Orbital Exenteration : a series of cases

Objective: To describe causes of orbital exenteration in a Brazilian tertiary hospital. Methods: A retrospective study was done, involving patients submitted to orbital exenteration at the Clinical Hospital of Botucatu Medical School, between the years of 1993 to 2016. The surgeries have been performed under general anesthesia, by a multidisciplinary team, composed by ophthalmologists, otolaryngologists and head and neck surgeons. Results: Fourteen cases of orbital exenteration occurred in the period of the study, with a mean age of 63.36 ± 13.18 years and nine were men (64.3%). All exenteration were due to malignant tumors, being more frequent the squamous cell carcinoma (7 cases 50.0%). The most common primary sites were the eyelids (50.0%) followed by the conjunctiva (28.6%). The majority of the surgeries was extended exenteration type (57.1%) and most of the reconstructions was made by spontaneous granulation (64.3%). The survivor rate in 1 year was 78,6% and in 5 years was 71.4%. Conclusion: The main cause of orbital exenteration was squamous cell carcinoma and the most frequent primary site was the eyelids. Extended exenteration was necessary for the majority of cases, most of them with free margins.


T
he orbital exenteration is a radical procedure that consists in removing the content of orbit including the eye bulb, orbital fat, periocular muscles, conjunctival fornix and all or part of the eyelid. (1)here are three types of exenteration: total, when the removal is of all content of orbit, with or without removal of the eyelids; subtotal, when the removal of the orbital content is partial, but with sacrifice ocular bulb; and extended exenteration, when adjacent bone walls or paranasal sinuses are also removed . (2)he classic indication for this procedure is malignant tumors, and it may be required due to orbital invasion secondary to malignant neoplasms of ocular annexes like the eyelids, 1- 3 conjunctival lesions, 4 primary orbital tumours, 5 and less commonly benign lesions which have intractable pain, inflammatory disease or pseudotumors. 1fter the exenteration it is necessary to reconstruct the orbital cavity, with the possibility of using the temporal muscle transposition or skin flaps in the region adjacent to coat the orbital cavity or wait for healing by spontaneous granulation. 1 Exenteration studies are scarce in the literature, especially in the Brazilian population, with the objective of this study being to describe the cases of exenteration of a Brazilian tertiary hospital.

METHODS
This is a retrospective study involving patients undergoing orbital exenteration at Hospital das Clínicas da Faculdade de Medicina de Botucatu (HC-FMB-UNESP), between the years of 1993 and 2015.The study was approved by the Ethics Committee of the institution.
The data was recovered from the electronic medical record of the patient, being important age, gender, diagnosis, previous treatments, date of surgery, surgical technique, method of reconstruction, result of pathology and evaluation of surgical margins.
The surgeries were performed under general anesthesia by a multidisciplinary team comprising ophthalmologists, otolaryngologists and head and neck surgeons.
The data was transferred to the Microsoft Excel 2010 spreadsheet, and analyzed by the program IBM Statistical Package for the Social Sciences (SPSS) version 20.Continuous variables are expressed by mean values and standard deviation, and the qualitative ones by frequency and absolute number.We considered p<0.05 as significant.

RESULTS
In the period of evaluation 14 exenterations were carried out in 14 patients, being the average age of 69.36 ± 13.18 years (ranging from 45 and 88 years).The study population comprised nine men (64.3%) and five women (table 1).
All exenterations carried out were for treatment of malignant tumors.The histopathological analysis showed five different types of tumors, and squamous cell carcinoma (SCC) was the most frequent with 7 cases (50.0%), followed by three cases of esclerodermiform basal cell carcinoma (BCC) and two melanomas (table 2).The melanomas were the only injuries that did not derive from the annexes, but from the eye itself.
Rev Bras Oftalmol.2016; 75 (6): 452-5 The average age of patients with SCC was 65.86 ± 14.40, and with BCC was 72.00 ± 4.58 years, that is, tumors that have resulted in orbital exenterations took place mainly in the elderly.
The primary sites of tumors are listed in table 3, with the most frequent being the eyelids (seven cases), followed by conjuctiva (four cases).In relation to the surgical margins after exenteration, 13 cases (92.9%) presented free margins in the pathological study (table 1).
Half of the patients (seven cases) had undergone previous treatment, with previous surgical excision being the most frequently modality, isolated, associated to radiation therapy or to the use of 5-Fluoracil, corresponding respectively to four, one and one case (table 4).
The most accomplished type of surgery was the extended exenteration (seven procedures), followed by total exenteration (five procedures), and one case of subtotal exenteration.Regarding laterality, there was no difference between the sides, with seven cases on the left (50.0%) (Table 1).The most widely used method of reconstruction was granulation by secondary intention (nine cases), with the remaining five cases being performed by flap rotation (Table 1).
Of the patients studied, four missed follow-up during treatment, so there is no information regarding the time of followup and outcome.
In the remaining patients, the time of follow-up after surgery was 35.36 ± 35.10 months (ranging from 0.00 to 90.63 months).There was a total of five deaths during the follow-up period, with a survival rate in 1 year of 78.6%, and of 71.4% in 5 years.The deaths were secondary to a case of adenocystic carcinoma, two of melanoma, one of BCC and one of SCC, however unrelated to the type of tumor (p>0.005).In addition, these patients registered a case of recurrence and one of metastasis, being a case of adenocystic carcinoma and one of melanoma, respectively.

