Leiomioma orbitario gigante en un paciente pediátrico: reto diagnóstico y terapéutico / Giant orbital leiomyoma in a pediatric patient: Diagnostic and therapeutic challenge

Este manuscrito presenta un caso de un leiomioma orbitario de larga evolución en un joven de 14años. El tumor era inusualmente grande y causaba una proptosis severa y una afectación importante de la musculatura ocular. El paciente presentaba amaurosis, una oftalmoplejía completa, dolor ocular espontáneo e incapacidad para cerrar los párpados. Debido al tamaño del tumor y a su progresión, se realizó una exenteración orbitaria derecha para eliminar todo el contenido orbitario, incluyendo el tumor y el globo ocular. El procedimiento quirúrgico tenía como objetivo prevenir la recurrencia del tumor y mejorar la calidad de vida del paciente. El análisis histopatológico confirmó el diagnóstico de leiomioma orbitario. Este caso presenta un interés particular por el grado de evolución que ha alcanzado. La extirpación completa del tumor y un seguimiento a largo plazo son necesarios para prevenir la recurrencia y garantizar resultados óptimos para el paciente. Además, este caso refleja las grandes diferencias en el acceso a la sanidad en las diferentes regiones del mundo. (AU)

This manuscript describes an exceptional case of a long-standing orbital leiomyoma in a 14-year-old male. The tumor was unusually large, causing severe proptosis and significant involvement of the ocular muscles. The patient presented with amaurosis, complete ophthalmoplegia, spontaneous eye pain, and the inability to close the eyelids, leading to psychological distress. Due to the tumor's size and progression, a right orbital exenteration was performed to remove all orbital contents, including the tumor and the eyeball. The surgical procedure aimed to prevent tumor recurrence and improve the patient's quality of life. The histopathological analysis confirmed the diagnosis of orbital leiomyoma. This case presents a particular interest due to the degree of evolution it has reached. Complete tumor excision and long-term follow-up are necessary to prevent recurrence and ensure optimal patient outcomes. This report underscores global healthcare disparities and the complexity of managing rare orbital neoplasms in diverse country settings. (AU)

Clinicodemographic profile of orbital exenteration in a tertiary eye care center - A 20-year experience.

PURPOSE: This study aimed to determine the clinical indications for orbital exenteration, demographic profile of these patients, and clinicopathologic correlations in the current times and to compare these results with previous published data. METHODS: It was a retrospective study. All exenterations performed at a tertiary eye care center over a period of 20 years (from January 2001 to June 2020) were retrospectively evaluated. Patient records were reviewed to obtain demographic data, presenting symptoms and their duration, laterality, and clinical and histopathologic diagnosis. RESULTS: A total of 352 cases (males:females = 222:130) who underwent exenteration were identified. Patients age ranged from 11 months to 87 years (mean: 43.86 years, median: 50 years). The most common indication for exenteration was found to be eyelid malignancy in 54.36%, followed by retinoblastoma in 18.75% and primary orbital tumors in 14.49%. Out of 156 cases of eyelid malignancies requiring exenteration, squamous cell carcinoma (SCC) was the most common histologic subtype ( n = 94, 60.26%), followed by sebaceous gland carcinoma ( n = 40, 25.64%) and basal cell carcinoma ( n = 20, 12.82%). The most common primary orbital tumors requiring exenteration were adenocystic carcinoma of the lacrimal gland in adults and rhabdomyosarcoma in the pediatric age group. Benign conditions requiring exenteration included fulminant fungal orbital infections and lymphangioma among others. CONCLUSION: The number of exenterations performed have significantly increased in terms of absolute numbers. However, the ratio of exenteration to other tumor-related surgeries, mainly excision biopsy, has reduced compared to that reported from a previous study. The most common indication in our study remains eyelid malignancy followed by intraocular malignancy. However, SCC has emerged as the most common tumor histologic subtype requiring exenterations.

Orbital and periocular complications in patients with sinonasal tumours with orbital invasion.

