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A 28-year-old male presented to the emergency room suffering an ocular burn injury from a welding rod. Given the mechanism of injury, severe delayed injury of the ocular adnexa occurred, requiring enucleation, partial exenteration of the superior orbit, and extensive reconstruction. Histopathology of the affected tissue was analyzed. This is the first report that details the clinical course of a patient with delayed high amperage and low voltage electrical burn injury.

A 77-year-old Asian female with a history of left orbit exenteration and lid-sparing reconstruction for recurrent sebaceous carcinoma presented with fluid-like sensation of the left orbit. Magnetic resonance imaging (MRI) demonstrated bright T2 signal and a cyst-like cavity within the exenterated orbit. Decision was made to proceed with surgical exploration and excision. A calcified, bone-like cavity was encountered intraoperatively and removed. Histopathology revealed dense fibrous connective tissue with areas of calcification without osseous metaplasia, suggestive of retained blood in the orbit that underwent dystrophic calcification. This case report illustrates a rare occurrence of a bone-like calcific cyst following exenteration.

Purpose: Orbital exenteration of periocular tumors complicated by orbital invasion is a heavy burden for patients and leads to disfiguring cosmesis and loss of vision. Here, we report our experience with globe-sparing surgery in a series of patients with periocular malignancies other than basal cell carcinoma (BCC), all exhibiting anterior orbital invasion., Methods: In this consecutive case series, we examined medical records of all patients between 2000 and 2018 with periocular malignancies (other than BCC) invading the anterior orbit (without extraocular muscle or scleral invasion) treated by one orbital surgeon (DP). The main outcome measures included local recurrence, regional and distant metastasis, survival, and visual acuity., Results: Nine patients were identified. Of the non-BCC cancers invading the orbit, squamous cell carcinoma (SCC) (44.4%) was the most prevalent type in our series. Excision included the removal of visibly distinguishable tumor and a free clinical margin of up to 5 mm with histological confirmation of radicality of the invasive tumor component. Reconstruction was achieved by a variety of oculoplastic reconstructive procedures. At a mean follow-up of 70 months (range 11-177 months), 8 out of 9 patients were still alive. Recurrence occurred in two patients with conjunctival melanoma (CM), and they were again treated with wide excision. Postoperative visual acuity remained stable or improved., Conclusion: This retrospective case series demonstrates that globe-sparing excisional surgery can be considered in selected cases of periocular malignancies other than BCC with anterior orbital invasion, thus avoiding cosmetic disfigurement and loss of vision due to orbital exenteration.

A 52-year-old male presented with right eye prominence of 1-year duration. Right eye examination revealed proptosis and limitation of extraocular movements. Magnetic resonance imaging (MRI) of the orbit showed soft tissue mass in the inferomedial orbit with medial rectus involvement and involving lacrimal sac. The patient underwent an incisional biopsy. Histopathology confirmed a diagnosis of primary orbital adenocarcinoma. Positron emission tomography computed tomography (PET CT) did not show the involvement of any other organ. Right orbital exenteration was done for disease control. On histopathology the mass was found to be arising from the lacrimal sac and was reported as apocrine adenocarcinoma. The diagnosis was further confirmed by immunohistochemistry with the tumor staining positive for gross cystic disease fluid protein 15 (GCDFP 15). The patient received adjuvant radiotherapy and is alive without any recurrence at 1 year of follow up. Apocrine adenocarcinomas are rare malignant skin adnexal tumors. Involvement of lacrimal sac is extremely rare with only one case reported in the literature till date. Immunohistochemistry utilizing GCDFP 15 stain can confirm the diagnosis.

