Surgical Management of Tumors of the Foramen Magnum

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Chapter 43 Surgical Management of Tumors of the Foramen Magnum

The foramen magnum (FM) comprises a bony channel formed anteriorly by the lower third of the clivus, the anterior arch of the atlas, and the odontoid process. The lateral limits are the jugular tubercle (JT), the occipital condyle (OC), and the lateral mass of the atlas. Lastly, the FM is limited posteriorly by the lower part of the occipital bone, the posterior arch of the atlas, and the two first intervertebral spaces.13

The FM encloses the vertebral arteries (VAs) and their meningeal branches, the anterior and posterior spinal arteries, the lower cranial nerves (IX, X, and XI), and the roots of the C1 and C2 vertebrae. The neural structures located at the FM are the cervicomedullary junction, the cerebellar tonsils, the inferior vermis, and the fourth ventricle.13 It is surrounded by veins, venous sinuses, and the jugular bulb. Hence, when approaching this region, surgeons must avoid manipulation and retraction of those neurovascular structures and, consequently, preserve anatomy and function.

There is a broad spectrum of intra- and extradural surgical pathologies of the FM. Tumors represent almost 5% of spinal and 1% of intracranial neoplasms,1 which consist mostly of meningiomas, neurinomas, and chordomas.15

In the past, these lesions were approached posteriorly and eventually via the transoral route; however, the results of these techniques were disappointing.6 The introduction of computed tomography (CT) scan and magnetic resonance imaging (MRI) allowed the improvement of anatomic knowledge and the development of microsurgical techniques and skull base approaches. Therefore, treatment of these tumors has evolved and remarkable improvement in surgical results has been achieved. Nevertheless, despite these advances, surgery of FM tumors is still associated with a high rate of morbidity.

Clinical Presentation

The clinical presentation associated with FM tumors is insidious. Because of their slow-growing pattern, their indolent behavior, and the wide subarachnoid space at this level, the mean length of symptoms before diagnosis is 30.8 months.3,7 In early stages, patients complain of occipital headache and cervical pain. This pain is described as deep and is aggravated by neck motion, coughing, and straining. As the tumor grows, sensory and motor deficits develop. The classic syndrome of FM tumors, mainly of meningiomas placed anteriorly, is an asymmetrical deficit defined by weakness, paresthesis, and spasticity, first in the ipsilateral arm and progressing to the ipsilateral leg, then to the contralateral leg, and finally to the contralateral arm. Long tract signs characteristic of upper-motor lesions are the presence of atrophy in the intrinsic muscles of the hands. Later findings include spastic quadriparesis, respiratory dysfunction, and lower cranial nerve deficits.6,7 In extradural tumors, especially in cranial base chordomas, diplopia is the symptom most commonly reported and headache is the second-most-common symptom.5

Classification of the Tumors

Tumors of the FM are classified according to their origin. They can arise in the FM itself or secondarily from surrounding areas. Most classifications focus on meningiomas and usually do not regard bone tumors. Among the many classifications of meningiomas of the FM,68 the one most frequently used by neurosurgeons is the classification from Bruneau and George.3 The main objective of this system is to define the surgical strategy preoperatively. Based on this classification, meningiomas of the FM are classified as intradural, extradural, or intra- and extradural. According to their insertion on the dura, meningiomas are anterior if insertion happens on both sides of the anterior midline, anterolateral if insertion occurs between the midline and the dentate ligament, or posterior if insertion is posterior to the dentate ligament. The other landmark used for classification is the relation to the VAs, because meningiomas of the FM can develop above, below, or on both sides of the VAs. Intradural meningiomas are the most common type, and most of them arise anterolaterally;48 these are followed in frequency by posterolateral tumors. Tumors that arise purely posteriorly and anteriorly are rare.7

The surgical approach to extradural tumors is based on their relationship with the C1 lateral mass, OC, clivus, intradural extension, cavernous sinus, jugular foramen, retropharynge, VAs, and carotid artery. Although there are various kinds of tumors, they present a similar surgical aspect. A position in front of, or lateral to, the cervicomedullary junction; a closer relationship with the VAs and their branches; lower cranial nerves; and complex articulation between the occipital bone and the C1 and C2 vertebrae are some of these surgical aspects. The size, position, and nature of the tumors define the surgical approach and steps, such as drilling the lateral wall of the FM and transposing the VAs. The definition of the space between the cervicomedullary junction and the lateral wall of the FM, the so-called surgical corridor,7 is also an important consideration. Large tumors, either anterior or anterolateral, push the cervicomedullary junction posteriorly, creating a surgical avenue for tumor removal. In contrast, small tumors and an elongated FM may require additional space, which can be obtained via the condyle or the lateral mass of C1, with transposition of the VAs.5

