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Purpose To report the epidemiology, indications and surgical outcomes of oculoplastic surgeries in older adults (≥ 90 years old). Methods A retrospective study was conducted reviewing the medical charts of 114 patients aged 90 years old and older who underwent oculoplastic procedures from 2010 to 2020. Data retrieved from the medical records included: past medical and ocular history, indication for surgery, type of surgery, intra and post-operative complications, pathological analysis for removed tissues, and surgical outcome in the last follow-up. Results One hundred and twenty eight surgeries were performed on 114 patients (male: female = 1:1). The mean age was 92.95 years old (± 3.12 SD). Six patients (5.2%) were older than 100 years old. The most common indication for surgery was lower lid malpositioning (32%). Mass/lesion excision was performed in 34 procedures (25%). Forty-three biopsies were analyzed and basal cell carcinoma was found to be the most common pathological diagnosis (32%). Hypertension was the most common associated systemic comorbidity (79 patients, 69%). 80% of the surgeries were performed under local anesthesia. Surgical revision was required in seven patients (5.2%). One patient had suffered from a minor stroke one day after the surgical pocedure. The same patient had orbital-skin fistulas after orbital exenteration Conclusion In our experience, oculoplastic surgeries among the elderly population are safe without significant complications and can usually be performed under local anesthesia. Advanced age should not prevent surgery, especially if the procedure may improve vision and quality of life.

Malignant tumours arising in the paranasal sinuses or maxilla usually spread to the surrounding regions. The skull base and the anterior cranial fossa are frequently affected as well. When the resection of a tumour involves an orbital exenteration, a transconjunctival-perilimbic incision can be added to a coronal approach in order to preserve the eyelids and the conjunctiva, avoiding cutaneous midfacial incisions. Patients with a diagnosis of malignant tumour affecting the orbit, upper jaw, paranasal sinuses, and/or anterior skull base were eligible for this technique. Tumoural invasion of the eyelids, conjunctiva, lacrimal system, or surrounding skin was considered a contraindication for this technique. A retrospective study of the clinical records was performed and age, type of tumour, location, and reconstructive technique were evaluated. Eight patients referred to the study department between 2015 and 2019 were selected. All patients underwent craniofacial surgery and orbital exenteration. The transconjunctival-perilimbic approach was combined with a coronal incision in all cases. In our experience, the transconjunctival-perilimbic approach to orbital exenteration proposed in this paper can be used successfully in skull base surgery. Combined with a coronal and transmandibular approach, it allows wide access to the facial skeleton/anterior skull base while avoiding skin incisions in the midface.

BACKGROUND AND OBJECTIVE Orbital metastases are an uncommon condition. They may be the clinical presentation of a previously unknown malignancy. Depending on the series, the rate of orbital metastasis as a first manifestation of a malignant tumour is 20-42%. The clinical presentation and survival are presented in a series of 11 cases of orbital metastasis corresponding to 10 patients. MATERIAL AND METHODS Descriptive retrospective study of a series of 10 adult patients diagnosed with orbital metastasis from solid tumours during a 9-year period. Metastases involving the orbit were included, and lymphomas and contiguity invasions from adjacent structures were excluded. A note was made on whether the clinical picture was the first sign of tumour onset. Signs and symptoms at clinical debut were registered, as were primary tumour location, distance seeding, orbital structures involved, and survival time since the diagnosis was established. RESULTS One-half (50%) of the patients were women. Mean age at diagnosis was 60.9years (range 42-82). In nine cases (90%), the metastasis was unilateral, while in the remaining one the involvement was bilateral. The most frequent primary tumour location was the breast (36% of the cases), followed by the bladder (27%), lung (18%), and ovary and cavum (9%). Seventy percent of the patients had a previously diagnosed neoplasm; in 3 cases the metastasis was the first malignancy manifestation. Most frequent symptoms were diplopia (60%), visual impairment (40%), and pain (30%). The most common signs were resistance to ocular retropulsion (60%), presence of a mass on orbital palpation and ocular dystopia (50%), and bulbar hyperaemia and proptosis (40%). The most employed management modality was clinical observation (5 patients). In 3 patients radiotherapy was administered, combining chemotherapy plus hormonal therapy in one case, and orbital exenteration in another one. In 2 cases, chemotherapy was administered as an isolated regime. Median survival time was 4.8months since the diagnosis. There was a statistically significant difference between the survival time in the observation group (median 2.5months) and in the active treatment group (median 29.2months), P=.034. CONCLUSIONS In the series presented, 27% of the cases established the clinical debut of the malignant neoplasm. The ophthalmologist plays an essential role when this condition is suspected, diagnosing it, and proposing its management together with the Oncology Service.

