Orbital Exenteration

L’exentération orbitaire a-t-elle encore sa place en 2019 ?

Orbital Exenteration is a major surgical procedure that consists of the removal of the orbital bone, orbital fat, eyeball, and its contents including extraocular muscles. It is an extensive and morbid surgical procedure. Our aim is to systematically review the indications, complications and reconstruction methods utilised for orbital exenteration. An objective electronic database search was conducted in PUBMED Central, MeSH, NLM Catalog, Bookshelf, and PUBMED published in 20years period from 1999 till 2019. A total of 29 articles were shortlisted for the present review. Most of the studies have eyelid and canthus as most common primary site of malignancy leading to orbital exenteration. Basal cell carcinoma and squamous cell carcinoma being most common pathology. Other intraocular pathology was Retinoblastoma and melanoma. There were various reconstruction methods used by different authors and Sino-orbital fistula was most commonly occurring in majority of studies. Inspite of being a morbid surgery, Orbital Exenteration had acceptable survival and good quality of life. The aggressive pathology that requires orbital exenteration worldwide is mostly periorbital skin, sinus, and intraocular malignancies. The morbidity of the procedure is high with many surgical complications. However, in properly selected patients it can give better outcomes and survival.

The aim of this study was to evaluate the indications for surgery, patient and healthcare delays, ophthalmic status, challenges of management, complications, disease-free survival (DFS), and overall survival (OS) of patients treated by orbital exenteration at a tertiary care centre. Patients who underwent orbital exenteration during an 18-year period (2005-2022) were identified from the Helsinki University Hospital (Helsinki, Finland). Demographic and medical data were retrieved. Sixty-nine patients were included. Median age was 74 years (range, 7-93 years). The most common operative indication for exenteration was a malignant tumour (99%). Histopathological types that were most frequently encountered were uveal melanoma in 10 (14%), basal cell carcinoma in 9, (13%) and cutaneous squamous cell carcinoma in 8 (12%) patients. Orbital exenteration could have been avoided in 32 (46%) patients if the primary surgery had been performed with sufficient margins and without delay. The median follow-up time was 16 months (range, 0-169 months). At the end of follow-up, 34 (49%) patients had no evidence of disease, 12 (17%) were alive with disease and 23 (33%) had died of the disease. Kaplan-Meier analysis revealed 1- and 2-year DFS of 74% and 67%, respectively. One- and 2-year OS were 77% and 69%, respectively. Selected periorbital malignancies, especially those with insufficient primary treatment or with treatment delay, may warrant exenteration. Given the diverse origins of malignancies, a multidisciplinary approach, including ophthalmological consultation, appears to be the optimal strategy for these patients.

Sinonasal malignancies account for <1% of all cancers, typically presenting at an advanced stage and affecting the paranasal sinuses and surrounding structures. The association between head-and-neck cancers and pregnancy is uncommon. Anaesthetic management of pregnant patients undergoing non-obstetric surgery is complex and demanding, requiring meticulous planning to safeguard both mother and foetus. Here, we describe the anaesthetic management of a 35-year-old pregnant woman (G4P3L2A1) at 32 weeks of gestation, admitted with left-sided sinonasal adenocarcinoma with intracranial and orbital extension, scheduled for craniofacial resection with orbital exenteration. Managing such cases demands a multidisciplinary approach and poses challenges for anaesthesiologists, as all management must prioritise the safety of both mother and baby.

Cavernous sinus meningiomas are rare, and radiotherapy is considered because the risk of postoperative complications is high. Radiotherapy is useful for short-term control of meningiomas, but hydrocephalus may appear as a long-term complication. A 71-year-old male patient suffered from a cavernous sinus meningioma with orbital involvement and communicating hydrocephalus. Radiotherapy was administered thrice due to tumor progression. Right intraorbital meningioma increased markedly, and right eye bulging and visual deterioration were aggravating. Tumor removal with orbital exenteration was performed to prevent left visual impairment due to tumor progression. The pathology was atypical meningioma (WHO grade 2). Postoperative cerebrospinal fluid (CSF) leakage from the right orbit, so-called oculorrhea, was difficult to repair due to hydrocephalus, requiring eyelid sutures and a lumboperitoneal shunt. The tumor never developed into the opposite cavernous sinus. Radiation-induced hydrocephalus can lead to intractable postoperative CSF leakage from orbit after tumor removal with orbital exenteration and require surgery. In these cases, hydrocephalus treatment may lead to a cure for intractable CSF oculorrhea.

