Fig. 32.1
Tectal plate glioma cases with i.v. contrast MR study. (a–c) does not have contrast enhancement and (d) minimal, whereas (e, f) demonstrates significant contrast enhancement
MRI is more reliable than CT for the visualization of tectal tumors and it should be ordered in every child and adolescent with hydrocephalus [9, 23]. MRI reliably detects a tectal mass and it is a reliable imaging modality for follow-up [15, 24]. There has been no consensus on significance of contrast enhancement in the tumorous region yet [10]. Contrast enhancement at presentation is seen in up to 18 % of cases [24, 25]. It is speculated that there is no correlation between contrast enhancement in the first imaging and tumor progression during follow-up. On the other hand, there is contrast enhancement in all cases with tumor enlargement during follow-up (Fig. 32.1d–f) [19, 24]. The T2-weighted images give more detailed anatomical information about these lesions particularly growth direction and relationship of the tumor with cerebellum and tegmentum (Fig. 32.2a–f).
Fig. 32.2
The T-2 weighted images give more detailed anatomical information. In the six cases shown above (a–f) the boundaries of the tectal lesions are very clear in T2 sequences
32.1.4 Surgical Treatment
The goal of treatment in tectal plate gliomas is to reverse the signs and symptoms of hydrocephalus and improve neurologic deficits and to prevent further neurologic damage [10]. One of the earliest treatment options is shunting of cerebrospinal fluid to peritoneum via a ventriculoperitoneal (VP) shunt [26].
Pollack al. [18] describe hydrocephalus for this group of patients to be more challenging than the usual hydrocephalic patient.
18.75 % of the patients (3 out of 16) in this report developed subdural hematomas.
Chapman [27] encountered similar problems in his shunted cases with 28.57 % (2 out of 7) cases resulting in subdural hematomas. Subsequent management is open-ended follow-up with neuroimaging. The second option is endoscopic third ventriculostomy (ETV). It is the gold standard for the management of hydrocephalus induced by tectal plate gliomas (Fig. 32.3a–d) and additionally allows the surgeon to take a biopsy from the lesion [28–30]. The third but less frequently used option is endoscopic aqueductoplasty with or without stenting [10].
Fig. 32.3
Tectal plate glioma case presenting with hydrocephalus (a, b). After endoscopic third ventriculostomy (c, d)
There are well-described safe and unsafe entry zones for open midbrain surgery [31]. It is advocated to use supracollicular incision, infracollicular incision, and lateral mesencephalic sulcus to enter the mesencephalon, whereas it is not advised to use incisions through superior and inferior colliculi [31].
The lesions in the tectal plate can be reached easily by occipital transtentorial and infratentorial supracerebellar approaches.
Occipital transtentorial approach has been adopted by many neurosurgeons for tumors located in this region, to avoid possible damage to colliculi (Fig. 32.4a–d). In this approach a right occipital craniotomy is performed and tentorium is incised just adjacent to the straight sinus. The direct visualization of the fissure between the superior vermis, quadrigeminal plate, and superior medullary velum is the advantage, whereas a probable visual defect resulting from occipital lobe retraction is the disadvantage of this approach [32, 33]. The same area can be also easily reached through standard infratentorial supracerebellar approach which is first described for pineal region tumors [33, 34]. Infratentorial supracerebellar approach can be used for more caudally located median tumors with infracollicular entry to avoid ocular and auditory disturbances [32]. Ternier et al. [35] centered their decision based on tumor volume. Tumors less than 4 cm3 are less likely to progress (hamartomas); lesions with volumes greater than 10 cm3 warrant consideration for an early intervention. Those in between, 4–10 cm (hamartomas or tumors), will benefit from frequent initial imaging.
Fig. 32.4
Tectal plate glioma case with documented tumor growth (a, b) treated via occipital transtentorial approach (c, d)
Possibility exists that the majority of these tumors increase and cause new deficits; thus, diligent long-term follow-up becomes a part of the armamentarium of the surgeon. Pollack et al. [14] recommend MR imaging and clinical examinations every 6 months for the 1st year after the diagnosis and then yearly for 4 years in patients who are asymptomatic. From the 6th year onwards, yearly clinical evaluation is sufficient and imaging studies are obtained every 2–3 years or in the interim if new symptoms develop. It is vital that the patients are warned there is a possibility of tumor progression (6.9 years from shunt insertion and 11.5 years from the onset of initial symptoms) in a certain percentage of patients, despite the so-called indolent nature of their tumors, and hence not to neglect follow-up [14].
