Total orbital exenteration - Experience from a tertiary cancer care center in Northern India

Yogendra Singh Bhakuni; Kailash Chand Sharma; Suhas Kodasoge Rajappa; Dharma Ram; Ajay Kumar Dewan; Rashika Chand; Udip Maheshwari; Ankush Jajodia; Venkata Pradeep Babu Koyyala

doi:10.4103/oji.oji_11_21

ORIGINAL ARTICLE

Year : 2021 | Volume : 5 | Issue : 2 | Page : 67-70

Total orbital exenteration - Experience from a tertiary cancer care center in Northern India

Yogendra Singh Bhakuni¹, Kailash Chand Sharma¹, Suhas Kodasoge Rajappa¹, Dharma Ram¹, Ajay Kumar Dewan¹, Rashika Chand², Udip Maheshwari³, Ankush Jajodia⁴, Venkata Pradeep Babu Koyyala³
¹ Department of Surgical Oncology, Rajiv Gandhi Cancer Institute and Research Centre, New Delhi, India
² Department of Pathology, Rajiv Gandhi Cancer Institute and Research Centre, New Delhi, India
³ Department of Medical Oncology, Rajiv Gandhi Cancer Institute and Research Centre, New Delhi, India
⁴ Department of Radiology, Rajiv Gandhi Cancer Institute and Research Centre, New Delhi, India

Date of Submission	14-Feb-2021
Date of Decision	17-May-2021
Date of Acceptance	26-Jun-2021
Date of Web Publication	21-Aug-2021

Correspondence Address:
Venkata Pradeep Babu Koyyala
Department of Medical Oncology, Rajiv Gandhi Cancer Institute and Research Centre, New Delhi - 110 085
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/oji.oji_11_21

Abstract

Introduction: Orbital exenteration (OE) is a defacing procedure reserved for advanced head and neck malignancies involving the orbit. Even though it is cosmetically unappealing and associated with complete loss of vision in one eye, the procedure has low surgical morbidity and recurrence rates and should be considered in appropriate patients after adequate patient counseling. Due to relative rarity of the procedure, there is a paucity of literature, particularly in India. Aim: To analyze the role and indications of OE, operative details, morbidity, and postoperative complications in patients who had undergone total OE in a tertiary care cancer hospital. Materials and Methods: We retrospectively reviewed 5-year (2011–2016) data of those who had OE from our computerized database. The retrieved data were analyzed for demographic profile, operative details, morbidity, and survival rates. Results: Out of 20 patients analyzed, 13 were male and 7 were female. Majority of the patients have secondary eyeball extension (15/20). Most frequent indication for exenteration was tumor of eyelid origin. Reconstruction after surgery was done by temporalis flap (n = 10), anterolateral thigh-free flap (n = 6), and split skin graft (n = 4). None of the patients had any intraoperative complications. One patient developed flap necrosis and managed by flap revision. One patient had wound dehiscence and managed conservatively. At a mean follow-up of 20 ± 9.6 months, two patients had recurrence of primary disease. Mortality occurred in two patients; 1 case for disease related and another one for other medical condition. Conclusion: The OE has still a role and is indicated for a variety of conditions. The surgical procedure remains safe, and major morbidity is dictated by the type of reconstruction. Although skin graft is the simplest reconstructive option, many a times, myocutaneous/free flap is required in advanced cases.

Keywords: Eye lid tumors, flap reconstruction, paranasal sinus

How to cite this article:
Bhakuni YS, Sharma KC, Rajappa SK, Ram D, Dewan AK, Chand R, Maheshwari U, Jajodia A, Babu Koyyala VP. Total orbital exenteration - Experience from a tertiary cancer care center in Northern India. Oncol J India 2021;5:67-70

How to cite this URL:
Bhakuni YS, Sharma KC, Rajappa SK, Ram D, Dewan AK, Chand R, Maheshwari U, Jajodia A, Babu Koyyala VP. Total orbital exenteration - Experience from a tertiary cancer care center in Northern India. Oncol J India [serial online] 2021 [cited 2023 Mar 29];5:67-70. Available from: https://www.ojionline.org/text.asp?2021/5/2/67/324229

Introduction

Bartisch described orbital exenteration (OE) as early as 1583.^[1] OE involves removal of the contents of the eye socket including the eye ball, orbital nerves, extraocular muscles, lacrimal glands, as well as the orbital bones in advanced disease. Although the surgical procedure itself is straightforward, it is a disfiguring, morbid procedure and the psychological impact on the patient is considerable. The challenging aspect is how to counsel the patient to accept the procedure and reconstruction of the orbit and eyelids. The most common indications for exenteration are malignant conditions such as advanced ocular melanoma and metastases to orbit. Most of the patients undergoing OE have secondary rather than primary malignancies of the orbit.^[1],[2] In Indian setup, there is a paucity of data on this surgical entity, and owing to the varied nature of the indications, comprehensive analysis is difficult.

