at 6 months follow-up. Postoperatively, hearing loss was found in one female patient. The offending vessel was both anterior inferior cerebellar artery (AICA) and posterior inferior cerebellar artery (PICA) loop, which was transpositioned during surgery, and the patient was spasm free postoperatively.Conclusion:The incidence of hearing loss following MVD can be minimized using proper surgical techniques and various intraoperative adjuncts such as brainstem auditory evoked responses monitoring, use of endoscope, and indocyanine green or dual-image video angiography.

Endoscope-Assisted Retromastoid Intradural Suprameatal Approach for Trigeminal Schwannoma.

Dumbbell trigeminal schwannoma is the second most common type of intracranial schwannomas. Herein, we are describing and presenting a video of left trigeminal dumbbell schwannoma with a predominant posterior fossa component (modified Samii's grade C1), operated through left Retromastoid Intradural Suprameatal Approach (RISA). We demonstrate a step by step technique of a previously defined procedure for educational purpose. The patient was placed in the right lateral position, and left retromastoid craniotomy was done. Dura was opened based on transverse and sigmoid sinus to expose tumors in the suprameatal region. The tumor removed piecemeal under the microscope, and later endoscope was introduced to identify and remove the residual tumor in the superolateral part of Meckel's cave. The patient had an uneventful recovery. Trigeminal schwannoma with a predominantly posterior fossa component can be excised through retromastoid sub-occipital craniotomy. Endoscopic-assisted microsurgery should be considered in all skull base tumors. Aim for complete excision, but safe maximal resection with GKRS for residual can be considered in difficult cases.

P118 A novel technique for repair of the vertebral artery during cranial surgery

ObjectivesTo demonstrate a novel/alternative technique for repair of the vertebral artery intraoperatively.DesignLeft-sided retromastoid craniotomy in one patient.SubjectsA 61-year-old male patient with meningioma in the foramen magnum encasing the vertebral artery on the left side.MethodsStandard retrosigmoid craniotomy performed with preservation of transverse and sigmoid sinuses. The dura was opened and the cerebellum was retracted with CSF release. The tumour was debulked gradually. The attempt to peel the tumour from the vertebral artery resulted in arterial bleeding. The hole in the artery was sealed using an N-hydroxysuccinimide functionalised polyethylene glycol-coated collagen patch (Hemopatch®, Baxter Healthcare Ltd) as an onlay, applying pressure for 2 minutes. Another patch was applied for further support and the artery checked for haemostasis. The dura was closed with 3–0 vicryl and Hemopatch® onlay after further tumour debulking.ResultsHaemostasis of the vertebral artery was achieved at 140 mmHg SBP and a catheter angiogram found no dissection or false lumen. The patient did not suffer postoperative haemorrhage and after three months of inpatient rehabilitation was discharged with significantly improved cranial nerve function. There was no CSF leak.ConclusionsHaemostasis was successfully achieved in the vertebral artery with this novel technique. It can prove to be important in such scenarios where direct cross-clamping and arterial repair is technically challenging and can lead to brainstem ischemia.

Ocular Tilt Reaction in Compensated Vestibular Schwannoma

Ocular Tilt Reaction in Compensated Vestibular Schwannoma

Dolicho ectasia of vertebro basilar artery presenting as Trigeminal neuralgia: Case report

We present a case of trigeminal neuralgia caused by vertebrobasilar dolicho ectasia treated with micro vascular decompression. A 56-year-old presented with recurrent lancinating left facial pain in V2 and V3 trigeminal territories. On Evaluation, found to have Dolicho ectasia of vertebro basilar artery system causing compression over trigeminal nerve. On initial conservative management patient had temporary relief with resurgence of pain in increased in severity and number of episodes of facial pain. He underwent Retro mastoid craniotomy and micro vascular decompression of trigeminal neuralgia.

Comparison of the antiemetic effect of ramosetron with ondansetron in patients undergoing microvascular decompression with retromastoid craniotomy: a preliminary report.

