Periocular Desmoplastic Malignant Melanoma and Orbital Invasion With Uncontrollable Disease: A Case Report.

Primary orbital melanoma and metastatic cutaneous melanoma of the orbit are extremely rare. Desmoplastic melanoma (DM) is an infrequent variant of melanoma that can extend from a superficial location into deep tissues by neurotropic mechanisms. A 78-year-old male was referred to us with a periocular mixed malignant melanoma (spindle cell melanoma with desmoplastic reaction) in his left lower eyelid with uncontrollable disease (orbital and inferior orbital rim invasion) despite treatment. The surgical technique consisted of an extended orbital exenteration, maxillectomy, and ethmoidectomy, with a 2 cm macroscopic surgical margin. We performed a delayed socket reconstruction with a temporalis muscle flap using a transorbital approach. The patient remained disease-free for 1.5 years with a good quality of life since exenteration surgery. At this time, he presented a recurrence in the area of the malar scar with a new orbital invasion, and finally, he died due to mediastinal, pleural, and pulmonary metastasis. The treatment of a cutaneous melanoma arising in the periocular region is a challenging reconstructive problem and it may compromise the globe and visual function.

Eyelid Eccrine Poroma: A Case Report and Literature Review.

Eccrine poroma is a rare benign adnexal tumor arising from intradermal cells of eccrine sweat ducts. At least two-thirds of eccrine poromas present on the extremities, most commonly on the palms and soles. They are scarcely found on the face; to date, only 11 cases of eyelid poromas have been reported in PubMed. Biopsy excision with a free margin is necessary to distinguish it from malignant lesions and avoid recurrence with possible transformation to porocarcinoma. We present the case of a 23-year-old male with a histopathological confirmation of poroma using staining with hematoxylin-eosin on the eyelid, previously clinically diagnosed with molluscum contagiosum. After four years, he has not experienced a recurrence.

Superior orbital fissure syndrome after deep lateral orbital wall decompression in Graves' ophthalmopathy.

PURPOSE: The superior orbital fissure contains cranial nerves III, IV, VI, and V1 with their three branches: frontal, lacrimal, and nasociliary. Superior orbital fissure syndrome (SOFS) is rare and can occur as a result of compression of these nerves due to trauma, bleeding, or inflammation in the retrobulbar space, but no cases of SOFS after deep lateral orbital wall decompression (DLOWD) have been reported. The aim of this paper is to describe this pathology, its possible causes, management, and outcome. METHODS: Retrospective study of 575 DLOWD in patients with disfiguring exophthalmos due to Graves' ophthalmopathy performed in our hospital between 2010 and 2023. Three cases of postoperative SOFS were identified based on clinical presentation, history, physical examination, and radiological study. All patients were observed for a minimum of 12 months. RESULTS: SOFS was diagnosed with the presence of ophthalmoplegia, ptosis, fixed and dilated pupils, hypo/anesthesia of the upper eyelid and forehead, loss of corneal reflex, and no loss of vision after DLOWD. Fractures, edema, and hemorrhages were excluded. They were treated with high-dose intravenous steroids and the patients recovered completely. CONCLUSIONS: DLOWD challenges orbital surgeons because it requires removing bones near the globe or neurovascular structures. SOFS may occur due to the proximity and increased pressure on these structures.

Advanced Periocular Basal Cell Carcinoma with Orbital Invasion: Update on Management and Treatment Advances.

Purpose: Basal cell carcinoma (BCC) is the most frequent malignant periocular tumor. It is associated with exposure to ultraviolet radiation, and its incidence is gradually increasing. It may occasionally display more aggressive behavior and result in orbital or intracranial invasion. Mortality from periocular BBC with orbital invasion is very low, but the associated morbidity can be significant, from disfigurement to blindness. Traditionally, these cases have been treated with orbital exenteration or with radiotherapy (RT), but in recent years, hedgehog pathway inhibitors (HPIs) have emerged, are effective in more serious cases, and are used primarily or combined with surgery, changing our perspective on the management of these patients. Methods: We studied 24 cases of periocular BCC with orbital invasion, some primary and others recurrent, which were treated between 2011 and 2021 in the same hospital. All patients had clinical or radiological evidence of orbital invasion. Orbital exenteration was performed on 9/24 of the patients (1 received vismodegib after surgery), and 12/24 were treated, surgically preserving the eyeball, with 3 of them receiving adjuvant vismodegib. Three of the twenty-four patients were treated exclusively with vismodegib (Erivedge®, Genentech). Results: One patient died due to poor tumor evolution, but the rest evolved favorably and they have had no recurrences. Vismodegib was generally well tolerated, except for in one patient who discontinued treatment due to the side effects. Conclusions: In advanced BBC with orbital invasion, mutilating surgical treatments such as exenteration or potentially vision-threatening treatments such as RT remain as options. In recent years, however, very promising new medical therapies have emerged, such as HPI, which can be used effectively instead of surgery or in combination with it, preserving the eye and vision, which implies a new approach to treatment.

