Congenital orbital teratoma: a rare case with intracranial extension.

INTRODUCTION: Teratoma is the most common congenital tumor, but the orbital location is rare. It is composed of tissues from ectoderm, mesoderm, and endoderm. CLINICAL PRESENTATION: Congenital orbital teratoma commonly presents as unilateral proptosis, with rapid growth, leading to exposure keratopathy. DIAGNOSIS: Prenatal ultrasound may detect the orbital mass, computed tomography (CT) scans, and magnetic resonance (MR) imaging are better in demonstrating multilocular cystic and solid mass, without bone erosion. Laboratory tests should include alfa-fetoprotein (AFP) and B-human chorionic gonadotropin (B-HCG), and histopathologically, it contains all three germ cell layers components. The management is surgical removal of the lesion, the mature teratoma has a benign behavior, and the immature has a poor prognostic. We describe a rare case of congenital orbital teratoma with intracranial extension of the lesion, in which was treated with orbital exenteration. After surgery, AFP levels decreased, the middle face displacement has improved and development milestones were appropriate.

Orbital preservation in the treatment of acute invasive fungal sinusitis.

Acute invasive fungal sinusitis (AIFS) is an aggressive disease with significant mortality and morbidity. Surgical debridement is a mainstay of treatment. However, orbital involvement may limit its efficacy and is an independent risk factor for mortality. Traditionally, orbital exenteration has been utilized in cases with orbital invasion and ophthalmoplegia or vision loss. Retrobulbar liposomal amphotericin B injection may improve disease control and has the potential to spare the morbidity associated with exenteration. In this video article, we document the use of serial endonasal debridement with retrobulbar injections to salvage the eye in a patient with significant orbital involvement. A 28-year-old immunocompromised female patient presented with acute onset restricted right extraocular movement, progressive orbital pain, V2 trigeminal numbness, and 20/40 vision. The patient underwent recurrent debridement and retrobulbar injections of liposomal amphotericin B. Her serial exams, including changes in extraocular muscle appearance and gradual improvement in extraocular movement, were documented. The exam six months after initial presentation demonstrated 20/20 vision, minimal extraocular movement restriction, and proper healing of the orbit and ethmoid. The salvage of the patient's orbit suggests that liposomal amphotericin B injections with debridement may be a viable treatment alternative in patients with acute invasive fungal sinusitis and orbital involvement.

Preservation of orbit in tumor invasion through the periorbita in sinonasal malignancy.

OBJECTIVE: One of the possible risks of sinonasal malignancy is its possible spread in the orbit. However, there is no clear consensus among the different departments as to whether it is necessary to exenterate the orbit in limited tumorous infiltration of periorbital fat. The purpose of the study was to demonstrate that periorbital infiltration and periorbital fat invasion without involvement of deeper orbital tissues are not the indication of orbital exenteration. MATERIALS AND METHODS: Retrospective analysis was performed over a 17-year period of patients undergoing surgical treatment for sinonasal malignancy with histologically verified periorbital infiltration or deeper invasion into the orbit. A total of 32 patients were included in the study. For each group, the following data were analysed: sex, age, preoperative imaging studies, histological findings, site of origin, stage, surgical reconstruction, oncological treatment, survival, cause of death, number of recurrences in the orbit and functional status of preserved eyes. RESULTS: Based on our criteria for orbital exenteration, orbital preservation was feasible in 18 patients. Orbital exenteration was performed in 14 patients with deeper tumor infiltration. There was a statistically insignificant difference in survival between the two groups. The 5-year overall survival (OS) was 44% for the orbital preservation group (only 2 patients died from local tumor recurrence) and 34% for the orbital exenteration group. The groups did not differ in other observed factors other than the extent of orbital infiltration. In 11 (61.1%) patients, vision was without significant change after radiation therapy. In 2 (11.1%) patients, visual function was impaired due to diplopia. 5 (27.8%) patients had severely impaired vision due to optic nerve atrophy after radiation therapy. CONCLUSIONS: Our results show a relatively high survival rate in the group of patients with orbital preservation with a high chance of vision preservation, which justifies our approach to orbital preservation even in some tumors with periorbital infiltration.