BackgroundMicrovascular decompression with retromastoid craniotomy carries an especially high risk of postoperative nausea and vomiting. In this study, we compare the antiemetic efficacy of ramosetron and ondansetron in patients undergoing microvascular decompression with retromastoid craniotomy.MethodsUsing balanced anesthesia with sevoflurane and remifentanil infusion, ondansetron 8 mg (group O, n = 31) or ramosetron 0.3 mg (group R, n = 31) was administered at the dural closure. The incidence and severity of postoperative nausea and vomiting, required rescue medications and the incidence of side effects were measured at post-anesthetic care unit, 6, 24 and 48 hours postoperatively. Independent t-tests and the chi-square test or Fisher's exact test were used for statistical analyses.ResultsThere were no differences in the demographic data between groups, except for a slightly longer anesthetic duration of group R (P = 0.01). The overall postoperative 48 hour incidences of nausea and vomiting were 93.6 and 61.3% (group O), and 87.1 and 51.6% (group R), respectively. Patients in group R showed a less severe degree of nausea (P = 0.02) and a lower incidence of dizziness (P = 0.04) between 6 and 24 hours.ConclusionsThe preventive efficacy of ramosetron when used for postoperative nausea and vomiting was similar to that of ondansetron up to 48 hours after surgery in patients undergoing microvascular decompression with retromastoid craniotomy. A larger randomized controlled trial is needed to confirm our findings.

Intraparenchymal Schwannoma Involving the Brainstem in a Young Woman

Schwannomas are tumors derived from the Schwann cells, which form the myelin sheath of the peripheral nerves. Fewer than 1% of these tumors occur within the brain parenchyma without arising from the cranial nerves. Only 55 cases have been published after the first recorded case. We report a 17-year-old girl with a 3-month history of unspecific dizziness, unsteadiness, and headache. Magnetic resonance imaging showed a heterogeneous cystic lesion involving midbrain, pons, and left cerebellar peduncle. The patient underwent a retromastoid craniotomy with complete resection of the tumor. Pathologic examination was compatible with intraparenchymal schwannoma. Since the first case of intraparenchymal schwannoma involving the brainstem was described in 1980, only seven others have been reported. Diagnosis of intraparenchymal schwannoma is almost never made preoperatively. Immunohistochemical staining is crucial in distinguishing a Schwannoma from a meningioma, glial tumor, or metastatic tumor. Pathologic findings are those typical of acoustic neurinomas. Histogenesis of intraparenchymal schwannoma remains unclear, and several theories have been proposed to explain their origin. The recognition of this curable tumor and its differentiation from brainstem glioma, which generally has a less favorable outcome, is of obvious importance.

Microvascular Decompression in Patient With Atypical Features of Hemifacial Spasm Secondary to Compression by a Tortuous Vertebrobasilar System

We report a case of hemifacial spasm in a patient who had associated hearing loss, numbness throughout the face, tinnitus, and vertigo, all of which occurred when turning his head to the left. To our knowledge, these symptoms have not occurred in this pattern and with a single trigger. A 45-year-old man presented with a 3-year history of right-sided hemifacial spasm initially treated with botulinum toxin. One month before presentation, he had an episode of acute hearing loss in the right ear when turning his head to the left, followed by multiple episodes of transient hearing loss in his right ear, numbness in his right face in all distributions of the trigeminal nerve, tinnitus, and vertigo. He was found to have decreased sensation in nerves V1 to V3 and House-Brackmann grade 3/6 weakness in his right face, despite not having botulinum toxin injections in more than a year. Magnetic resonance imaging/angiography showed an ectatic vertebrobasilar system causing compression of the fifth, seventh, and eighth cranial nerves. The patient underwent a retromastoid craniotomy and microvascular decompression. Postoperatively, he had complete resolution of his symptoms except for his facial weakness. The benefit has been long-lasting. Multiple, simultaneous cranial neuropathies from vascular compression are rare, but this case is an example of safe and effective treatment with microvascular decompression with durable results.

