Bilateral primary orbital xanthogranulomas: A case report and comparison of xanthomatous conditions.

This report describes an unusual and diagnostically challenging case of subcutaneous soft tissue xanthogranulomas of bilateral orbits of a 58-year-old female patient seen in a private oculoplastics practice. Accurate and timely diagnosis is crucial in xanthogranulomatous diseases so that any systemic manifestations can be identified and addressed in a multidisciplinary fashion. Periorbital xanthogranuloma is a frequent early manifestation of adult xanthogranulomatous disease, and its association with life-threatening systemic disease requires accurate diagnosis and prompt work-up. This case describes an otherwise asymptomatic patient who presented with bilateral orbital masses causing visually significant ptosis, initially diagnosed as soft tissue xanthomas, and later identified as xanthogranulomas. It is important for physicians of all fields, from primary care to surgical subspecialty, to be aware that xanthogranulomatous disease may first present as periorbital lesions and/or orbital masses, and that further work-up for vision and life-threatening systemic disease is warranted.

Orbital Artifacts on MRI.

PURPOSE: To describe artifacts on orbital MRI, which led to an incorrect radiology report. METHODS: Retrospective chart review of patients identified from the orbital databases at the Royal Adelaide Hospital and University of Wisconsin Hospital. Patients who had artifacts on orbital MRI that led to an incorrect radiology report were included. Records were evaluated for age at imaging, gender, MRI sequence, laterality, and location of artifact, radiological characteristics and misdiagnosis, and cause of artifact. RESULTS: Data were collected from 7 patients (3 male) who had a median age of 61 years at the time of imaging. Five artifacts resulted from fat-suppression failure with 4 of these cases misdiagnosed as inflammatory changes and 1 misdiagnosed as neoplastic infiltration. The OD was involved in 4 cases. Six cases were in the inferior orbit region. CONCLUSIONS: Fat-suppression failure artifacts may arise in the inferior orbit region and can be mistaken for inflammatory or neoplastic orbital disease. This may prompt additional investigations such as orbital biopsy. Clinicians should be aware of artifacts which can affect orbital MRI and lead to potential misdiagnosis.

Congenital Orbital Fibrosis With Spontaneous Regression of Orbital Tumor.

Congenital orbital fibrosis (COF) is a rare disorder characterized by an infiltrating orbital mass with secondary involvement of the extraocular muscles that may present with extraocular muscle dysfunction, and globe and eyelid abnormalities in infancy. This condition is thought to be a nonprogressive process and literature on longitudinal assessment of COF is limited. The authors describe a case of COF which was followed for 15 years. The patient had stable symptoms of ocular dysmotility and ptosis but was noted to have spontaneous regression of the orbital mass on serial MRI.

Orbital Vascular Malformations: Relationship Between Enophthalmos and Clinically Apparent Distensibility with Valsalva.

PURPOSE: Determining the hemodynamic characteristics of an orbital vascular malformation is a critical step in management. The purpose of this study is to assess the relationship between enophthalmos and clinically apparent distensibility of orbital vascular malformations, to optimize imaging and treatment. METHODS: In this cross-sectional cohort study consecutive patients at a single institution were screened for study entry. Data extracted included age, sex, Hertel measurements, presence or absence of distensibility during the Valsalva maneuver, whether lesions were primarily venous or lymphatic based on imaging, and location of the lesion relative to the globe. Enophthalmos was defined as ≥ 2 mm difference from the opposite side. Parametric and nonparametric statistics were used, and linear regression was performed to examine factors predictive of Hertel measurement. RESULTS: Twenty-nine patients met the inclusion criteria. Relative enophthalmos ≥2 mm was significantly associated with distensibility ( p = 0.03; odds ratio = 5.33). Distensibility and venous dominant morphology were the 2 most important factors associated with enophthalmos on regression analysis. The relative position of the lesion anterior or posterior to the globe did not have a significant bearing on baseline enophthalmos. CONCLUSIONS: The presence of enophthalmos increases the likelihood that an orbital vascular malformation is distensible. This group of patients was also more likely to be characterized by venous dominant malformations. Baseline clinical enophthalmos may serve as a useful surrogate marker for distensibility and venous dominance, which may be useful in guiding the selection of appropriate imaging.

Systemic sarcoidosis presenting as acute rapidly progressive proptosis.

Sarcoidosis is a generalized systemic chronic inflammation that rarely involves the orbit. As a chronic inflammation, sarcoidosis typically manifests with an insidious onset and slowly progressive course. We report a case of acute-onset proptosis resulting from a rapidly growing diffuse orbital mass that simulated malignant growth, which was biopsy proven to be the first manifestation of systemic sarcoidosis. The patient demonstrated complete resolution of proptosis and systemic involvement with long-term corticosteroid treatment.

Clinical characteristics, diagnosis, and outcomes of orbital biopsies in a single Irish centre.