Purpose: To review the clinical features and treatment-associated outcomes of primary orbital melanoma among cases reported in the literature and to present a case treated with orbital exenteration and post-operative radiotherapy. Methods: Case reports and case series on primary orbital melanoma published in the literature between 1980 and 2020 were reviewed. Data collected included patient demographics, presenting ocular symptoms, diagnostic imaging, histology, management, and outcomes. Results: Eighty-eight cases of primary orbital melanoma were reviewed. The average age at presentation was 45 years and 58% of patients were male. The most common presenting symptoms and signs were proptosis (73%), decreased visual acuity (32%), pain (14%), diplopia (15%), and palpable mass (9%). Imaging frequently showed a well-circumscribed enhancing lesion. Diagnosis was made by histology in all cases, and orbital blue nevus was identified in 42%. In the majority of cases, treatment consisted of orbital exenteration (54%) or excision (38%). Adjuvant radiotherapy was given in 47% of cases. For the 72 patients with reported outcomes, 36% had metastases, 15% had local recurrence, and 32% died of metastatic disease. Patients who received surgery and radiotherapy had improved survival compared to those who received surgery alone ( p = .01). There was no difference in survival between those who underwent orbital exenteration or excision ( p = .16). Conclusions: Primary orbital melanoma is a rare malignancy and should be considered in patients with a history of unilateral proptosis and a well-defined orbital mass on imaging. Surgery remains the mainstay of treatment. Adjuvant radiotherapy may improve patient survival.

Purpose: Orbital exenteration is a radical oncological surgery that is usually indicated for advanced primary orbital tumors or invasion from local malignancy. We report a 5-year series from a tertiary head and neck center with particular focus on our ablative and reconstructive approach., Methods: We performed a clinicopathological review of patients referred to Guy's and St Thomas' NHS Foundation Trust Head and Neck multidisciplinary team for management input of an orbital malignancy during the period of 2013 to 2018. Cases involving local invasion from sinonasal malignancy were excluded. The reconstructive approach, perioperative complications, disease-free and overall survival were analyzed., Results: 27 patients were identified and of those treated surgically, a radical extended orbital exenteration was required in almost half (44.4%), with squamous cell carcinoma being the most common pathology (55.6%). A concurrent neck dissection and parotidectomy were commonly performed with confirmed or suspected regional disease, or in the presence of high-risk pathological features. This approach resulted in favourable 2-year overall survival in these advanced stage cases of 84.6% and disease-free survival of 73.2%, with 92% achieving a negative surgical margin. The majority of treated patients required a free flap reconstruction, especially when an extended exenteration defect or adjuvant treatment was anticipated. The anterolateral thigh flap was the most commonly used donor site, and we present our algorithm for reconstruction of these defects., Conclusions: A multidisciplinary approach to advanced orbital malignancy with a comprehensive approach to surgical resection and reconstruction results in favorable oncological outcomes and addresses functional and cosmetic patient rehabilitation.

Microcystic adnexal carcinoma (MAC) is a rare malignancy of sweat glands that has been reported most often on the face in the form of a cutaneous lesion, with the potential for deeper invasion. The synonyms of MAC include sclerosing sweat duct carcinoma, syringomatous carcinoma, and malignant syringoma. Clinically, MAC in the periocular area has been misdiagnosed as basal cell carcinoma, squamous cell carcinoma, or even chalazia. We report a case of MAC presenting clinically as sebaceous gland carcinoma with pagetoid spread for the first time in literature.

Purpose: To determine the changes in indications for orbital exenteration over 20 years and to assess its impact on patient survival. Evolving techniques of rehabilitation of the orbit in our institution were also evaluated., Methods: This was a retrospective review of hospital records of patients who underwent orbital exenteration from 1995 to 2015 in a tertiary care center. Data extracted included primary location of the tumor, preoperative treatments, interval between initial diagnosis and exenteration, status of surgical margins, presence of metastatic disease, and postoperative survival. The types of prosthesis utilized over the years were also reviewed. Cox regression analysis was performed for categorical variables. Kaplan-Meier analysis was used to estimate post-exenteration survival., Results: Over a 20-year period, orbital exenteration was performed on 100 orbits of 100 patients. The mean age was 39.4 years (range: 2 months to 90 years). The most common indications among 98 malignant causes were retinoblastoma, squamous cell carcinoma, basal cell carcinoma, extraocular extension of uveal melanoma, and conjunctival melanoma. Postoperative survival was significantly related to age and tumor location but independent from gender, surgical margin, histopathological diagnosis, previous treatment modality, and preoperative interval. In the whole cohort, 1-year and 5-year survival rates were 97% and 84%, respectively., Conclusions: Exenteration appears to be life-saving in children with orbital extension of retinoblastoma. While patients exenterated for malignant eyelid tumors have the best chance of survival, those with orbital extension of uveal melanoma and adenoid cystic carcinoma of the lacrimal gland have the worst prognosis.