Choosing the Best Approach

The principal factors that determine access to the lesions placed at the craniovertebral junction are the nature, position, and size of the tumors and the shape of the FM.5 Tumors located posteriorly or posterolaterally to the cervicomedullary junction can be approached from the posterior midline, which allows an extensive sagittal view from the skull base to the entire cervical spine; however, this approach does not work well for tumors located anterolaterally. This midline route does not allow control of the VAs when the bone needs to be removed ventrolaterally. Anterior approaches via transcervical or transoral routes have been used but are not accepted widely. The transoral approach is essentially a midline and extradural approach to the inferior clivus and upper cervical spine that, combined with maxillotomy or labiomandibulotomy and glossotomy, can provide access from the superior clivus to the middle cervical spine. Nevertheless, this approach is limited laterally from both carotid arteries and VAs at the clival and spinal levels. Removal of an intradural pathology carries a high risk of cerebrospinal fluid (CSF) leakage. The dura is difficult to repair, because it comprises a limited amount of soft tissue and the subarachnoid space is exposed to the contaminated field.9 Anterior approaches are suitable for small extradural and bony lesions without VAs and carotid artery involvement.4,6,7,10

However, minimization of the cervicomedullary retraction and of risk of CSF leakage, a firm watertight closure, and management of the OC and of the VAs are the main factors considered when choosing the approach. Among the approaches available to the FM, the so-called far lateral approach, the extreme transcondylar approach, and its variants of the lateral suboccipital approach meet these criteria. These approaches can be combined with petrosal, retrosigmoid, transtuberculum, transfacetal, and infratemporal approaches, according to the rostrocaudal extension and nature of the tumor.6,11

Extradural tumors located frontal to the cervicomedullary junction that present with or without involvement of the VAs and the lower cranial nerves, that invade the dura, and that invade and/or destroy the OC and the articulation among OC, C1, and C2 can be approached via the same route; however, these tumors often require combined approaches.6,911

Far Lateral Approach

Positioning

Surgical results depend on the positioning of the patient. Malpositioning may result in a narrow microsurgical view, cerebral edema, increased bleeding because of impairment of venous return, and lesions of the eye, peripheral nerves, and spinal cord.12 At our institution, we adopt the three quarter–prone position to perform lateral approaches.6 The side of the approach is ipsilateral to the lesion. If the lesion is placed midline, the side of the approach is usually the side of the nondominant VAs and the nondominant jugular bulb. The body is placed in a lateral position, falling to the side of the craniotomy, and the arm contralateral to the operating side is placed out of the operating table and toward the floor and is padded with an axillary roll to avoid peripheral nerve damage. The knees and other pressure points are also padded to avoid damage to the peripheral nerves, and the legs are flexed to protect the femoral nerves. To avoid displacement of the patient’s body during operating table movements, adhesive tape is attached to the operating table and then applied at the hip and shoulder. The position of the head is crucial, as the surgeon needs good exposure of the occipitocervical region for a good angle of view of the contents of the posterior fossa. A three-point head holder is placed so that the mastoid bone is at the highest point of the approach. The neck should be slightly flexed and the vertex angled down, up to 30 degrees, with the face rotated slightly ventrally. The head should not be flexed more than two to three fingers from the thyroid and should not be rotated more than 45 degrees to prevent impairment of venous drainage. The results of this positioning are the cerebellum falling away from the operating field and the contents of the lateral aspect of the FM and posterior fossa being placed right under the surgeon’s view. Intraoperative monitoring is composed of somatosensory evoked potentials, auditory evoked responses, facial nerve monitoring, and monitoring of the X, XI, and XII cranial nerves, although their use is based on surgeon preference and acceptance.4,5,7,10

Skin Incision and Muscular Dissection

An inverted hockey stick–shaped incision is made, as it provides good exposure of the muscular layers, brings the surgeon closer to the surgical field, and requires lesser retraction of the soft tissues of the head and neck. A linear incision can also be used. The incision starts at the midline of the neck 5 cm below the inion and extends superiorly toward this reference. At the level of the inion, the incision is moved laterally right above the superior nuchal line and ends at the top level of the ear, about 2 to 3 cm medial to the pinna in the sagittal plane. From the last point, the incision extends down toward the mastoid and straight down over the posterior border of the sternocleidomastoid muscle. Knowledge of the muscular layers and of the neurovascular structures of the posterior neck is essential for the performance of the posterolateral approach and its variants. Dissection of each muscle is not recommended, as it creates extra dead space, increases the risk of infection, and causes muscle ischemia, which leads to wound dehiscence. We can summarize the muscular stage in three main steps: (1) elevation of the superficial muscles to expose the suboccipital triangle, (2) dissection of the suboccipital triangle to expose the VAs, and (3) transposition of the VAs if needed. At our institution, we avoid the use of cauterization to dissect the musculature, as it can damage the VAs with an abnormal loop out of the suboccipital triangle and disrupt the boundaries of the muscular layers, which damage the venous plexuses of the posterior neck, increasing bleeding and the risk of air embolism.

The skin flap is usually composed of the incised skin and galea, which are first elevated to expose the underlying pericranium. This structure, in addition to the superficial fascia of the neck, is elevated to expose the musculature of the posterior neck. The pericranium should be preserved to make a fascial graft for dural closure at the end of the operation. The first muscular layer that is exposed using this maneuver is composed by the sternocleidomastoid and trapezius muscles. They are incised near their insertion at the superior nuchal line and mastoid, leaving a cuff of tissue that is used later for closure of the incision. The underlying muscular layer (second or middle layer) is composed by the splenius capitis, longissimus capitis, and semispinalis capitis muscles. They are also incised and reflected as a single layer to expose the third layer, which forms the suboccipital triangle. The triangle is formed medially by the rectus capitis posterior major muscle, inferiorly by the inferior oblique muscle, and superolaterally by the superior oblique muscle; the VAs and its venous plexus are located in its center. Anatomic knowledge of the suboccipital triangle muscles and their insertions is essential to provide the best and safest exposure of the VAs. The rectus capitis major muscle inserts onto the inferior nuchal line and the spinous process of C2 and should be detached from the inferior nuchal line and reflected posteriorly. The inferior oblique muscle inserts onto the transverse process of C1 and onto the spinous process of C2 and the superior oblique muscle inserts at the inferior nuchal line and onto the transverse process of C1. Both muscles should be detached from the transverse process of C1 and reflected posteriorly. This maneuver exposes the C1 lamina, the VAs, the VAs venous plexus, and the C1 root.13