To evaluate the survival benefit of orbital exenteration in periocular malignancy, taking account of preoperative intent. Patients undergoing exenteration had retrospective chart review for demographics, clinical features, radiology, histology, and outcome. Based on systemic tumor status, the patient was either "Class I" (with absent or well-controlled systemic disease) or "Class II" (incurable active metastatic disease), and based on the extent of orbital disease and exenteration intent , was classed as either "Group A" (locally curative) or "Group B" (locally palliative). One hundred thirty-three patients (78 females; 59%) underwent exenteration at an average age of 61 years (median 64; range 5-91) for sebaceous, squamous and basal cell carcinomas, or for melanoma (22%, 19%, 11%, and 28%, respectively). There were 20% systemically incurable patients (26/133; Class II), and incurable local disease ("Group B") in 5% (5/107) of Class I and 15% (4/26) Class II patients. The overall survival (OS) was 88% at 12 months, 57% at 5 years, and 41% at 10 years, prognosis being worse with age more than 70 years ( p = 0.005), prior local radiotherapy ( p = 0.005) or positive resection margins ( p = 0.002). The mean OS for Type IA exenteration (145 months; 95% CI 122-168) was significantly different to 50 months for Type IB (95% CI 22-79; p = 0.02); likewise, OS for Type IIA procedures (31 months; 95% CI 11-51) was different to Type IIB (19 months; 95% CI 2-36) ( p = 0.001). Exenteration confers a significant survival with advanced periocular malignancies, even in patients with uncontrollable systemic disease, or where the local disease is deemed incurable.

Sebaceous carcinoma (SC) is an aggressive, malignant tumor derived from the adnexal epithelium of sebaceous glands that can occur in ocular/periocular (75 % of all SC) and extraocular variants. Its incidence varies according to the ethnicity. In Caucasians, SC accounts for 1.5–5 % of eyelid malignancies, while in Asiatic people, it is the second most common eyelid malignancy after basal cell carcinoma with an incidence of 38 %. Ocular SC occurs more frequently in women (3:1), while the extraocular form has a predilection for men (2:1). The age of presentation varies from 60 to 80 years. SC may occur in the setting of Muir–Torre syndrome, after radiotherapy or in immunocompromised patients such as HIV-infected patients.

The primary objective was to investigate the trends in orbital exenteration rates at a large tertiary care center, particularly in the context of recent advancements in immunotherapy, targeted agents, and globe-sparing surgical techniques, which have significantly impacted patient management. The authors conducted a retrospective cohort study at the University of Miami. Patients who underwent orbital exenterations from 2011 to 2024 were identified by obtaining surgical coding data via institutional data brokers and validated through a rigorous surgical chart review. The authors used Poisson regression with robust standard errors to compare incidence trends, using the Florida state population as an offset. The analyses compared 2011 to 2017 with 2018 to 2024 and examined year-by-year variation, ensuring a robust and reliable study. A total of 179 exenteration cases were identified. Compared with 2011 to 2017, total exenterations decreased by 39% in 2018 to 2024 (incidence rate ratio [IRR] = 0.61; p = 0.004). Significant declines were also observed from 2018 to 2024 for cutaneous squamous cell carcinoma (IRR = 0.49; p = 0.023) and ocular surface squamous neoplasia (IRR = 0.17; p = 0.002), while sebaceous gland carcinoma increased (IRR = 2.94; p = 0.015). Year-over-year analysis estimated a 3.9% annual decrease in total exenterations. This study revealed a significant decline in total orbital exenterations from 2018 to 2024 and diagnosis-specific declines in exenterations for cutaneous squamous cell carcinoma and ocular surface squamous neoplasia compared with 2011 to 2017. This trend coincides with the introduction of Programmed Cell Death Protein 1 (PD-1) inhibitors and other immunotherapies, first approved by the United States Food & Drug Administration in 2018, and suggests they may have contributed to the observed decline. These findings reflect shifting oncologic treatment paradigms and underscore important implications for surgical training and resource planning.

Background: Eyelid tumors present with a diverse histopathological spectrum, varying across geographic and ethnic groups. In Asia, sebaceous gland carcinoma (SGC) often rivals basal cell carcinoma (BCC) in incidence. This study evaluates the clinicopathological profile of eyelid tumors from Northeast India. Objective: To analyse the histopathological distribution, demographic profile, and malignant versus benign ratio of surgically excised eyelid tumours over six years at a tertiary care centre. Methods: A retrospective review was conducted on histopathologically diagnosed eyelid tumors excised between March 2008 and February 2014. Clinical and demographic data were retrieved. Routine H&E staining was performed, with immunohistochemistry as needed. Tumours were classified according to the WHO 5th edition classification. Results: Of 132 excised eyelid lesions, 124 were neoplastic (81.5% benign, 18.5% malignant), and 8 were inflammatory chalazia. Benign tumors included epidermal cyst (23.8%), dermoid cyst (21.8%), haemangioma (14.9%), and squamous papilloma (11.9%). Among malignancies, basal cell carcinoma (BCC) predominated (39.1%), followed by squamous cell Carcinoma (SCC) (30.4%), Sebaceous gland carcinoma (SGC) (21.7%), and malignant melanoma (8.6%). Malignant tumors showed a female preponderance. Comparative analysis with regional and global studies demonstrated higher proportions of SGC and SCC in this cohort. Conclusion: Benign lesions remain predominant in eyelid tumors. However, sebaceous carcinoma and squamous cell carcinoma constitute a significant malignant burden in this Northeast Indian population. Early biopsy and histopathological diagnosis are essential for timely intervention.

