Fungal Infections
John C Watkinson, Raymond W Clarke, Louise Jayne Clark, Adam J Donne, R James A England, Hisham M Mehanna, Gerald William McGarry, Sean Carrie in Basic Sciences Endocrine Surgery Rhinology, 2018
Surgical treatment (endoscopic or open) aims to confirm the diagnosis and remove non-viable tissue. Debridement is carried out until clear, bleeding margins are observed. There is little evidence that radical resection, including orbital exenteration and radical maxillectomy, improves survival. In fact, in the systematic review carried out by Turner et al.,20 patients who underwent endoscopic resection had significantly improved survival compared to those who underwent open surgery, although this may partly be due to the fact that patients undergoing open surgery had far more advanced disease. There is little consensus as to the role of orbital exenteration, although most agree that it should be reserved for those with a completely non-functioning eye.25 A multidisciplinary approach utilizing ophthalmology, maxillofacial and neurosurgical expertise is paramount.
Ocular and adnexal tumours
Pat Price, Karol Sikora in Treatment of Cancer, 2014
Rhabdomyosarcomas (RMSs) are the most common soft-tissue sarcomas of childhood and are of unknown cause. Over one-third of all new cases present with head and neck primaries. Orbital RMS accounts for one-quarter of all head and neck primary sites,42 and this particular primary RMS has long been recognized as having a later pattern of dissemination than tumours arising at other sites. Early surgical series demonstrated that, following orbital exenteration, up to 18% of patients could remain long-term survivors. Similarly, Sagerman et al.43 found an excellent local control rate of 91% following radical radiotherapy. However, in the late 1960s it was found that combination cytotoxic chemotherapy was extremely powerful against this condition. Following the success of adjunctive chemotherapy in Wilms’ tumour, cytotoxic drugs quickly achieved a place in the standard therapy of all patients with RMS. Furthermore, it became conventional practice to deliver one or two pulses of chemotherapy prior to radiotherapy to the orbit, to achieve cytoreduction and perhaps improve vascularity of the tumour at the time of irradiation. Together, chemotherapy and radiotherapy have improved the prognosis of RMS at all sites and, for patients with primary orbital RMS, orbital exenteration is now reserved as a salvage procedure.
Rhabdomyosarcoma
Mark Davenport, James D. Geiger, Nigel J. Hall, Steven S. Rothenberg in Operative Pediatric Surgery, 2020
Orbital tumors should be biopsied with the help of an ophthalmologist. Orbital exenteration is reserved for the few patients who have refractory or recurrent disease. In the interests of preserving vision, RT should be limited to the conjunctiva, cornea, and lens. Survival is 90–95%.
Locally advanced sinonasal adenoid cystic carcinomas: endoscopic endonasal surgery-centered comprehensive treatment provides benefits
Published in Acta Oto-Laryngologica, 2023
Jin Wang, Meng Zhang, Wenqi Yi, Liang Li, Liangyu Li, Chuan Pang, Lei Chen
Orbital invasion is a typical pattern of locally advanced SNACCs (Figure 3). In patients harboring sinus malignancies, orbital invasion via the periorbita is generally associated with poorer OS and DFS rates. Indeed, orbital invasion is associated with worse patients outcomes even following salvage surgery, with reported 5-year survival rate was no more than 40% in patients exhibiting orbital invasion [12]. Orbital exenteration is a procedure that is frequently performed for patients exhibiting tumor invasion of sinus structures, particularly in cases of the medial rectus muscle, optic nerve, or ocular bulb invasion. However, the benefits of this procedure to patients OS or DFS remain unclear, particularly in individuals with residual visual function. As orbital exenteration was not performed in this study, the survival benefits of our procedures were not assessed. However, we achieved orbital tumor recurrence rates of just 13% (3/23), and the total rate of local recurrence (7/44) was not significantly associated with the 5-year OS of patients, indicating that orbital resection may not be so radical during EES for locally advanced SNACCs.
Orbital exenteration: Institutional review of evolving trends in indications and rehabilitation techniques
Published in Orbit, 2018
Orbital exenteration traditionally denotes the surgical act of removing the whole orbital content with the periorbita including conjunctiva, globe, extraocular muscles, and the lacrimal apparatus.1 Depending on the underlying disease, the eyelids may or may not be included and resection of bony orbital walls and adjacent sinuses may become necessary.1 The foremost goal of exenteration must be to obtain tumor-free surgical margins before distant metastases or intracranial invasion develop, not only to justify this mutilating procedure but more importantly to provide a disease-free survival since the most common indications are malignant neoplasms arising from the periocular, orbital, and intraocular tissues.2 However, exenteration may not be curative in spite of clear surgical margins, as overall survival rates of 97% at 1 year, 55–65% at 5 years, and 37% at 10 years were reported.3,4
Transcutaneous retrobulbar injection of amphotericin B in rhino-orbital-cerebral mucormycosis: a review
Published in Orbit, 2022
Akshay Gopinathan Nair, Tarjani Vivek Dave
Controlling the underlying immunodeficiency condition is the foremost step in the management of ROCM. Simultaneously, intravenous antifungal medications and endoscopic sinus debridement with adjuvant local intraoperative amphotericin B (AMB) administration are considered the standard of care in ROCM.14 Orbital exenteration is typically recommended in patients who show progression of disease in spite of medical and surgical treatments.14,15 In the literature, indications for orbital exenteration have included ophthalmoplegia, proptosis, cranial involvement, and ocular involvement.16–21 Some have even reported that exenteration could increase patients’ survival in the presence of intracranial spread and rapid progression.18 At the same time, it has also been reported that orbital exenteration, by itself, does not affect the patients’ survival in ROCM.15 A possible explanation being that orbital exenteration is typically performed for patients in the end-stage disease.15,19 Hence, it was noted that no standard of care currently exists to guide physicians on when exenteration may benefit a mucormycosis patient.22 In summary, there are no clear guidelines for the effective management of the orbital component in ROCM.23
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