AIMS: To review the distribution of histopathological diagnoses and visual outcome of orbital biopsy in an Irish tertiary referral centre over a 10-year period. METHODS: This was a retrospective, clinical-histopathological case series. Clinical records of all patients who underwent orbital biopsy between January 2008 and January 2018 in the Mater Misericordiae University Hospital were reviewed using data collected from theatre logbooks and hospital-based medical records. RESULTS: A total of 83 orbital biopsies in 77 patients were included for analysis in this study. The mean age was 55.7 ± 18.41 years. The mean follow-up period was 1.87 ± 2.097 years. The most common presenting symptoms and signs were pain (22.3%) and proptosis (27.6%). Most lesions were located in the extraconal space (65%), with incisional biopsy (65%) being the most common technique used to gain a sample for histopathological diagnosis. Histopathology analysis of the biopsies revealed malignant tumours (27, 32.5%), benign tumours (7, 8.4%), inflammation (26, 31.3%), and other diagnoses (23, 27%). Excluding patients who underwent exenteration procedures, no study patients suffered visual loss following orbital biopsy. CONCLUSIONS: Orbital biopsy serves as a safe diagnostic tool in managing orbital diseases. The breakdown of diagnosis in our patients is in line with international studies. No patients in our series suffered vision loss as a result of their orbital biopsy. This emphasises its use as a safe procedure in the diagnosis and management of patients with the orbital disease. Our data provides helpful guidance to clinicians when counselling patients for orbital biopsy.

A high-pressure situation.

An 8-year-old boy presented with acute visual loss in the right eye and nausea, vomiting, and diplopia. Imaging revealed a right orbital apex mass. Biopsy showed Langerhans cell histiocytosis (LCH), and the patient was diagnosed with isolated orbital LCH causing an orbital apex syndrome. A 12-month cytarabine chemotherapy course was begun, during which the patient developed bilateral optic disc edema. He was diagnosed with cytarabine-induced intracranial hypertension, which was successfully treated with acetazolamide. The cytarabine course was completed with complete resolution of the LCH lesion. The ophthalmologic relevance of this rare disorder is discussed.

The transconjunctival orbitotomy: A versatile approach to the orbit and beyond.

In the management of orbital disorders and defects, minimally invasive surgical approaches have become increasingly efficient for their reduction of operative trauma and access without compromise of therapeutic benefit or diagnostic yield. Various approaches have focused on bone- and canthal-sparing techniques and concealed and small skin incisions. We review the current state of knowledge of procedures to enter the orbit via the conjunctiva. Any quadrant of the orbit can be accessed via the conjunctiva. Surgical incisions involve the orbital palpebral, forniceal, and bulbar conjunctiva. According to the location, nature, and size of the lesion, the transconjunctival orbitotomy can be used as a single procedure, in combination with a caruncular approach or as an adjunct in a multidisciplinary procedure for lesions extending deep into or outside the orbit. The working space and field of operating view can be expanded by releasing the horizontal tension of the eyelid with a lateral cantholysis, lateral paracanthal blepharotomy, or medial lid split procedure. Complications related to the conjunctival incision are reduced to dry eye disease.

Immunosuppression is a Double-Edged Sword in COVID-19 Treatment: a Case Report of Rhino-Orbito-Cerebral Mucormycosis.

BACKGROUND: Mucormycosis is a life-threatening fungal infection mostly occurring in immunosuppressed patients such as organ transplant or diabetic patients. In this paper, we described a case of COVID-19 with rhino-orbito-cerebral mucormycosis. METHODS: The nucleic acid amplification test (NAAT) from a nasopharyngeal sample for SARS-CoV-2 was done. Demographic data, biochemical tests, paranasal sinuses (PNS) CT scan, brain CT scan, chest CT scan, and palate biopsy were performed. RESULTS: The NAAT was positive for SARS-CoV-2. PNS CT scan revealed mucosal thickening of all paranasal sinuses, brain CT scan showed hypodense area in antero-inferior cortex, and chest CT scan revealed diffuse ground glass opacity in favor of COVID-19 infection. Palate biopsy revealed fibroconnective tissue with broad pauciseptated ribbon-like hyphae. CONCLUSIONS: In this paper, a case of COVID-19 with rhino-orbito-cerebral mucormycosis was described. The treatment with immunosuppressive drugs predisposed this patient to secondary fungal infection. Immunosuppression is a double-edged sword in COVID-19 treatment and immunosuppressive drugs should be prescribed only in severely ill patients and for a short period.

First report of rhino-orbital-cerebral mucormycosis after COVID-19 infection in Bulgaria.

Mucormycosis is a relatively rare infection but with a high mortality rate due to the difficult and time-consuming diagnostic and therapeutic process. The authors present the first case of rhino-orbital-cerebral mucormycosis, histologically and microbiologically proven, in a patient after COVID-19 infection in Bulgaria.

Elastoma of the Orbit: A Case Report.

