Giant cavernoma of the orbit: clinical and surgical considerations

Cavernoma gigante da órbita: considerações clínico-cirúrgicas

SCIENTIFIC NOTES

Giant cavernoma of the orbit: clinical and surgical considerations

Cavernoma gigante da órbita: considerações clínico-cirúrgicas

Feres Chaddad Neto^I; Armando Lopes^II; Mario Machado Filho^III; Aguinaldo Catanoce^III; José Maria Campos Filho^IV; Evandro de Oliveira^V

^INeurocirurgião do Instituto de Ciências Neurológicas, São Paulo, Brasil (ICNE); Neurocirurgião-Assistente da Disciplina de Neurocirurgia do Departamento de Neurologia da Universidade de Campinas, Campinas SP, Brasil (UNICAMP)

^IIMédico Residente do Centro de Neurocirurgia de Coimbra, Centro Hospitalar de Coimbra, Portugal (CHC)

^IIIMédico do Instituto de Ciências Neurológicas, São Paulo, Brasil (ICNE)

^IVMédico Residente de Neurocirurgia da Faculdade de Ciências Médicas da Universidade Estadual de Campinas, Campinas SP, Brasil (UNICAMP)

^VDiretor do Instituto de Ciências Neurológicas, São Paulo, Brasil (ICNE); Professor e Chefe da Disciplina de Neurocirurgia da Faculdade de Ciências Médicas da Universidade Estadual de Campinas, Campinas SP, Brasil (UNICAMP)

ABSTRACT

Cavernous hemangiomas of the orbit are benign, hamartomatous, vascular lesions, more frequent in middle-aged women, representing the most common benign primitive neoplasm of the orbit. Several therapeutic modalities and surgical approaches have been described, in order to preserve the normal orbital structures. We describe the case of a patient with a volumous orbital cavernoma, approached by a fronto-orbito-zigotomy. A review on diagnostic modalities and surgical approaches is also presented.

Key words: cavernoma, surgical approach, fronto-orbito-zigotomy.

RESUMO

Os hemangiomas cavernosos da órbita são lesões vasculares hamartomatosas benignas, mais frequentes em doentes do sexo feminino e de meia idade, constituindo a neoplasia benigna primitiva da órbita mais comum. Diversas modalidades terapêuticas e abordagens cirúrgicas têm sido descritas com a finalidade de preservar as estruturas orbitárias. Apresentamos o caso de uma doente com volumoso cavernoma da órbita esquerda, intervencionado por abordagem fronto-órbito-zigomática. É efetuada ainda revisão das modalidades de diagnóstico imageológico e das diferentes estratégias cirúrgicas descritas na literatura.

Palavras-chave: cavernoma, abordagem cirúrgica, fronto-órbito-zigomática.

Cavernous hemangiomas of the orbit are benign vascular lesions accounting for approximately 5% of all primitive orbital neoplasms¹. They usually occur in adult-age, where they represent the most common vascular orbital tumor². They are more frequent in females and may grow during pregnancy. They differ from the brain cavernomas in their behavior and radiological features³, and may coexist with venous angiomas in the same location suggesting a common origin and development⁴. Multiple cavernomas of the orbit are rare entities that can recur after complete excision, and may exist with concurrent systemic vascular tumors⁵. The posterior part of the orbit contains a number of important and delicate strutures including the optic nerve, the ophthalmic artery and veins, the ocular muscles and their motor nerves, which makes the surgical access to this area quit difficult.

The case we present is illustrative.

CASE

39 years-old woman, presenting with recent, progressive, painless, proptosis in the absence of other clinical / neurological features.

MRI revealed a space occupying lesion in the left orbit, indicative of orbital cavernous hemangioma (Figs 1 and 2).

The lesion was removed by a fronto-orbito-zigotomy extended to the medial wall of the contra lateral orbit. The procedure was uneventful and a cranioplasty with methylmetacrilate was performed for esthetical correction (Figs 3 to 6).

The patient remained neurologically intact after the procedure.

Histopathological analysis revealed a cavernous angioma of the orbit. Follow up MRI show no reminiscent, and, three years after surgery, the patient is still asymptomatic.

DISCUSSION

Histopathology – Vascular tumors of the orbit result from new formation of blood vessels, proliferation of tissue components of the vessel wall and hyperplasia of cellular elements ordinarily concerned with genesis of vascular tissue⁶. They are well delimitated benign vascular hamartomas, formed by sinusoidal vascular channels with irregular walls.

Clinical features – Painless and progressive proptosis associated or not with visual impairment, causing a variable degree of hyperopia, is the usual clinical presentation⁷. Cranial nerves III - VI palsy and diplopia is uncommon.

