Histiocytosis and adult-onset orbital xanthogranuloma in 2023: a review of the literature and mini case series.

PURPOSE: Within the large umbrella of histiocytosis are a few similar yet heterogenous entities involving the orbit and periocular tissues with or without systemic infiltration, termed adult onset xanthogranuloma or orbital xanthogranuloma. Due to rarity of these conditions, different classifications in use, diverse clinical presentations and still unknown etiology, the aim of this paper was to provide an up-to-date literature review of the actual understanding of histiocytosis and its subgroups involving the orbit and periocular area, diagnostic strategies and therapeutic modalities. METHODS: We present a review of literature and small case series comprising four patients diagnosed and treated in the period from 2001 until 2023 in our hospital. Clinical files of 4 patients with adult-onset xanthogranulomatous disease of the orbit and ocular adnexa (AOXGD) were reviewed retrospectively. Clinical, laboratory, radiological, histopathological, and immunohistochemical findings were reexamined. RESULTS: Reviewing medical records of our patients with AOXGD, we found significant overlap between histiocytosis and different immune disorders. A broad workup should be considered in these patients as they can harbour severe immune disfunctions and hematologic disorders. Preferred treatment modality depends on a histopathologic type of AOXGD, clinical presentation and systemic involvement and should be conducted multidisciplinary. CONCLUSION: The diagnosis is often delayed because of its rarity and diverse clinical findings. Development of molecular genetic tests, detection of BRAF V600E mutation and different types of kinase mutations, mutations in transcriptional regulatory genes as well as tyrosine kinase receptors have shed a new light on the etiopathogenesis and potential targeted treatment of histiocytosis.

Marginal Full Thickness Blepharotomy for Management of Orbital Compartment Syndrome.

PURPOSE: To assess the utility of a marginal full thickness blepharotomy (MFTB) for the treatment of orbital compartment syndrome. METHODS: An experimental study design employing a cadaver model for orbital compartment syndrome was used to assess the efficacy of an MFTB. Elevated orbital compartment pressures were created in 12 orbits of 6 fresh cadaver heads. Intraocular pressure, as an analog of orbital pressure, was measured before and after inferior and superior MFTBs were performed. Statistical analysis was performed on the collected data to assess the efficacy of the procedure. RESULTS: Both procedures were found to significantly lower the orbital compartment pressure. MFTB of the inferior lateral eyelid decreased orbital compartment pressure by an average of 62.2 mm Hg (95% CI, 56.9-67.5). MFTB of the superior lateral eyelid following MFTB of the inferior lateral eyelid decreased the orbital compartment pressure by an additional average of 10.3 mm Hg (total average reduction of 72.5 mm Hg; 95% CI, 68.1-76.9). CONCLUSIONS: Orbital compartment syndrome is a time-sensitive vision-threatening emergency that requires prompt diagnosis and intervention to prevent irreversible vision loss. The authors describe the MTFB, a simple one-step procedure that when performed correctly results in a significant decrease in orbital compartment pressure, making it a viable option when canthotomy and cantholysis fails or is unable to be performed.

ACR Appropriateness Criteria® Orbital Imaging and Vision Loss-Child.

Orbital disorders in children consist of varied pathologies affecting the orbits, orbital contents, visual pathway, and innervation of the extraocular or intraocular muscles. The underlying etiology of these disorders may be traumatic or nontraumatic. Presumed location of the lesion along with the additional findings, such as eye pain, swelling, exophthalmos/enophthalmos, erythema, conjunctival vascular dilatation, intraocular pressure, etc, help in determining if imaging is needed, modality of choice, and extent of coverage (orbits and/or head). Occasionally, clinical signs and symptoms may be nonspecific, and, in these cases, diagnostic imaging studies play a key role in depicting the nature and extent of the injury or disease. In this document, various clinical scenarios are discussed by which a child may present with an orbital or vision abnormality. Imaging studies that might be most appropriate (based on the best available evidence or expert consensus) in these clinical scenarios are also discussed. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision process support the systematic analysis of the medical literature from peer reviewed journals. Established methodology principles such as Grading of Recommendations Assessment, Development, and Evaluation or GRADE are adapted to evaluate the evidence. The RAND/UCLA Appropriateness Method User Manual provides the methodology to determine the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where peer reviewed literature is lacking or equivocal, experts may be the primary evidentiary source available to formulate a recommendation.

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 intramuscular hydatid cyst causing compressive optic neuropathy: a case report and literature review.