AIMS: The purpose of this study was to determine the frequency and associated risk factors of orbital/periocular complications in patients with sinonasal tumour with orbital invasion managed with eye-sparing treatments. METHODS: A retrospective case series of patients with primary sinonasal tumour with orbital invasion from January 2008 to December 2018. Patient factors were compared between the following groups: (1)patients with orbital/periocular complications versus those who did not and (2) patients who needed secondary oculoplastic surgical procedures versus those who did not. RESULTS: Out of 80 patients, 48 had eye-sparing surgery, 8 had orbital exenteration and 24 were managed non-surgically. The most common histology was squamous cell carcinoma (n=28, 35%). Among the eye-sparing treatment group, 51/72 patients experienced one or more orbital/periocular complication(s), with motility deficit (N=26, 36%) being the most frequent. Factors associated with higher risk of complications included tumour involving the orbital floor (p=0.019), clinical disease stage III/IV (p=0.038), maxillectomy (p=0.004), resection of the orbital floor (p=0.027) and cigarette smoking (p=0.041). Tumour involving the orbital floor had an OR of 3.9 (95% CI 1.3 to 11.6, p=0.016) in predicting orbital/periocular complication. In the eye-sparing surgery group, the most frequent secondary oculoplastic procedures was dacryocystorhinostomy (n=6, 13%). The use of a free flap in reconstruction had an OR of 8.2 (95% CI 2.1 to 31.8, p=0.002) in predicting need for secondary oculoplastic surgery. CONCLUSION: Majority of patients with sinonasal tumours and secondary orbital invasion were managed with eye-sparing multidisciplinary treatments. Preservation of the eye can lead to reasonably good functional outcome despite expected orbital and periocular complications.

Extended orbital exenteration, epithetic restoration, and patient supply: A cross-sectional study of a historic cohort.

BACKGROUND: The aim of this study was to investigate the clinical course and to redefine an optimized algorithm for OE cases until epithetic restoration. METHODS: Indication, defect type according to Kesting, reconstructive technique, incidence of postoperative complications and peri-implantitis, patients' quality of life, timing of periorbital implant insertion, incidence of and interval to implant loss, and time until epithetic restoration were analyzed in 43 patients. RESULTS: A significant correlation was detected between wound dehiscence and defect type. Out of 24 patients, 7 were implanted secondarily with a median time interval of 399 days (270-2015) after OE. Eleven out of 83 placed implants were lost in 8 patients with a median time interval of 586 days (264-4485) after insertion. The majority of epithesis carriers had no or few restrictions in their quality of life. CONCLUSIONS: We recommend our modified treatment algorithm to further improve and shorten the clinical course.

Tumi Flap: A Novel Modification to Frontal Flap for Orbital Exenteration Defects.

Orbital exenteration remains a significant disfigurement in the face. Many reconstructive options were reported for one stage covering the defects. Local flaps are used primarily in elderly patients who are not candidates for microvascular procedures. Local flaps generally close the gap without achieving 3-dimensional adjustment perioperatively. Secondary procedures or shrinking by time are needed for better orbital adaptation. In this case report, we describe a novel frontal flap design influenced by a Tumi knife, an ancient Peruvian trepanation instrument. The design helps us to create a conic shape that can resurface the orbital cavity at the time of the operation.

Congenital mature orbital teratoma: A case report.

Teratomas are gonadal or extragonadal neoplasms, derived from the three embryonic tissues, composed of germ cells of the neuroectoderm, mesoderm and ectoderm. Congenital orbital teratoma (OCT) commonly affects the left orbit, primarily affecting women over men at a ratio of 2:1. We present the case of a female patient of 9 days of extrauterine life with a left mature congenital orbital teratoma. The orbit and oculoplastic service performed an orbital exenteration and total resection of the tumor mass.

Orbital exenteration reconstruction using a superficial temporalis muscle flap: The "Carpaccio flap", an innovative approach.

INTRODUCTION: Malignant orbital diseases may lead surgeons to practice an orbital exenteration associated with chemotherapy and/or radiotherapy to ensure curative treatment. That radical procedure makes physicians consider reconstructive filling in order to allow prothesis wearing and reduce esthetic and social after-effects. We first describe the clinical case of a 6-year-old patient who presented an orbital rhabdomyosarcoma and underwent an orbital exenteration with immediate reconstruction by a superficial temporal pedicled on the middle temporalis muscle flap. TECHNICAL NOTE: Through that case-report, we propose an original temporal flap to repair ipsilateral midface defects which may reduce donor site side effects and allow furthers corrections. DISCUSSION: In pediatrics cases, our Carpaccio flap was an available regional tool to rehabilitate an irradiated orbital socket with an appropriate bulking and vascularization effect after subtotal exenteration. Furthermore, we prescribe that flap as a posterior orbital filling, when eyelid and conjunctiva are spared, to prepare orbital prosthesis implementation. A mild sunken temporal fossa appears with our procedure but by preserving the deep layer of the temporalis muscle, autologous reconstruction such as lipofilling are permitted in post-radiotherapy condition to enhance esthetic sequelae.

