A unique case of a double intra-conal cavernous hemangioma

Abstract

Background

Cavernous hemangiomas are benign vascular malformations, probably representing the most common intraorbital and intraconal tumors in the adult population.

Case presentation

We report the case of a 49-year-old female with two intra-conal lesions. We performed a total resection using Ulivieri's extended lateral approach. The postoperative course was uneventful and the patient was discharged three days after surgery.

Conclusions

To the best of our knowledge, we report here the first case in the literature of a double intra-conal lesion.

Introduction

Cavernous hemangioma is a benign vascular malformation, which probably represents the most common intraorbital and intraconal tumor in the adult population [1,2,3]. When the lesion is small, it can be followed up with clinical examination and serial neuroimaging. If the lesion reaches a considerable size with the onset of neurological deficits, surgical removal becomes mandatory. In our Department of Neurosurgery we usually perform, as in this case, an extended lateral approach to the orbit [4, 5].

Case presentation

We present the case of a 49-year-old woman who presented with a history of severe orbital pain of a few months duration and the presence of a progressive exophthalmos on the left eye. Neurological examination revealed no neurological deficits or impairment in visual acuity except for an upward and outward 22-mm proptosis of the left eyeball. A computed tomography (CT) scan of the brain showed the concurrent presence of two well-defined and circumscribed intra-conal lesions on the left side of the orbit (Fig. 1). The tumor was surgically removed by Ulivieri’s extended lateral approach with cryoprobe technique (Fig. 2). Histopathological examination showed a benign vascular lesion consisting of a cavernous hemangioma.

Fig. 1

A computed tomography (CT) scan of the brain showing the concurrent presence of two well-defined and circumscribed intra-conal lesions on the left side of the orbit

Fig. 2

Surgical removal of the tumor by Ulivieri’s extended lateral approach with cryoprobe technique

The postoperative course was uneventful, without any complication and the patient was discharged three days after the operation.

Conclusions

Hemangiomas are the most common intraorbital tumors in the adults and the second most common cause of proptosis after thyroid disease [6]. Patients typically present in their 3rd to 5th decade with unilateral, painless proptosis. Decrease in visual acuity from mild compression of optic nerve is often subtle. These lesions more commonly occur in women who may experience clinical worsening during pregnancy [7]. Pre-operative diagnostic work-up includes complete ophthalmologic examination, and neuroimaging examinations such as CT or magnetic resonance imaging (MRI).

Hemangiomas are defined as intraconal tumors as they are most commonly located between the optic nerve and the extraocular muscles.

Histopathology shows a benign vascular malformation characterized by multiple large vascular channels covered by endothelial cells and abundant stroma. The vascular lumen is filled with blood and variable regions of intralesional thrombosis, reflecting vascular stasis and/or very slow blood flow. Endothelial cells appear as mature vascular elements. The stromal structure shows perivascular hypercellularity or hyperplastic elements related to neovascular activity [8].

To the best of our knowledge, we report here the first case in the literature of a double intra-conal lesion.