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.

Summary Purpose To evaluate our patients with rhino-orbito-cerebral mucormycosis according to a new scoring system and to compare those who underwent orbital exenteration persuant to a new threshhold. Methods Mucormycosis is a fungal disease that can be of acute onset, aggressive, and angioinvasive. Aggressive surgical debridement, long-term systemic antifungal therapy, and treatment of underlying predisposing factors are essential to the treatment. However, orbital exenteration is a very difficult decision to make, particularly in patients with orbital involvement, because there is little information in the literature, resulting in limited support for making this aggressive surgical decision. In this study, our 43 cases of mucormycosis were evaluated in terms of orbital exenteration using a scoring system (including clinical signs and symptoms, ophthalmoscopic findings, and radiologic results) developed by Shah et al., which establishes indications for orbital exenteration in mucormycosis. Results According to our study, if the threshold score for exenteration is 19.0, the sensitivity was 100% and specificity was 97%, providing better results than the 23.0 threshold score determined by the reference study for exenteration. When these two score thresholds were compared, there was a statistically significant difference. Conclusion We believe that this scoring system may be beneficial to use for orbital exenteration in patients with mucormycosis. Prospective studies in large case series are required to determine the most appropriate threshold score.

Objective This study aims to evaluate malignant and benign diseases that lead to orbital exenteration. Patients From December 1999 to September 2017, patients undergoing orbital exenteration were included in this retrospective study. All of them were evaluated on clinical symptoms, indications, tumour localizations, pathologies, reconstruction techniques, complications, recurrences, and survival. Results Of the 205 patients enrolled in this study, 94 had a carcinoma, 73 melanoma, 9 a sarcoma, 14 some other malignant disease, and 15 a benign medical condition. Sixteen patients underwent reconstruction using a local eyelid skin flap (7.8%), 6 with a split-thickness graft (2.9%), 144 with a local flap (70.2%), and 25 with a microvascular graft (12.2%), whereas 14 patients did not undergo reconstruction (6.8%). The most common complications were wound dehiscences (25 cases), pain (17 cases), and partial flap necroses (13 cases). Moreover, 62% of the patients were treated with different facial prostheses or artificial eyes. Given these results, it appears that lymph nodes and distant metastases, as well as lymphatic invasion into vessels, perineural invasion, and non-cleared resection margins, seem to affect overall survival after orbital exenteration. Conclusion Different reconstruction techniques can be used to provide the patient with maximum functionality and aesthetics after orbital exenteration. Individual concepts should be discussed at the beginning of the treatment. Using primary reconstruction and providing osseointegrated implant-retained prostheses remain the gold standard.

Orbital exenteration is a disfiguring procedure that often results in free tissue transfer for reconstructive purposes. The reconstructive focus is the obliteration of dead space while sparing the nasal airway, particularly if the medial orbital wall was resected. Prolapse of transferred tissue into the nasal airway may cause breathing difficulties drastically compromising quality of life. The objective of this study was to demonstrate the effectiveness and feasibility of temporary nasal septum splints as mechanical support for transferred tissue, to prevent airway obstruction. This novel application technique was employed in three patients between 2017 and 2018. No flap loss or sino‐orbital fistulas were observed. On postoperative MRI and endoscopy, a patent nasal airway was observed at all times. Temporary nasal splinting in combination with free tissue transfer proved to be a simple, but effective reconstructive option for securing the nasal airway following orbital exenteration with resection of the medial orbital wall.