The purpose of this article is to evaluate the three different surface coating on cohesive silicone gel implants in eviscerated rabbit eye sockets. Forty-five albino rabbits underwent right eye evisceration and received hemisphere-shaped cohesive silicone gel implants with smooth (Group 1), textured (Group 2), or polyurethane-coated surface (Group 3) in the socket. The animals were euthanized at 7, 30, and 90 days postoperatively. Computed tomography of the orbits was performed prior to euthanasia. Subsequently, the orbital contents were removed and underwent histologic and morphometric examination. Data were statistically analyzed. There were no adverse effects throughout the study. The majority of implants in the Group 1 exhibited 180° rotation. The Group 3 experienced an intense inflammatory reaction around the implant and implant deformation probably due to pseudocapsule contraction. Cohesive silicone gel implants had good integration into the scleral socket. Optimal results were obtained with cohesive silicone gel textured implants (Group 2). Smooth implants (Group 1) rotated significantly, whereas polyurethane (Group 3) coated implants precipitated an intense inflammatory reaction and were deformed postoperatively.

To describe the novel use of a bilayer dermal substitute to reconstruct exenterated orbits. A retrospective chart review was performed in the practices of two surgeons (RET and PDL) of all patients who had undergone orbital exenteration between April 2014 and June 2016 and whose subsequent reconstruction included lining the socket with Integra bilayer. Patient demographics, pathologic diagnoses, surgical and post-operative complications, graft integrity, and patient acceptance were recorded. The charts of 7 patients (4 men and 3 women, ages 60-87 years) were reviewed. In all cases, the Integra graft had taken well to the socket bed at the time of silicone removal 3-4 weeks after surgery. Epithelialization of the socket occurred rapidly over the Integra graft (within several weeks) without incident in each case and with minimal postoperative management. No intraoperative or postoperative complications were noted. Integra dermal substitute is an ideal graft for the lining of an exenterated orbit. It is readily available in large quantities, handles easily, lines the socket smoothly, epithelializes rapidly, and requires minimal postoperative care. It offers minimal morbidity compared to skin grafting or free flap reconstruction, but greatly speeds epithelialization compared to laissez faire management. Surgeons should consider reconstructing exenterated orbits with the Integra matrix, both for its ease of use and its ability to epithelialize rapidly.

Orbital exenteration can lead to significant disfigurement causing considerable functional, cosmetic and psychological disturbances to the patient. Orbital prosthesis is important for the patient's cosmetic and psychological rehabilitation. A 49-year-old man received orbital exenteration and postoperative radiotherapy for malignant orbital carcinoma. In view of uncertain suitability and survival of osseointegrated implant in his case, a novel design of ocular implant consisting of a self-retaining magnetic spring retainer and a coupling silicone prosthesis was used. This novel design was shown to provide the patient with ease of use, good cosmetic outcome and better quality of life.