Risk Factors for Orbital Exenteration in Periocular Basal Cell Carcinoma

Androgen Receptor Identification in the Diagnosis of Eyelid Sebaceous Carcinomas

Eyelid sebaceous gland carcinoma is rarely observed in young Caucasian men. A 28-year-old man was referred for a recurring chalazion of the eyelid that had been operated four times in the past two years. The chalazion-like lesion of the external third of the upper left eyelid was associated with a cystic lesion. Cytology of the fluid in the cyst and histology of the lesion were compatible with a sebaceous cell carcinoma. Magnetic resonance imaging revealed that the cystic lesion associated with the eyelid tumour was extending into the orbit. According to current clinical practice and experience, to increase the chance of survival of the patient, an orbital exenteration was conducted to remove the sebaceous cell carcinoma in total. Although rare, one should be aware that an eyelid sebaceous cell carcinoma can occur in a young Caucasian man and this diagnosis should be evoked in case of a recurrent chalazion.

We present the management of asevere case of recurrent periocular basal cell carcinoma, orbital invasion and exenteration. The present case is of arecurrent basal cell carcinoma in a84-year-old male presenting with non-healing lesion above right eyelid. Atumor excision was performed in May 2014. Histopathology revealed abasal cell carcinoma (dg. C44.1 ICD-10-CM) with positive margins. The re-excision of the lesion was performed. After two years, there was alocal recurrence and orbital invasion. Indication for external curative radiation therapy. Plastic surgery of the upper eyelid. Orbital exenteration was indicated in January 2018. After another year, arecurrence of the tumor was once again noted. Histopathology revealed abasal cell carcinoma (dg. C44.1 (TNM 7, pMx, pNx, pTx)). The patient was indicated for external radiotherapy. There were no indications for biological treatment. After another year, aprogression of the local finding was noted. Basal cell carcinoma (BCC) is the most common non-melanoma skin cancer of the periocular region. Primary treatment of basal cell carcinoma is surgical. Advanced lesions require extensive surgical interventions and other available treatment modalities. In some cases, mutilating surgery - exenteration of the orbit is inevitable. Despite arelatively small percentage of invasive diseases today, advanced stages may still occur; either as aresult of the patient'slate presentation, inadequate initial therapy aimed at maintaining critical periorbital structures, or due to high tumor aggression. The case report highlights necessity of radical resection of primary tumor with histological examination.

28158 Multiple cutaneous neoplasms and uveal melanoma in a patient with Muir-Torre syndrome

BackgroundMucormycosis is a potentially lethal, angioinvasive fungal infection caused by the Mucoracea family comprising Mucor, Rhizopus, and Absidia species. It is commonly associated with uncontrolled diabetes mellitus, the use of corticosteroids, immunosuppressive drugs, and Covid-19 infection. The occurrence of mucormycosis in an immunocompetent patient is rare. Also, only a few case reports have been published where patients developed mucormycosis with associated malarial infection.Case presentationA young female presented with a 3-weeks history of painful swelling and outward protrusion of the right eye with complete loss of vision. She had a history of P.vivax malaria two weeks before her ocular symptoms. On ocular examination, there was proptosis and total ophthalmoplegia with loss of corneal sensations in the right eye. Hematological examination revealed normocytic normochromic anemia and thrombocytopenia. MRI was suggestive of right-sided pansinusitis and orbital cellulitis with right superior ophthalmic vein thrombosis and bulky cavernous sinus.Nasal biopsy was negative for fungal culture. An emergency surgical debridement of all the sinuses was done with right orbital exenteration. Histopathology confirmed the diagnosis of mucormycosis and the patient improved post-operatively on systemic antifungals.ConclusionSuch an association of mucormycosis with malaria infection is rarely reported in the literature and is hypothesized to be a result of immunosuppression caused by malaria. Also, emphasis is laid upon having a high index of suspicion for fungal infection in the setting of pansinusitis even if the risk factors are absent.We hereby report a case of rhino-orbital mucormycosis following P.vivax malaria in a 20-year-old female with anemia and thrombocytopenia.