Surgical removal of orbital tumors by orbital approach using ultrasonic surgical system SONOPET®.

PURPOSE: Orbital tumor surgery can be challenging when the tumor is located in a very narrow surgical field and close to important structures, such as nerves or extraocular muscles that can be damaged during surgery. Advances in technology and special surgical techniques help to avoid such damage. We describe our experience using SONOPET® ultrasonic surgical aspirator to remove 12 different orbital tumors that were difficult to treat due to their poorly defined borders, adhesions, or location. METHODS: This is a retrospective case series that describes 12 operations that occurred between March 2016 to December 2018 using an ultrasonic device to debulk or remove orbital tumors. Different approaches and handpieces were used for each case depending on the location and consistency of the tumor. RESULTS: All patients experienced an improvement in preoperative signs and symptoms, pain, proptosis, diplopia, or lagophthalmos. Visual acuity, which had been reduced due to the tumor, was also recovered. There were no intraoperative or postoperative complications due to the use of the device. CONCLUSIONS: The ultrasonic aspirator is a safe, useful device that can successfully remove or debulk infiltrating orbital masses through any orbital access, regardless of their consistency. It is helpful in cases of difficult anatomical access or difficult extraction due to size or adhesions to the surrounding tissues.

Orbital Solitary Fibrous Tumor: Four Case Reports-Clinical and Histopathological Features.

PURPOSE: To retrospectively describe the clinical characteristics, management, and outcomes of four cases of orbital solitary fibrous tumor (SFT). In one patient, we present an ultrasonic aspirator system for tumor removal. METHODS: Four patients with orbital SFT were selected: one patient with orbital SFT, another patient with frontal and ethmoidal SFT and orbital affectation with high rates of recurrence, the third patient with frontal lobe SFT and orbital invasion with multiple recurrences, and the fourth case with a history of craniopharyngioma surgery and SFT located on the orbital apex. RESULTS: All cases showed proptosis, eye movement restriction, and, in three cases, visual acuity alteration. Different treatments were applied: in three cases, excision was performed, one of them with an ultrasonic aspirator system, and in the remaining case, an exenteration was done (in two cases, radiosurgery treatment was also applied). The immunohistochemical study revealed SFT, similar to hemangiopericytomas (HPCs). No recurrence has been observed after surgical treatment. CONCLUSION: The SFT is a spectrum of different tumors with similar histopathological characteristics. The use of immunohistochemical markers is very helpful in the diagnosis. The main problem of orbital involvement is the risk of damaging important structures adjacent to the tumor during the surgical removal. The ultrasonic aspirator system allows elimination of the tumor without damaging other orbital structures.

Localized Bilateral Superior and Inferior Orbital Neurofibroma in the Absence of Neurofibromatosis.

Localized or isolated neurofibromas are peripheral nerve sheath tumors. They are rare in the orbit and occur without a systemic neurofibromatosis. There are few cases of bilateral tumors reported but none affecting both supraorbital and infraorbital nerves. We report a 45-year-old female who presented an extraconal mass in the right orbit as an incidental finding in a head computer tomography, without ocular symptoms. Magnetic resonance image showed a well-defined oval mass in the right supraorbital and infraorbital nerves, of similar characteristics, as well as smaller masses in the left supraorbital and infraorbital nerves. A progressive increase in size of the left supraorbital and infraorbital tumor motivated their surgical excision. The histological result was compatible with a neurofibroma. These uncommon orbital tumors are slow growing and affect the sensory nerves of the trigeminal nerve. Neurofibromas usually present progressive symptoms due to the orbital mass, proptosis, or visual changes although not in this case. Surgical removal is the only definitive treatment.

Giant Cell Reparative Granuloma of the Orbit: Clinicopathological Characteristics and Treatment.