DISCUSSION
The purpose of this study was to show the profile of individuals who suffer orbital exenteration, since there are few data in the literature on the subject, especially in the Brazilian population.
As the orbital exenteration is a mutilating procedure carried out in cases of advanced disease, their prescriptions are limited.In our study, all prescriptions were due to malignant tumors.The prescription can also be for benign tumors or pseudotumors, but always with a greater frequency for malignant neoplasms, 6 as in Manchester, where among 69 exenterations, 92.7% were due to malignant neoplasms. 1In another study involving 16 exenterations, all were due to malignant tumors. 7Currently, more conservative treatments for SCC and BCC, as local excisions, indicate similar tumor control. 3The search for alternative treatments is probably due to the mutilation induced by exenteration, reflecting on the quality of life compared to the general population. 8onsidering the subtypes of malignant tumors that most require this type of surgical approach, literature studies differ between SCC and BCC, 1,7,9 with the majority pointing to the BCC as the most common subtype [10][11][12] However, squamous cell tumor corresponded to 50.0% of our cases, finding similar to the Australian survey showing the SCC as responsible for 48.0% of exenterations carried out 9 , and another Brazilian study also showing SCC in 54.2% of cases. 13BCC is approximately 87% of palpebral tumors 14 , and although the SCC is less common than the BCC, it is a more aggressive subtype that may be present wih early perineural invasion, with a greater chance of orbital invasion and faster than the BCC.In our study there was a trend of SCC

Table 4 Frequency of previous treatments performed
in patients with tumors that required orbital exenteration cases being operated in younger patients, but without statistical significance, perhaps due to the reduced sample.However, another study showed this finding. 3sually this type of procedure is performed by ophthalmologists, head and neck surgeons, otolaryngologists, plastic surgeons, among others.The exenterations performed by ophthalmologists tend to be of the total or subtotal type, with greater tissue preservation. 9In our study, most exenterations performed were of the extended type (57.1%), which can be explained by the fact that we work in a multidisciplinary team.
In addition, another difference is that ophthalmologists opt for healing by granulation, but non-ophthalmologists use other techniques such as temporal muscle flap, skin graft of total or partial thickness, derma-fat graft, among others. 10he need for adjuvant treatment after surgery like radiotherapy influences the type of reconstruction because, if required, it is necessary to reconstruct it with flaps. 12Healing by second intention (granulation) is more time consuming and does not allow the completion of radiation therapy.However, it allows early identification of recurrence and a more uniform color of the cavity.The use of flaps and grafts, besides allowing early radiotherapy, leads to a faster healing, with the disadvantage of complicating the diagnosis of recurrences and sometimes complicating the adaptation of prostheses.In our study, 64.3% of cases were reconstructed by second intention healing.
All of our patients are referred for facial prosthesis adaptation after surgery, which can give a better appearance and improve the quality of life.
During the period of study, among patients without tracking loss, 5 deaths were evidenced, with a 1 year survival of 78.6%, and of 71.4% in 5 years.There was no statistical difference between the types of tumors, probably because of the small sample.However, the two cases of melanoma have evolved to death, one of the seven cases of SCC (14.3%), and one of the three cases (33.3%) of BCC.These findings are similar to a study of Massachusetts who found a survival in 1 year of 72%, and a higher mortality in melanomas (85.7%). 15mong the limitations to our study are having a retrospective design and presenting a small number of patients, possibly justified by the low performance of this type of procedure, making it difficult to have a prospective study with the largest number of patients.Another limitation was the loss to follow-up of four patients, which could add information on survival and time of follow-up.

CONCLUSION
The exenteration indication of a Brazilian tertiary hospital took place predominantly with SCC, and the main primary site was located on the eyelids.The most accomplished procedure was the extended exenteration, with the great majority reaching free margins.