Alternating pattern of rhino-orbital-cerebral mucormycosis with COVID-19 in diabetic patients.

PURPOSE: Our aim was to analyze the patients with diabetic rhino-orbital-cerebral mucormycosis that we have treated in our clinic in the last 5 years, and to reveal the altering conditions with COVID-19. MATERIALS AND METHODS: A retrospective study was conducted on 39 rhino-orbital-cerebral mucormycosis (ROCM) patients with diabetes mellitus between 2017 and 2022. The patients were divided into two groups as those associated with and not associated with COVID-19 and compared. RESULTS: Thirty-nine diabetic patients were included in the study, with 15 (38.5%) of them being COVID-19 associated mucormycosis (CAM) group. CAM patients showed higher orbital involvement and cavernous sinus involvement (p = 0.002 and p = 0.013, respectively). The mortality rate in the non-COVID-19 associated mucormycosis (non-CAM) group was statistically higher in patients with intracranial and cavernous sinus involvement (p = 0.015 and p = 0.033, respectively). The difference between the overall survival of the CAM patients and non-CAM patients was not statistically significant (p = 0.741). CONCLUSIONS: With COVID-19, progressive mucormycosis accompanied by orbital and intracranial involvement is observed more frequently. However, on the contrary, the mortality rate in COVID-19-associated mucormycosis is considerably lower than expected. The fact that temporary immune suppression can be ameliorated with adequate supportive treatment and liposomal amphotericin-B which can be given to patients in high doses may explain this situation. It has been attained hints that the essential factor in the treatment of COVID-19-associated ROCM is the control of the underlying disease and to be cautious in the decision of early aggressive surgery.

Rhino-orbital mucormycosis in a patient with no susceptibility following P.vivax malaria infection-a case report.

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

An orbital calcific cyst following exenteration.

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.

Outcomes of orbital exenteration for craniofacial lesions.

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).

Efficacy and safety of an artificial dermal graft for the reconstruction of exenterated sockets: a preliminary report.

PURPOSE: The purpose of this study is to report our experience with the use of artificial dermis grafts for orbital socket reconstruction following orbital exenteration (OE). METHOD: A retrospective study was conducted in our ocular oncology centre from May 2018 to June 2020 in patients undergoing OE for orbital malignancies in whom an artificial dermis device (Integra® template, 2 layers) was used for reconstruction. Data recorded included demographics, previous and adjuvant treatments, aetiologies, surgical procedure, surgical reconstruction, complications and follow-up. The main outcome measure was the time between OE and the full granulation of the cavity. RESULTS: Ten patients (mean age, 71.3 years [43-92]) were included. Tumours originated from the conjunctiva (n = 5, 50%), eyelid (n = 3, 30%) and orbit (n = 2, 20%). Nine patients underwent total OE, and one required enlarged OE. Orbital reconstruction was performed using an artificial dermis alone (n = 9, 90%) or combined with regional flaps (n = 1, 10%). The mean granulation time was 3.3 weeks (2-4). Three (30%) patients received adjuvant radiotherapy 1 month post-surgery. The mean time to spontaneous epithelialization was 9.4 weeks (6-12). Preoperative and postoperative radiotherapy was not associated with a delayed epithelialization of the socket (p = 0.463 and p = 0.236, respectively). One (10%) and 2 (20%) patients experienced postoperative socket infection and an ethmoidal fistula, respectively. The mean follow-up was 11.6 months (6-16). CONCLUSION: Using artificial dermis grafts alone or with regional flaps appears to be a viable surgical procedure for orbital socket reconstruction. They reduce surgical morbidity and hospital stay. Preoperative and postoperative radiotherapy does not seem to delay socket healing.

Factors related to survival outcomes following orbital exenteration: a retrospective, comparative, case series.

