Multimodal Treatment of Orbital Tumors

Chapter 50 Multimodal Treatment of Orbital Tumors



The surgical management of intraorbital tumors requires a thorough understanding of not only orbital anatomy but also the objectives of surgical intervention. The orbital contents abut the skull base, paranasal sinuses, and intracranial compartment or anterior cranial fossa. The orbit is a quadrangular compartment that can be approached through a variety of trajectories along one of its four walls or posteriorly from its apex. From the surgical standpoint, the orbit represents an anatomic compartment that is encountered by several specialties—neurosurgery, ophthalmology and otolaryngology—with their own approaches derived from the pathologic processes they usually encounter.


From the neurosurgical perspective, the orbit is exposed in the surgical management of pathologic processes involving the orbit, in addition to the skull base or intracranial cavity. The neurosurgeon often becomes involved in orbital disease cases when tumors or vascular lesions are present. Orbital tumors are often benign (i.e., meningioma, cavernous hemangioma, and schwannoma) but may be locally invasive or infiltrative. Malignant lesions (i.e., cranial base sarcomas, esthesioneuroblastomas, and squamous cell carcinomas) are even more likely to require a multidisciplinary approach because of their spread across multiple anatomic compartments. Primary orbital tumors are often discrete from normal orbital structures and may engulf, although not necessarily invade, the extraocular muscles and/or optic nerve; secondary tumors extend into the orbit along normal anatomic structures (often nerves) or by bony destruction. As such, the surgical objectives for processes involving the orbit range from the need for negative margins or aggressive resection to purely orbital decompression.


Selecting the most appropriate surgical treatment for orbital disease processes requires an understanding of the various open and endoscopic surgical approaches and their advantages, indications, and limitations. The primary considerations are the location and size of the tumor, including its relationship to crucial orbital structures, the site of origin, the other extraorbital anatomic compartments involved, and histologic diagnosis.



Orbital Anatomy



Orbital Bony Anatomy


The bony orbit not only lies close to other compartments in the facial skeleton but also contains several foramina through which critical neurovascular structures pass1 (Fig. 50-1). The orbital roof is formed by the frontal bone and lesser wing of sphenoid; its superior surface is the floor of the anterior cranial fossa. The roof also lies below the frontal sinus. The orbital plate of the maxillary bone forms most of the orbital floor, in addition to the roof of the maxillary sinus; the palatine and zygomatic bones also contribute to the floor. Medially, the medial wall, also known as the lamina papyracea, forms a thin wall separating the orbit from the ethmoid sinus anteriorly and the sphenoid sinus more posteriorly, a structure through which most endoscopic approaches to the orbit can be directed. It is comprised of four bones: the maxillary, lacrimal, ethmoid, and lesser wing of sphenoid. The medial wall of the orbit also contains the foramina through which the anterior and posterior ethmoidal arteries pass. The lateral orbital wall is covered externally by the temporalis muscle and formed by the frontosphenoid process of the zygomatic bone, in addition to the greater wing of the sphenoid. The apex of the orbit is directed in a medial oblique direction and contains three critical foramina: optic canal, superior orbital fissure, and inferior orbital fissure. The optic canal bridges the intracranial space and orbit inferomedial to the anterior clinoid process while it is bordered laterally by the lesser wing of the sphenoid. The superior orbital fissure is bordered laterally by the greater wing of the sphenoid.




Muscle Cone and Annulus of Zinn


The annulus of Zinn serves as the origin of six of the seven extraocular muscles (Fig. 50-2). Superiorly, the superior rectus arises from the annulus, which at this point is fused with the dura of the optic nerve. The levator palpebrae arises medial and superior to the superior rectus muscle but remains intimately associated with it. More medial and inferior to this are the origins of the medial rectus and superior oblique muscles. Although it is firmly fused to the optic nerve dorsally, the annulus of Zinn loops widely around the nerve laterally and inferiorly, giving rise to the lateral rectus muscle, in addition to the inferior rectus. The space between the insertion sites of these two muscles is known as the oculomotor foramen. Based on this arrangement, there are evident portals of entry of neurovascular structures into the orbit: the optic canal and the superior orbital fissure.




Optic Nerve and Orbital Nerves


The optic nerve, throughout its entire course from the chiasm to sclera, is covered with a pial membrane (providing vascular supply) and associated subarachnoid space (Fig. 50-3). As the optic nerve enters the optic canal, the intracranial dura splits, with the outer leaf forming the orbital periosteum and the inner leaf remaining with the optic nerve. The superior orbital fissure contains the remaining cranial nerves that enter the orbital compartment. The trochlear nerve and the frontal and lacrimal branches of the trigeminal nerve enter through the superior orbital fissure above the extraocular muscles and annulus of Zinn. The remaining nerves—the oculomotor nerve (superior and inferior divisions) and the abducens nerve—pass through the superior orbital fissure and annulus of Zinn.



Because of this arrangement, it is evident that the optic nerve can be approached directly through the medial compartment, between the medial rectus and the levator muscles, without fear of injury to the nerve supply of any extraocular muscle (Fig. 50-4).




Case Selection


The treatment strategy for orbital lesions is primarily determined by the nature of the lesion. An overview of the types of orbital lesions is provided in Table 50-1.2 While medical management is best suited for infectious and inflammatory processes involving the orbit, definitive surgical treatment remains the mainstay for a majority of symptomatic orbital tumors and dysthyroid orbitopathy.


TABLE 50-1 Overview of Orbital Lesions








































































Category Lesions
Vascular Capillary hemangioma
  Cavernous hemangioma
  Hamartoma
  Hemangiopericytoma
  Lymphangioma
Nerve sheath Neurilemomas
  Neurofibroma (plexiform and solitary)
Osseous and cartilaginous Osteoma
  Osteogenic sarcoma
  Chondroma
  Fibrous dysplasia
  Aneurysmal bone cyst
Neuroepithelial Optic nerve glioma
Meningioma  
Mesenchymal Rhabdomyosarcoma
  Lipoma
  Liposarcoma
Inflammatory Nonvasculitic
  Vasculitic, nongranulomatous
Carcinoma  
Cystic  
Other  

The diagnostic workup proceeds logically after clinical examination and primarily consists of imaging. Magnetic resonance imaging (MRI) with fat suppression provides excellent definition of orbital pathology.3,4 Fat suppression eliminates the T1 bright signal associated with orbital fat that can obscure intraorbital pathology. Contrast-enhanced MRI provides excellent soft tissue resolution and is the best modality to determine intracranial extension of orbital pathology.5,6 Computed tomography (CT) imaging provides excellent definition of orbital pathologic process and regional bone anatomy. An adequate study includes thin cuts through the orbits and shows normal orbital anatomy, including the size and position of the globe, optic nerve, and extraocular muscles. CT is superior to MRI in surgical planning because of its ability to show bony anatomy, but MRI is preferred when optic nerve involvement by the tumor or disease process must be examined.


Once a thoughtful sequential diagnostic workup has been completed, the location and extent of the pathologic process must be defined and following questions considered:


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Jul 12, 2018 | Posted by in NEUROSURGERY | Comments Off on Multimodal Treatment of Orbital Tumors

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