Male patient, 52 years old with no history of interest, attended out service presenting exophthalmos that had been evolving for five years. He had recently been suffering from retroocular pain, which appeared recently in the right eye. He also reported a decrease in visual acuity at the end of the day, with no associated symptomatology.

On physical exploration proptosis or the right eye was observed with slight diplopia on down-gaze. The remaining ophthalmologic examination was absolutely normal. The ocular echography showed an alteration in the curvature of the eye. Nuclear Magnetic Resonance (RMN) was carried out with and without gadolinium contrast, with cuts in the three planes of space, which showed a retroocular and intraconal position in the right orbit measuring 3 x 3 x 2 cm, with well defined borders, which was leaving an impression on the posterior pole of the ocular globe causing an anterior displacement, and which was at the same time causing the optic nerve to be displaced medially and the inferior rectus muscle. The signal was intermediate on T1 weighted sequences and it was hyperintense on T2 weighted sequences, and it showed enhancement following the administration of gadolinium (Fig. 1,2,3). The radiologic diagnosis established a retroocular intraconal orbital mass on the right side that did not seem to infiltrate the optic nerve or the muscles. Carrying out Fine Needle Aspiration (FNA) was rejected due to the inaccessibility of the lesion.

Orbital cavernous hemangioma.
Bicoronal approach and lateral orbitotomy
Hemangioma cavernoso orbitario.
Abordaje bicoronal y orbitotomía lateral

R. González García¹, V. Escorial Hernández¹, A. Capote Moreno¹, J. Sastre Pérez²,
F.J. Rodríguez Campo², S. Hyun Nam³, M. Muñoz Guerra², L. Naval Gías²

1. Médico Residente de Cirugía Oral y Maxilofacial
2. Médico Adjunto de Cirugía Oral y Maxilofacial
3. Médico Residente de Anatomía Patológica
Hospital Universitario de la Princesa, Madrid, España

Correspondencia:
Raul González García
c/ Los Yébenes, 35 - 8ºC
28047 Madrid, España
E-mail: raugg@mixmail.com

Following a complete preoperative study that did not contraindicate surgery, a decision was taken to operate the patient with general anesthesia using a bicoronal approach from the ipsilateral preauricular region to the contralateral preauricular region, separating the skin mass underneath the subperiosteal and temporal fascia and starting from the zygomatic arch and frontal eminence. An approach was made through the entire external rim of the right orbit and right malar area, together with an osteotomy of the malar bone (lateral orbitotomy). The periorbit was then incised and an intraconal approach was made to the lesion, which had the appearance of a hemangioma and was well defined. It was resected and the surrounding tissue pulled away. The reconstruction of the zygomatic arch and the frontal eminence was carried out with flat miniplates.

The macroscopic examination of the histologic study showed a nodular formation with a diameter of 2.5 cm, which was well defined, brownish-black in color and with a hemorrhagic appearance. The microscopic examination revealed the existence of large cavernous vascular structures with hematic content, which were separated by a fine stroma of connective tissue (Fig. 4). The definitive anatomopathologic diagnosis is of cavernous hemangioma.

Discussion

Orbital cavernous hemangioma (OCH) is a benign vascular tumor that grows slowly but progressively, and which represents 9.5 to 15% of the primary expansive lesions of the orbit. It is a vascular malformation characterized by the presence of sinusoids with fine walls, which contain an accumulation of blood with no apparent arterial or venous inflow.¹

Its incidence peaks between the ages of 40-50 years and, according to various authors,^1-3 a greater percentage of women are affected. In relation to this data, hormones have been considered to influence the development of this entity, especially if we take into account the none too frequent tumor acceleration during pregnancy.⁴

Clinically, the most common finding is the presence of longterm proptosis. In this sense, the presence in our patient of exophthalmos that had been evolving for 5 years was clear. For along time there was no associated symptomatology in concordance with the observations of other authors.⁵

The appearance of other signs and associated symptoms, such as recent retroocular pain, visual acuity and diplopia on down-gaze, is in accordance with the literature. Likewise, the appearance of headaches, choroidal folds, retinal striae and edema of the optic disc have been described,^1,3,5 but which were not observed in our case. The appearance of hemorrhages is infrequent, and our patient did not refer any episode of bleeding during the time [the hemangioma] was evolving. The existence of abundant fibrous tissue in the structure of the OCH seems to explain this fact, unlike observations made of hemangioma in different locations.