32.2 Tegmental Tumors
Tumors arising from the tegmentum may extend upward to the thalamus or downward to the pons. They displace but do not infiltrate intrinsic mesencephalic nuclei and tracts although they may reach enormous size so that they may be candidates for surgical resection (Figs. 32.6a–c, 32.7a–c, and 32.10a–c) [32].
They are almost always focal low-grade astrocytomas. Therefore, total excision should be the aim for surgery of these tumors.
32.2.1 Basic Anatomy
Tegmentum consists of two cerebral peduncles, substantia nigra, red nuclei, oculomotor nerve nuclei, and medial and lateral longitudinal fasciculus. A tumor originating from tegmentum may show many different clinical presentations upon involvement of these structures [32]. On the other hand posterior cerebral and superior cerebellar arteries encircle the midbrain and are in close relationship with the oculomotor and trochlear nerve thorough their course. It is important to decide the most appropriate surgical approach that allows sufficient exposure of the chosen entry area while minimizing the damage to normal neurovascular structures [32, 36].
32.2.2 Clinical Presentation
The patient may present with hemiparesis, hemihypoesthesia, headache, bilateral papilledema, vertical gaze deficiency, oculomotor paresis, or paralysis upon involvement of midbrain structures [37].
32.2.3 Radiology
Tegmental gliomas are more frequently focal, round-shaped hypo- or isointense on T1-weighted MRI series, whereas they appear hyperintense on T2-weighted MRI series (Fig. 32.5a–c). They generally show no or little contrast enhancement on contrasted MRI scans (Fig. 32.6a–c) [19]. But some rare cases can present with strong enhancement (Figs. 32.8a–c and 32.10a–c). Cystic tumors should be evaluated in detail about their cyst configuration and location, since entering through a right area in the cyst will give a nice exposure for the removal of the solid part (Figs. 32.5a–c and 32.10a–c).
Fig. 32.5
Left sided tegmental cystic tumor with mural nodule (a–c). After tumor resection (d–f)
Fig. 32.6
Tegmentum tumor without contrast uptake (a–c). Staged surgery via pterional transsylvian and median suboccipital craniotomies (d–f)
32.2.4 Surgery
Surgical exposure to the mesencephalon is a challenge. With such a complex anatomy and a variety of sites where tumors may arise, there is no single approach that can provide optimal pathway.
The mesencephalic tegmental tumors can be divided into three groups:
(a)
Dorsal
(b)
Dorsolateral
(c)
Lateral
Success depends on detailed study of imaging and selection of appropriate route according to the location of the pathology that will facilitate maximal safe resection with minimal morbidity [38, 39].
Porter et al. [40] uses a two-point method to select the best approach. A line is drawn between the point where the tumor is closest to the surface and another at the center of the lesion. This will give an indication of which approach to utilize to excise the lesion. Ultimately, route to be selected should allow for excellent exposure and contribute to minimal retraction of brain tissue, vasculature, and cranial nerves ensuring complete excision of tumor with no or minimal complications.
Dorsal tumors of tegmentum can be reached by occipital transtentorial, infratentorial supracerebellar, or midline telovelar approach.
Occipital transtentorial approach is used for lesions superior to pontomesencephalic junction. In dorsal lesions there is a very thin amount of tissue under the pia. The supracollicular area is the entry point into mesencephalon, avoiding causing visual and oculomotor disturbances. In case of a cystic mass, the entry point should give a good vision to the solid part (Fig. 32.5d–f). Since use of a retractor will be not possible in such a deep area, a long plated bayonet can be used for retraction [41, 42]. During the resection of the tumor, pulling the tissue is not recommended; instead an ultrasonic aspirator should be used.
Infratentorial supracerebellar approach can also be used in removal of tegmental lesions. To reach tegmental lesions with this approach, the dural opening should be extended more laterally close to sigmoid sinus. Careful dissection of arachnoid allows wide opening of cerebellomesencephalic fissure. It is important to identify the lateral mesencephalic vein, which lies in the lateral mesencephalic sulcus. To avoid pyramidal tract injury, the entry zone should be posterior to lateral mesencephalic sulcus [32].
In case tumor is extending downward to the pons and the IV ventricle, a midline telovelar approach can be used (Fig. 32.6d–f). The disadvantage of this approach is the long distance to the lesion and working through the IV ventricle. The floor of the IV ventricle should be covered with cotton patties to avoid possible damage during the tumor removal. For big lesions which expand to the suprasellar area, the treatment can be staged and for the second step, a pterional approach can be utilized (Fig. 32.6).