Orbital histiocytosis; From A to Z.

PURPOSE: Histiocytosis is one of the most challenging diseases in medical practice. Because of the broad spectrum of clinical manifestations, systemic involvements, unknown etiology, and complex management, different types of histiocytosis are still a big question mark for us. Orbital histiocytosis is characterized by the abnormal proliferation of histiocytes in orbital tissues. It could affect the orbit, eyelid, conjunctiva, and uveal tract. Orbital histiocytosis can cause limited eye movement, proptosis, decreased visual acuity, and epiphora. In this study, we review the novel findings regarding the pathophysiology, diagnosis, and treatment of different types of histiocytosis, focusing on their orbital manifestations. METHOD: This review was performed based on a search of the PubMed, Scopus, and Embase databases or relevant published papers regarding orbital histiocytosis on October 9th, 2023. No time restriction was proposed, and articles were excluded if they were not referenced in English. RESULTS: 391 articles were screened, most of them being case reports. The pathophysiology of histiocytosis is still unclear. However, different mutations are found to be prevalent in most of the patients. The diagnostic path can be different based on various factors such as age, lesion site, type of histiocytosis, and the stage of the disease. Some modalities, such as corticosteroids and surgery, are used widely for treatment. On the other hand, based on some specific etiological factors for each type, alternative treatments have been proposed. CONCLUSION: Significant progress has been made in the detection of somatic molecular changes. Many case studies describe various disease patterns influencing the biological perspectives on different types of histiocytosis. It is necessary to continue investigating and clustering data from a broad range of patients with histiocytosis in children and adults to define the best ways to diagnose and treat these patients.

Ocular and orbital manifestations in VEXAS syndrome.

BACKGROUND: VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) is a hematoinflammatory disease that typically affects adults. It results from a somatic mutation of the E1 ubiquitin conjugating enzyme encoded by the UBA1 gene. VEXAS is frequently accompanied by myelodysplastic syndrome (MDS). The purpose of this study is to describe the ocular and orbital manifestations of VEXAS patients in a case series in our medical centre. METHODS: A retrospective chart review was performed for all patients who were diagnosed with VEXAS syndrome in a tertiary medical centre over two years. RESULTS: Eight patients were identified with VEXAS. In six patients, the diagnosis was confirmed by genomic sequencing. Two patients were identified based on their phenotype. All patients were males. The mean age at diagnosis was 78.7 years. In two patients, the ocular manifestation was the presenting symptom for VEXAS. Seven patients (87.5%) had history of MDS. Systemic inflammation manifestations include: skin rash (n = 5), recurrent fevers (n = 2), relapsing polychondritis (n = 2), pleuritis and pleural effusion (n = 2), poly arteritis nodosa- PAN (n = 1) and thrombophlebitis (n = 1). Seven (87%) patients were presented with periorbital oedema. Three patients showed orbital inflammation. Dacryoadenitis was observed in two patients, and extraocular muscle (EOM) myositis was detected in two patients. Four patients demonstrated ocular inflammation such as: episcleritis, scleritis and anterior uveitis. CONCLUSION: ocular manifestations in VEXAS include orbital inflammation, dacryoadenitis, myositis, uveitis, scleritis, episcleritis and periorbital oedema. We recommend that in old male patients, with history of haematological disorder, presenting with ocular symptom, VEXAS investigation should be taken into consideration.

Orbital complications of sinusitis in children - Retrospective analysis of an 8.5 year experience.