A 78-year-old man presenting for revision ptosis surgery was found to have an asymptomatic left inferomedial orbital mass visible below the left lower eyelid on external inspection, and subconjunctivally on examination. This was subsequently diagnosed as an isolated elastoma. A mass in a similar location was excised 60 years previously. His other ophthalmological history included stable diplopia corrected with prism, left-sided ectropion, bilateral sequential phacoemulsification, and past bilateral ptosis which has been persistent on the left side despite surgical repair and revision. His examination revealed left hypertropia but was otherwise largely unremarkable. However, imaging demonstrated the soft tissue lesion abutting the left globe. An anterior orbitotomy was performed, and the lesion was biopsied and specimens sent for histopathological examination and immunohistochemistry. This is the first case of an elastoma of the orbit reported in the literature to the best of the authors' knowledge.

[Orbital cranial fasciitis in a child].

A 4-year-old patient with a left orbital mass was admitted to the Department of Ophthalmology of the First Affiliated Hospital of Zhengzhou University. After clinical and imaging examinations, the left orbital mass lesion was removed under general anesthesia. Postoperative pathological examination confirmed orbital cranial fasciitis. The appearance and imaging result of the patient were normal at postoperative 7 months.

Orbital Aspergillosis, a great masquerader: a series of three unusual cases and a review of the literature.

Fungal infections of the orbit are infrequent. They typically affect immunocompromised individuals but, in some instances, can also affect an immunocompetent person. They mimic much orbital pathology clinically. Ours is a series of three patients eventually diagnosed with orbital aspergillosis but who, initially, were thought to have some other pathology.

Congenital Conjunctival Cysts of the Orbit.

PURPOSE: To evaluate the clinical presentation, anatomical location, and histological features of congenital conjunctival cysts of the orbit. The location and the histological features of inflammation in these patients were compared with those for 293 orbital dermoid cysts. PATIENTS AND METHODS: Retrospective review of the clinical details, imaging, and histopathology for patients who had excision of conjunctival cysts from their orbit between 1992 and 2020; patients with a history of trauma or surgery were omitted. RESULTS: Twelve patients (7 male; 58%) with congenital conjunctival cysts were identified, the patients presenting at an average age of 16 years (median 26; range 1-61) with a symptoms for a mean duration of 20 months (median 24; range 6-36). The commonest symptoms were peribulbar lump (6/12 patients; 50%), and eyelid swelling and blepharoptosis (6/12 patients; 50%). An orbitaxl mass was palpable in 10 patients (83%), 3 patients (25%) had mild proptosis (1-3 mm), and the cysts were most commonly located superiorly (6/12 patients; 50%) or superonasally (3/12; 25%) in the anterior half of the orbit. Imaging was performed in 7 cases, this showing an intimate relation to the common sheath of the superior rectus/levator complex in 3 patients (25%) and to the trochlea in 1 (8%). All cysts were excised completely, and no patient had postoperative complications or recurrence. Chronic mild and nonspecific inflammation was evident within the cyst wall in 7 cases (54%), but-unlike 55% of the 293 dermoid cysts-none showed granuloma formation. CONCLUSION: Congenital conjunctival cysts are rare and usually present with a palpable mass in the upper eyelid sulcus. A significant proportion of these cysts have an intimate relationship with the trochlea, or the superior rectus, levator palpebrae or superior oblique muscles and, to minimize the risk of postoperative diplopia or ptosis, particular care must be exercised during surgery.

Orbital Cyst with Ependymal Differentiation Associated with Microphthalmia.

BackgroundOrbital cysts associated with microphthalmia are colobomatous lesions that typically present unilaterally and posterior to the globe. Case Report: A male infant had an orbital cyst associated with microphthalmia located anterior to the globe composed of a neuroglial wall, ependymal-like epithelial lining, with synaptophysin-positive cells resembling the retinal neuronal layer. Conclusion: This orbital cyst may represent a malformation of the eye rather than an encephalocele.

Lymphoma and inflammatory disorders presenting in the orbit- a comparison of characteristics from a 10-year series in a tertiary hospital.

BACKGROUND/AIMS: To explore differences in clinical presentations and outcomes between patients with biopsy-proven orbital lymphoma and orbital inflammatory disease. METHODS: Clinical records of all patients who underwent orbital biopsy at Bristol Eye Hospital during a 10-year period were reviewed. Comparisons were made between patients diagnosed with orbital lymphoma and orbital inflammatory disease (OID). RESULTS: 35 patients were diagnosed with orbital lymphoma, undergoing 40 biopsies over the 10-year period studied. 62 patients had OID, undergoing 66 biopsies. Sub-analysis identified six patients with sarcoidosis, four with granulomatosis polyangiitis (GPA), eight with IgG4-related orbital disease, and 44 with idiopathic orbital inflammation (IOI). Patients with sarcoidosis and IOI were statistically younger at presentation than those with lymphoma, with means of 44 and 52 years, respectively, compared with 71 years. Lid swelling/puffiness was more common in OID than lymphoma. More patients with lymphoma lost vision during follow-up than those with OID. CONCLUSIONS: Although younger age of presentation and presence of lid swelling are more likely to indicate orbital inflammation than orbital lymphoma, orbital biopsy remains the gold standard to distinguish these two entities. In the case of orbital inflammation, identification of a specific diagnosis allows appropriate ongoing referral and investigations for potential systemic involvement and helps guide immunosuppressive treatment.