A 20-year-old girl came to the Tianjin Medical University Eye Hospital because of progressive herniation of her right eye for 3 months. Examination revealed a huge mass behind the right eyeball, which was clinically diagnosed as a space-occupying lesion of the right orbit. Because the tumor was close to the nerve, most of the tumor was resected and pathologically diagnosed as a poorly differentiated leiomyosarcoma. Orbital evisceration was performed later. There was no recurrence during the 10-year follow-up.20岁青年女性因右眼渐进性突出3个月就诊。检查发现右眼球后巨大肿物，临床诊断为右眼眶占位性病变。因肿物紧靠神经，故行肿物大部分切除，组织病理学确诊为平滑肌肉瘤，低分化型。再次手术行眼眶内容摘除。术后密切随访，未行其他治疗，随访10年无复发。.

BACKGROUND Orbital exenteration (OE) is an ablative procedure used in the management of malignancies of the orbit of either primary or secondary origin. Publications evaluating this procedure have suffered from small patient numbers, heterogeneity of pathologies, and poor patient follow-up. The purpose of this study was to assess patient outcomes in a large cohort of patients undergoing OE at a tertiary cancer center. METHODS A retrospective review was conducted of 180 consecutive patients who underwent OE at the authors' institution. Overall survival (OS) was the primary end point measured in the study. Time to locoregional recurrence (progression-free survival [PFS]) and disease-free survival were secondary end points. RESULTS Between the years 1993 and 2011, 180 consecutive patients received OE for craniofacial malignancy at the authors' institution. The median follow-up for the cohort was 9.7 years (116 months). The median OS was 73 months, and the median PFS was 96 months. The presence of perineural invasion was associated with shorter OS (P = .01) and PFS (P < .01). Magnetic resonance imaging was predictive of perineural invasion (P < .01). Positive margins were associated with shorter PFS than negative margins (P < .01) but with no change in OS (P = .15). The overall complication rate was 15%. The major complication rate (Clavien-Dindo 3b or greater) was 2.8% (n = 5), and there was 1 death observed (0.6%). CONCLUSIONS Used judiciously in the setting of a multidisciplinary management plan, OE for tumor control is a safe therapy. LAY SUMMARY Between the years 1993 and 2011, 180 consecutive patients received orbital exenteration for craniofacial malignancy at the MD Anderson Cancer Center. The median follow-up for the cohort was 9.7 years. The presence of perineural invasion was associated with shorter overall survival (P = .01) and progression-free survival (P < .01). Magnetic resonance imaging was predictive of perineural invasion (P < .01). Positive margins were associated with shorter progression-free survival than negative margins (P < .01). The overall complication rate was 15%. The major complication rate (Clavien-Dindo 3b or greater) was 2.8% (n = 5).

Reconstruction options after orbital exenteration can be challenging, time-consuming, and require intensive postoperative care. Engineered dermal acellular matrices offer a quick and easy option for wound healing that has proven to be successful in various settings. Specifically, the porcine urinary bladder matrix has demonstrated success in periocular and orbital wound healing. This report describes a pediatric patient who underwent repair with porcine urinary bladder matrix after orbital exenteration for recurrent alveolar rhabdomyosarcoma. The patient did not require any additional reconstructive procedures. To our knowledge, this is the youngest patient to receive a porcine urinary bladder matrix after 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.