With this background, the present retrospective study conducted to evaluate indications, clinical, pathological characteristics, and types of reconstructive surgeries in patients who underwent total OE.

Materials and Methods

This is a descriptive, retrospective study. The primary objective of this study was to assess demographics, clinical features, and outcomes of OE surgeries done in a tertiary care cancer center at New Delhi. A retrospective chart review was conducted for the patients who had undergone total OE for various indications from August 2011 to July 2016. The study was conducted according to the ethical principles stated in the latest version of Declaration of Helsinki and the applicable guidelines for good clinical practice. The patients with age below 13 years, medical comorbidities, or medically unfit patients were excluded.

The following baseline characteristics were assessed: age at diagnosis, sex, surgery details, indications for surgery, and follow-up. Steps in OE are as follows:

Skin is incised encircling the eyelids and all visible tumors after applying the tarsorrhaphy sutures
The incision is extended through periosteum around the orbital rim
Periosteal elevator is used to separate periorbita from the bony socket
The eye and all orbital contents are cut at the orbital apex and removed
The orbital socket following removal of all soft tissues
A split skin graft (SSG) lines the exenterated socket
The socket and skin graft are splinted with Telfa and cotton balls soaked in antibiotic solution
Aseptic dressing done.

Data collected were used to determine outcome and postexenteration survival details. The details were recorded in MS Excel sheet and analyzed using SAS 8.02 (SAS Institute Inc., Cary, NC, USA) were used to analyze the data.

Results

A total of 20 patients met the inclusion criteria and were eligible for analysis who underwent total OE during the 5-year study period. Among these 20 patients, 13 were male and 7 were female [Table 1]. The mean age of the patients undergoing exenteration was 55.3 years within the age range of 13–80 years. Majority of them had secondary involvement of the orbit (15/20), while the remaining five patients had primary orbital tumors which were the two cases of orbital rhabdomyosarcoma, two patients had choroidal melanoma, and one patient had recurrence of retinoblastoma previously treated with enucleation and chemoradiation. The eyelid was the most common site of tumor origin in secondary cases, accounting for 12 patients (60%); remaining three patients were carcinoma of ethmoid sinus (2 cases) and adenoid cystic carcinoma of the lacrimal gland (1 case).

Table 1: Demographic and clinical features of patients who underwent orbital exenteration

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Out of the 20 patients, 11 had past treatment history and presented with recurrent disease, out of which seven had undergone wide local tumor excision for basal cell carcinoma (BCC), one patient underwent enucleation, and one underwent functional endoscopic sinus surgery. Rest two patients of squamous cell carcinoma (SCC) of the eye lid had received concurrent chemoradiation. All the remaining patients underwent upfront surgery.

Temporalis flap was used for reconstruction in 10 patients, anterolateral thigh (ALT)-free flap was used in six patients, and remaining four patients underwent SSG [Table 2]. None of the patients had any intraoperative complications. Margins of the resections in 18 patients were negative for tumor on histopathology. Rest two patients were found close margin on histology, for which adjuvant radiation therapy was given. In overall, three out of 20 patients received adjuvant radiation and one patient received adjuvant chemotherapy as per the histopathology report. One patient who had undergone ALT flap had flap necrosis after 45 days which was managed by flap revision using temporalis flap. Wound dehiscence was seen in one patient and was managed conservatively.

Table 2: Types of reconstruction done after exenteration surgery and complications

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Mean duration of follow-up of the patients was 20 ± 9.6 months. Two patients had recurrence of primary disease during follow-up [Table 3], one of which was a case of orbital rhabdomyosarcoma that recurred after 12 months after surgery and was addressed with wide local excision followed by adjuvant chemotherapy. Another patient was a case of adenoid cystic carcinoma who developed widespread metastases at a follow-up of 4 months and eventually succumbed to it. One other patient analyzed died due to an unrelated medical condition.

