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What is the diagnosis?
¿Cuál es su diagnóstico?

Thirty-two year old male with no history of interest to this case, was referred to our Oral and Maxillofacial department by the Ophthalmology department because of presentation of progressive ocular proptosis of the left [orbit] which had been evolving over the past three years. The patient had occasional retro-ocular pain and had recently started experiencing diplopia. On physical examination a tumor with a diameter of approximately 3 cm was felt at a superoexternal angle to the left orbit, together with ocular dystopia, unilateral exophthalmos of the left [orbit], with slight limitation of supraduction, and diplopia on upgaze (Fig. 1). The patient presented isochoric and normoreactive pupils.

Complementary explorations were performed with CT and NMR and a superolateral extraconal mass in the left superolateral [orbit] was observed above the lacrimal duct, measuring 3.1x2x2.5 cm. It had lobulated borders and internal signals that were mildly heterogeneous, hyperintense on T1 weighted sequences and intense areas mixed with others that were hypointense (Fig. 2 and 3) on T2 weighted sequences. The lesion was compressing and displacing the ocular globe downwards and inwards, but there were no signs of it extending into muscle structures nor into the ocular globe. Imaging tests also revealed bone remodelation and thinning of the frontal and left malar bones. Everything was compatible with a dermoid tumor.

Chronic hematic cyst of the orbit
Quiste hemático crónico orbitario

B. Peral Cagigal¹, J.I. Crespo Pinilla¹, R. Álvarez Otero¹, L.M. Redondo González²,
L.A. Sánchez Cuéllar², A. Verrier Hernández³

1 Médico Residente.
2 Médico Adjunto.
3 Jefe de Servicio.
Servicio de Cirugía Oral y Maxilofacial.
Hospital Universitario del "Río Hortega". Valladolid. España.

Correspondencia:
Dra. Peral Cagigal
Servicio Regional de Cirugía Oral y Maxilofacial,
Hospital Universitario del Río Hortega,
C/ Cardenal Torquemada s/n, 47010 Valladolid, España.
E-mail: beaperal77@yahoo.es

With this suspected diagnosis a decision was made to surgically remove the orbital mass for posterior anatomopathological analysis of the lesion. The patient underwent surgery, and a left hemicoronal incision was made in order to approach the orbital mass. The tumor was then resected as a block together with the supraorbital border due to the considerable thinning of the bone produced by the tumor mass. The tumor was dark in color with yellowish areas inside, and with a very friable consistency (Fig. 4). The supraorbital border was later reconstructed with an autograft of skullcap that was fixed with microplates and covered with a galea flap.

The anatomopathological study showed the existence of a cystic formation with a fibrous wall, abundant hematic content which was old and recent, and in which numerous cholesterol crystals could be identified. There was a focal accumulation of histiocytes with wide, frothy cytoplasm and associated inflammatory infiltrates, with mixed characteristics and a predominance of lymphocytes (Figs. 5 and 6). The lesion showed no evidence of proliferation using immunohistochemical techniques, and histiocytes stained [positive] with CD68 were reported, with no evidence of epithelium in any of the fragments studied. This all confirmed the diagnosis of chronic hematic cyst of the orbit.

Discussion

The hematic cyst of the orbit, which is also called cholesterol granuloma of the orbit, cholegranuloma or cholesteatoma of the orbit, is a rare condition (less than 1% of orbital tumors) and with an unclear pathogenesis.¹ Characterized by the presence of a progressive enlarging lesion due to the incomplete reabsorption of a hemorrhage through trauma, surgery, a foreign body or vascular lesion (lymphangioma o hemangioma) which sets off a granulomatous reaction against the metabolites in the blood, [leading to a] fibrous encapsulation and recurring hemorrhages.²

Middle aged males are particularly affected by the lesion, as in our case, which typically results in progressive proptosis (this being the most frequent symptom),³ periorbital edema, diplopia because of limitation of elevation and ocular discomfort that can last from weeks to years. In some cases pain can appear, especially supraorbital pain, as the most frequent location of the tumor is in the orbital roof,⁴ as in the case of our patient. An extraconal mass tends to appear on the CT scan which is homogenous, subperiostal, well-defined, smooth, extending over the bone of the orbital roof, which produces osteolysis in the frontal bone.⁵ The erosion of the orbital bone can sometimes lead to the suspicion that the lesion is malignant.

MR is the most specific test, showing blood residue products within the cystic lesion together with bone erosion. MR tends to show a welldefined mass, isointense on T1 weighted sequences and hypointense on T2 with regard to orbital fat6 although there is much variability in this respect. Diagnostic confirmation was reached by means of an anatomopathological study, which revealed a dense fibrous pseudo-capsule with no epithelial cells and which was enveloping old and recent blood, blood degradation products, inflammatory cells and cholesterol crystals.⁷

In the differential diagnosis entities such as the dermoid cyst, epidermoid cyst, mucocele, lipoma, lymphangioma, tumors of the lacrimal duct and any other entity that can produce a similar clinical and/or radiological expression have to be taken into account. Of all of these, the most important to keep in mind is the dermoid cyst.

The dermoid cyst is the most frequent congenital cyst of the orbit. It tends to be located in the supero-temporal and anterior part of the orbit, or in the tail end of the eyebrow, and it generally contains epidermal structures and skin appendages with variable sebaceous material; it is present from birth and it progresses very slowly during early life producing a painless, unilateral exophthalmos. Dermoid cysts are often accompanied by a considerable inflammatory reaction, which is sometimes similar to a cholesterolinic granuloma. It is therefore important not to break the capsule when it is extirpated, as this would increase the risk of recurrence and, in addition, the remains of the cyst wall would continue to secrete keratin, which would result in a serious granulomatous inflammatory response.⁸

The normal course of evolution of the hematic cyst of the orbit is of gradual growth. In this case there had been three years of progressive development that had produced exophthalmos and diplopia. When extirpation is carried out depends on the visual and/or aesthetic discomfort of the patient. Extirpation is generally carried out through an orbitotomy, together with aspiration of the cystic content and curettage of the bed.⁹ This treatment is generally definitive, as these types of lesions do not tend to recur,¹⁰ and the prognosis is therefore excellent. In the case we are reporting, the patient is asymptomatic, and his physical and radiological examinations have proved normal during the six months of postsurgical follow-up.

The hematic cyst of the orbit is therefore a rare entity, but with typical clinical and radiological characteristics; the histological study is decisive in determining the presence of a cystic accumulation of inflammatory cells, which are a product of blood degradation and cholesterol crystals in the absence of epithelial cells.

Acknowledgements

To Dr. Angeles Torres Nieto, anatomopathologist at the Rio Hortega Hospital in Valladolid, for her help in the analysis and in obtaining histologic images.

References

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