We describe the techniques and outcome of three different approaches to transfer the posterior 2/3 rd temporalis muscle pedicle flap for orbital socket reconstruction following total orbital exenteration. A retrospective interventional series of 9 patients operated between February of 2000 and 2006. We describe three different techniques, namely supraorbital, transorbital and transorbitectomy approach. All patients were followed for minimum of 3 years and muscle trophism with periorbital contour was clinically studied for outcome. There were 6 males and 3 females with a mean age of 42 years. Three patients each underwent the three mentioned approaches of socket reconstruction following total orbital exenteration performed mainly for oculo-adenexal malignancies with orbital extension (77.78%). Intraoperative, tumor-free histopathological margins were ensured. Postoperatively, bulky lateral orbital rim was noticed in all 3 patients of supraorbital approach, while progressive temporalis flap atrophy was noticed in all with transorbital approach over a period of 6 months. No such complications were observed in transorbitectomy approach and reasonably good periorbital cosmetic appearance with optimum preservation of muscle trophism was obtained. The mean follow-up period was 7 years. Temporalis muscle flap provides adequate orbital volume restoration in an exenterated socket. It also helps in better skin graft uptake, socket health and appearance. The transorbitectomy approach appeared as a reliable one stage surgical technique with reasonably acceptable anatomical and cosmetic outcome over a long-term follow-up. The choice of posterior portion of temporalis muscle as a flap offers satisfactory temporal fossa appearance.

This article compares the perception of health and beauty of patients after exenteration reconstruction with free flap, eyelid-sparing, split-thickness skin graft, or with a prosthesis. Cross-sectional evaluation was performed through a survey sent to all students enrolled at the University of Iowa Carver College of Medicine. The survey included inquiries about observer comfort, perceived patient health, difficulty of social interactions, and which patient appearance was least bothersome. Responses were scored from 0 to 4 for each method of reconstruction and an orbital prosthesis. A Friedman test was used to compare responses among each method of repair and the orbital prosthesis for each of the four questions, and if this was significant, then post-hoc pairwise comparison was performed with p values adjusted using Bonferroni's method. One hundred and thirty two students responded to the survey and 125 completed all four questions. Favorable response for all questions was highest for the orbital prosthesis and lowest for the split-thickness skin graft. Patient appearance with an orbital prosthesis had significantly higher scores compared to patient appearance with each of the other methods for all questions (p value < 0.0001). Second highest scores were for the free flap, which were higher than eyelid-sparing and significantly higher compared to split-thickness skin grafting (p value: Question 1: < 0.0001; Question 2: 0.0005; Question 3: 0.006; and Question 4: 0.019). The orbital prosthesis was the preferred post-operative appearance for the exenterated socket for each question. Free flap was the preferred appearance for reconstruction without an orbital prosthesis. Split-thickness skin graft was least preferred for all questions.

Aim: To evaluate the inter-observer agreement of the Manchester Orbital Exenteration Wound Assessment Tool (MOEWAT), a tool designed to facilitate grading of healing in exenterated sockets, scores from clinical photographs of exenterated sockets., Methods: Existing MOEWAT system was modified to allow grading of clinical photographs. Photographs of 36 different patients at different stages of healing following exenteration were identified. Scores for two masked observers, who independently graded the photographs were compared. The inter-observer agreement of scores was assessed using a Bland-Altman plot, with the average agreement and 95% confidence interval around the mean computed., Results: The average age of the 36 patients was 66 ± 29 years. Photographs were taken 32 ± 16 months after exenteration. Across all photographs, the averages score given by the first observer (4.3 ± 2.2) and second observer (3.4 ± 1.5) were similar (t = -1.9; p = 0.07). From the Bland-Altman plot, the mean difference in score (0.8 ± 1.8) was close to zero, with 95% confidence limits for agreement are indicated by the dashed lines and spanning from -2.6 to 4.3., Conclusion: MOEWAT can be successfully used to stage wound healing in exenterated sockets, with good inter-observer agreement.