BACKGROUND: Orbital exenteration is a disfiguring procedure that aims to achieve local control. It is commonly a part of the management of malignant orbital tumor which is a life-threatening condition. It is necessary to determine predictive factors associated with overall survival (OS) following orbital exenteration. METHODS: This was a retrospective, comparative, case series of 39 patients with malignant tumors who underwent orbital exenteration. Patient records were reviewed for age, clinical presentation, preoperative visual acuity (VA), tumor size, surgical margin, tumor invasiveness, recurrent disease, and status of distant metastasis. Kaplan-Meier curves were used to assess OS and event-free survival (EFS). The predictive factors related to OS were identified using multivariate analysis. RESULTS: The mean age was 62.9 years (range, 5.5 to 89.7 years), 68.4% presented with VA < 20/400. The mean size of all tumors was 32 ± 18 mm. Distant metastasis at diagnosis was reported in 11 patients (28.2%). Twenty-two patients died during follow-up. The median OS and EFS were 3.89 years and 3.01 years, respectively. The predictive factors for worse OS on multivariate analysis were preoperative VA < 20/400 (adjusted hazard ratio [aHR] 4.67, P = 0.003), tumor size larger than 20 mm (aHR 3.14, P = 0.022,) and positive distant metastasis at diagnosis (aHR 15.31, P <  0.001). CONCLUSIONS: The prognostic factors for poor survival outcome following orbital exenteration were a preoperative VA < 20/400, tumor size > 20 mm, and distant metastasis at diagnosis mostly due to patient negligence.

Two-stage rapid exenteration reconstruction to allow early radiation therapy for an aggressive orbital cancer.

PURPOSE: Describe a novel two-stage orbital exenteration technique using an INTEGRA dermal regeneration matrix. METHODS: A 63-year-old Hispanic male presented with multiple invasive right eyelid masses that incisional biopsy revealed was infiltrative basal cell carcinoma. The patient underwent a right orbital exenteration without lid sparing. An INTEGRA graft was sutured in place to cover the defect at the time of surgery and allowed to vascularize for 3 weeks. During this time, frozen section of tumor margins previously read as negative were found to have invasive basal cell carcinoma on permanent section re-evaluation. Three weeks after the initial exenteration, the patient returned to the operating room and the dermal matrix of the INTEGRA graft was found to be well integrated and vascularized. Further resection was performed in the areas which were found to have residual cancer on permanent section evaluation. After preliminary frozen section pathology demonstrated clear margins, full-thickness skin grafts harvested from the right and left supraclavicular regions were thinned, draped, and fixated over the INTEGRA matrix. RESULTS: The patient recovered well and experienced no immediate postoperative complications. Adjuvant radiotherapy began 5 weeks after initial exenteration with a fully epithelized exenterated socket. At postoperative week 16, our patient remained with full epithelization after completing radiation. As of postoperative week 47, our patient has had no complications. CONCLUSION: The use of INTEGRA with full-thickness skin grafting for orbital exenteration reconstruction presents several advantages over traditional reconstruction approaches including: quicker recovery, tumor surveillance by re-examining edges of the resection after INTEGRA dermal placement, easier postoperative care, and earlier initiation of radiation therapy.

Invasive Fungal Sinusitis Presenting as Acute Posterior Ischemic Optic Neuropathy.

Invasive fungal sinusitis causes painful orbital apex syndrome with ophthalmoplegia and visual loss; the mechanism is unclear. We report an immunocompromised patient with invasive fungal sinusitis in whom the visual loss was due to posterior ischaemic optic neuropathy, shown on diffusion-weighted MRI, presumably from fungal invasion of small meningeal-based arteries at the orbital apex. After intensive antifungal drugs, orbital exenteration and immune reconstitution, the patient survived, but we were uncertain if the exenteration helped. We suggest that evidence of acute posterior ischaemic optic neuropathy should be a contra-indication to the need for orbital exenteration in invasive fungal sinusitis.

Single-stage socket reconstruction with vascularised temporalis muscle flap following total orbital exenteration: Description of 3 surgical approaches.

We describe the techniques and outcome of three different approaches to transfer the posterior 2/3rd 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.

State-of-the-art reconstruction of midface and facial deformities.