OBJECTIVE: Orbital complications account for approximately 74-85 % of all complications of acute sinusitis, affect the pediatric population more frequently, and can have devastating consequences. In the years following the COVID-19 pandemic (2022, 2023), a high number of children presented to our clinic with orbital complications. 1)Has there been an increase in orbital complications in the post-covid era? 2)To what extent has the use of MRI reduced radiation in pediatric patients? DESIGN: In our retrospective data analysis, all pediatric patients (age 0-16 years) treated at a university ENT clinic during the period 01/2014-06/2023 who presented with an orbital complication of rhinosinusitis were included. The analysis was descriptive. RESULTS: Forty-four children with orbital complications of rhinosinusitis were treated during the study period, 14 females and 30 males. Most patients (n = 23, 52 %) presented during the years of the waning Covid-19 pandemic (01/2022 to 06/2023). MRI was the initial imaging modality (n = 22,50 %); CT was performed in 17 of 44 cases (39 %) when surgery was indicated. The most common germ detected was of the Streptococcus species, and the predominant antibiotic administered was amipicillin/sulbactam. CONCLUSION: The standard operating procedure (SOP) established at our hospital in 2014 was followed in 42/44 cases. Except for 2 cases, CT was performed exclusively when surgery was indicated. Imaging-related radiation could be avoided in 27 patients (61 %). There was a 30 % increase in orbital complications related to sinus infections postpandemically.

Role of Acute Rhinosinusitis in Periorbital Infections in Children.

OBJECTIVE: Periorbital cellulitis in children are commonly caused by acute rhinosinusitis (ARS). This study investigated the association of ARS and the severity of periorbital cellulitis in children. STUDY DESIGN: Retrospective case-control study of children with periorbital cellulitis with ARS versus periorbital cellulitis without ARS. SETTING: Patients were seen at West Virginia University Children's Hospitals between August 2011 to August 2022. METHODS: Patients were divided into cases and controls based on presence or absence of ARS. ARS was defined based on clinical symptoms with objective presence of disease on CT scan. Patients' characteristics, treatment, hospital length of stay, and readmission were collected. RESULTS: The sample consisted of 118 pediatric patients with orbital cellulitis. Patients with ARS were younger than patients without ARS (6.3 vs 8.5, p = 0.025), however there were no sex differences between two groups (p = 0.540). The ARS group had higher incidence of postseptal cellulitis (51.5% vs 9.6%, p < 0.001). As compared to patients without ARS, patients with ARS were more likely to be admitted (p < 0.001), have a longer length of stay (median of 3.5 days vs 0.5 days, p < 0.001), require IV antibiotics (95.3% vs 54.9%, p < 0.001), and require surgical intervention (23.1% vs 5.8%, p < 0.001). Readmission rate was similar between the two groups. CONCLUSION: Children presenting with acute periorbital cellulitis who have ARS tend to have more severe infection requiring higher level of care. ARS should be assessed and incorporated into the plan of care of pediatric patients with periorbital infections.

Ocular and orbital manifestations of granulomatosis with polyangiitis: a systematic review of published cases.

Objective: Granulomatosis with polyangiitis (GPA) is an autoimmune disorder characterized by necrotizing granulomatous inflammation of small and medium-sized vessels. This systematic review aimed to highlight the most common ophthalmic manifestations and to uncover their associations with antineutrophil cytoplasmic antibody (ANCA) positivity and the presence of granulomas. Methods: A literature search of PubMed, Web of Science, and Scopus electronic databases was performed from journal inception to March 21, 2021, for case reports and a series of ophthalmic GPAs. Cytoplasmic-ANCA (c-ANCA), perinuclear-ANCA (p-ANCA), and granulomas were analyzed against many ophthalmic signs and symptoms. 306 patients with GPA were retrospectively studied. Results: Granulomas were present in 47.7% of our sample, c-ANCA in 59.2%, and p-ANCA in 10.8%. Scleritis was significantly associated with higher odds for c-ANCA positivity. Eye discharge, episcleritis, proptosis, and central nervous system (CNS) involvement were each significantly associated with lower odds for c-ANCA positivity. Orbital mass was significantly associated with lower odds for p-ANCA positivity. CNS involvement was significantly associated with higher odds for p-ANCA positivity (OR:3.08, 95% CI:1.02, 9.36, p=0.047) and orbital mass was significantly associated with lower odds for p-ANCA positivity. Conclusions: We recommend that clinicians should consider ocular or orbital GPA in patients presenting with non-specific eye complaints, such as vision impairment, orbital mass, or proptosis, and obtain further assessments to determine the possible presence of granuloma, c-ANCA, or p-ANCA. Abbreviations: GPA = Granulomatosis with Polyangiitis, ANCA = antineutrophil cytoplasmic antibody, c-ANCA = cytoplasmic-ANCA, p-ANCA = perinuclear-ANCA, CNS = central nervous system, AAVs = ANCA-associated vasculitides, SD = standard deviation, GU = genitourinary, ENT = ear nose and throat, OR = odds ratio, CI = confidence interval.