Diagnosis – Nearly all cases of orbital cavernoma can be correctly diagnosed by pre-operative neuroimaging study. The combined use of ultrasonography and computerized tomography (CT) may establish the nature, extent and location of tumors and vascular malformations of the orbit, and can be useful for selection of the surgical approache⁸.

CT and magnetic resonance imaging (MRI) are of particular importance in the diagnosis of orbital vascular lesions. The improved image quality of CT and MRI along with dynamic CT angiography, MRI angiography, MRI venography, and multiphase dynamic contrast CT/MRI imaging, has proven useful to delineate and differentiate different orbital vascular lesions⁹.

From the analysis of MRI appearance of a intraconal well-defined mass, with homogeneous signal, isointense to muscle on T1-weighted sequence, hyperintense on T2-weighted sequence, and progressive filling on gadolinium enhanced sequences, in a patient presenting with progressive, painless proptosis, the diagnosis of cavernous hemangioma is highly suggestive¹⁰.

Scintigraphy with technetium Tc 99m (99mTc) labeled red blood cell, is a useful tool in the differential diagnosis of cavernous hemangioma from other orbital masses^{11, 12}.

A complete neuroimaging study with computerized tomography, magnetic resonance and scintigraphy, enables the correct pre-operative diagnosis of vascular orbital lesions

Treatment – In those cases presenting with visual impairment or significant exophthalmous, immediate treatment is indicated¹³. The remaining, with minimal or non existing exophtalmous, can safely be followed by observation. There are cases in which the incomplete removal of cavernous hemangioma led to eventual involution of the reminiscent, with clinical relief¹⁴.

Lateral orbitotomy has been widely employed for the removal of orbital tumors, being used in volumous hemangiomas, especially those located in the lateral compartment of the orbit and lateral apex¹⁵.

Anterior orbitotomy is useful in many cases, without significant complications and postero-inferior orbitotomy through the maxillary sinus¹⁶ can be used in small, well delimited lesions in the posterior and inferior orbit near the apex.

Resection of retro-bulbar tumors by transconjunctival approach is feasible, without relevant morbidity¹⁷.

Endoscopic transethmoidal approach of the orbit is a minimally invasive surgery for retro-bulbar orbital neoplasm, leading to excellent cosmetic results with less bleeding. However, the access to intraconal lesions near the apex may be difficult^18,19.

Trans-frontal, trans-fronto-ethmoidal and trans-maxillary approaches have the disadvantage of possible injuries to a number of nontumor structures ¹⁹. Displacement of tumor lesions to the maxillary sinus has been used to prevent the traumatic and dangerous excessive traction of hemangiomas²⁰.

Lateral suprabrow²¹, trans-orbital sub-frontal and pterional approaches are employed for removal of tumors affecting the posterior two thirds of the orbit and tumors originating in or intruding into the optic canal²².

Transcranial approaches offer an excellent surgical exposure and a good cosmetic effect and should be considered for big lesions located superiorly or medially to the optic nerve, especially those involving the apex¹³.

Fronto-orbito-zigotomatic approach leads to excellent functional results, being of particular importance in deep lesions and in those with intracranial component²³. The possibility of anterior clinoide removal and opening of the optic channel and superior orbital fissure, allows a wider surgical view and a safe transposition of the optic nerve.

Sclerosing endovascular therapy of low flow vascular lesions of the orbit has been advocated as a alternative to surgery in selected cases²⁴.

Cryosurgical extraction of cavernous hemangioma of the orbit, guided or not by stereotaxy has been described, but is not indicated for most orbital tumors^25,26.

The use in orbital surgery of neuronavigation systems offers three-dimensional coordinates of lesions, allowing a safer, controlled surgery²⁷.

Stereotactic radiosurgery is another treatment modality available for orbital and cavernous-sinus lesions²⁸.

In conclusion, cavernous hemangiomas of the orbit are benign lesions that usually occur in middle-aged women, presenting with progressive, painless proptosis. Due to improvement of neuroimaging techniques, it’s possible to obtain a correct pre-operative diagnosis in almost every case of vascular orbital lesions.

Despite of the employment of minimally invasive procedures in the treatment of these lesions, the authors consider that fronto-orbito-zigotomy is irreplaceable in volumous lesions of the two posterior thirds of the orbit, allowing an excellent cosmetic result with minimal morbidity.

Received 8 March 2007, received in final form 25 May 2007. Accepted 27 July 2007.

Dr. Feres Chaddad Neto - Praça Amadeu Amaral 27 / 5° andar - 01327-010 São Paulo SP - Brasil. E-mail: [e-mail]

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Publication Dates

• Publication in this collection
  06 Dec 2007
• Date of issue
  Dec 2007

History

• Accepted
  27 July 2007
• Reviewed
  25 May 2007
• Received
  08 Mar 2007