This study aims to analyse the various modifications of orbital exenteration. Patients undergoing orbital exenteration from March 1978 to October 2019 were included in this retrospective study. The patients were evaluated on the basis of the indication, type of exenteration, reconstruction technique, overall survival (OS), and disease-free survival (DFS). In total, 300 patients were enrolled in this study. As many as 24 patients had lid and conjunctiva sparing anterior exenteration, 16 had lid sparing anterior exenteration, 83 had anterior exenteration, 14 had lid and conjunctiva sparing total exenteration, seven had lid sparing total exenteration, 44 had total exenteration, one had lid and conjunctiva sparing extended exenteration, 23 had lid sparing extended exenteration, and 88 had extended exenteration. As many as 39 patients had a primary wound closure. Six patients underwent reconstruction with a split-thickness skin graft, 177 patients with a local or regional flap, and 40 patients with a microvascular flap. A total of 38 patients did not undergo reconstruction. The mean follow-up was 40 months (range 6–216 months). The OS rate was 82.2% after 1 year, 58.5% after 5 years, and 49% after 10 years for all patients with malignant tumours. The DFS rate was 67.7% after 1 year, 45.6% after 5 years, and 31.7% after 10 years. Individual types of orbital exenteration allow patient-adapted therapies. The preservation of uninvolved orbital tissue facilitates orbital reconstruction. The type of exenteration did not have any influence on overall survival.

PurposeOrbital 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.MethodsWe 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.Results27 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.ConclusionsA 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.

Objective. The current paper presents an interesting case of facial reconstruction after the excision of a giant basal cell carcinoma located in the orbitofrontal region. Methods. Performing the excision while securing the appropriate oncologic safety margin has determined the appearance of a soft tissue defect that required a complex reconstruction using three regional flaps: frontal, temporal fascial and temporal muscle flaps. Results. After the excision and reconstruction in a single surgical stage, the postoperative result was favorable, the 12 months assessment showing that the patient was satisfied with the aesthetic aspect. Conclusion. Including the orbital exenterations in the excisional treatment of giant neoplasms located in the facial region requires a complex reconstructive plan. The surgical team has to consider the relief of the anatomical structures that are targeted, as well as the necessity of achieving satisfactory aesthetic results while ensuring oncological radicality.

Purpose To report our 14-year experience with orbital exenteration and assess risk factors for poor prognosis by focusing on conjunctival melanoma. Patients and method A retrospective study was conducted in our tertiary care centre (Jules Gonin Eye Hospital, Lausanne, Switzerland) between 2003 and 2017. Inclusion criteria were patients aged ≥18 years with a follow-up >12 months, without metastatic spread at the time of surgery. Data recorded were age, gender, tumour histology, surgical technique, postoperative complications, surgical margin status, local recurrence, postoperative radiation beam therapy and metastatic status. Results Twenty-five patients with a mean age of 63.2 years (38–92) were included. Conjunctival melanoma was the most frequently identified tumour (n = 14, 56%) followed by conjunctival squamous cell carcinoma (n = 4, 16%), sebaceous carcinoma (n = 3, 12%), choroidal melanoma (n = 2, 8%) and basal cell carcinoma (n = 2, 8%). Eighteen tumours (72%) originated from the conjunctival tissue. Clear surgical margins were achieved in 21 (84%) patients. Fourteen (56%) patients experienced distant metastases and died from metastatic spread after a mean follow-up of 52.3 months (6–120). The 1-, 3- and 5-year overall survival (OS) was 96%, 72% and 60%, respectively. In the univariate analysis, positive surgical margins, local recurrence and metachronous metastases were associated with a decreased OS (p = 0.002, p = 0.005 and p = 0.007, respectively). In the multivariate analysis, positive surgical margins and metachronous metastases were also associated with a decreased OS (p = 0.02 and p = 0.042, respectively). Conjunctival melanoma was not associated with a poorer prognosis (p = 0.280). Conclusion Free surgical margins are needed to increase OS. To achieve clearer surgical margins, neoadjuvant targeted therapies/immunotherapies may be considered.