Revisiting the orbital complications of acute rhinosinusitis.

OBJECTIVE: The present study revisited three classification systems of orbital complications of acute rhinosinusitis (ARS) (Chandler, Mortimore & Wormald, and Velasco e Cruz & Anselmo-Lima) and observed which of them presented the best clinical applicability. METHODS: Clinical data and CT scan findings of patients with orbital infection were retrospectively collected. To compare the three classification systems, we revised and graded all CT images accordingly, and divided the patients into four groups: Eyelid cellulitis (EC), orbital cellulitis (OC), subperiosteal abscess (SA), and orbital abscess (OA). The groups were compared regarding the presence of sinus opacification, the need for hospitalization and/or surgical treatment, and the presence of further complications/sequelae. RESULTS: 143 patients were included. The median number of sinuses involved in patients in the OC, SA, and OA groups was 2.0. ARS was rarely associated with signs of EC (present in both Chandler's and Mortimore & Wormald's classifications. The hospitalization rate was significantly lower in the EC group compared to the other three groups. Surgery was performed in all cases in the OA group, in 58.1% in the SA group, 19.4% in the OC group, and 12.5% in the EC group (p-value < 0.0001). Complications were present at higher rates in the OA group compared to the other three groups. CONCLUSIONS: ARS was rarely associated with Eyelid Cellulitis. The stratification in the other three groups showed to be clinically relevant. Velasco e Cruz & Anselmo-Lima's classification system proved valid, simple, and effective for categorizing orbital complications of ARS.

Factors affecting clinical outcome in COVID-associated rhino-orbito-cerebral mucormycosis (CAROM) patients-An ambispective, single-arm, observational study.

BACKGROUND: COVID-associated Rhino-Orbito-Cerebral Mucormycosis (CAROM) appeared as an epidemic in India during the second wave of the COVID-19 pandemic during the months of March to May 2021. Though many reports have highlighted cross sectional and short-term attributes related to CAROM, long term follow up data is sparse. OBJECTIVE: This report aims to analyze the follow-up outcomes in consecutive patients presenting to us during the epidemic. PATIENTS AND METHODS: This was an ambispective observational analytical study, recruiting the consecutive patients admitted to our tertiary care centre during the period of the CAROM epidemic. The mortality rate during the follow-up and various factors affecting survival were studied using univariable and multivariable statistics with the Stata 14.0 software. RESULTS: Of the 189 patients studied, eight were lost to follow-up. The outcome analysis was performed for the 181 patients. 93.6 % (162/173) of the patients had diabetes. The All-cause mortality was 45 % (81/181), while the ROCM-specific mortality was found to be 24 % (46/181) at a median follow-up of 176 days (IQR: 21-217 days). With univariable analysis, increasing age, higher serum IL-6 levels, presence of additional comorbidities (in addition to Diabetes and hypertension), bilateral disease, skin necrosis, palatal involvement, infratemporal fossa involvement, and impaired vision/ocular movements were found to be associated with increased mortality. However, on multivariable analysis, only 1) increasing age, 2) raised serum IL-6 levels, and 3) bilateral disease were predictive of increased mortality. Surgical debridement (endoscopic, palatal removal, orbital exenteration, neurosurgical intervention) was associated with significantly reduced mortality on both univariable and multivariable analysis. CONCLUSION: Our intermediate-term follow-up data showed advanced age at presentation, raised IL-6 levels, and bilateral sinonasal involvement to be predictive of increased mortality, while surgical debridement is significantly protective from mortality in CAROM patients.

Frontal osteomyelitis post-COVID-19 associated mucormycosis.