Knowledge of the muscular layers of the posterior neck is useful to prevent bleeding from the vascular structures of this region. Each muscular layer covers a vascular layer composed by a venous plexus and muscular arterial branches. Arterial blood supply for the muscles is provided by the occipital artery and by the muscular branches of the VAs. As the muscular branches of the VAs pass through the suboccipital triangle to reach the muscles, this is a crucial point for homeostasis. The main source of bleeding and air embolism in this region is the venous network. The venous system of the posterior neck is divided into two connected plexuses: (1) the suboccipital venous plexus and (2) the plexus around the VAs. The suboccipital venous plexus is superficial and is located in a space formed by the splenius capitis muscle superiorly and the longissimus capitis semispinalis capitis muscles inferiorly. The suboccipital plexus reaches the suboccipital triangle via the muscular cleft between the latter muscles and drains it into the plexus, thus surrounding the VAs through the anterior vertebral vein.14 Using the scalpel blade to cut through the muscles allows easier identification of these vascular layers and enables coagulation before bleeding and air embolism to occur.

Exposure of the Extradural VAs

The VAs is divided into four segments. V1 is the segment that runs from the origin of the artery at the subclavian artery and ends at the vertebral foramen of C6. V2 runs within the vertebral foramina from C6 through C1. V3, which is the horizontal segment of the vessel, begins at the transverse foramen of the atlas, runs through a groove on the upper surface of the posterior arch of the atlas, and ends by piercing the dura of the posterior fossa, medial and to the right of the OC. V4 is the intradural segment of the VAs and joins the opposite side vessel to form the basilar artery13 (Fig. 43-1).

Exposure and transposition of the VAs is not needed in the basic far lateral approach, in which drilling of the OC is not required.15,16 To transpose the vessel in the other variations of the far lateral approach, dissection and manipulation of the venous plexus around the VAs, which is sometimes referred to as the suboccipital cavernous sinus, is needed. The suboccipital cavernous plexus is connected to the suboccipital plexus through the suboccipital triangle and via the anterior vertebral vein.4 It is also connected to the internal vertebral venous plexus, posterior and anterior condylar veins, and occipital marginal sinus. To avoid intense bleeding from the plexuses, subperiosteal detachment of the VAs from its groove in C1 is recommended. To transpose the VAs, unroofing of the C1 transverse process is also mandatory. After detachment of the VAs and plexus, laminectomy of the C1 arch as laterally as possible can be performed to expose the OC for drilling.1,2,4,5,10

The V3 segment of the VAs has some branches that need to be coagulated during the approach. The first and largest is the anterior VAs, which passes through the suboccipital triangle to reach the muscles of the posterior neck. The posterior meningeal artery is another branch that can be coagulated. Care should be taken not to coagulate a posteroinferior cerebellar artery (PICA) or a posterior spinal artery that arises extradurally from the V3.

Osseous Stage: Suboccipital Craniectomy and Hemilaminectomy

The target points of the osseous stage of the approach are (1) exposure of the borders of the sigmoid and transverse sinuses, (2) resection of the ipsilateral margin of the FM, (3) resection of the squama of the occipital bone to the midline, and (4) resection of the ipsilateral border of the posterior arch of C1. If additional lateral space is needed, the OC can be removed in a subsequent step.

The landmarks for orientation of the craniotomy are (1) the asterion, (2) the midline, (3) the posterior border of the mastoid, (4) the inion, and (5) the superior nuchal line. The asterion is closely related to the lateral portion of the sulcus of the transverse sinus, especially with its inferior margin. To expose the lateral angle of the junction between the transverse and the sigmoid sinuses, a bur hole is placed immediately posterior and inferior to the asterion. This retrosigmoid point is the keyhole to the lateral suboccipital approach and exposes the posterolateral border of the cerebellar hemisphere.17 The inferior margin of the transverse sinus is located over a 50-mm line beginning at the inion and running across the superior nuchal line. This is the upper limit of the lateral suboccipital approach.

A high-speed drill is used to thin the squama of the occipital bone, and rongeurs are used to perform an occipital craniectomy. Another option is to perform a craniotomy of the posterior fossa. The mastoid air cells are the lateral limit of the suboccipital approach and are drilled until the borders of the sigmoid sinus and jugular bulb are exposed. The ipsilateral border of the FM is removed, and the occipital bone is removed to the point where it joins the OC.

To improve the inferior dural exposure, a hemilaminectomy of C1 is performed after detachment of the VAs and its plexus from its groove in C1. If additional exposure is needed, the C2 and C3 laminas can be removed.

