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.

Orbital Cerebrospinal Fluid Leak After Dog Bite: A Case Report

A 4-year-old boy was referred to our tertiary hospital after a penetrating adnexal injury by a large-breed dog to the left orbital area. There was an increase in lacrimation, which was thought to be due to an inflammatory reaction. However, it was discovered that the lacrimation increased in the reverse-Trendelenburg position and with the Valsalva maneuver. Halo sign and beta transferrin test were positive, which led to the diagnosis of cerebrospinal fluid (CSF) fistula, and the patient was operated using a supraorbital craniotomy. A dural tear was visualized and sutured appropriately, then fibrin glue and an autologous galeal graft were applied to the tear. The CSF oculorrhea stopped postoperatively, and the patient was discharged after 10 days of follow-up. The patient had no recurrent CSF leakage at 4-year follow-up. Although CSF oculorrhea is rare and may be difficult to discern from lacrimation, the presence of pneumocephalus and halo sign should suggest fistula repair.

Nontraumatic orbital hematoma secondary to labor and childbirth: a case report and review of the literature.

PURPOSE: Non-traumatic orbital hemorrhage without underlying vascular malformations or predisposing conditions is uncommon, and particularly rare in the context of maternal labor. This study combines a novel case report and retrospective review to analyze reported cases and propose insights. METHODS: This study is both a unique case report and literature review examining PubMed publications with articles traced back to original sources through citations for inclusion. Analysis included clinical presentation, visual examination, hematoma characteristics, neuroimaging, management strategies, and outcomes. RESULTS: We present a 37-year-old multigravida woman at 40 weeks gestation who developed acute right-sided proptosis, diplopia, retrobulbar pain, and periorbital edema during the second stage of labor. Computed tomography (CT) revealed a subperiosteal hemorrhage, with subsequent magnetic resonance imaging (MRI) excluding vascular anomalies. Symptoms resolved within two months. Only 14 cases of maternal orbital hematoma associated with labor have been reported. The average age was 28 with 42% (6/14) being primigravid. Including our case, forty percent (6/15) developed symptoms during the second stage of labor, 40% (6/15) immediately postpartum, and 20% (3/15) over 24 hours postpartum. Overall, 33% (5/15) had potentially contributing conditions including coagulopathies, delivery complications, or vascular malformations. Unilateral orbital hemorrhage occurred in 87% (13/15). Surgical intervention was necessary in 13% (2/15). Most (87%, 13/15) underwent observation or medical management with full recovery of symptoms. CONCLUSIONS: Non-traumatic orbital hematomas associated with maternal labor are rare and likely related to increased valsalva during delivery and heightened blood volume in pregnancy. Neuro-imaging and systemic workup are recommended to assess for vascular anomalies or underlying coagulopathies. The overall prognosis is favorable with most having full recovery.

The association between sinonasal anatomical variants and the laterality of orbital complications in pediatric acute rhinosinusitis.

INTRODUCTION: As the role of sinonasal anatomical variants as predisposing factors in determining the lateralization of acute rhinosinusitis-related orbital complications (ARS-OC) in pediatrics remains a topic of debate, this study further explores the potential association between anatomical variations and ARS-OC. METHODS: A retrospective study was conducted on children who had been admitted with ARS-OC using medical records and sinus CT scans to compare anatomical differences between the affected and contralateral sides. This study aimed to identify bony anatomical disparities that may impact OC laterality secondary to ARS. The anatomical features examined included septal deviation, concha bullosa, lamina papyracea dehiscence (LPD), and uncinate process abnormalities. RESULTS: The CT scans of 57 pediatric patients (114 sides) were reviewed. Our results indicated that bony anatomical variations were associated with ARS-OC laterality (63 % vs. 37 %, P = 0.006), yielding an odds ratio of 2.91. Additionally, our study revealed a significant association between ipsilateral LPD with the increased risk of ARS-OC (39 % vs. 1.8 %, P < 0.05), with an odds ratio of 34.3 compared to the opposite side. CONCLUSIONS: LPD might play a role in the pathophysiology of pediatric ARS-OC, as it is associated with a significantly higher risk of affecting the ipsilateral side. Further research is necessary to determine whether LPD is a causative factor or a result of ARS.

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.

Case series of central retinal artery occlusion in COVID-19-associated rhino-orbital-cerebral mucormycosis.

Rhino orbital Mucormycosis caused by filamentous fungus of mucoraceae family was considered a rare disease affecting immunocompromised and diabetics with ketoacidosis until the recent COVID 19 pandemic. We are presenting a series of six cases of Rhino orbital cerebral Mucormycosis with central retinal artery occlusion. All six cases had common history of COVID 19 infection in recent past with sinusitis, proptosis and total ophthalmoplegia with central retinal artery occlusion on presentation. MR imaging showed invasive pan sinusitis with orbital and cerebral involvement. Urgent debridement was done and histopathological examination showed broad, filamentous aseptate fungi suggestive of Mucormycosis. All patients inspite of intravenous Amphotericin B with local debridement did not show any improvement and expired within a week of presentation. Hence our study shows poor prognosis of post covid 19 associated Mucormycosis with central retinal artery occlusion.

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.