Giant cell reparative granuloma (GCRG) is a rare fibroosseous lesion uncommonly seen in the orbital area. Although benign, it is known to be recurrent and locally destructive. We report two cases of GCRG of the orbit. In both cases, computed tomography revealed a heterogeneously growing well-defined mass, arising from the roof of the orbit, affecting the cortex, and invading the orbit. In the first case, the mass extended into the anterior cranial fossa. Magnetic resonance imaging with gadolinium showed, in both cases, a cystic character of the lesion with fluid levels. The surgical treatment was performed via an upper crease incision. An ultrasonic aspirator system was used to remove the tumor tissue and its extension into cranial fossa. Careful histopathologic analysis established the diagnosis of GCRG. Symptoms resolved completely with no evidence of recurrence after a follow-up of 18 and 14 months, respectively. We present the clinicopathological and radiological findings, and we describe the surgical approach. As a rare entity, GCRG of the orbit should be considered in differential diagnosis of fibroosseous orbital masses. Complete surgical excision carries a low risk of recurrence.

Orbital-Conjunctival-Eyelid Hemorrhage-Hematoma due to Inappropriate use of FFP2/NK95 Facial Mask in COVID-19.

A non-traumatic or spontaneous orbital hemorrhage (NTOH) is usually caused by the presence of an orbital mass, an inflammation, an infection, a bleeding disorder or those called idiopathic. This entity usually affects elderly adults and some risk factors can be identified. The NTOH normally acts like a benign and self-limited process, but attending to its anatomical pattern, may need specific management. A 64-year-old male referred to us with sudden binocular double vision, without loss of visual acuity (VA) or pain immediately after fitting the FFP2/NK95 facial mask for air protection during the pandemic COVID-19. He presented sudden orbital-subconjunctival-eyelid cutaneous hemorrhage-hematoma with conjunctival protrusion from the palpebral fissure without proptosis. There was also limitation in adduction and a cutaneous hematoma in the inner third of the lower eyelid. After 48 hours, the diplopia had resolved and ocular motility was completely re-established with persistence of a massive hyposphagma. No radiological image test was performed due to the COVID-19 epidemiological situation, as the patient was in good systemic situation and it was not a vital emergency. The evolution was favorable with conservative treatment, cold application, and moisturizing eye drops. After 10 days, the bruising was almost completely gone. During the ongoing novel coronavirus disease (COVID-19) pandemic caused by the novel enveloped RNA virus named severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), face mask use has drastically increased among the healthcare professionals and the general population. The importance of this case lies in the new adverse effects caused by the misuse of mandatory face masks in the general population.

Eyelid Eccrine Poroma: A Case Report and Review of Literature.

Poroma is a rare benign tumor of the epidermal sweat duct unit with predilection for the head and neck. Only six cases with eyelid location have been described in the literature (PubMed). A 34-year-old male presented with a single tumor on the left upper eyelid. It was skin-colored, nodular, solid, tender with some telangiectatic vessels, and showed no ulcerated lesion. Clinical diagnosis was basal cell carcinoma. This type of lesion can mimic a malignancy. Complete excisional biopsy revealed features consistent with eccrine poroma. After three year of follow up, no recurrence was observed. The authors reviewed all the cases reported in the literature and made a summary comparing them.

Deep lateral rim-sparing orbital wall decompression in spontaneous globe subluxation associated with shallow orbits and eyelids laxity.

Purpose: To share our experience on deep lateral wall rim-sparing orbital decompression for the prevention of further spontaneous globe subluxation, in patients with shallow orbits and eyelid laxity.Methods: This is a retrospective, interventional case series review. We report the results of deep lateral wall rim-sparing orbital decompression in 7 patients with recurrent spontaneous globe subluxation, operated in our department between 2010 and 2016. The orbital morphology was established by computed tomography scan images, and all patients with shallow orbit configuration and who in addition had eyelid laxity were included. Patients with thyroid eye disease were excluded.Results: No significant intraoperative and postoperative complications were encountered. In all cases, the patients were satisfied with the aesthetic result and none reported further episodes of globe subluxation.Conclusions: Deep lateral wall rim-sparing orbital decompression is a safe and effective decompressive procedure associated with minimal complications, which can be performed successfully in patients with spontaneous globe subluxation associated with shallow orbits with enough eyelid laxity.

Deep Lateral Wall Partial Rim-Sparing Orbital Decompression with Ultrasonic Bone Removal for Treatment of Thyroid-Related Orbitopathy.

