Orbital Apex Syndrome: A Case Series and Review of the Literature

Orbital apex syndrome (OAS) is an uncommon disorder characterized by ophthalmoplegia, proptosis, ptosis, anesthesia of the forehead, and vision loss. It may be classified as part of a group of orbital apex disorders that includes superior orbital fissure syndrome (SOFS) and cavernous sinus syndrome (CSS).  Complete SOFS presents similarly to OAS, without the accompanying optic nerve impairment.  CSS may include anesthesia of the cheek in addition to the signs seen in OAS.  CSS can occur bilaterally.  Each of these disorders are differentiated chiefly by the anatomical location of the causative process.

While historically described separately, these three disorders share similar causes, diagnostic evaluation, and management strategies¹. Each requires prompt recognition to ensure timely intervention and to maximize patient outcomes.

The purpose of this study is to report three cases of orbital apex disorders recently treated and to review the literature related to these conditions.  The authors describe a case of SOFS during a modified Le Fort III osteotomy, OAS secondary to a rapidly progressive maxillary sinus infection with orbital involvement, and CSS as a result of carotid-cavernous fistula formation after a motor vehicle collision. 

Inflammatory and vascular disorders, neoplasm, infection, and trauma are potential causes of orbital apex disorders². Management is directed at the causative process.

The cases described represent a rare but important group of disorders seen by the oral and maxillofacial surgeon.  A review of the clinical presentation, etiology, and management of these conditions may prompt timely recognition and treatment.

1. Lenzi GL, Fieschi C. Superior orbital fissure syndrome. Review of 130 cases. Eur Neurol. 1977;16(1-6):23-30.

2. Yeh S, Foroozan R. Orbital apex syndrome. Curr Opin Ophthalmol. 2004 Dec;15(6):490-8.