Small tumors without rostral extension require small craniectomy; however, craniotomy is preferred for tumors with rostral extension, as replacing the bone protects the dura and limits the postoperative occipital pain.

Condylar Stage

The OC, which is an oval-shaped osseous structure located at the base of the occipital bone, articulates the skull in relation to the cervical spine. The anterior portion of the condyle is directed anteriorly and medially toward the basion. The posterior portion ends at the level of the middle portion of the FM and blocks the angle of view to an anterior portion of the FM and of the craniovertebral junction. The resection of the posterior aspect of the condyle increases the angle of exposure, reduces brain stem retraction, and increases the working area of the posterior fossa.48,10,18,19 The presence of small anterior tumors, an elongated FM, a short distance between the foramen and the brain stem, and relatively large OCs represent the ideal conditions for resection of the condyle.20

A high-speed drill is used to remove the posterior portion of the condyle after displacement of the VAs to avoid injury of the vessel. The amount of condyle that can be safely removed is controversial; however, biomechanical studies showed that the removal of more than 50% of the condyle leads to considerable hypermobility of the craniocervical junction, in which case fusion is indicated.5,21 The removal of the cortical bone (which forms the external capsule of the condyle) exposes cancellous bone (which forms the core of the condyle). Drilling of this bone exposes the lateral aspect of the intracranial portion of the hypoglossal canal; this landmark is approximately at the limit of the posterior third of the condyle. Another maneuver that can be used to achieve a better view of the anterior portion of the clivus is the removal of the JT, a bony prominence situated above the hypoglossal canal, in cranial and medial extensions of the tumor.8,11

Many variants of the far lateral approach have been proposed, according to the amount of bony resection at the condylar region.4,5,10,18 The basic far lateral approach comprises the steps described earlier, without condylar drilling. An occipitoatlantal transarticular transcondilar approach is performed after the condyle and the C1 superior articular facet are removed. The occipital–transcondylar approach exposes the clivus and the lower medulla and is performed after drilling the atlanto-occipital joint, condyle, and lower border of the hypoglossal canal. A supracondylar variant increases the exposure of the lateral aspect of the clivus and is directed above the condyle. During the transtubercular approach, the JT above the hypoglossal canal is removed to expose the area in front of the lower cranial nerves. The paracondylar approach is achieved via drilling of the area lateral to the condyle to resect lesions of the jugular process and of the posterior aspect of the mastoid.5,11,13

Intradural Exposure

The dura is opened parallel to the sigmoid sinus, crossing the circular sinus at the FM. Extreme care must be taken when opening the circular sinus, as the large venous plexus renders homeostasis more difficult and the risk of embolism increases.

All procedures performed after opening of the dura are carried out under the microscope. The arachnoid is opened and kept in place to facilitate the dissection or identification of the following neurovascular structures: the VAs and PICA (identification and dissection), the anterior spinal artery, and the cranial nerves (spinal division of the XI, IX, X, and XII cranial nerves, as well as identification and dissection, if possible, although sometimes the lower cranial nerves and the VAs are encased by the tumor). In general, tumors that encase the VAs can be removed via an arachnoid plane. In tumors located below the VAs, the lower cranial nerves may be identified in the superior part of the tumor. In contrast, the position of these nerves cannot be anticipated in tumors with superior extension.

The tumor is approached first via the side of the main vasculature at the dural attachment. The tumor is devascularized and removed piecemeal using an ultrasonic aspirator, with protection of the neurovascular structures involved. The bone and the dura involved by the meningioma attachment are also removed, if possible, to avoid recurrence. A meticulous homeostasis is performed, and the dura is closed in a watertight manner with the aid of patches from the pericranium or of dural substitutes. The mastoid bone, if open, is filled with bone wax, pieces of muscle, and fibrin glue. To avoid dead space, the posterior part of the aponeurotic-muscle flap is made and is sutured onto the dura.

Illustrative Cases

Patient 1

A 63-year-old woman presented with chronic upper neck pain, headaches, and neck stiffness. She also complained of weakness of the limbs. Thorough neurologic examination revealed hypotrophy of the tongue, tetrahyperreflexia, bilateral paresis of the trapezium, hypophonia, and left-palate deviation during phonation. MRI revealed the presence of a right anterolateral extramedullary tumor of the FM, with superior extension toward the jugular foramen and along the right hypoglossal canal, and development on both sides of the VAs. The tumor was hypointense on T1-weighted MRI and hyperintense on fluid-attenuated inversion recovery and T2-weighted MRI, with homogenous enhancement after the infusion of gadolinium. CT angiography was performed to obtain relevant information on the VAs and the sigmoid sinus. CT with bone windows was also performed to assess the shape of the FM. Stereotactic image guidance was used. The surgical procedures were planned using an imaging database that included stereotactic CT and MRI scans (Fig. 43-2).