Diagnosis of Facial Nerve Schwannoma by Magnetic Resonance Imaging Enhancement of the Geniculate Ganglion

diagnosis of facial nerve schwannoma was based on this enhancement pattern. The patient had a retromastoid craniotomy and intentional subtotal tumor resection. Intraoperatively, the normal-appearing portion of the right facial nerve was identified and noted to be contiguous with the tumor. The pathologicdiagnosiswasschwannoma.No operative complications occurred. Ten months postoperatively, the patient had radiosurgery of the residual tumor. One year after radiosurgery, the patient had full facial sensation, no eye dryness, and mild facial asymmetry. Follow-up imaging showed that the residual tumor had decreased in size (Figure 2), and the patient has returned to work. COMMENT

Paroxysmal otalgia due to compression of the intermediate nerve: a distinct syndrome of neurovascular conflict confirmed by neuroimaging

✓The authors present the case of a 52-year-old female patient with a 6-year history of intractable paroxysmal otalgia. Preoperative magnetic resonance (MR) angiography demonstrated an anterior inferior cerebellar artery loop compressing the intermediate nerve in the seventh/eighth cranial nerve complex inside the internal auditory canal. The pain resolved completely after a microvascular decompression via a retromastoid craniotomy. To the best of the authors' knowledge, the combined neuroimaging and intraoperative findings of this case provide a unique demonstration that vascular compression of the intermediate nerve can be the exclusive cause of paroxysmal otalgia. Magnetic resonance imaging and MR angiography can establish the causative mechanism and distinguish this otalgia due to vascular compression of the intermediate nerve from other pain syndromes that are designated as geniculate neuralgia (GN). The present case indicates that intermediate nerve neuralgia is a distinct syndrome of neurovascular conflict and a variant of GN. The causative classification of GN should be reexamined with the use of advanced MR imaging.

Paroxysmal otalgia due to compression of the intermediate nerve: a distinct syndrome of neurovascular conflict confirmed by neuroimaging

The authors present the case of a 52-year-old female patient with a 6-year history of intractable paroxysmal otalgia. Preoperative magnetic resonance (MR) angiography demonstrated an anterior inferior cerebellar artery loop compressing the intermediate nerve in the seventh/eighth cranial nerve complex inside the internal auditory canal. The pain resolved completely after a microvascular decompression via a retromastoid craniotomy. To the best of the authors' knowledge, the combined neuroimaging and intraoperative findings of this case provide a unique demonstration that vascular compression of the intermediate nerve can be the exclusive cause of paroxysmal otalgia. Magnetic resonance imaging and MR angiography can establish the causative mechanism and distinguish this otalgia due to vascular compression of the intermediate nerve from other pain syndromes that are designated as geniculate neuralgia (GN). The present case indicates that intermediate nerve neuralgia is a distinct syndrome of neurovascular conflict and a variant of GN. The causative classification of GN should be reexamined with the use of advanced MR imaging.

