Orbital Vascular Anomalies and Vascular Tumours

Periorbital vascular malformations and tumours are a heterogeneous group of conditions with a complex nomenclature and classification. This chapter aims to clarify the current classification, as well as the clinical presentation, diagnosis, and management of the principal conditions within this group. Infantile haemangioma is the most common benign orbital vascular lesion in the paediatric population. It tends to regress in most cases. Oral propranolol is considered the treatment of choice in persisting cases. Solitary encapsulated venous-lymphatic malformation (SEVLM), traditionally known as cavernous haemangioma, is the most common orbital lesion in adult patients. It is usually diagnosed incidentally, and treatment can be postponed until significant proptosis or visual symptoms appear; in those cases, the surgical excision is the treatment of choice. The group of vascular malformations includes a broad spectrum of lesions that very in terms of histology, size and location, blood flow, and management. Included within this group are venous malformations, SEVLM, lymphangioma, orbital varices, arterio-venous malformations. Arterio-venous fistulae or shunts are characterised by an abnormal communication of the arterial into the venous systems without the normal capillary bed, which leads to an increase in the venous pressure and congestive signs and symptoms. Other rare conditions described in this chapter, are sinus pericranii, a vascular anomaly that results when there is communication between intracranial and extracranial venous systems, and orbital vascular occlusion, which may occur in the context of underlying vascular anomaly, injury, or infection.