The tumor was resected using a far lateral suboccipital approach. The patient was placed in the three quarter–prone position, with the head fixed with a three-pin head holder (Fig. 43-3). Excessive superior shoulder traction was avoided. The head of the patient was tilted slightly downward to open the space between the mastoid and the neck. The incision ran laterally in an inverted hockey stick–shaped fashion, from the right mastoid process to the occipital protuberance, and then curved medially to the spinous process of C4. After exposure of the VAs (V2 to V3), a right far lateral suboccipital craniectomy was performed that included the rim of the FM. The JT was drilled because of the upward extension of the tumor. A right C1 hemilaminectomy without condyle resection was tailored. The dura was opened in a linear shape over the cerebellum, after identification of the VAs entry point. The next step consisted of draining the CSF by opening the cisterna magna. The procedure led to the spontaneous sinking of the cerebellum, which rendered significant retraction unnecessary. The dentate ligament was sectioned before the initiation of tumor removal and widening of the surgical corridor. A small portion of the V4 was identified before tumor involvement, and the dissection was performed by following the artery inside the tumor along an arachnoid plane. Microsurgical resection of a firm, consistent, and hypervascularized lesion with dural attachment was performed. The bulk of the tumor was removed using microscissors, cupped forceps, and an ultrasonic aspirator and was continued piecemeal. The tumor matrix was then coagulated, the basal dura was partially resected, and Simpson grade II resection was achieved. The only nerve that was identified initially was the spinal portion of cranial nerve XI and the posterior rootlets of the first cervical nerve. All of them were preserved. The other nerves and the VAs branches were identified during tumor removal. Control MRI and CT scan showed complete resection of the tumor (Figs. 43-4 and 43-5). After surgery, in the immediate postoperative period, videolaringoscopy showed that paresis of the lower cranial nerves worsened, providing evidence that a tracheostomy and gastrostomy were needed. The patient’s symptoms resolved, with the exception of some mild facial paresis and transient right brachial paresis (brachial plexus neuropraxis). Histopathologic analysis revealed the presence of a transitional meningioma.

Patient 2

A 44-year-old male patient was admitted for the first time in 2003. At that time, he complained of progressive weakness of the legs and arms (mainly of the left arm) for a few months prior to admission. At neurologic examination, he exhibited mild tetraparesis and hyperreflexia. Imaging demonstrated the presence of a vast lesion based on the anterolateral portion of the FM, which was suggestive of a meningioma that compressed the medulla. He was operated on in 2003 via a suboccipital extreme lateral approach and the lesion was partially removed. The surgery was stopped when the resection caused transitory cardiac arrhythmia.

The patient was supposed to undergo follow-up; however, he did not comply in full, as he was afraid of a second surgery, which was recommended and necessary. Five years later, the patient returned because his symptoms had worsened. He was tetraparetic, in a wheelchair, and unable to stand up. In addition, he had dysphonia, dysphagia, and nocturnal apnea, with paralysis of the IX, X, XI, and XII cranial nerves, suggesting the presence of pyramidal-tract deficits and brain stem compression. Imaging revealed that the lesion had grown considerably and had infiltrated the medulla, engulfing the VAs. At that time, he finally accepted the second operation, as he had no other options. We started by performing a traqueostomy, as a fibroendoscopy examination showed that he had pulmonary microaspiration. He was reoperated on with complete monitorization of the affected neurologic functions and by using the same approach; however, we extended the surgery laterally and extradurally, as the tumor infiltrated the extradural portion of the VAs. The tumor was radically removed after we found an irregular and incomplete plane between the tumor and the brain stem. The postoperative period was uneventful. He recovered partially from his deficits, and at the last follow-up, he was able to stand up and walk, with the only remaining symptom being cranial nerve XII paresis (Fig. 43-6).

Patient 3

A 13-year-old girl presented with difficulty in swallowing and a severe occipital headache that was aggravated by physical activity. The symptoms started 15 months before examination. Neurologic examination was normal. MRI showed the presence of a widespread retropharyngeal tumor. The tumor involved both carotid arteries, displaced both VAs, and extended caudally to C3. The tumor seemed to replace the OCs bilaterally, as well as the arch of C1 and the inferior border of the clivus, from whence it invaded the subarachnoid space posterolaterally and ventrolaterally. CT showed intense bone involvement.

Because the symptoms were the result of the retropharyngeal location of the tumor, we thought that midline anterior and posterolateral exposure was required. First, we felt that the midline anterior approach would be the best for the removal of the anterior mass. Thus, an extended transoral–transpalatopharyngeal approach was performed. After retraction of the pharyngeal mucosa and of the longus colli and longus capitis muscles, a gelatinous yellow mass was found that did not have a capsule. The tumor, which was suckable and firm, was removed piecemeal using a drill and an ultrasonic aspirator under microscopic view. The anterior arch of the atlas was resected, as were the inferior border of the clivus, which is limited bilaterally by the JT and the lateral mass of C1 and C2. The midline dura was infiltrated. Careful closure was performed, during which the longus colli and the longus capitis muscles were covered with the pharyngeal mucosa. The palatal layers were closed, and a nasogastric feeding tube was placed under direct vision. A postoperative MRI scan showed a decompression of the retropharyngeal mass; however, marked ventral and posterolateral compression of the cervicomedullary junction remained.

The second stage of the tumor resection was performed using the far lateral approach. A midline incision was performed because the tumor invaded the spinal canal bilaterally. Gross tumor resection was achieved that included part of the OC (on the right side) and the JT, with bilateral transposition of the VAs. The tumor that invaded the subarachnoid space was removed after careful dissection from the lower cranial nerves. A dorsal occipitocervical fusion was performed that spanned C1 and C2 and reached the fusion at the C3 or C4 level. Postoperatively, the patient exhibited swallowing deterioration; thus, gastrostomy and traqueostomy were performed. The traqueostomy was discontinued after the end of the third week after surgery, and the gastrostomy was discontinued after 3 months. Histopathologic examination revealed the presence of a chordoma. Postoperative MRI showed the presence of a residual mass, without compression (Fig. 43-7). The patient was able to return to her normal life and underwent MRI-based follow-up periodically.