Purpose To evaluate the surgical outcome and safety of acellular human dermal allograft as a new lining material to the exposed orbit after exenteration. Methods Retrospective case series of patients who underwent orbital exenteration followed by reconstruction with meshed-type acellular dermal allograft from 2009 to 2018 in a single tertiary institution. Results There were 14 eyes (2 right, 12 left) of 14 patients (6 men, 8 women). Mean age at operation was 69.1 ± 16.5 years. Indication for surgery was malignancy in all patients. One patient underwent subtotal exenteration, while the rest underwent total exenteration. SureDerm Meshed was used in 12 patients, AlloDerm Meshed in 1, and CGDerm Meshed in 1. Mean follow-up period was 12.1 months. Full or nearly full epithelialization occurred in 10 of 14 patients (71.4%) at 1 month and 9 of 12 patients (75.0%) at 3 months. There was delayed epithelialization in 3 patients due to poor wound care (n = 1), adjuvant radiotherapy (n = 1), and adjuvant radiotherapy followed by cerebrospinal fluid leak (n = 1). Conclusions Meshed acellular human dermal allograft showed good success in reconstruction after orbital exenteration and may be considered as an alternative lining material to split-thickness skin graft after orbital exenteration.

A 78-year-old Caucasian woman presented with pain in her right and only eye that was worse on abduction. Her history was significant for a choroidal melanoma affecting her left eye for which she underwent an orbital exenteration 12 years previously. Computed tomography and magnetic resonance imaging of the right orbit identified a mass lesion affecting the medial rectus, suspicious for metastatic melanoma. A histopathological diagnosis of metastatic melanoma was subsequently made following biopsy of the right medial rectus.

Background Studies of eyelid anatomy suggest an absence of supratarsal folds and a redundancy of retromuscular tissue in many Asians. Aggressive retromuscular tissue resection during senile blepharoplasty can lead to complications such as hematoma and extra fold formation. A thin fold is considered aesthetically pleasing, but upper eyelid hollowness is undesirable. Therefore, senile blepharoplasty allows relatively little room for retromuscular resection. Methods Between January 2016 and March 2019, blepharoplasty without a brow procedure was performed in 48 patients aged 55 years and older. In some patients with thin eyelids, the upper retromuscular tissue was explored, and the surgeon decided intraoperatively whether to perform resection. In total, 36 of 48 patients underwent retromuscular tissue resection, which was confined to the central and lateral aspects of the eyelid within 7–10 mm from the incision in the superior direction to create a crisp, thin fold without hollowness. The skin was closed, with the closure including the levator palpebrae superioris (levator) muscle, to create a weak, natural-looking, and dynamic fold instead of the buried dermo-levator suture typically created during double eyelid surgery. Results In the 72 eyelids that underwent resection, hematoma or extra fold formation was absent. In all cases, thin folds were achieved without an increase in the hollowness of the upper eyelid. Most patients were satisfied with their natural-looking postoperative appearance. Conclusions When indicated, limited retromuscular resection is recommended in cases of senile blepharoplasty. The resection should be confined to the central and the lateral aspects of the eyelid, within 7–10 mm in the superior direction from the incision, to create a crisp thin fold without hollowness.

Background Congenital orbital teratomas are extremely rare, usually benign neoplasms, comprised of cells originating from all three germ cell layers. Clinically the tumor appears solid, most of the times is intraconal and presents as a rapidly growing mass leading to a massive unilateral axial proptosis, chemosis, exposure keratopathy, markedly distended eyelids and often, loss of vision. To prevent these complications, tumor excision usually involves enucleation or even orbital exenteration. Case presentation We report a case of a 1-day old infant who presented with dramatic proptosis at birth due to a true congenital orbital teratoma. We describe the clinical findings, the preoperative neuroimaging, the surgical management which included complete tumor resection with preservation of the globe to allow for optimal orbital growth, the histopathological evaluation, and the clinical course during 18 years of follow up. Conclusion Every effort to salvage the globe should be made to achieve the best possible orbito-facial development. Furthermore, the value of prompt surgical management with a less invasive transconjunctival globe sparing procedure can be appreciated in our case.

