Skull Base Approaches to Clival Chordomas



Fig. 26.1
Preoperative bony window sagittal CT scan



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Figs. 26.2 and 26.3
Preoperative MRI scan




26.5 Anatomy of the Approach


A precise knowledge of the anatomy of the nose, paranasal sinuses, sellar and parasellar region, as well as clival, paraclival, and CCJ areas with all associated crucial neurovascular structure is mandatory. Skull base neurosurgeons are used to the transcranial perspective of anatomical structures such as the ICA, but the endonasal view of it can be confusing for beginners. It definitely requires some effort, some practice in an anatomical laboratory, and the assistance of an ENT surgeon to safely identify crucial anatomical structures while performing such approaches. The use of neuronavigation system cannot replace a strong knowledge of the anatomy.

Landmarks are mandatory to navigate in the nasal cavities. One crucial landmark to begin with is the choanal arch. The choanal arch should be considered as a light in the dark for the beginners, and every time the surgeon get lost into the nasal cavities, going posteriorly, along the nasal fossa floor to the choanal arch, is particularly helpful to confirm that the line of sight of the approach is not too high toward the anterior skull base. The next major landmark is the middle turbinate. When the middle turbinate is mobilize medially, the uncinate process and, immediately behind, the bulla ethmoidalis come into view. One should keep in mind that the lateral wall of the bulla ethmoidalis is the thin medial wall of the orbit. The maxillary sinus will be reached behind and lateral to the inferior part of the uncinate process.

The sphenopalatine foramen (SPF) is a notch between the orbital and sphenoidal processes of the ascending process of the palatine bone. Following backward the ethmoid crest, which is a small spur of the bone of the medial surface of the ascending process of the palatine bone, the SPF with the sphenopalatine artery (SPA) running through is identified.

Following backward the nasal septum, the ostium of the sphenoid is located about 1.5 cm above the roof of the choanal arch. Below the level of the ostium runs the artery that vascularizes the mucosa of the nasal septum: the posterior septal artery (terminal branch of the sphenopalatine artery).

Once into the sphenoid sinus, some major landmarks may be recognized. Below the sella is the clival recess that is bordered laterally by the prominences created by the paraclival segment of the ICA. Those two prominences appear in case of a well pneumatized sphenoid sinus as two pillars on both side of the clival recess. The paraclival segment of the ICA corresponds to the posterior ascending segment of the cavernous segment of the ICA (C4 segment) that follows the lacerum segment of the ICA (C3 segment) [29]. The junction between the lacerum segment and the posterior segment of the ICA is also called the anterior or second genu of the ICA. The sellar floor limits superiorly the clival depression. On both sides of the anterior wall of the sella can be identified the slight prominence of the horizontal segment of the cavernous ICA followed by the recess formed by the middle clinoid process and, above it, the prominence of the clinoidal segment of the ICA. Above the prominence of the sella is the depression formed by the tuberculum sellae. Lateral to it is the medial opticocarotid recess that corresponds on the intracranial surface to the lateral tubercular crest. The opticocarotid recess that corresponds to the pneumatization of the optic strut (Inferior root of the anterior clinoid process) lies between the prominence of the clinoidal segment of the ICA and the prominence of the optic canal.

One should keep in mind that the shape of the posterior wall of the sphenoid sinus is highly variable from one patient to another. Two factors contribute to such variations: Firstly, the degree of pneumatization is variable, and in case of a conchal form of sphenoid sinus, none of those landmarks may be identified clearly. Secondly, the shape of the cavernous ICA is extremely variable [29], and the paraclival segment of the ICA can either run straightly vertical, slightly medially, or laterally or can be, rarely, tortuous. Those variations dictate the presence and shape of the clival depression and are important to consider preoperatively when planning an endoscopic transclival approach.

While resecting a tumor located into the clivus, the structure at high risk of damage is the abducens nerve (CN VI). From its emergence from the brain stem at the level of the bulbopontine sulcus (approximately at the level of the vertebral convergence which is a straightforward landmark after opening the dura mater in the midline), CN VI runs upward and slightly laterally. Following the same direction, it then runs into a dural canal in which CN VI is followed by a sleeve of arachnoid for a variable distance (abducens nerve cistern). Into Dorello’s canal, below Gruber ligament, CN VI changes direction and became more horizontal and moves lateral to the posterior ascending segment of the ICA. Then, it courses slightly upward along the medial and inferior surface of the horizontal segment of the cavernous sinus ICA (Fig. 26.4). When looking at the CN VI from an endoscopic endonasal 2D perspective with a 0° scope, the CN VI in its cisternal segment is medial to the paraclival ICA until it reaches Dorello’s canal. It then runs lateral to the paraclival segment of the ICA. The region at most risk for the CN VI nerve in case of clival chordomas is located immediately before and into Dorello’s canal. CN VI nerve monitoring is therefore critical and helpful to identify CN VI. However, the efficiency of CN VI nerve monitoring is often diminished by a preoperative CN VI paresis, which is one of the most frequent presenting symptoms of clival chordomas.

