Impact of Food and Drug Administration Approval of Vismodegib on Prevalence of Orbital Exenteration as a Necessary Surgical Treatment for Locally Advanced Periocular Basal Cell Carcinoma.

PURPOSE: To test the hypothesis that the US Food and Drug Administration approval of vismodegib in early 2012 has reduced the prevalence of orbital exenteration for locally advanced periocular basal cell carcinoma (BCC). METHODS: Following institutional review board approval, the authors reviewed clinical and pathological data of patients with locally advanced periocular BCC (T4 per the eyelid carcinoma classification in the 8th edition of the AJCC Cancer Staging Manual) treated by the senior author during 2006-2018. Patients were grouped into those who were treated before February 2012 ("before vismodegib approval") and those who presented later ("after vismodegib approval"). RESULTS: Forty-two patients with locally advanced periocular BCC were treated during the study period, of whom 31 were men. The median age at presentation was 66 years (range, 43-90). Twenty-two patients had T4a and 20 had T4b tumors. Thirteen patients were treated before and 29 were treated after vismodegib approval. The 2 groups did not differ in age distribution (p = 0.164), sex distribution (p = 0.270), prevalence of recurrent tumor at presentation (p = 0.317), or duration of treatment with vismodegib (p = 0.605). Orbital exenteration was significantly more prevalent in patients treated before vismodegib approval than after (46% vs. 10%, p = 0.016), and vismodegib treatment was significantly more prevalent in patients treated after vismodegib approval than before (when vismodegib was given in clinical trials; 69% vs. 23%, p = 0.008). There was a trend toward more patients retaining their eyes at last follow-up in patients treated after vismodegib approval (83% vs. 54%, p = 0.066). CONCLUSIONS: The prevalence of orbital exenteration as a necessary surgical procedure in patients with a locally advanced periocular BCC has fallen since the Food and Drug Administration approval of vismodegib. Although vismodegib is not specifically approved for organ-sparing, it has changed the authors' practice and enabled eye preservation in patients with locally advanced periocular BCC, who would otherwise require an orbital exenteration.

Orbital exenteration: The 10-year Massachusetts Eye and Ear Infirmary experience.

The authors report their experience with orbital exenteration surgery at one academic institution over a 10-year period and review the literature. This retrospective cohort study monitored outcomes of all patients who underwent orbital exenteration surgery at Massachusetts Eye and Ear Infirmary between January 2003 and January 2013. Patients with no follow-up data or survival data were excluded from the study. The main outcome measures were surgical complications, disease status of surgical margins, need for adjuvant treatment, local recurrence, metastases and survival. 23 patients with malignancy and 2 with mucormycosis met inclusion criteria for the study. Surgical procedures included non-lid sparing total exenteration (44%), lid-sparing total exenteration (32%), non-lid sparing partial exenteration (8%) and lid-sparing partial exenteration (16%). 44% underwent additional extra-orbital procedures. Survival rates were 72% at 1 year, 48% at 3 years, and 37% at 5 years. Of patients with malignancies, 48% had clear margins after exenteration. There was no statistically significant difference in survival between patients with negative surgical margins compared to positive margins (p = 0.12). Mortality was highest in patients with melanoma (85.7%) and lowest in patients with non-squamous cell lid malignancies (0%). Our study suggests that the type of disease has a much greater impact on the survival of patients undergoing exenteration surgery than the type of exenteration surgery or the disease status of surgical margins. Patients with non-squamous cell lid malignancies and localized orbital disease have the best prognosis for tumor eradication from this radical and highly disfiguring surgery.

Risk factors for endophthalmitis requiring evisceration or enucleation.