Oncologic resection involving the midface results in potentially disfiguring and challenging defects for the reconstructive surgeon. As with any defect, restoration of form and function is critical, but is particularly difficult in the midface, which is paramount to a patient's physical appearance and identify, as well as the patient's ability to breath, speak, see, and eat. Here, we present an algorithmic approach for optimizing outcomes in midfacial reconstruction utilizing the state-of-the-art reconstructive techniques. J. Surg. Oncol. 2016;113:962-970. © 2016 Wiley Periodicals, Inc.

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.

The implications of orbital invasion in sinonasal tract malignancies.

In recent decades, the management of sinonasal tumors abutting the orbit has been widely discussed. A real guideline has yet to be proposed, as prospective randomized studies on this topic are very difficult to organize, given the relative rarity of this pathology, the wide spectrum of histologic patterns, and the different clinical behavior of tumors. Nevertheless, in recent years, a better assessment of tumor extension has been obtained thanks to the refinement of preoperative imaging tools and, therefore, more conservative approaches could be adopted, with no worsening of the oncological outcomes and, at the same time, with more attention given to the post-surgical quality of life. Currently, tumors that extend to the bony orbital walls with or without focal infiltration of the periorbit are amenable to orbital preservation. On the other hand, infiltration of extraocular muscles and neurovascular structures are an indication to orbital exenteration. The ideal surgical treatment in cases of limited involvement of orbital fat still remains a matter of debate. We report and discuss the recent English literature on this interesting topic.

Rehabilitation of orbital cavity after orbital exenteration using polymethyl methacrylate orbital prosthesis.

Squamous cell carcinoma of the eyelid is the second most common malignant neoplasm of the eye with the incidence of 0.09 and 2.42 cases/100 000 people. Orbital invasion is a rare complication but, if recognized early, can be treated effectively with exenteration. Although with advancements in technology such as computer-aided design and computer-aided manufacturing, material science, and retentive methods like implants, orbital prosthesis with stock ocular prosthesis made of methyl methacrylate retained by anatomic undercuts is quiet effective and should not be overlooked and forgotten. This clinical report describes prosthetic rehabilitation of two male patients with polymethyl methacrylate resin orbital prosthesis after orbital exenteration, for squamous cell carcinoma of the upper eyelid. The orbital prosthesis was sufficiently retained by hard and soft tissue undercuts without any complications. The patients using the prosthesis are quite satisfied with the cosmetic results and felt comfortable attending the social events.

Reliability and Effectiveness of the Manchester Orbital Exenteration Wound Assessment Tool: Inter-Observer Agreement.

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.

Rapid fabrication of silicone orbital prosthesis using conventional methods.

Restoration of orbital defects with silicone prosthesis has been a well-documented and accepted treatment option. Adhesive retained prosthesis offer the patients with adequate retention and treatment satisfaction. However, marginal breakdown and discoloration are common problems associated with these prostheses, necessitating their refabrication. Fabrication of a silicone orbital prosthesis is time consuming and requires multiple clinical and laboratory procedures. This technical article describes simple and cost effective steps for rapid fabrication of a silicone prosthesis using conventional methods.

Maxillary reconstruction: Current concepts and controversies.

Maxillary reconstruction is still an evolving art when compared to the reconstruction of the mandible. The defects of maxilla apart from affecting the functions of the speech, swallowing and mastication also cause cosmetic disfigurement. Rehabilitation of the form and function in patients with maxillary defects is either by using an obturator prosthesis or by a surgical reconstruction. Literature is abundant with a variety of reconstructive methods. The classification systems are also varied, with no universal acceptance of any one of them. The oncologic safety of these procedures is still debated, and conclusive evidence in this regard has not emerged yet. Management of the orbit is also not yet addressed properly. Tissue engineering, that has been hyped to be one of the possible solutions for this vexing reconstructive problem, has not come out with reliable and reproducible results so far. This review article discusses the rationale and oncological safety of the reconstructing the maxillary defects, critically analyzes the classification systems, offers the different reconstructive methods and touches upon the controversies in this subject. The management of the retained and exenterated orbit associated with maxillectomy is reviewed. The surgical morbidity, complications and the recent advances in this field are also looked into. An algorithm, based on our experience, is presented.