Orbital Meningiomas Meningiomas Orbitários Carlos Eduardo Da Silva, M.D
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31 Revisão Orbital Meningiomas Meningiomas Orbitários Carlos Eduardo da Silva, M.D. 1 Paulo Eduardo Freitas, M.D. Ph.D.2 Alicia Del Carmem Becerra Romero, M.D.3 Tâmen Moyses Pereyra4 Vicente Faraon Fonseca4 Willian Alves Martins4 Márcio Aloisio Bezerra Cavalcanti Rockenbach4 Fáberson João Mocelin Oliveira4 ABSTRACT RESUMO Orbital meningiomas usually invade the orbit as an extension Meningeomas orbitários invadem a órbita, na maioria dos ca- of the sphenoid wing meningiomas, clinoidal meningiomas, sos, como uma extensão de meningeomas da asa do esfenóide, cavernous sinus meningiomas and tuberculum sella tumors. meningeomas do seio cavernoso, meningeomas da clinóide e They also arise into the orbit originating from the optic sheath do tubérculo da sela. Eles também podem ser originados do or as ectopical lesions. The authors present a review of clini- revestimento dural do nervo óptico ou como lesões ectópicas cal aspects and surgical treatment of the orbital meningio- intraorbitais. Os autores apresentam uma revisão dos aspectos mas. Material and methods: The authors present a literature clínicos e cirúrgicos dos meningeomas orbitários. Material e review of the anatomical, clinical, and surgical aspects of métodos: Os autores apresentam uma revisão da literatura dos the orbital meningiomas, add illustrative cases, pointing aspectos anatômicos, clínicos e cirúrgicos dos meningeomas their principal concerns about the treatment of such tumors. orbitários, com casos ilustrativos, apresentando suas principais Results: Exophthalmos and unilateral visual loss are the most preocupações no manejo destes tumores. Resultados: Exoftal- common features of the orbital meningiomas. There are two mia e perda visual unilateral são os achados mais frequentes important surgical routes to approach such tumors, which are nos meningeomas orbitários. Existem duas importantes vias de extracranial (transorbital) and transcranial approaches. Re- acesso para tais tumores, que são a extracraniana (transorbi- currence is higher in sphenoorbital meningiomas and related tária) e a transcraniana. A recorrência no grupo dos meningeo- to bone and neurovascular invasion. Conclusions: The ana- mas esfeno-orbitários é maior e ralacionada a invasão do osso tomical structures involved by orbital meningiomas challenge e das estruturas neurovasculares. Conclusões: As estruturas the microsurgical resection. Total removal should be the goal anatômicas envolvidas pelos meningeomas orbitários tornam at the first surgical approach. Orbital reconstruction should estes tumores verdadeiros desafios para ressecção microcirúr- be performed in cases where the orbital floor is not preserved gica, sendo a ressecção total o objetivo durante a primeira or in extensive lateral and roof removal with no preservation cirurgia. A reconstrução orbitária deve ser efetuada nos casos of the periorbit. em que o assoalho orbitário não é preservado, assim como em casos de ressecções extensas da face lateral e do teto da órbita, Keywords: Orbital tumors, orbital anatomy, orbital menin- sem a preservação da periórbita. giomas, skull base surgery. Palavras-chave: Tumores orbitários, anatomia da órbita, meningeomas orbitários, cirurgia de base de crânio. 1- Neurosurgeon Coordinator, Neurosurgical Fellowship for Medical Students, Department of Neurosurgery and Skull Base Surgery, Hospital Ernesto Dornelles. Neurosurgeon, Department of Neurosurgery, Hospital Cristo Redentor, GNSHC. Porto Alegre, Brazil. 2- Neurosurgeon Senior, Department of Neurosurgery, Hospital Ernesto Dornelles. Porto Alegre, Brazil. 3- Neurosurgeon, Department of Neurosurgery, Hospital Ernesto Dornelles. Porto Alegre, Brazil. 4- Medical Student, Medical Student Fellow, Department of Neurosurgery and Skull Base Surgery, Hospital Ernesto Dornelles. Porto Alegre, Brazil. Recebido em novembro 2009, aceito em dezembro 2009 Silva CE, Freitas PE, Romero ADCB, Pereyra TM, Fonseca VF, Martins WA, Rockenbach MABC, Oliveira FJM - Orbital Meningiomas J Bras Neurocirurg 21 (1): 31-38, 2010 32 Revisão IntroductIon anatomIcal consIderatIons Orbital tumors are classified according to their original site ORBITAL BONE ANATOMY in: primary lesions, arising from the orbit itself; secondary le- The orbit is composed by 7 bones: frontal, ethmoid, sphenoid, sions, originating from surrounding structures; and metastatic maxilla, zygomatic, lacrimal and palatine. The superior border lesions. Orbital meningiomas comprise 3 to 10% of all intra- of the orbital opening is formed by the frontal bone, which orbital tumors. 