MANAGEMENT OF PETROUS ENDOSTOSIS IN POSTERIOR FOSSA PROCEDURES FOR TRIGEMINAL NEURALGIA

Within a series of 440 consecutive patients who underwent posterior fossa procedures for trigeminal neuralgia (TN), the site of neurovascular conflict was obscured by petrous endostosis in 15 patients. The surgical management and clinical outcomes of these patients are presented. We retrospectively analyzed the prospectively collected data of all patients with a diagnosis of TN from 1980 to 2005. Clinical presentation, preoperative imaging, intraoperative findings, surgical technique, and outcomes were recorded. A postal follow-up questionnaire and a visual analog scale of 100 points were used for outcome assessment and patient satisfaction. All 15 patients presented with typical TN, had preoperative imaging suggestive of vascular compression of the trigeminal nerve root, and underwent standard retromastoid craniotomy. The juxta-petrous portion of the trigeminal nerve root was obscured by petrous endostosis. The neurovascular conflict was revealed and dealt with after drilling of the endostosis in 11 patients, and four patients had endoscopic-assisted exploration of the region of the endostosis. A pontotrigeminal vein caused compression in most cases. Three patients had endoscopic-assisted division of the vein. All patients had immediate relief of pain and were 100% satisfied with the results of surgery after a mean follow-up period of 38.6 months. Four patients developed a cerebrospinal fluid leak, and five patients experienced postoperative headaches. Petrous endostosis is an uncommon finding in posterior fossa procedures for TN (3.4%). However, it can obscure the region of neurovascular conflict, which is venous compression in these cases. We have found that drilling away endostosis or using endoscopic-assisted microsurgery increases the chances of good outcome and avoids unnecessary rhizotomy. All magnetic resonance imaging scans should be inspected for evidence of petrous endostosis and, when present, a bone window computed tomographic scan should be performed to clarify the image and check for the presence of petrous air cells. If the endostosis is drilled, failure to detect and to seal these air cells thoroughly can result in postoperative cerebrospinal fluid rhinorrhea. Attention to these details will optimize the surgical results. However, the use of an angled endoscope avoids this problem and is now our preferred method.

Downbeat Nystagmus Associated With Brainstem Compression by Vertebral Artery

Rarely, downbeat nystagmus can occur due to compression of the lower brainstem by the ectatic vertebral artery and be resolved by microvascular decompression. We present a case of a 67-year-old man with downbeat nystagmus associated with brainstem compression by ectatic vertebral artery. He presented with oscillopsia and vertigo. When he turned his head upward, his symptoms were aggravated and a gait disturbance occurred. Magnetic resonance imaging and computed tomographic angiography demonstrated compression of the medulla oblongata by the left ectatic vertebral artery and other medical causes of downbeat nystagmus were ruled out. Retromastoid craniotomy was performed and after lifting the vertebral artery off the medulla, a trough-shaped indentation in the lower brainstem was identified. The ectatic vertebral artery was repositioned and a Teflon was inserted between the brainstem and the ectatic vertebral artery. Postoperatively, downbeat nystagmus had disappeared.

Three-dimensional MRI of hemifacial spasm with surgical correlation.

MRI was used to investigate 100 patients with hemifacial spasm, using 3D-FT T2-weighted (CISS) and contrast-enhanced 3D-FT T1-weighted (turbo-FLASH) sequences in all cases. MR angiography was performed in 54 patients, using 3D-MT FISP images. Decompression of the facial nerve through a retromastoid craniotomy was performed in all patients. Hemifacial spasm caused by tumours in the cerebellopontine angle was not included. Vascular contact with the facial nerve root-exit zone or at the internal auditory canal was present in 96 of 100 patients with hemifacial spasm. The vessel responsible was the vertebral artery (VA) in 18 cases, the posterior inferior cerebellar artery (PICA) in 23, the anterior inferior cerebellar artery (AICA) in 22, the VA and PICA in 24, VA and AICA in 3, PICA and AICA in 1, VA, PICA and AICA in 4, and a vein in 1 case. CISS images showed compressive vascular loops better than contrast-enhanced turbo-FLASH images alone. The sensitivity of MRI was high, since only one false-negative case was found among the 100 patients who underwent surgery.

Clivus chordoma in childhood

A 9-year-old boy with mild left hemiparesis and left abducens nerve palsy was found to have chordoma of the clivus. Magnetic resonance imaging (MRI) was most useful not only for diagnosis but also for selection of operative approaches. The tumor was subtotally resected by a transoral-transpharyngeal approach followed by a retroauricular retromastoid craniotomy, and postoperative radiation therapy was administered. Intracranial chordomas are rare in children; only 12 cases have previously been reported in detail. Eleven patients were male and one was female. Progressive cranial nerve involvement and long tract signs without increased intracranial pressure constitute the cardinal symptomatology of intracranial chordomas, but a separation of cranial sutures and enlargement of the head may be seen in children. The role of MRI in diagnosis and patient management cannot be overemphasized.