Endophthalmitis has devastating sequelae resulting in blindness and even loss of eyeball. Although the prognosis of endophthalmitis has much improved with the advances of antibiotics and vitreoretinal surgery, of the number of patients that required evisceration or enucleation is still significant. We retrospectively reviewed the charts of 210 eyes of 210 patients with endophthalmitis andcompared the group that required evisceration or enucleation with those that received salvaging therapies. Regression analysis was used to identify the risk factors for evisceration or enucleation. Thirty eyes (14.3%) underwent enucleation or evisceration. The group of eviscerated or enucleated eyes were older (58.7 vs. 42.2 years, p < 0.001), had more women (56.7% vs. 22.2%, p = 0.003), had poorer initial visual acuity (2.79 vs. 2.10 LogMAR, p < 0.001), and had longer duration before intervention (18.03 vs. 5.74 days, p = 0.031). The most common primary indications for endophthalmitis were infections from corneal ulcer (50.0% vs. 4.4%, p < 0.001) andfrom endogenous source (23.3% vs. 5.6%, p < 0.001). Less common indications were trauma (26.7% vs. 67.8%, p < 0.001) and postoperative (6.7% vs. 22.2%, p = 0.049) endophthalmitis. After adjusting for confounding factors, corneal ulcer-related endophthalmitis, endogenous endophthalmitis and initial visual acuity were the independent risk factors for evisceration or enucleation.

Pattern of orbital exenteration at a tertiary eye care centre in North India.

OBJECTIVE: To analyze cases undergoing orbital exenteration in terms of demographic and socioeconomic profile of patients, indications for surgery, histopathological diagnosis and to assess the magnitude of eyelid malignancies as indication of orbital exenteration. MATERIALS AND METHODS: A case record analysis of 25 patients who underwent orbital ex enteration at a tertiary care centre in India between October 2002 and October 2007 was undertaken. The case records were reviewed to obtain demographic data, presenting symptoms, duration of symptoms, laterality, best-corrected visual acuity (BCVA), and the clinical and histopathological diagnosis. RESULTS: The age group of patients undergoing exenteration ranged from 1 year to 78 years (Mean 46.92 +/- 10 years). Proptosis and/or a palpable mass were the main presenting complaints in 80 % of cases. The duration of symptoms was more than 5 years in 80 % cases. Primary orbital malignancies were the commonest indication for exenteration and were diagnosed in 11 cases (44 %), followed by lid malignancies in 8 cases (32 %), retinoblastoma in 4 cases (16 %) and conjunctival malignancies in 2 cases (8 %). Sebaceous gland carcinoma was the commonest lid malignancy followed by basal cell carcinoma and squamous cell carcinoma. CONCLUSION: Lid malignancies contribute the majority of the patients undergoing orbital exenteration in this part of the world, leading to an extreme sacrifice of eyes with good visual acuity. Increase in awareness, early diagnosis and prompt referral may help to overcome this grim scenario.

Review of 345 eye amputations carried out in the period 1996-2003, at Rigshospitalet, Denmark.

PURPOSE: The aim of this study was to identify the number of eye amputations, and the causative diagnoses, indications for surgery and surgical techniques applied, and to evaluate a possible change in surgical technique in a tertiary referral centre in Denmark. METHODS: The hospital database was screened using surgery codes for patients who had undergone bulbar evisceration, enucleation or orbital exenteration in the period 1996-2003. Patient records were reviewed for gender, age, time since surgery, causative diagnosis (the disease process leading to the indication for amputation), indication for eye amputation, type of surgery and whether an implant was applied. RESULTS: A total of 345 patients were identified as having undergone eye amputation during the 8-year period. Indications for eye amputation were: painful blind eye (127); neoplasm (119); infection (42); recent injury (25); disfiguring blind eye (25); prevention of sympathetic ophthalmia (5), and other reasons (2). Surgical procedures included 174 eviscerations, 154 enucleations and 17 orbital exenterations. The mean number of surgeries per year was 43.1. An orbital implant was applied in 168 patients. CONCLUSIONS: The most frequent indications for eye amputation were painful blind eye (37%) and neoplasm (34%). During the study period, the annual number of eye amputations was stable, but an increase in bulbar eviscerations was noticed. Orbital implants were used in 33% of patients in 1996 and 67% in 2003.

Indications for orbital exenteration in mucormycosis.