6,8 They usually invade the orbit as an extension presents a notch or foramen for the supratrochlear and supra- of the sphenoid wing , cavernous sinus , clinoid and tubercu- orbital vessels and nerves. The medial border is formed by lum sella tumors. They may also arise from the optic sheath or the frontal bone superiorly and the frontal process of the ma- as ectopical rests of the arachnoid cells within the retrobulbar xilla inferiorly. Lateral border is formed by the frontal process space 8. The anatomical structures involved by orbital menin- of the zygomatic bone and a small portion superiorly by the giomas make them formidable challenges for microsurgical zygomatic process of the frontal bone. The inferior border is resection. The authors present a review of clinical aspects and composed by the maxilla medially and the zygomatic bone la- surgical treatment of the orbital meningiomas. terally.16 (Fig. 1) epIdemIology Meningiomas represent 18 to 20% of all intracranial tumors. Primary orbital meningiomas comprise 0.4 to 2% of all menin- giomas and secondary lesions present the distribution accor- ding to their initial sites. Sphenoid wing meningiomas com- prise between 16 to 20% of the intracranial meningiomas and the suprasellar region originates 7 to 30% of all meningiomas. Such data evidence that orbital meningiomas are secondary Fig. 1. - Orbital Bone Anatomy (frontal view). Left - Skull; Right - 3D CT. lesions most of the time. Females are more affected, with 73 FB - frontal bone; SOF - superior orbital fissure; OC - optic canal; LSW - lesser to 84% of the cases. Meningiomas are uncommon in children sphenoid wing; IOF - inferior orbital fissure; GSW - greater sphenoid wing; fpZB - frontal process of the zygomatic bone; fpMax - frontal process of maxillar and neurofibromatosis is a possible risk factor associated to an bone; nlc - nasolacrimal canal; MaxB - maxillar bone; ZB - zygomatic bone; IOfo - 5 6,8 orbital meningioma in infants. infraorbital foramen. The orbital roof is composed by the orbital plate of the frontal bone and the lesser wing of the sphenoid bone. The orbital floor is formed by the orbital plate of the maxilla, the orbital surface clInIcal aspects of the zygomatic bone and the orbital process of the palatine bone. The lateral wall is formed by the greater sphenoid wing Primary and secondary orbital meningiomas present similar and the frontal process of the zygomatic bone. Posteriorly, the clinical presentation. Exophthalmos and unilateral visual loss inferior orbital fissure (IOF) separates the lateral wall and the are the most common features described in the literature. (5) Di- orbital floor. The superior orbital fissure (SOF) separates the plopia, pain, swelling and headaches are frequently observed lateral wall and the roof of the orbit. The nasolacrimal canal in such cases. Nausea and vomiting are symptoms associated perforates the anteromedial portion of the orbital floor. to intracranial hypertension. Optic nerve atrophy can be obser- At the posterior orbital portion there are three bone apertures, ved as a result of direct compression of the nerve. Diplopia is which allow the neurovascular structures to access the orbit secondary to direct involvement of the cranial nerves or extra- from the temporal fossa, cavernous sinus, suprasellar region ocular muscle disfunction. In large secondary orbital menin- and infratemporal fossa. The cavernous sinus is directly rela- giomas, intracranial mass effect and hypertension can produce ted with the orbit and the cranial nerves III, IV, V and VI enter 5 papilledema in both optic nerves. the posterior portion of the orbit through the superior orbital fissure. (Fig. 2) Silva CE, Freitas PE, Romero ADCB, Pereyra TM, Fonseca VF, Martins WA, Rockenbach MABC, Oliveira FJM - Orbital Meningiomas J Bras Neurocirurg 21 (1): 31-38, 2010 33 Revisão CRANIAL NERvES ANATOMY The oculomotor nerve originates two roots at the SOF, which enter the orbit inside the annular tendon with the extra-ocular muscles and vessels, at the central portion of the SOF. Such central region is called oculomotor foramen. 4,16 (Fig. 3) Fig. 2. - Cranial nerve anatomy at SOF and cavernous sinus - digital schematic representation. ON - optic nerve; ICA - internal carotid artery; CS - cavernous si- nus; C2 and C3 - portions of the ICA; III - oculomotor nerve; IV - trochlear nerve; V - trigeminal nerve; VI - abducens nerve; OS - optic strut. The optic canal is located inferiorly to the anterior clinoid pro- cess and the optic nerve leaves the canal medially to the C2 Fig. 3 -Orbital Cranial Nerve Anatomy (frontal view - Superior Orbital Fissure) III - oculomotor nerve; IV - trochlear nerve; Fr. N - frontal nerve; Lac. N - lacrimal (4) portion of the internal carotid artery. (Fig. 2) nerve; Nasoc. N - nasociliary nerve; V - trigeminal nerve; VI - abducens nerve; ON - optic nerve. The blue line corresponds to the annular tendon. The optic canal opens into the superomedial portion of the or- bital apex and