Table 3: Clinicopathologic, follow-up, and outcomes of patients who underwent orbital exenteration

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Discussion

OE is a defacing surgery reserved for locally advanced head and neck cancers involving orbit. It is generally psychologically and cosmetically challenging for the patients to accept and equally challenging for the surgeons to counsel the patient and family for surgery. However, in certain clinical indications, this surgery is associated with good outcomes, particularly in head and neck malignancies. The contraindications for OE are invasion of cavernous sinus and/or internal carotid artery by high-grade malignancy, bilateral orbital invasion (especially when patient is not blind), patients with widespread metastatic disease, patients with tumors expected to have a high likelihood of local-regional recurrence despite OE, elderly patients with multiple medical comorbidities, or patients with a short life expectancy.

OE involves removal of the orbital contents with periorbital appendages, eyelids, and adjoining skin if involved. This is divided into two types in the literature according to the extent of surgery as total and subtotal types. Yeatts^[3] divided OE into two categories: total exenteration as removal of the entire orbital contents with or without sacrifice of eyelid skin, and subtotal exenteration as partial removal of orbital tissues with sacrifice of the eye, which can be considered as an extended enucleation. All the patients in this study underwent total OE.

Most of the patients in our study had secondary involvement of orbit, out of which malignancies of eyelid accounted for 60% (12/20). Seven patients had BCC (58.3%), three had sebaceous carcinoma, and two had SCC in final histopathology. In a study of 429 cases undergoing OE by Günalp et al.,^[4] it was seen that BCC was the most common histology followed by SCC. In another series, Rahman et al.^[5] reported BCC in 40% cases. SCC of eyelid is less frequent as compared to BCC.^[6] Both histologies have aggressive course with tendency to invade orbit locally. A study by Howard et al.^[7] has reported the orbital invasion rate from cutaneous BCC and SCC ranging from 0.8% to 8.2%. Out of 12 patients in this study, seven patients presented with recurrent disease after inadequate previous resection and five patients had neglected disease.

Retinoblastoma is a rare indication for OE. In our study, one patient underwent exenteration for recurrent orbital invasion from retinoblastoma previously treated with enucleation and chemoradiation. Günalp et al.^[4] reported retinoblastoma as an indication for exenteration in 18.6% of cases. Exenteration is often reserved for the advanced retinoblastoma who had undergone previous enucleation or chemoradiation.

OE for orbital rhabdomyosarcoma is indicated in patients with massive tumor infiltration and periorbital spread with accompanying proptosis.^[8] In our study, two such patients underwent OE for rhabdomyosarcoma who presented with advanced disease.

Malignant melanoma is another indication for OE and melanoma of the choroid and conjunctiva are the commonest indications for OE.^{[9],[10],[11]} In our study, two patients underwent exenteration for choroidal melanoma.

In our study, OE was performed for two patients with advanced carcinoma of the ethmoid sinus with orbital involvement. Malignant tumors of the lacrimal gland usually have grave prognosis. Exenteration is not a curative procedure in these conditions as most of them have perineural or bone invasion at presentation.^[12]

Reconstruction is an important part of this surgery which itself is a disfiguring procedure. Reconstruction with different types of flaps has improved the cosmetic outcomes of surgery. Reconstruction surgery involves various techniques ranging from skin graft, local flaps to microvascular-free flaps. Local reconstructive techniques are simpler to perform, and recurrences are more easily detected compared to other reconstructive techniques.^[13] Regional reconstruction procedure including forehead flap,^[14] temporalis muscle flap,^[15] latissimus dorsi, and pectorals flap has shown improved results.^[16],[17] In our study, 10 patients had undergone reconstruction with temporalis flap.

With advent of microvascular anastomosis, free tissue transfer is being frequently used for reconstruction. Free flaps are a safe and reliable method of repairing the large defect created by OE. Complete flap failure rates are low, and complication rates are within an acceptable range and have little to no impact on the convalescence time.^[18] In our study, six patients underwent ALT-free flap and four were reconstructed with SSG. One patient of ALT flap had flap necrosis, for which flap revision was done using temporalis muscle flap.

