**Abstract**

Extended orbital exenteration is a highly disfiguring operation which entails complete removal of the orbital contents including periorbita, eyelids and involved surrounding bony walls with variations tailored to the specific clinical circumstances. The aim of such an extensive surgery is to achieve local control of the lifethreatening progressive neoplasms, when other treatment modalities fail to achieve disease control. Eyelids can be preserved in posterior orbital pathology, while it may not be possible in neoplasms arising from the anterior eye tissues. Depending on the clinical circumstances, if the neoplasm is invading the surrounding bony orbit, the involved bony and soft tissue structures are removed en bloc to achieve complete resection (R0 resection). Although the steps of the orbital exenteration are well defined, the same is not true for extended orbital exenteration. We demonstrate the details of extended orbital exenteration in different clinical scenarios for the malignancy of orbit and periorbital tissues invading surrounding orbital walls.

**Keywords:** extended orbital exenteration, orbital neoplasms, paranasal sinuses, skin malignant tumors, reconstruction, rehabilitation

### **1. Introduction**

Any ocular or periocular tumor, if neglected, can invade the orbit and raise the probability of various forms of orbital exenteration. Approximately 2–4% of the periocular malignancies invade the orbit and are candidates for orbital exenteration or extended procedures [1–6]. Frezzoti et al. classified orbital exenterations into subtotal, total and radical (**Table 1**) [7]. The radical resections or extended orbital exenteration have been classified as Type V (removal of bony walls) and VI (removal of bony walls and adjacent structures) (**Table 1**) [7]. Exenteration or its variations are psychologically and anatomically disfiguring, hence reserved to treat potentially life-threatening malignancies unresponsive to other treatments. Extended orbital exenteration has wide variations to the basic technique and is tailored to the clinical circumstances. These variations depend on saving or sacrificing different tissues within or around the orbit. Clinical and radiological findings guide the surgeon to tailor make the resection needed to completely remove the tumor with negative margins along with the selected sections of the orbital bone. Extended orbital exenteration, although highly disfiguring surgery, offers the best chance of cure as it aims to achieve local control of extensive disease when other treatment modalities fail to halt the progression of the disease [8]. The


### **Table 1.**

*Categorization of orbital exenteration based upon surgical technique.*

basic technique is somewhat similar to the orbital exenteration which removes all orbital contents including the periorbita along with part or complete eyelids. But variations are tailor made depending on the extent of the disease as assessed by the CT or MRI scan [9, 10]. Eyelids may be spared if the pathology present posteriorly not infiltrating them [11]. We describe different indications and step by step approach to different clinical presentations of orbital tumors.