Purpose: To report a series of patients with fungal orbital cellulitis who underwent exenteration surgery and describe presenting features, management and outcomes at a referral center., Methods: Retrospective case series., Results: From November 2011 to March 2014, four patients underwent orbital exenteration for fungal orbital cellulitis at the University of Illinois. Three patients had mucormycosis and one had aspergillosis. All patients were treated with intravenous antifungals and underwent orbital exenteration. Two patients were successfully treated with supplemental intra-orbital catheter delivery of amphotericin B. Presenting visual acuity in the affected eye ranged from 20/25 to no light perception. Some level of ophthalmoplegia was present in three patients. Significantly elevated intraocular pressure was found in two patients. All patients with mucormycosis were found to have uncontrolled diabetes mellitus. One patient had a history of myelodysplastic syndrome, chronic hepatitis C infection, polysubstance abuse and Crohn's disease. Another patient had a history of alcoholic liver cirrhosis, Crohn's disease treated with systemic immunosuppression and renal cell carcinoma. The patient with aspergillosis had myelodysplastic syndrome and portal hypertension, and the initial presentation resembled giant cell arteritis. Two of four patients died during their hospitalization., Conclusions: Fungal orbital cellulitis has a high mortality rate despite aggressive antifungal treatment and orbital exenteration performed soon after the diagnosis is confirmed. Patients often have a history of immunosuppression and the onset may be insidious. There must be a high rate of suspicion for fungal orbital cellulitis given the appropriate signs and medical history in order to avoid treatment delay.

Osteoradionecrosis (ORN) occurs in an estimated 2% of head and neck-irradiated patients. It is seen most commonly in the mandible with other reported sites including the maxilla, temporal bone, clavicle, and vertebrae. It is defined as an area of exposed devitalised irradiated bone, with failure to heal during a period of at least 3 months, in the absence of local neoplastic disease. We report 2 cases of ORN following postoperative radiotherapy given to patients who had undergone an orbital exenteration. ORN can develop spontaneously in one-third of cases, although in the majority of patients, it is induced by secondary trauma. Radiotherapy induces an endarteritis in the small blood vessels of bone, thus favouring the generation of small thrombi that obliterate the vascular lumen and interrupt tissue perfusion. Likewise, irradiation impairs the function of osteoblasts, manifesting as osteopenia, with impairment of the repair and remodelling capacity of bone. Prior radiation exposure can thus decrease bone vascularity and injure its reserve reparative capacity. It is important to differentiate ORN from local recurrence of malignancy, bone metastasis, radiation-induced sarcoma, and infection. CT and MRI are effective diagnostic tools. Clinical management of ORN is complex and unsatisfactory. Treatment remains difficult, and prevention is paramount. A history of radiotherapy should alert clinicians to detect bone exposure or excessively prolonged socket healing. Early diagnosis with a high index of suspicion can achieve higher control rates with conservative management. Our case series reports a rare, previously unreported, but important complication of radiation therapy of the exenterated orbit.

Primary signet ring cell carcinoma of the eyelid (PSCE) is a very rare tumour. Only a few cases having been reported to date. The majority of patients have been middle-age to elderly men. Different treatment modalities have been applied in cases of PSCE. Here, we report a case of PSCE in a man treated with orbital exenteration and radiotherapy.

Haemangiopericytoma (HPC) is a rare soft tissue tumour of fibroblastic origin and is part of the solitary fibrous tumour spectrum. The tumour is generally considered to be benign, but can behave clinically as if sarcomatous -- with relentless infiltrative local growth. HPC generally presents in adulthood (median age 45 years for orbital disease) and is equally frequent in both sexes. HPC can arise in any site in the body and presents as a slowly growing, painless mass. We report a case of a 20 year old African male seen at Kikuyu Eye Unit, Kenya, with a 12 year history of a gradually enlarging, painless orbital mass. The patient underwent skin-sparing orbital exenteration with complete tumour excision; histology confirmed diagnosis of HPC.

Purpose: To describe the clinicopathologic features and management of an unusual case of orbital teratoma., Methods: A 7-year-old girl presented with a history of an orbital mass since birth. CT scan showed a large mass lesion involving the right orbit, with absence of the eyeball. An ectopic tooth was identified within the tumor. Lid-sparing exenteration surgery was performed., Results: Histopathologic examination of the excised mass showed presence of elements from all three germ layers, consistent with a diagnosis of mature orbital teratoma. Normal ocular structures were not identified on histopathology. At one year follow-up, there was no tumor recurrence., Conclusion: We report an extremely rare and interesting case of a mature orbital teratoma, which was associated with primary anophthalmos and an ectopic tooth.