Results

Our group of authors treated 22 patients with FM tumors. The mean age of the 14 women and eight men was 57.3 years. There were 12 meningiomas, all of them located intradurally and 10 arising from the anterior or anterolateral rim. One tumor was located in the posterior midline, and another had a posterolateral origin. One hypoglossal schwannoma had intradural and extradural components, and a C1 neurofibroma completed the intradural tumors in this series. Chordomas were the most common type of extradural tumors. The most common symptom was suboccipital neck pain and/or headache. Other symptoms included motor weakness, gait imbalance, myelopathy, and numbness. Table 43-1 lists the types of tumors, and Table 43-2 shows the clinical presentations of the patients in our cohort.

TABLE 43-1 Distribution of Pathology

Tumor Type Patients
Meningioma 12
Schwannoma 1
Neurofibroma 1
Chordoma 5
Glomus tumor 1
Chondrosarcoma 1
Metastatic kidney carcinoma 1
Total patients 22

TABLE 43-2 Clinical Presentation of Patients with FM Tumors

Signs and Symptoms Cases by Presented Symptom (Confirmed after Examination)
Suboccipital neck pain 12
Headache 8
Motor weakness 9 (5)
Gait imbalance and myelopathy 5 (2)
Numbness 5 (6)
Cranial nerves  
 Swallowing difficulty 7 (5)
 Tongue atrophy 3 (2)
 Speech problems 3 (2)
 Diplopia 1 (2)
 Hearing deficit 1 (2)
Hand deficit 1 (1)
Vomiting 1 (1)

The posterior midline approach was performed in 2 patients with posterior and posterolateral meningioma, respectively. The far lateral approach was used in the other 10 patients. The rostrocaudal extension tailored the size of the craniotomy. For tumors with upward extension or located above the intracranial VAs, a combined retrosigmoid approach was performed in four cases and a petrosal approach was used in three cases, all of which had predominantly extradural tumors. An extended transoral–transpalatopharyngeal approach was used in two patients, both with chordomas. These patients required additional petrosal and far lateral transcondylar access. Drilling of the JT was performed in four cases that had FM meningiomas with cephalad extension. Partial resection of the lateral mass of C1 was performed in one patient with a C1 neurofibroma. The OC was partially resected in three patients with meningioma and in one patient with hypoglossal schwannoma (Table 43-3).

For intradural tumors, the VAs was mobilized in five cases, although it was encased or displaced in all cases. This maneuver was performed at the dural entry of the artery at the FM. For extradural tumors, the VAs was encased in four patients with chordomas, and it was displaced in another four patients. In extradural tumors, the VAs was mobilized in all cases, either for dissection or for improved exposure. In these cases, the VAs was transposed at the C1 transverse foramen and mobilized medially. There were no injuries in the VAs, neither in extradural nor in intradural tumor cases.

Seven patients with extradural tumors had some degree of condylar resection. The resection of the condyle performed in these patients was done because of the invasive nature of the tumors and/or direct involvement by the tumors in five patients with a chordoma. A complete condylar resection was performed in one patient with metastatic kidney carcinoma. One chordoma patient underwent a partial condylar resection because of extensive bone invasion; the purpose of the surgery was decompression. In the case of a glomus tumor, the lesion was approached more anteriorly and required partial condylectomy and resection of the JT.

Occipitocervical fusion was performed in four chordoma patients and in one patient with metastatic kidney carcinoma who had complete resection of the condyle. These surgeries were performed after the removal of their tumors using an elective fusion procedure.

The grade of tumor resection for intradural tumors was based on Simpson’s classification.21 For nonmeningiomas, the degree of resection was divided into three categories: (1) total resection, in which the entire tumor was removed; (2) subtotal resection, in which a small fragment of the tumor remained on vital structures, such as the VAs, cranial nerves, or the brain stem; (3) partial resection, in which the bulk of the tumor remained.5

For meningiomas, radical excision (Simpson grades I and II) was achieved in 10 cases (83.3%). In most cases, the dura insertion was coagulated or removed. Two patients had Simpson grade III resection. The incomplete resection performed in one patient was attributable to a firm adhesion of the lower cranial nerves to the VAs and poor dissection planes; in another patient, intentional decompression was planed because of the clinical condition. Other patients underwent two surgeries (see the illustrative case of patient 2). The patient with hypoglossal schwannoma had a total resection, and the patient with a dumbbell-shaped neurofibroma of C1 had total tumor removal via a transfacetal approach (Fig. 43-8).

Total resection was achieved in two cases of chordoma tumors; the remaining three patients had a partial removal because of the extensive nature of the tumor, which occupied multiple compartments, invaded the jugular foramen and the cavernous sinus, and encased the carotid arteries. The patient with chondrosarcoma also had partial tumor removal because of the firm adherence to the lower cranial nerves (Table 43-4).