PURPOSE: To describe the results of thyroid-related orbitopathy (TRO) treated by ultrasonic deep lateral wall bony decompression with partial rim sparing (DLW-PRS). METHODS: A review was carried out, from January 2015 to September 2017, of all patients treated with ultrasonic DLW-PRS decompression using a SONOPET® (Stryker, Kalamazoo, MI, USA) ultrasonic aspirator, using a lateral, small triangle flap incision for access. The primary outcome was the change in proptosis (measured by the difference in Hertel exophthalmometry measurements). Other secondary outcomes were changes in visual acuity (VA) (using Snellen scale, decimal fraction), presence of lagophthalmos, eyelid retraction (measured by upper eyelid margin distance to the corneal reflex (MRD1) and lower eyelid margin distance to the corneal reflex (MRD2), and presence of exposure keratopathy). RESULTS: A total of 58 orbital decompressions in 35 patients were reviewed, with 23 patients (65.7%) having bilateral decompressions. There was a female preponderance with 26 patients (74.2%), and the mean age ± standard deviation was 52.6 ± 13.9 years. Mean proptosis was 24.51 ± 1.76 mm preoperatively, reduced to 19.61 ± 1.27 mm in final follow-up. The mean reduction was 4.9 ± 1.54 mm. VA improved from 0.8 ± 0.14 to 0.9 ± 0.12, p=0.039. 5 of 13 patients (38.4%) with preoperative diplopia reported improvement or complete resolution after surgery. MRD1 was reduced from 5.25 ± 0.88 mm to 4.49 ± 0.7 mm. MRD2 was also reduced from 6.3 ± 0.88 mm to 5.0 ± 0.17 mm. Presence of lagophthalmos was reduced from 35 eyes (60.3%) to five (8.6%); the presence of epiphora was also reduced from 20 patients (57.1%) to 3 (8.5%) following decompression. Complications of the surgery included zygomatic hypoaesthesia in 14 (40%) patients in the early postoperative period and chewing alterations in 10 (28.5%) of the patients. All of these complications were resolved at the 6-month follow-up visit. We noted no surgical complications such as ocular or soft tissue damage, infection, inflammation, or visual loss. CONCLUSIONS: The SONOPET® ultrasonic bone curette can be used safely and effectively for DLW orbital decompression surgery. The main benefits were good visualization and handling of tissues and speed and ease of use of the equipment. This trial is registered with ClinicalTrials.gov identifier: NCT04025034.

Rhino-Orbito-Cerebral Mucormycosis: Two Cases with Amaurosis as Presentation, Medical Surgical Management and Follow-Up.

Purpose: Mucormycosis is an infection caused by fungi to the class Zygomycetes that usually appears in immunosuppressed patients. Diagnostic confirmation is often delayed, with fatal prognosis in cases in which treatment is not rapidly established. Case report: We present two clinical cases of rhino-orbito-cerebral mucormycosis with an atypical presentation form, consisting of a unilateral complete sudden vision loss. Intravenous treatment with liposomal amphotericin B was started and total orbital exenteration surgery was performed. The removed surgical area was filled with gauze impregnated with liposomal amphotericin B and was left open for cures every 12 hours. Due to the good clinical evolution, a reconstruction of the orbital exenteration defect was performed in Case 1 with a temporal muscle flap and a skin island pedicled flap. In Case 2, reconstruction was not performed due to the poor evolution of the patient. Discussion: As it is a very aggressive surgery, the aesthetic and functional sequelae are very important. When the survival of the patient is achieved, we should offer reconstructive solutions that improve their quality of life. The reconstruction carried out using a flap of the temporal muscle can be made in a single act without requiring microvascular surgery.

Reconstruction of Upper Eyelid Defects Secondary to Malignant Tumors with a Newly Modified Cutler-Beard Technique with Tarsoconjunctival Graft.

PURPOSE: We describe a modification of Cutler-Beard's technique, using a contralateral tarsoconjunctival graft, in patients who underwent excision of large malignant tumors of the upper eyelid. METHODS: This is an interventional retrospective review (clinical study) of ten cases, with age range from 64 to 86 years (mean: 71.2 years ± 6.49) with malignant upper eyelid tumors, operated by the same surgeon (AB-G), between 2011 and 2016. The resulting defects were reconstructed using a modification of Cutler-Beard's technique. A tarsoconjunctival graft was harvested from the contralateral upper eyelid, with an extra 3 mm of conjunctiva from the superior edge of tarsus and was attached to the upper eyelid defect, different from that previously described. Follow-up ranged between 24 and 60 months (mean: 41.6 ± 9.87). Functional, cosmetic outcomes and postoperative complications were evaluated. RESULTS: No upper eyelid retraction, eyelid margin entropion, or graft retraction was observed in any of the cases. All of the patients were satisfied with the aesthetic result. CONCLUSION: This technique allows us to safely inset a suitable graft on the ocular surface, with sufficient blood supply, resulting in a stable margin and good contour.