Globe-sparing surgical treatment for periocular malignancies with anterior orbital invasion: a consecutive case series.

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.

Surgical outcomes of orbital evisceration with primary orbital implant placement in patients with endophthalmitis.

OBJECTIVES: This study reports the surgical outcomes of evisceration with primary orbital implant placement in patients with endophthalmitis and analyses the association with implant exposure and extrusion. METHODS: A retrospective, multicentre, Chinese cohort study. Review of medical records and orbital images of patients who underwent evisceration with primary orbital implant placement between January 2005 and January 2021. RESULTS: Out of 79 patients who underwent orbital evisceration with primary orbital implant placement, 26 (26 eyes) of them (male = 13) suffered from endophthalmitis. The duration from endophthalmitis diagnosis (19 = exogenous, 7 = endogenous) to evisceration was 9 standard deviation ± 5 (range: 1-15) days. The follow-up was 70 ± 46 (24-180) months after operation. The orbital implant size was 17 ± 3 (14-20) mm, and silicone was the most used material (69%, 18/26 of patients). The most frequent post-operative complication was orbital implant exposure (42%, 11/26), followed by orbital implant extrusion (12% 3/26) and ptosis (8%, 2/26). Implant exposure or extrusion was more commonly associated with endophthalmitis in comparison to non-endophthalmitis patients that required evisceration and primary orbital implant placement (54% versus 17%, P < 0.05). Univariate analysis showed single scleral closure technique (100% versus 58%, P < 0.05) and endogenous endophthalmitis (50% versus 0%, P < 0.05) were associated with implant exposure or extrusion, and only endogenous endophthalmitis was significant with multivariate analysis (P < 0.05). CONCLUSIONS: Primary implant placement during evisceration should be avoided in eyes with endophthalmitis especially in those with an endogenous source, and double scleral closure technique may be a better alternative for primary orbital implant placement in infected eyes.

Orbital Extenteration Defects: Ablative and Reconstructive Flowchart Proposal.

ABSTRACT: Orbital exenteration is a radical and disfiguring operation. It is still under debate the absence of correlation between the term describing the resulting orbital defect and the type of reconstruction. Authors' goal was to propose a consistent and uniform terminology for Orbital Exenteration surgery in anticipation of patients' tailored management. Twenty-five patients who underwent orbital exenteration between 2014 and 2020 were reviewed. A parallel comprehensive literature review was carried on. Five different types of orbital exenteration where outlined. Multiple reconstructive procedures were enclosed. An algorithm for orbital reconstruction was proposed based on anatomic boundaries restoration. Eyelid removal was first considered as an independent reconstructive factor, and both orbital roof and floor were indicated as independent reconstructive goals, which deserve different defect classification. In our opinion, this algorithm could be a useful tool for patient counseling and treatment selection, which might allow a more tailored patient care protocol. LEVEL OF EVIDENCE: Level III.

Psychosocial factors among patients undergoing orbital exenteration.