There was no surgical mortality in this series. The patient with metastatic kidney carcinoma died because of multiple metastases. The second death was a chordoma patient who was in poor condition and in whom tumor removal was intentionally partial.

A postoperative CSF leak occurred in four patients and required surgical repair in one case. A new deficit of the accessory nerve was observed in one patient, and a deficit of the hypoglossal nerve occurred in another individual who underwent partial recovery. Dysphagia developed in four patients; however, their recovery was complete. Transient hoarseness occurred in two patients, and abducens nerve palsy occurred in two subjects (one of whom developed a permanent deficit). Three patients required postoperative traqueostomy, and two underwent gastrostomy. After 6 months, these problems were resolved completely. Periodical clinical examination and MRI scans were performed for all patients. The mean Karnofsky performance scale score improved in 13 cases (63.6%), was kept stable in 8 cases (36.3%), and worsened in 1 case.

Comments

Surgical treatment is the best approach to treat tumors of the FM, especially meningiomas, schwannomas, neurofibromas, and chordomas. Most lesions can be removed using posterior approaches; however, when the tumors are located anteriorly or anterolaterally, resection using traditional approaches becomes more difficult. Some authors used posterior approaches to reach posterolateral and anterolateral tumors located at the FM,22,23 as large tumors provide a working space that is sufficient for resection of the tumor. However, there is not enough space to control the VAs between C2 and C3 and to reach essential anterior midline and small tumors.

Intradural extramedullary tumors, which are located anterolaterally or ventrally and are mostly meningiomas, generally tend to extend cranially and caudally. For this reason, and to obtain sufficient exposure of the cervicomedullary junction, tumor interface, rostrocaudal extension, and attachment at the dura, as well as to achieve dissection of the lower cranial nerves and control of the VAs and their branches, approaches that are more lateral are required. Among them, the far lateral approach and its variants, such as the transcondylar, transtubercle, and transfacetal methods, met the criteria. Resection or drilling of the condyle posterior to and below the hypoglossal canal provides an enhanced view of the target area. The extreme lateral transcondylar approach, which implies drilling the condyle, was used by some neurosurgeons.46,8,9,24 This procedure is not required for most intracranial tumors because there is risk of vertebral artery injury.16

Transposition of the VAs is another controversial point. Injury of this vessel or transposition of atherosclerotic VAs may result in permanent or transient neurologic deficit.16 For intradural tumors in patients with a normal VAs, the artery can be mobilized at the dural entry but not transposed posteromedially, because this implies drilling the condyle and unroofing the transverse foramen of C1. For patients with extradural tumors, resection of the condyle is not as controversial; in such patients, drilling the bone is part of the surgical treatment.5,10

Incomplete resection is a challenge for neurosurgeons. The size and the rostral extension from the tumor to the midline of the clivus are factors that influence morbidity.24 Remnants of tumors with strong adherence to the brain stem or to the VAs and their branches should not be resected. We prefer to follow the patient periodically instead of sending the patient to radiation therapy because of the risk of complications and because a second procedure would be more difficult.5,16,25

Nowadays, the entire circumference of the FM can be accessed. Anterior extradural lesions located on the midline can be exposed and removed from transoral approaches combined with mandibular splitting and maxillotomy.

The far lateral approach with or without condylar resection has the advantage of being combinable with the petrosal, retrosigmoid, and infratemporal approaches. It also allows the removal of most of tumors, either intradural or extradural lesions.

Key References

Bassiouni H., Ntoukas V., Asgari S., et al. Foramen magnum meningiomas: clinical outcome after microsurgical resection via a posterolateral suboccipital retrocondylar approach. Neurosurgery. 2006;59:1177-1187.

Boulton M.R., Cusimano M. Foramen magnum meningiomas: concepts, classifications, and nuances. Neurosurg Focus. 2003;14(6):10.

Bruneau M., George B. Foramen magnum meningiomas: detailed surgical approaches and technical aspects at Lariboisiére Hospital and review of the literature. Neurosurg Rev. 2008;31:19-33.

George B.. Foramen magnum tumors, Shmidek H.H., Roberts D.W. Schmidek and Sweet. Operative Neurosurgical Techniques: Indications, Methods, and Results, 5th ed., Philadelphia: WB Saunders, 2005.

Gupta S.K., Khosla V.K., Chhabra R., et al. Posterior midline approach for large anterior/anterolateral foramen magnum tumors. B J Neurosurg. 2004;18(2):164-167.

Margalit N.S., Lesser J.B., Singer B.A., Sen C. Lateral approach to anterolateral tumors at the foramen magnum: factor determining surgical procedure. Neurosurgery. 2005;56(ONS suppl 2):ONS-324-ONS-326. pp. 324-336

Menezes A.H. Surgical approaches: postoperative care and complications “posterolateral–far lateral transcondilar approach to the ventral foramen magnum and upper cervical spinal canal”. Childs Nerv System. 2008;24:1203-1207.

Rhoton A.L.Jr. The posterior cranial fossa: microsurgical anatomy & surgical approaches. Neurosurgery. 2000;47(suppl 3):S131-S153.

Roberti F., Sekhar L.N., Kalavaconda C., et al. Posterior fossa meningiomas: surgical experience in 161 cases. Surg Neurol. 2001;56:8-21.