Rhino-orbito-cerebral mucormycosis (ROCM) is the most commonly noted form of mucormycosis, which is the most common secondary fungal infection following severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. Osteomyelitis is one of the rare sequelae of ROCM, frontal osteomyelitis being the rarest. We present four patients of coronavirus disease 2019 (COVID-19)-associated mucormycosis, who presented with frontal bone osteomyelitis after being treated for ROCM surgically and medically. This is the first case series highlighting this complication in post-COVID-19 mucormycosis patients and needs utmost attention as it can be life-threatening and can cause extreme facial disfiguration. All four patients are alive with salvage of the affected globe and vision being preserved in one patient. If identified early, disfiguration of face and intracranial extension can be avoided.

Seasonal variations, acute rhinosinusitis and orbital infections in children.

BACKGROUND: Orbital infections in children are commonly secondary to acute bacterial rhinosinusitis (ABRS). It is unclear whether seasonal variations can predispose to these complications mirroring acute rhinosinusitis incidence. OBJECTIVE: To determine the incidence of ABRS as a cause of orbital infections and whether seasonality is a risk factor. METHODS: A retrospective review of all children who presented to West Virginia University children's hospital between 2012 and 2022 were reviewed. All children with CT evidence of orbital infection were included. Date of occurrence, age, gender, and presence of sinusitis were reviewed. Children with orbital infection secondary to tumors, trauma, or surgery were excluded. RESULTS: 118 patients were identified with mean age of 7.3 years with 65 (55.1 %) males. 66 (55.9 %) children had concomitant sinusitis on CT scan, and the distribution of orbital complications per season showed 37 (31.4 %) cases occurred in the winter season, followed by 42 (35.6 %) cases in spring, 24 (20.3 %) cases in summer, and 15 (12.7 %) in fall. Children with orbital infections during winter & spring had sinusitis in 62 % of children vs. 33 % in other seasons (P = 0.02). Preseptal cellulitis was present in 79 (67 %) children, 39 (33 %) children with orbital cellulitis, and 40 (33.9 %) children with abscesses. 77.6 % children were treated with IV antibiotics and 94 % with oral antibiotics, and 14 (11.9 %) with systemic steroids. Only 18 (15.3 %) children required surgery. CONCLUSIONS: There seems to be a seasonal predisposition for orbital complications mainly in the winter and spring seasons. Rhinosinusitis was present in 55.6 % of children presenting with orbital infections.

Orbital abscess a rare complication after bichectomy surgery.

Bichectomy is the partial removal of the Bichat's fat pad for the aim of smoothing the facial contour. The complications of bichectomy include soft tissue infections, hematoma, facial paralysis (especially buccal branch paralysis), stenon canal injuries and related complications such as sialocele and sialoadenitis, however these are not common complications in clinical practice. Here we report a case of a 29-year-old white female with a right sided orbital abscess following bichectomy. Right sided orbital abscess and orbital cellulitis was managed with endoscopic decompression and drainage. The patient healed completely with no sequela. The frequency of major complications leading to moridity after bichectomy is rare in the literature. This is the first reported intraorbital complication of bichectomy.

Impact of Season on Incidence of Sinusitis-related Orbital Cellulitis.

PURPOSE: To explore the impact of season on the incidence of presentation to emergency departments with sinusitis-related orbital cellulitis in the United States. METHODS: The National Emergency Department Sample was queried to identify cases of patients with sinusitis-related orbital cellulitis. Patient's age, location, and the month of presentation were recorded. Statistical correlations were analyzed via a dedicated software package. RESULTS: A total of 439 patients with sinusitis-related orbital cellulitis were identified. The overall incidence was higher during the winter months ( p < 0.05); while children were more likely to develop this disease during the winter ( p < 0.05), season was not statistically correlated with its incidence among adults ( p = 0.16). The incidence of orbital cellulitis was higher during the winter in the midwest and south regions of the United States ( p < 0.05 for each region), although this correlation did not apply in the northeast and west ( p = 0.60 and 0.99, respectively). CONCLUSIONS: While sinusitis incidence increases during the winter, the relationship between season and orbital cellulitis is complex and varies by age and geographic location. These findings may help to facilitate screening protocols for this disease and to define staffing issues for emergent ophthalmic care.

High risk and low prevalence diseases: Orbital cellulitis.