CLINICAL RELEVANCE: Patients undergoing exenteration have significant anxiety and depression, and lower quality of life that worsens after surgery. Therefore, counselling by an eye health-care provider along with the team of psychologist and psychiatrist could be provided pre-operatively to assess baseline psychosocial status and be continued post-operatively to look for any deterioration. BACKGROUND: Orbital exenteration comes with psychological trauma of disfigurement of face. The study aimed to evaluate the quality of life, anxiety and depression in patients undergoing orbital exenteration, before and after the procedure; and also compared the same with patients previously rehabilitated by spectacle-mounted prosthesis after exenteration. METHODS: It was a prospective cohort study. Group I included patients who were planned to undergo orbital exenteration, and group II were those already using spectacle-mounted prosthesis post orbital exenteration for at least 3 months. Level of depression was assessed using Patient Health Questionnaire-9(PHQ-9), anxiety using Generalised Anxiety and Depression scale-7(GAD-7), and quality of life was assessed using Functional Assessment of Cancer Therapy- General (FACT-G) score. RESULTS: A total of 19 patients were recruited in group I and 10 patients in group II. The baseline demographic factors were comparable. The scores for PHQ-9 and GAD-7 were significantly higher at 3-month post-exenteration follow-up, indicating significantly higher levels of anxiety and depression after orbital exenteration in group I. Both these scores were significantly lower in group II compared to group I (3 months follow-up), indicating lower levels of anxiety and depression after rehabilitation with prosthesis. The FACT-G and its subscores were all significantly higher in group II patients compared to post-operative scores of group I patients, indicating higher overall quality of life after rehabilitation with prosthesis. CONCLUSIONS: Patients undergoing exenteration have a significant level of anxiety and depression, and lower quality of life that worsens after orbital exenteration.

Orbital Exenteration Reconstruction for Medically Complex Patients: Bilaminate Dermal Substitute as an Alternative to Major Surgery.

Reconstructive strategies after orbital exenteration range from the use of skin grafts to tissue flaps, depending on the classification of the defect, patient's goals, and tolerance for surgery. We present a case of a 68-year-old, medically complex patient who underwent orbital exenteration, radiation therapy, and reconstruction with a bilaminate dermal substitute. Despite the placement in an irradiated area without periosteum, the bilaminate dermal substitute graft demonstrated acceptable integration and provided an adequate base for subsequent split thickness skin grafting, sparing a medically fragile patient from a morbid operation. This case highlights the advantages of a less invasive reconstructive modality when managing a patient with multiple comorbidities and a significant chance of cancer recurrence.

Risk factors and prognosis of orbital exenteration in conjunctival melanoma.

OBJECTIVES: To identify the risk factors of orbital exenteration and to evaluate the prognosis of exenterated patients with conjunctival melanoma (CM). METHODS: 79 consecutive CM patients treated at our centre from January 2000 to September 2021 were included. The demographic, clinical and pathological characteristics were compared between eye-sparing patients and orbital exenteration patients. Main outcomes including progression-free survival (PFS), distant metastasis-free survival (DFS) and disease specific survival (DSS) were assessed in exenterated patients. RESULTS: The mean follow-up period was 46 ± 39 months. Risk factors for orbital exenteration were identified as worse cT category (OR, 50.75; 95% CI, 5.40-477.07; P = 0.001) and greater tumour thickness (OR, 1.27; CI, 1.04-1.55; P = 0.02). Of the 32 patients who underwent orbital exenteration, three (9.4%) had local recurrence; six (18.8%) experienced regional metastasis; sixteen (50.0%) suffered distant metastasis and fifteen (46.9%) died of metastatic disease. In patients who received orbital exenteration, palpebral conjunctiva involvement (PFS: P < 0.01; DFS: P < 0.05; DSS: P = 0.04), histological ulceration (PFS: P = 0.03; DFS: P = 0.01; DSS: P = 0.03) and regression (PFS: P = 0.01; DFS: P < 0.01; DSS: P = 0.04) were identified as risk factors for poor prognosis. Caruncle involvement (P = 0.01) was also associated with increased risk of melanoma related mortality in exenterated patients. CONCLUSIONS: Histopathological factors should be taken into account when formulating surgical plans for orbital exenteration and when evaluating patients' prognosis following exenteration. For CM patients with caruncle or palpebral conjunctiva involvement, orbital exenteration should be considered for unresectable disease.

[Primary orbital leiomyosarcoma: a case report].

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.

Use of Porcine Urinary Bladder Matrix in Socket Reconstruction After Pediatric Orbital Exenteration.

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.

Oculoplastic Surgeries in Patients Older than 90 Years of Age.

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.

Orbital metastasis: Clinical presentation and survival in a series of 11 cases.

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 is 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. Metastasis involving the orbit was 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 60.9 years (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 4.8 months since the diagnosis. There was a statistically significant difference between the survival time in the observation group (median 2.5 months) and in the active treatment group (median 29.2 months), 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.