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Fig. 26.4
Abducens nerve trajectory


26.6 Surgical Technique



26.6.1 Preoperative Planning


The approach should be carefully planned to achieve the three main goals of the surgery: (1) exposition, (2) resection, and (3) reconstruction. Considering those three steps, which are closely linked together, we have to consider several critical issues:



  • If the patient had already prior surgery, the availability of vascularized flaps has to be evaluated: nasoseptal flap (NSF) in case of previous endonasal surgery and temporoparietal fascia flaps in case of previous craniotomy.

    A vascularized flap is mandatory in almost all cases because the procedure will often lead to at least a “naked” ICA or a dural defect. The vascularized flap serves as a remodeling tissue to reconstruct any dural defect and to prevent any secondary ICA rupture due to the inflammation and erosion of the wall of the ICA.

    The presence of scar tissue may also impede the tumor dissection as well as the identification of critical vascular and nervous structures.


  • In case of previous radiotherapy, the issue of having a reliable vascularized flap is even more critical. Patients are at risk of radionecrosis, and the healing process is usually impaired with a higher risk of CSF leak. The identification and control of critical neurovascular structures such as the ICA surrounded by scar tissue is also at higher risk in case of previous irradiation.

Then, the side of the NSF harvest should be planned before the surgery, and it depends on several factors: its availability on both sides, the side of the main lateral extension, the presence of a septal deviation, and the size of the both nasal cavities.

The CT anatomy of the nasal cavities should be carefully reviewed, searching for septal deviation, Onodi cells, and bone defect over the ICA and studying the pneumatization of the sphenoid sinus. A CT angiography should be considered systematically as it offers a precise evaluation of the main intra- and extracranial vessel trajectories (ICA in all its segments, VA and BA, PCom) as well as their relationship with the tumor. The need of a proximal control of the ICA prior tumor resection must also be anticipated especially when the tumor is extending laterally and posteriorly to the paraclival ICA. In such cases an additional transpterygoid approach, following the vidian nerve posteriorly toward the lacerum segment of the ICA, may be necessary before tumor resection.

Depending on the anatomy of the nasal cavities, the exact location and extent of the tumor, and the need of a NSF, one could consider a one nostril or binostril approach. In case of a small extradural chordomas, it may be possible to achieve a complete resection through only one nostril, keeping one nasal cavity intact and reducing the postoperative discomfort of the patient. In case of a large chordomas, with intradural extension, a binostril approach is almost unavoidable allowing a proper exposure of the tumor, enough space for the movement of the instruments, and a good control of the critical structures.


26.6.2 Surgical Technique and Pitfalls



26.6.2.1 Tools and Patient Preparation


The endonasal endoscopic approach is systematically performed using image-guided surgery (IGS) with MRI (contrast T1-weighted and T2-weighted) and CT imaging fusion (Fig. 26.5) as well as a microvascular ultrasound Doppler probe to accurately localize the internal carotid arteries. The precision of the navigation system should be confirmed regularly during the procedure. Before starting the surgery, we find very useful to test the accuracy of the neuronavigation using the groove between both superior incisive that are clearly visible on the properatice CT scan. Deeper, the accuracy is checked along the rail of the septum and using the bony septations into the sphenoid sinus. This is mandatory when working at high depth at the level of the lower clivus and CCJ.

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Fig. 26.5
Image-guided surgery (IGS) with MRI and CT imaging fusion

Monitoring of the cranial nerve is also critical, especially the monitoring of CN VI that is most often involved in clival chordomas. In case of lower clivus chordomas with significant lateral extension toward the condyl and hypoglossal canal, monitoring of cranial nerve XII is also mandatory. It is even more important in case of unilateral cranial nerve palsy to avoid a bilateral XII nerve palsy that is a devastating complication for the patient. In case of brain stem compression or infitration, monitoring of motor evoked potential may also be helpful.

Standard 4 mm, 30° and inverted 30° scope is commonly used (KARL STORZ GmbH & Co., Tuttlingen, Germany). 70° scope may be very helpful for lesion located laterally, typically behind the horizontal segment of the ICA. Scope with variable direction of view from 0° to 120° might be also extremely useful to check the deep corners of the surgical field (KARL STORZ GmbH & Co., Tuttlingen, Germany). This ability to inspect such corners with multidirection scope may reduce the need of additional surgical steps required to expose those deep regions using regular 0° or 30° scopes.

Binostril approach is most commonly used. With experience and training, a one nostril approach can be considered in case of small well-defined lesion. However, the functional benefit for the patient of sparing one nasal cavity should not be overestimated and should not limit the ability to achieve a radical resection that is even more critical for the patient. The need for two, three, or four hands depends on the step of the surgery. The initial ENT steps of the approach most often require only two or three hands. The main surgeon is holding the scope while performing the approach with its other hand in a sequential use of the instruments. A third hand may be needed for suction of blood or smoke caused by the use of bipolar or monopolar coagulation. When drilling is required, the same combination can be used with one surgeon holding the scope and the drill and another one holding the suction. Another option is for the main surgeon to work with both hands with another surgeon holding the scope. Self-irrigating drills are mandatory. When working deep at the level of the CCJ, long instruments and an angled drill handpiece are most useful to navigate the curve of the palate.