INTRODUCTION: Orbital cellulitis is an uncommon but serious condition that carries with it a potential for significant morbidity. OBJECTIVE: This review highlights the pearls and pitfalls of orbital cellulitis, including presentation, diagnosis, and management in the emergency department (ED) based on current evidence. DISCUSSION: Orbital cellulitis refers to infection of the globe and surrounding soft tissues posterior to the orbital septum. Orbital cellulitis is typically caused by local spread from sinusitis but can also be caused by local trauma or dental infection. It is more common in pediatric patients compared to adults. Emergency clinicians should first assess for and manage other critical, sight-threatening complications such as orbital compartment syndrome (OCS). Following this assessment, a focused eye examination is necessary. Though orbital cellulitis is primarily a clinical diagnosis, computed tomography (CT) of the brain and orbits with and without contrast is critical for evaluation of complications such as abscess or intracranial extension. Magnetic resonance imaging (MRI) of the brain and orbits with and without contrast should be performed in cases of suspected orbital cellulitis in which CT is non-diagnostic. While point-of-care ultrasound (POCUS) may be useful in differentiating preseptal from orbital cellulitis, it cannot exclude intracranial extension of infection. Management includes early administration of broad-spectrum antibiotics and ophthalmology consultation. The use of steroids is controversial. In cases of intracranial extension of infection (e.g., cavernous sinus thrombosis, abscess, or meningitis), neurosurgery should be consulted. CONCLUSION: An understanding of orbital cellulitis can assist emergency clinicians in diagnosing and managing this sight-threatening infectious process.

Predictors for surgical intervention in orbital complications of pediatric rhinosinusitis.

OBJECTIVES: Orbital complications are the most common complication of acute rhinosinusitis, especially among pediatric patients. While most cases are treated with antibiotics alone, severe presentation may demand surgical intervention. Our goal was to determine which factors predict the need for surgery and to investigate the role of computerized tomography in the decision process. METHODS: A retrospective review of all children hospitalized between 2001-2018 with orbital complications of acute rhinosinusitis in a university-affiliated children's hospital. RESULTS: A total of 156 children were included. Mean age was 7.9 years (1-18 years). Twenty-three children (14.7%) were surgically treated, and the rest were conservatively treated. High fever, ophthalmoplegia and diplopia in association with minimal or no response to conservative treatment were predictive for surgical intervention, as well as higher inflammatory indices. Eighty-nine children (57%) underwent imaging during hospitalization. Presence of a subperiosteal abscess, as well as its size and its location were not found to be predictors for surgery. CONCLUSION: Clinical and laboratory findings in association with minimal or no response to conservative treatment predict the need for surgical intervention in cases of orbital complications of acute rhinosinusitis. As Computerized Tomography scans can have long-term implications in the pediatric population, caution and patience should be practiced when deciding on the timing of imaging in this population. Thus, close clinical and laboratory monitoring should lead the decision-making process in these cases and imaging should be reserved for when the decision for surgery has been made.

[Diagnosis and management of orbital and cranial complications of pediatric acute rhinosinusitis].

Objective: To review the clinical characteristics, to illustrate diagnosis and management experience of orbital and cranial complications of pediatric acute rhinosinusitis. Methods: The clinical data of 24 children with orbital and cranial complications of acute rhinosinusitis who received endoscopic sinus surgery combined with drug treatment in Beijing Children's Hospital from January 2017 to December 2021 were retrospectively reviewed. There were 19 boys and 5 girls. The age varied from 13 to 159 months, with a median 47.5 months. The following diagnoses were obtained: 12 isolated subperiosteal orbital abscess, 2 associated with preseptal abscess, 2 associated with intraorbital abscess, 7 associated with optic neuritis, and 1 associated with septic cavernous sinus thrombosis. Clinical characteristics, organism isolated and outcomes were analyzed through descriptive methods. Results: All 24 patients presented with fever; 9 presented with nasal congestion and purulent discharge. The clinical manifestations of orbital infection included orbital edema, pain, proptosis and displacement of globe in all patients, while visual impairment was recognized in 7 children. Purulent drainage was cultured in 17 patients, among which 12 were positive. All patients underwent nasal endoscopic surgical interventions uneventfully, excluding one patient who required a second surgical procedure. Follow-up period ranged from 5 to 64 months. All patients resolved fully, with the exception of 2 children who got permanent blindness with visual loss preoperative. There was no recurrence or death. Conclusions: Orbital and cranial complications of pediatric acute rhinosinusitis could be severe with an occult onset. For patients with vison impairment, any signs of intracranial complications and a lack of response to conservative management, an urgent endoscopic intervention is needed.