PURPOSE: To determine whether evidence-based standards exist regarding the indications for orbital exenteration in patients with orbital mucormycosis. METHODS: A literature review was performed of 113 articles (1943 to 2004). Factors possibly related to patient survival were analyzed. Additionally, a survey was sent to all ASOPRS Fellowship Preceptors to ascertain the frequency, outcome, treatment modalities, and indications for exenteration by these practicing physicians. RESULTS: For published cases, parameter estimates (PE 46 years, frontal sinus involvement, and fever were less likely to survive compared with patients without these conditions. Patients treated with amphotericin B (OR, 4.476) and those with diabetes (OR, 4.987) were more likely to survive compared with patients without these conditions. Exenterated patients with fever were more likely to survive compared with nonexenterated patients with fever (P=0.0468). Thirty-four ASOPRS Fellowship Preceptors received surveys; 26 (76%) completed the survey. Responses to specific survey questions showed a varied experience and indication for exenteration throughout the country. CONCLUSIONS: : Our study underscores the lack of adequate data regarding the evaluation of treatment of orbital mucormycosis. No standard of care currently exists to guide physicians on when exenteration may benefit a mucormycosis patient. Further study is needed to determine which variables indicate the extent of disease and which variables or analytic scheme might predict the progression of orbital mucormycosis with or without exenteration.

Orbital exenteration: a 13 year Manchester experience.

BACKGROUND/AIMS: Orbital exenteration is a psychologically and anatomically disfiguring procedure reserved for the treatment of potentially life threatening malignancies or relentlessly progressive conditions unresponsive to other treatments. In this study the authors aimed to review their experience with exenteration, including indications, outcomes, and reasons for the increased rate of exenterations over the past 15 months. METHOD: This retrospective study reviewed operating department records via a computerised database to identify all patients who had undergone exenteration of the orbit from 1 January 1991 to 1 April 2004 inclusive, at the Manchester Royal Eye Hospital. Where case records were unavailable, attempts were made to obtain patient data from general practitioners, local health authorities, and referring hospitals. RESULTS: 69 orbits of 68 patients were identified. The mean age of the cohort was 68.2 years, with 33 males and 35 females having undergone exenterations. In total, 31 patients had previously undergone treatments undertaken by the referring specialty with a mean time from the primary procedure to exenteration of 115 months. 14 different tumours were encountered, of which basal cell carcinoma (28), melanoma (10), sebaceous cell carcinoma (nine), and squamous cell carcinoma (six) were the most common. An increasing incidence was observed in cases of BCCs requiring exenteration. 30 patients received orbital prosthesis within an 11 month period post-exenteration. CONCLUSION: Exenteration is a procedure performed with increasing frequency in this unit over the past 15 months, the majority the result of BCCs. A large proportion of these exenterations had undergone previous treatments under a variety of non-ophthalmic specialties in other units. Exenterations are disfiguring procedures that may, therefore, be reduced in incidence by aggressive removal at the time of primary removal. Once performed, the cosmetic rehabilitation is long, with multiple postoperative visits, independent of the method used to close the orbital defect.

Destructive ophthalmic surgical procedures in Onitsha, Nigeria.

OBJECTIVES: to determine the incidence, trend and causes for surgical removal of the eyeball at the Guinness Eye Center Onitsha, Nigeria. PATIENTS AND METHODS: Case files and theatre records of all patients who had eviscreration, enucleation or exenteration at the Guinness Eye Center Onitsha between 1995 and 2001 were reviewed. Information on age, sex, disease duration before hospital presentation, indication for surgery and the type of surgery were analysed. RESULTS: 56 eyes of 56 patients were surgically removed. Patients including some with penetrating eye injuries presented late for treatment; 37.5% and 53.5% had used traditional eye medicines (TEM) and eye drops possibly containing steroids, respectively, prior to hospital consultation. The indications for surgery were severe ocular infections, especially panophthalmitis; severe penetrating eye injuries and orbito-ocular tumours. The annual incidence ranged from 1.9 - 4.2% with an average of 3.4% . The downward trend observed in the later part of the study was associated with reduced incidence of ocular gunshot injuries, which in turn coincided with a reduction in violent crime in Onitsha and its environs. CONCLUSIONS: In spite of the observed downward trend in the rate of destructive eye surgery, the incidence of such procedures could be reduced further by timely intervention with modern microsurgical techniques; intraocular antibiotics and radiotherapy. The public should be educated on the harmful effects of TEM and abuse of eye drops. Those with eye problems should seek early hospital treatment.