OE is a disfiguring procedure with mortality and morbidity reported in some series. Rahman et al.^[19] showed overall 1-year survival of 93% and a 10-year survival of 37% assuring in that a proportion of individuals achieve surgical cure following exenteration. In our study, we had mortality of two patients at a mean follow-up of 20 ± 9.6 months. One patient had distant recurrence in liver after 1-year follow-up eventually died and another patient died due to ischemic heart disease. The remaining patients are doing well with no recurrence till the last follow-up.

Conclusion

In this study, we have reviewed the patients undergoing OE for different malignancies with the local/free flap reconstruction options and complications associated with it. Although OE is a disfiguring surgery and difficult for a patient to accept psychologically and cosmetically, it is associated with good outcome and survival rates in head and neck malignancies. It should be considered in appropriate conditions after multidisciplinary evaluation in psychologically fit patients. Awareness among the patients, early diagnosis and referral to appropriate tertiary cancer care centers in head and neck malignancies prevents the need of exenteration surgeries in majority of cases.

Limitations of the study

The main limitation of this study was this being retrospective analysis and shorter follow-up without the overall survival estimation.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1.	Bartisch G. Ophthalmodoulcia. Dresden 1583;3:208.
2.	Bartley GB, Garrity JA, Waller RR, Henderson JW, Ilstrup DM. Orbital exenteration at the Mayo Clinic. 1967-1986. Ophthalmology 1989;96:468-73.
3.	Yeatts RP. The esthetics of orbital exenteration. Am J Ophthalmol 2005;139:152-3.
4.	Günalp I, Gündüz K, Dürük K. Orbital exenteration: A review of 429 cases. Int Ophthalmol 1995;19:177-84.
5.	Rahman I, Cook AE, Leatherbarrow B. Orbital exenteration: A 13 year Manchester experience. Br J Ophthalmol 2005;89:1335-40.
6.	Aurora AL, Blodi FC. Lesions of eyelid. A clinicopathological study. Surv Ophthalmol 1970;15:94-104.
7.	Howard GR, Nerad JA, Carter KD, Whitaker DC. Clinical characteristics associated with orbital invasion of cutaneous basal cell and squamous cell tumors of the eyelid. Am J Ophthalmol 1992;113:123-33.
8.	Günalp I, Dürük K, Gündüz K. Orbital rhabdomyosarcoma: A twenty-year survey in Turkey. Jpn J Ophthalmol 1993;37:499-504.
9.	Haskins CP, Nurkic S, Fredenburg KM, Dziegielewski PT, Mendenhall WM. Primary orbital melanoma treated with orbital exenteration and postoperative radiotherapy: A case report and review of the literature. Head Neck 2018;40:E17-20.
10.	Levin PS, Dutton JJ. A 20-year series of orbital exenteration. Am J Ophthalmol 1991;112:496-501.
11.	Jovanovic P, Mihajlovic M, Djordjevic-Jocic J, Vlajkovic S, Cekic S, Stefanovic V. Ocular melanoma: An overview of the current status. Int J Clin Exp Pathol 2013;6:1230-44.
12.	Wright JE. Factors affecting the survival of patients with lacrimal gland tumours. Can J Ophthalmol 1982;17:3-9.
13.	Levin PS, Ellis DS, Stewart WB, Toth BA. Orbital exenteration. The reconstructive ladder. Ophthalmic Plast Reconstr Surg 1991;7:84-92.
14.	Dortzbach RK, Hawes MJ. Midline forehead flap in reconstructive procedures of the eyelids and exenterated socket. Ophthalmic Surg 1981;12:257-68.
15.	Holmes AD, Marshall KA. Use of the temporalis muscule flap in blanking out orbits. Plast Reconstr Surg 1979;63:336-43.
16.	Donahue PJ, Liston SL, Falconer DP, Manlove JC. Reconstruction of orbital exenteration cavities. The use of the latissimus dorsi myocutaneous free flap. Arch Ophthalmol 1989;107:1681-3.
17.	Ariyan S. Further experiences with the pectoralis major myocutaneous flap for the immediate repair of defects from excisions of head and neck cancers. Plast Reconstr Surg 1979;64:605-12.
18.	Rajak S, Figueira E, Forster N, Greenwell T, Rees G, Selva D, et al. Free flaps reconstruction after orbital exenteration: A single centre case series. Int J Ophthalmol Clin Res 2015;2:3.
19.	Rahman I, Maino A, Cook AE, Leatherbarrow B. Mortality following exenteration for malignant tumours of the orbit. Br J Ophthalmol 2005;89:1445-8.