Salas E., Sekhar L.N., Ziyal I.M., et al. variations of extreme-lateral craniocervical approach: anatomical study and clinical analysis of 69 patients. J Neurosurg (Spine 2). 1999;90:206-219.

Wanebo J.E., Chicoine M.R. Quantitative analysis of the transcondylar approach to the foramen magnum. Neurosurgery. 2001;49:934-943.

Numbered references appear on Expert Consult.

References

1. George B.. Foramen magnum tumors, Shmidek H.H., Roberts D.W. Schmidek and Sweet. Operative Neurosurgical Techniques: Indications, Methods, and Results, 5th ed., Philadelphia: WB Saunders, 2005. pp. 1755-1765

2. George B., Lot G. Anterolateral and posterolateral approaches to the foramen magnum: technical description and experience from 97 cases. Skull Base Surg. 1995;5:9-19.

3. Bruneau M., George B. Foramen magnum meningiomas: detailed surgical approaches and technical aspects at Lariboisiére Hospital and review of the literature. Neurosurg Rev. 2008;31:19-33.

4. Arnautovic K., Al-Mefty O., Husain M. Ventral foramen magnum meningiomas. J Neurosurg. 2000;92:71-80.

5. Margalit N.S., Lesser J.B., Singer B.A., Sen C. Lateral approach to anterolateral tumors at the foramen magnum: factor determining surgical procedure. Neurosurgery. 2005;56(ONS suppl 2):ONS-324-ONS-326.

6. David C.A., Spetzler R.F. Foramen magnum meningiomas. Clin Neurosurg. 1997;44:467-490.

7. Boulton M.R., Cusimano M. Foramen magnum meningiomas: concepts, classifications, and nuances. Neurosurg Focus. 2003;14(6):10.

8. Borba L.A.B., Oliveira J.G., Giudicissi-Filho M., et al. Surgical management of foramen magnum meningiomas. Neurosurg Rev. 2009;32:49-60.

9. Miller E., Crockard H.A. Transoral transclival removal of anteriorly placed meningiomas at foramen magnum. Neurosurgery. 1987;20:966-968.

10. Roberti F., Sekhar L.N., Kalavaconda C., et al. Posterior fossa meningiomas: surgical experience in 161 cases. Surg Neurol. 2001;56:8-21.

11. Salas E., Sekhar L.N., Ziyal I.M., et al. variations of extreme-lateral craniocervical approach: anatomical study and clinical analysis of 69 patients. J Neurosurg (Spine 2). 1999;90:206-219.

12. Rozet I., Vavilala M.S. Risks and benefits of patient positioning during neurosurgical care. Anesthesiol Clin. 2007;3:631-653.

13. Rhoton A.L.Jr. The posterior cranial fossa: microsurgical anatomy & surgical approaches. Neurosurgery. 2000;47(3 suppl):S131-S153.

14. Reis C.V., Deshmukh V., Zabramski J.M., et al. Anatomy of the mastoid emissary vein and venous system of the posterior neck region: neurosurgical implications. Neurosurgery. 2007;61(suppl 2):193-200. discussion 200–201

15. Nanda A., Vincent D.A., Vannemrredy P.S.S.V., et al. Far-lateral approach for intradural lesions of the foramen magnum without resection of the occipital condyle. J Neurosurg. 2002;96:302-309.

16. Bassiouni H., Ntoukas V., Asgari S., et al. Foramen magnum meningiomas: clinical outcome after microsurgical resection via a posterolateral suboccipital retrocondylar approach. Neurosurgery. 2006;59:1177-1187.

17. Ribas G.C., Yasuda A., Ribas E.C., Nishikuni K., Rodrigues A.J.Jr. Surgical anatomy of microneurosurgical sulcal key points. Neurosurgery. 2006;59(suppl 4):ONS-177-ONS-210.

18. Menezes A.H. Surgical approaches: postoperative care and complications “posterolateral–far lateral transcondilar approach to the ventral foramen magnum and upper cervical spinal canal”. Childs Nerv System. 2008;24:1203-1207.

19. Pamir M.N., Kilic T., Ozduman K., et al. Experience of a single institution treating foramen magnum meningiomas. J Clin Neurosci. 2004;11(8):863-867.

20. Wanebo J.E., Chicoine M.R. Quantitative analysis of the transcondylar approach to the foramen magnum. Neurosurgery. 2001;49:934-943.

21. Simpson D. The recurrence of intracranial meningiomas after surgical treatment. J Neurol Neurosurg Psychiatry. 1957;20:22-39.

22. Gupta S.K., Khosla V.K., Chhabra R., et al. Posterior midline approach for large anterior/anterolateral foramen magnum tumors. B J Neurosurg. 2004;18(2):164-167.

23. Goel A., Desai K., Muzumdar D. Surgery on anterior foramen magnum meningiomas using a conventional suboccipital approach: a report on an experience with 17 cases. Neurosurgery. 2001;49:102-107.

24. Kano T., Kawase T., Horiguchi T., Yoshida K. Meningiomas of ventral foramen magnum and lower clivus: factors influencing surgical morbidity, the extent of tumor resection, and tumor recurrence. Acta Neurochir. Sep 25, 2009.

25. Ganz J.C., Reda W.A., Abdelkarim K. Adverse radiation effects after gamma knife surgery in relation to dose and volume. Acta Neurochir. 2009;151:9-19.