The advent of computed tomography (CT) and of nuclear magnetic resonance (NMR) has enabled the diagnosis of orbital tumors to be established very reliably, together with the causes of proptosis. This permits careful surgical planning and, as a result, damage with regard to vision, ocular motility and vascular structures is avoided. In this case of ours, NMR was carried out and a well-defined image was obtained showing an intraconal retro-orbital localization in a lateroposterior position. On T1 weighted sequences an intermediate signal was obtained, unlike the low signal density that some authors have described.^6,7 The hyperintensity of the signal on the T2 weighted sequences did coincide, as well as the existence of enhancement on the administration of gadolinium. The CT image showed a dense lesion that became stronger with the administration of intravenous contrast [material].

Despite conservative treatment being described based on the periodic observation of asymptomatic OCH,⁸ we feel that the best therapeutic attitude is surgical resection, and that the surgical risks are minimized if an adequate approach is used and if the surgeon is trained in this field. In any event, conservative management is justified for very small orbital masses, providing they are always subject to thorough radiologic control, and that surgery is carried out in all cases if there is an increase in size or if the first signs and symptoms appear.

Various types of approaches have been described for removing OCH. With the superior approach a coronal

approach and a transorbital craniotomy are carried out. This is indicated in patients with OCH located in the upper region or in the orbital apex, and in OCH with intracranial extension or of the optic nerve channel. A variation of this technique has been described using a suprabrow⁹ cutaneous incision which, unlike the first approach, leads to a better aesthetic result and to better postsurgical recovery. The anterior approach through the superior or inferior orbital rims is indicated in intra or extraconal OCH located in the anterior two-thirds of the orbit. The transconjunctival approach,¹⁰ which is a variation of the anterior approach, can be used for the excision of OCH of the orbital base, inferomedial and lateral orbital face. Cutaneous incisions have been described that go directly into the caruncular area, with or without retracting the internal rectus muscle, for approaching lesions of the midface of the orbit. The Lefort 1 orbitotomy provides an optimum approach to the inferonasal apex,¹¹ with little morbidity if in the hands of experienced surgeons. It provides certain advantages such as the absence of visible scarring (intraoral approach), absence of a depression in the temporal region because of muscle atrophy following craniotomy, and lack of risk of damaging intraorbital structures. The posterior-inferior orbitotomy permits limited exposure through a Caldwell-Luc incision through the maxillary sinus to the orbital apex.¹² Lastly, the lateral approach, which is performed through a frontal zygomatic orbitotomy, raising the lateral wall of the orbit or of the greater wings of the sphenoid, ¹ allows optimal exposure for intraconal OCH not having a medial location. The lateral localization of the OCH in our case, meant that an approach using a lateral orbitotomy with a bicoronal incision was more advantageous, as it allowed an ample surgical field, with adequate control of the osteotomy of the malar [bone] and posterior repositioning with low profile miniplates.

Conclusions

The OCH is a common cause of primary expansive lesions of the orbit The presence of proptosis, diplopia, visual changes or retro-ocular pain, together with a NMR and/or CT image that is of a well-defined dense mass, should make us suspect the diagnosis, which will be confirmed by the anatomopathologic study of the resected specimen. We propose a bicoronal approach with a lateral orbitotomy for the resection of intraconal OCH in a non-medial localization, as a wider surgical field is gained and because it represents a reliable technique with predictable results. Other more complex approaches require a greater degree of training in surgery of the orbit.

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