Purpose: To report a unique approach to the orbital apex through the contralateral orbit., Methods: A 65-year-old male presented with right compressive optic neuropathy due to nasopharyngeal carcinoma spread. Clinical evaluation and imaging showed a right orbital apex mass. Surgery included apical orbital tumor debulking, and orbital decompression performed via the contralateral exenterated side. Postoperatively, symptoms were relieved., Conclusions: Orbitotomy via the contralateral exenterated orbit should be considered as a surgical option in these unique patients requiring direct access to the orbital apex.

Purpose: Indications for orbital exenteration performed over a 10-year period from a tertiary eye care center in Western India., Method: Retrospective review of medical records of all patients who underwent exenteration of orbit over a period from January 1999 to April 2009, at a tertiary eye care center in western India., Result: Out of total 236 major orbital surgeries performed, 15 (6%) underwent orbital exenteration. Age ranged from 19 years to 75 years. Of these 9 were male and 6 female. Except for one case all exenteration were for malignant neoplasm. Lesions originated from eyelid in 8 (54%) cases, orbit in 5 (33%) cases and bulbar conjunctiva in 2 (13%) cases. Histopathological classification of the neoplasm were sebaceous carcinoma (4 cases), basal cell carcinoma (3 cases), squamous cell carcinoma (3 cases), adenoid cystic carcinoma (1 case), transitional cell carcinoma (1 case), malignant fibrous histiocytoma (1 case), lymphoma (1 case) and mucormycosis (1 case)., Conclusion: Majority of exenterations performed were for neglected ocular or adnexal malignant lesions that could have been avoided provided patients reported earlier for treatment. Exenterations can be reduced by aggressive patient education about eye malignancy.

Mesenchymal chondrosarcoma, an uncommon lesion in bone and extraskeletal tissue, is extremely rare in the orbit. Two cases of orbital mesenchymal chondrosarcoma in young adults presenting with proptosis and diminution of vision are reported. The diagnosis was established by histopathological examination in both cases, which showed undifferentiated mesenchymal cells with islands of cartilage. Both patients underwent exenteration followed by chemotherapy and radiation therapy and are alive with healthy orbits after two years of follow-up.

Purpose: To evaluate the results of the porous polyethylene (Medpor) orbital implantation technique combined with the use of a primary patch graft of acellular dermis., Methods: The study design was a retrospective, non-comparative single surgeon case series of 27 patients. The Medpor orbital implant was placed with a primary anterior sub-Tenon's acellular dermis patch after evisceration (23/27), enucleation (2/27) or implant exchange/secondary implant procedure (2/27) over a period of 3 years. Risk factors for dehiscence/complications were defined as trauma, infection, inflammation, previous ocular surgery, radiotherapy, secondary orbital implantation, and removal of extruding implants. The primary outcome measure was the development of implant exposure. Other complications were also recorded, including postoperative conjunctival wound dehiscence., Results: Twenty-five patients (93%) had more than 2 risk factors. Mean follow-up time was 22 months (median 24 months, range 4-34 months). Three (11%) patients needed further intervention, which included pyogenic granuloma excision (1) and fornix formation (1). Conjunctival wound dehiscence followed by implant exposure occurred in 1 of 27 eyes (3.7%). The cosmetic appearance was satisfactory in 26/27 patients., Conclusions: Our results with primary acellular dermis grafts when coupled with Medpor orbital implantation are encouraging.

Purpose: To describe a technique for reconstructing the orbital bony architecture after invasion by tumour., Methods: Orbital bone invaded by tumour was osteotomized (post-exenteration), autoclaved to remove tumour cells, and then refixated in order to re-establish the normal orbital anatomy., Results: Despite some shrinkage of the bone fragment in the autoclaving process, after refixation the contour and topography of the bony orbit was essentially normal., Conclusion: Autoclaved bone can be used to reconstruct the exenterated orbit; it is a fast and technically simple strategy for maintaining orbital anatomy when faced with bony invasion by tumour.