Facial intraosseous haemangioma

A 64-year-old female patient who reported discomfort in right orbit for about three months, with progressive evidence of exophthalmos.  She does not refer to double vision or loss of visual acuity.

CT (computed tomography) images show in the right orbit, a bone lesion affecting the greater and lesser right wing of the sphenoids, affecting pterygoid apophysis. The bone is insufflated and occupied by a tumour with small trabeculae. The lateral wall of the right orbit is also affected, the lesion produces a thinning of the cortex and grows inside the orbit, in an extraconal situation. All this conditions produce a medial displacement of the optic nerve, external rectus muscle and ocular proptosis. On MR (magnetic resonance) images, lesion has a heterogeneous appearance, very hyperintense on T2-weighted images and with hyperintense zones on T1-weighted images that translate into fat replacement. After gadolinium show strong homogenous enhancement on T1-weighted images, better seen on fat-saturated images. No extraosseous mass is evident.

Intraosseous haemangioma are very slow-growing tumours that account for only 1% of all bone neoplasms. Spine is the most frequent location of haemangiomas and skull is the second most frequent location [1]. Facial bone involvement is very rare. When haemangiomas appear in the facial bones, the most frequent location is in the maxilla, mandible, and nasal bones. There is a 3:1 female predominance [2] and their prevalence peak is during the fourth and fifth decades of life [3]. The pathogenesis is not clear, but a possible antecedent of trauma is postulated. Haemangiomas are benign vascular tumours  which can be histologically divided into 3 types: cavernous, capillary  and mixed.  Cavernous haemangioma are made up of clusters of dilated blood vessels, which are separated by fibrous septa, this type are usually presented in skull. Haemangiomas in  vertebras are usually of thecapillary type, which are rich in small vascular luminas without  much fibrous septa [4,5]. Haemangiomas are generally asymptomatic but can manifest as painful, palpable masses [2] and can cause facial deformation. When it affects the orbit, it can produce exophthalmos, diplopia, and loss of sight. CT is the best imaging technique to define trabecular and cortical features in bone lesions. Haemangioma on CT, usually appears as a sharply expansive lesion with thin margins and a sunburst pattern of radiating trabeculae. ‘‘Soap bubble’’ and ‘‘honeycomb’’ patterns may also occur [1]. Although the inner and outer tables are often intact, outer layer could appear eroded. After contrast administration they generally show marked and homogenous enhancement.  Haemangiomas on MR imaging usually appear as isointense lesions on T1-weighted images with scattered hyperintense foci due to fat components. Due to the presence of vascular lacunae, haemangioma on T2-weighted images show as hyperintense lesion with multiple lobules. As a vascular lesion, haemagiomas show strong homogenous enhancement on gadolinium T1-weighted images, better seen on fat-saturated images. Total surgical excision is the treatment of choice, although  haemangiomas are slow growing tumours, treatment is not always necessary. Indications for surgery include  correction  of  a  mass effect, control of haemorrhage and esthetical problem [2,3,6]. In this case the patient underwent a biopsy of the lesion. The anatomopathological diagnosis of the fragments of tissue obtained by curettage of bone lesion in the lateral orbital wall was a vascular tumour congruent with cavernous haemangioma. Prognosis after complete excision is excellent and recurrence is usually rare. Malignant transformation has only been reported  after radiation treatment, in recurrent cases [2].

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