Evaluation of the etiology of ocular globe atrophy or loss.

This survey investigated the etiology of atrophy or loss of the ocular globe in patients assisted at the Maxillofacial Prosthetics Clinic of two Schools of Dentistry in São Paulo State, Brazil. A total of 238 patients were examined and their clinical files were reviewed. The etiology of eyeball atrophy/loss was assessed with respect to gender, age group, affected side and type ophthalmologic surgery performed. The greatest incidence of ocular globe loss was due to traumatic etiology (57.14%), followed by pathogenic (36.13%) and congenital (5.04%) etiologies. Comparing the genders, a predominance of male patients was observed (61.76%; p<0.01). The age group most frequently affected was between 21 and 40 years (42.01%; p<0.01). For all types of etiologies investigated in this study, enucleation was the most commonly used surgical procedure for removal of the ocular globe (66.38%; p<0.01). Loss of the left eye was predominantly seen (55.04%), even though no statistically significant difference was found between sides (p>0.01).

Pattern of orbital exenteration in a tertiary eye care centre in India.

PURPOSE: This study aimed to determine the clinical indications for orbital exenteration, profile of these patients and clinicopathological correlations, and to compare these results with previous published data. METHODS: A retrospective analysis was conducted of exenterations performed in adults at a tertiary eye care centre in India over a period of 10 years (January 1990 to December 2000). Patient records were reviewed to obtain demographic data, presenting symptoms and their duration, laterality, and clinical and histopathological diagnosis. RESULTS: A total of 26 cases were identified. Patients ranged in age from 32 to 72 years (mean +/- SD 58.7 +/- 9.23 years). No sex predilection was observed. Classification of cases on histopathological criteria showed that exenterations were performed mostly for squamous cell carcinoma (10 cases), followed by sebaceous gland carcinoma (six cases) and basal cell carcinoma (two cases) of the eyelid. Conjunctival malignant melanoma contributed to 5 of 26 cases of exenteration. Adenoid cystic carcinoma of the lacrimal gland (one case), neurofibroma (one case) and orbital fungal infection (one case) were the other indications of exenteration. The clinicopathological correlation was 100% for squamous cell carcinoma, 100% for basal cell carcinoma, 80% for malignant melanoma and 75% for sebaceous gland carcinoma. CONCLUSION: Exenteration is mainly performed as a life-saving treatment for advanced malignant tumours with epithelial tumours being the commonest. In comparison to previous published data, the indications of surgery in India differed, as squamous cell carcinoma and sebaceous gland carcinoma were the commonest indications for exenteration.

Orbital exenterations and squamous cell carcinoma of the conjunctiva at Sekuru Kaguvi Eye Unit, Zimbabwe.

OBJECTIVES: The aim of the audit was to determine the common orbital diseases necessitating orbital exenterations at Sekuru Kaguvi Hospital (SKH) between January and December 1999 and recommend ways of reducing the numbers of procedures done annually. DESIGN: Retrospective cross sectional study. SETTING: Department of Surgery, Parirenyatwa Hospital, University of Zimbabwe. SUBJECTS: 23 patients who underwent orbital exenterations at SKH over a one year period. MAIN OUTCOME MEASURE: The nature of orbital tumours requiring orbital exenteration in Zimbabwe. RESULTS: A total of 23 patients underwent orbital exenteration during the period under review. Of the 23 patients, 13 (56.5%) had squamous cell carcinoma (SCCA) of the conjunctiva with orbital extensions, 69% of the 13 patients were females and the mean age of these patients was 37.2 years (interquartile range 28, 48). CONCLUSIONS: People in tropical regions, where there is a high risk of squamous cell carcinoma of the conjunctiva in terms of increased predisposition to solar radiation, high prevalence of HIV and inadequate eye care services, should seek medical attention as soon as they notice a persistent conjunctival growth. Conjunctival tumours should be widely excised and material sent for histology to confirm diagnosis and clarity of excision margins. All patients diagnosed as having SCCA of the conjunctiva should be closely monitored to facilitate early detection of recurrences and institute appropriate treatment.