Interdisciplinary care in orbital complications of acute rhinosinusitis in children.

Purpose: Orbital cellulitis is a serious condition with potentially severe complications. Treatment requires interdisciplinary care and early introduction of antimicrobial therapy. In our tertiary center, a team of pediatricians, pediatric ophthalmologists, and otorhinolaryngologists successfully participated in the management of pediatric periorbital/orbital cellulitis. This study aimed to demonstrate our interdisciplinary approach and to investigate clinical profile and management of pediatric periorbital/orbital cellulitis. Methods: A retrospective chart review was performed of all pediatric patients hospitalized for periorbital and orbital cellulitis in a tertiary hospital center from September 15, 2016, to March 15, 2020. Results: A total of 26 children-median age 2.7 years (range 0.5-12)-were treated during the study period. Disease presentation was unilateral, mainly during winter (n = 12) and autumn (n = 12), without ophthalmoplegia/proptosis. Seven patients had orbital cellulitis (Chandler classification of ≥III) and were older (6.5 years, P = 0.011) with sinusitis (P < 0.001), required surgery (P = 0.004), underwent longer antimicrobial treatment (13 days, P < 0.001), and had a longer length of hospital stay (13.43 days, P = 0.001). Orbital cellulitis occurred in a median of three days (range 1-12) of acute rhinosinusitis. Radiological survey was performed in 11 patients, whereas six patients were treated surgically. All intraoperatively collected cultures (sinus swabs) were positive, whereas Streptococcus pyogenes and Peptostreptococcus were isolated in five cases. All patients fully recovered. No recurrence was documented. Conclusion: Sinusitis is associated with severe orbital cellulitis and surgical management. Orbital cellulitis occurred early in the course of acute rhinosinusitis, as a distinctive presentation of rhinosinusitis. Interdisciplinary care and early management are crucial in treatment of pediatric periorbital/orbital cellulitis.

The eye is a common site of granulomatosis with polyangiitis. A collaborative study.

BACKGROUND: Ocular manifestations of granulomatosis with polyangiitis (GPA) have been reported in a limited number of studies and with largely variable frequency. Here we report on the clinical, diagnostic, and therapeutic features of a cohort of 63 GPA patients, with particular regard to 22 of them with ophthalmic involvement (35%). METHODS: Clinical manifestations, results of immunological findings, histopathological pictures, imaging data, Birmingham Vasculitis Activity Score, therapeutic regimens, and outcomes were retrospectively analyzed. At diagnosis, in addition to a structured clinical assessment, all patients underwent a comprehensive ophthalmologic examination. RESULTS: The most frequently involved organs were kidneys, lungs, ear/nose/throat, and eyes. Ocular manifestations were bilateral in 32%. The three most commonly diagnosed ophthalmologic manifestations were scleritis (36%), retro-orbital pseudotumor or orbital mass (23%), and episcleritis (13%). Ocular and systemic involvement were simultaneously present at onset in 41% of the patients; systemic involvement was followed by ocular lesions in 36%; ocular inflammation was followed by systemic manifestations in 18%; and an orbital mass in the absence of systemic disease characterized 5%. Glucocorticoids plus cyclophosphamide and glucocorticoids plus rituximab were the combined therapies most frequently employed during remission induction and remission maintenance, respectively. Persistent ophthalmologic and extra-ocular remissions were achieved in 77 and 64% of the patients, respectively. One to three systemic relapses were diagnosed in 7 patients (31.8%). At the last follow-up, a visual outcome 20/40 or better in 31 (70%) of 44 eyes was determined. CONCLUSIONS: The eye was involved in over one third of our patients with GPA. Increased awareness, early diagnosis, and multi-specialty collaboration are critical in achieving a favorable outcome of GPA.