The authors report the case of an 80-year-old male patient who had a modified left orbit exenteration with preservation of myocutaneus eyelid flap. The radical surgical treatment was performed due to high-grade recurrent pleomorphic conjunctival sarcoma with extension to the sclera and medial orbit. Five previous resection surgeries were performed, until the exenteration was considered. Prosthetic rehabilitation was planned integrating interdisciplinary biomedical knowledge, to create a new hybrid osteointegrated method with costume-made titanium miniplates and magnetic retention. During the second-year follow up, the system appeared osteointegrated. No failures from the retention mechanism have been reported. The patient reported to have a functional role inside and outside his house. Social interactions have not been limited by the anatomical facial defect. This new hybrid system is a feasible option to rehabilitate patients with orbital or facial defects, even for the elderly.

The conventional procedure of anterior orbital exenteration is unfavorable for eye socket reconstruction, whereas a modified procedure enables socket reconstruction and prosthesis fitting. Our study aims to compare the cosmetic outcomes between these 2 surgical techniques. We retrospectively recruited patients treated with modified or conventional exenteration during January 2015 to May 2021 in our hospital. The conventional approach was performed along with dermis-fat graft transplantation. The modified approach was conducted followed by eye socket reconstruction and eyelid blepharoplasty. The clinical data were collected and analyzed, including demographics, tumor characteristics, postoperative complications, tumor-related events, and cosmetic outcomes. Forty-nine patients were consecutively recruited in this study, including 22 cases of modified exenteration and 27 cases of conventional exenteration. Forty-four subjects (89.8%) were diagnosed with ocular surface malignancies (conjunctival melanoma and squamous cell carcinoma) and 5 subjects (10.2%) were diagnosed with extraocular stage of uveal melanoma. After follow-up for 31.8 ± 17.1 months, the 1-, 2-, 5-year overall survival rate was calculated as 100%, 79.2%, and 59.2% in the Modified group, and 94.2%, 73.8%, and 51.5% in the Conventional group. Comparison of the survival curves showed no significant differences. In the Modified group, all patients received orbital implant placement and eye socket reconstruction. The implant motility was satisfactory in 12 cases (54.5%) with movements in 3 to 4 directions. The eyelid function was acceptable in 17 cases (77.3%) with no entropion, ectropion or lower lid laxity. Ocular prosthesis was delivered in 17 cases (77.3%) with successful fitting in 11 cases (64.7%). The self-rated cosmetic score was statistically (t test, P

Purpose: Apocrine adenocarcinoma is a cutaneous adnexal malignancy which can rarely arise from ocular structures. In this retrospective study, we report our experience with four patients who had primary periocular apocrine adenocarcinoma initially presenting with an orbital tumor. Methods: Data extracted included demographics, clinical, imaging, and histopathological features, and treatment outcomes. Results: The definitive diagnosis was established after an incisional biopsy in all cases. Two patients were then managed with exenteration. The third patient underwent local resection followed by radiotherapy but had to be exenterated because of new tumor formation 7 years later. The fourth patient had to be managed with oral bicalutamide which kept the tumor stable for 3 years. Recurrence-free survival for the radical surgical treatment group was 10, 6, and 7 years respectively. Conclusion: Periocular apocrine adenocarcinoma may insidiously develop as an orbital mass without any clinically detectable primary eyelid skin or conjunctival lesions. This tumor must be in the differential diagnosis of medially located ill-defined orbital masses in patients over 50 years of age. Orbital exenteration appeared as an effective treatment of apocrine adenocarcinoma with orbital extension. Anti-androgenic treatment in an androgen receptor-positive tumor provided temporary local tumor control.