In case of recurrence and reoperation, neuronavigation and Doppler are mandatory as most of the anatomical landmarks, and the initial bone protection over the ICA may not be present anymore. The main rule in such “redo procedure” is to spot the bony limits from the previous surgery. The Doppler is crucial and has to be used at early stage in the surgery if the ICA is proved to be “naked” on the preoperative CT angiography.

The nostrils are prepared by 2 ml of 10 % naphazoline with adrenaline, applied topically with patties. The septum and lateral nasal wall is injected with 2 ml of 2 % lidocaine with adrenaline. The thorax is slightly elevated and the head is slightly flexed, rotated, and tilted toward the surgeon and secured in a three-pin head holder. According to the navigation system that is used, the head has to be immobilized in a pin holder or could be moved freely. The pin holder headrest gives the advantage to freeze the position of the head. It’s also important to adapt the flexion of the head according to the location of the tumor. The flexion needs to be accentuated for lesion located at the lower clivus and CCJ. If a temporoparietal flap is needed, a non-fixed head is more convenient to harvest the flap and tunnelize it.

Intraoperative elevation of the head and thorax should also be anticipated in order to diminish the venous pressure and the venous bleeding that may occur at the level of the cavernous sinus or basilar venous plexus if necessary. The abdomen or anterolateral thigh is prepared for harvesting a fat and/or fascia lata graft to repair the bone and dural defects.


26.6.3 Surgical Approach



26.6.3.1 Endonasal Steps


The approach is initiated doing a review of the anatomy of the nostril. To gain space inferiorly for the endoscope, inferior turbinate may be out fractured and lateralized. Then, a middle turbinectomy is performed in the right nostril to provide enough space for the endoscope and the suction. The resection of the left middle turbinate may not be necessary and can be only fractured laterally.

Following the resection of the middle turbinate, a posterior ethmoidectomy can be performed on the side of the NSF in order to facilitate the harvesting of the flap. This step is best performed by a microdebrider. Before starting the ethmoidectomy, the ostium of the sphenoid sinus as well as the posterior root of the middle turbinate must be identified in order to envision the position of the SPA and the pedicle of the NSF. The uncinate process is resected and the bulla ethmoidalis is removed exposing the lamina papyracea. Behind the inferior part of the uncinate process, the maxillary sinus is entered, and the medial wall of the maxillary sinus, above the inferior turbinate, is removed from front to back toward the angle between the medial and posterior wall of the maxillary sinus. The basal lamella of the middle turbinate is opened to enter the posterior ethmoid, and the subsequent ethmoid air cells are removed from front to back. Finally, the superior turbinate is removed and the sphenoid sinus is entered. Care must be taken to remain above the level of the ostium not to injure the SPA and the pedicle of the NSF. Another option to locate the SPF and SPA is to elevate the mucoperiosteum over the ascending process of the palatine bone and to follow backward the ethmoid crest.

It is not always necessary to open the maxillary sinus. The opening of the maxillary sinus is required for orientation purposes, when an ipsilateral transpterygoid approach is planned or in order to store the NSF during the surgery.

Then, the NSF is harvested from back to front starting with the inferior mucosal cut so that no blood trickle will obscure the surgical field [17]. Section of the mucosa is made with a monopolar diathermy needle at minimum effective power to spare the olfactory mucosa as much as possible.

The incision starts above the superior lip of the Eustachian tube and follows the inferior aspect of the choanal arch toward the septum. Then, the incision runs anteriorly along the angle between the septum and the floor of the nasal cavity. In case of an expected large dural defect, the size of the NSF can be increased with the adjunction of the mucosa of the floor of the nasal cavity and eventually the mucosa of the inferior meatus. The superior cut starts above the ostium of the sphenoid and runs anteriorly along the nasal septum, 1.5–2 cm below the roof of the nasal cavity in order to spare the olfactory fibers.

Then, a vertical anterior incision cut joins the superior and inferior cut.

The flap is elevated from anterior to posterior and placed either in the nasopharynx or into the maxillary sinus through a wider antrostomy, depending on the extent of the tumor. It can also be positioned in the sphenoid sinus in case of tumor located at the level of the CCJ. The flap can be hold in the maxillary sinus with the placement of large cottonoid into the maxillary sinus.

A crucial step consists in removing the posterior septum allowing an easy introduction of instruments from the contralateral nostril without contamination of the optic by the posterior septum itself. A rescue flap may be secondarily performed with the contralateral septal mucosa that is cut posteriorly and reversed anteriorly to cover the remaining denuded septum [30].

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May 26, 2017 | Posted by in NEUROSURGERY | Comments Off on Skull Base Approaches to Clival Chordomas

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