Occult Orbital Injury with Dagger Fragment with Resulting Pneumocephalus.

Penetrating injuries of the cranium are relatively uncommon, only 0.4% of all head injuries. In patients with disturbed conscious level, an extensive examination should be performed in the emergency unit to rule out transorbital penetrating brain injury. A 25-year-old male was attacked with a dagger. He presented with ethylic intoxication and the physical examination demonstrated a small skin injury on the lateral canthus of the left eye with a large periocular hematoma which prevented eyelid opening. Cranial CT scan showed a metallic intraorbital foreign body consisting of a fragment of a dagger which perforated the eyeball, and penetrated through the superomedial wall of the orbit into the anterior cranial fossa. Reconstruction of the eyeball was performed and the fragment was removed. Orbital injuries with a knife in situ are very unusual. Early identification and removal of retained foreign bodies are essential.

Nasal Cavity or Alveolar Paranasal Sinus Rhabdomyosarcoma with Orbital Extension in Adults: 2 Cases.

Alveolar rhabdomyosarcoma (RMS) has a predilection for the deep soft tissues of the extremities and mainly occurs in children. Although the tumor may originate in other sites, such as the nasal cavity or paranasal sinus, invasion of the orbit is unusual. We describe the clinicopathological features of 2 cases of alveolar RMS of the nasal cavity or paranasal sinus in adult patients with orbital extension. These cases of alveolar RMS of the nasal cavity or paranasal sinuses are described in 2 men, both in the third decade of life. These patients were evaluated with radiological studies. The histological diagnosis was confirmed by immunohistochemical methods. Treatment consisted in a combination of chemotherapy and radiation therapy following excisional biopsy. Alveolar subtype RMS is an extremely aggressive neoplasm that rarely presents in the orbit or paranasal sinuses of adults but should be considered in the differential diagnosis of tumors with this localization. Myoglobin, Myo D1, and myogenin seem to be the most specific markers for RMS.

A Second New Choroidal Osteoma in the Same Eye: Differences between Them with New Imaging Techniques.

The authors introduce a 42-year-old woman with a choroidal osteoma. After 10 years the patient presented a second choroidal osteoma in the same eye; this osteoma has been growing in the last years. New tests that were unavailable during the first diagnosis were performed such as Fundus Autofluorescence or Enhanced Depth Imaging-Optical Coherence Tomography (EDI-OCT). These tests show characteristics of the tumors and allow us to realize a visual prognosis for the patient.

The use of autologous dermis grafts for the reconstruction of the anophthalmic socket.

PURPOSE: The main objectives of enucleation, evisceration or secondary orbital implants are to replace orbital volume and obtain good motility and adaptation of the implant and the external prosthesis. We describe our experience using autologous dermis graft sutured to Tenon and conjunctiva following evisceration, enucleation and any reconstruction requiring either a primary or secondary orbital implant, even those with large tissue loss. MATERIALS AND METHODS: A retrospective case series of patients who received an autologous dermis graft to assist in closure of a Tenon's capsule and conjunctiva at the time of placement of secondary orbital implants or evisceration or enucleation with fornices retraction or tension in tissues. We also describe how and from where to take the dermis graft. RESULTS: 72 patients were included and all received orbital porous polyethylene implants: 28 patients had secondary orbital implants, 36 patients had evisceration and 8 patients had enucleation. Implant size was 20 mm in most cases, but went to 22 mm. Follow-up ranged from 3 to 36 months. No intraoperative complications were observed in donor or receptor area. 8 cases had incipient ischemia of the dermis graft, but were treated and resolved with autologous serum. CONCLUSIONS: Implant exposure is due to bad surgical technique, an inadequate implant size or excessive tension on the suture. Dermis autologous graft allows moving the orbital implant anteriorly because it replaces surface to suture Tenon and conjunctiva without tension, so a good adaptation of a thinner external prosthesis is possible, resulting in better motility.