BACKGROUND: Echinococcosis, commonly known as hydatid disease, is a zoonotic infection resulting from the tapeworm Echinococcus granulosus. The occurrence of hydatid cysts in the orbital region is uncommon, representing less than 1% of all reported hydatid cases. This report details a unique case of an intramuscular hydatid cyst in the orbital region that led to compressive optic neuropathy. CASE PRESENTATION: A 22-year-old male from Kabul, Afghanistan presented with a five-month history of progressive proptosis in his left eye, associated with a gradual decrease in vision over the past three weeks. The left eye exhibited upward globe dystopia, ocular motility limitation, mild conjunctival injection, and chemosis. Diagnosis was achieved through imaging and histopathological examination. Treatment involves surgical removal of the cyst and prolonged albendazole therapy. The postoperative course showed significant improvement in the patient's condition and restoration of his vision. CONCLUSIONS: Despite its rarity, this case underscores the importance of awareness and knowledge of hydatid disease among physicians, especially those working in endemic areas. It emphasizes the importance of including hydatid disease in the differential diagnosis of orbital masses, particularly in endemic regions.

Orbital Giant Cell Reparative Granuloma: A Case Report.

Giant cell reparative granuloma has a very low incidence and is thought to be a response to trauma. While there have been only a few reported cases of orbital giant cell reparative granuloma, we recently observed such a case and analyzed 16 previously reported cases of this type. It is important to note that further investigation is necessary to fully understand the relationship between giant cell reparative granuloma and trauma.

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.

[Interdisciplinary Management of Orbital Diseases]. / Interdisziplinäre Therapie der Erkrankungen der Orbita.

Diagnosis and therapy of orbital diseases is an interdisciplinary challenge, in which i.e. otorhinolaryngologists, ophthalmologists, radiologists, radiation therapists, maxillo-facial surgeons, endocrinologists, and pediatricians are involved. This review article describes frequent diseases which both, otolaryngologists and ophthalmologists are concerned with in interdisciplinary settings. In particular the inflammatory diseases of the orbit including orbital complications, autoimmunological diseases of the orbit including Grave´s orbitopathy, and primary and secondary tumors of the orbit are discussed. Beside describing the clinical characteristics and diagnostic steps the article focusses on the interdisciplinary therapy. The review is completed by the presentation of most important surgical approaches to the orbit, their indications and possible complications. The authors tried to highlight the relevant facts despite the shortness of the text.

Features and evaluation of mucormycosis in COVID-19 patients from two referral hospitals in Iran.

OBJECTIVES: The present study aimed to assess the features, clinical characteristics, and species diversity among patients admitted to referral Hospitals for SARS-CoV-2 pneumonia and mucormycosis in Tehran, Iran, and the relationship between seasonal and species diversity was considered. METHODS: Confirmed COVID-19 patients with a positive reverse-transcriptase real-time (rRT-PCR) test for SARS-CoV2 were primarily included based on clinically suspected mucormycosis infection and confirmed by histopathology and mycology examination of biopsy specimens. The PCR technique was performed by the amplification of the high-affinity iron permease 1 (FTR1) gene for identification and discrimination between Rhizopus arrhizus and non- Rhizopus arrhizus isolates. In contrast, species identification of non-Rhizopus arrhizus was performed by sequencing of ITS rDNA region. RESULTS: Rhino-sino-orbital mucormycosis was identified in the majority of cases (n = 33), with 66 % and 34 % of the cases involving male and female patients, respectively. Rhizopus arrhizus was found to be the most prevalent (84.6 %), followed by Mucor circinelloides (7.6 %). Rhizopus arrhizus was the most prevalent species and present in all the seasons; however, Mucor circinelloides was only present in the autumn. The overall mortality of the total population was 24.6 % (16/ 65); the mortality rates occurring in patients diagnosed with rhino-sino-orbital infection and rhino-sinusal form were 21.4 % and 25 %, respectively. CONCLUSION: CAM can be a serious complication of severe COVID-19, especially in patients with uncontrolled diabetes. It is important to monitor the epidemiology of mucormycosis to raise awareness of the disease and improve diagnosis, treatment and prognosis, particularly in the setting of pandemic.

A Case of Lepromatous Leprosy (Lucio's Phenomenon) Presenting as Periorbital Edema.

Despite low prevalence of leprosy worldwide, new cases continue to present and require swift evaluation and diagnosis to prevent complications. Here, we describe a case of lepromatous leprosy with Lucio's phenomenon initially presenting with facial and periorbital edema. A 38-year-old Brazilian woman presented to the emergency department with facial swelling and erythema, initially treated as cellulitis. Due to rapid worsening despite broad-spectrum antibiotics, she underwent soft tissue exploration and biopsy due to concern for necrotizing fasciitis. During her course, she also developed retiform purpura of bilateral upper and lower extremities. Periorbital and lower extremity pathological specimens ultimately revealed acid-fast bacilli consistent with Mycobacterium leprae , and the patient improved with multidrug therapy. This case illustrates the diagnostic difficulty of lepromatous leprosy with Lucio's phenomenon, which can initially present with periorbital edema.

Traumatic terson syndrome with a peculiar mass lesion and tractional retinal detachment: a case report.

BACKGROUND: To report a case with bilateral Terson syndrome presented with a unique mushroom-like mass lesion on the optic disc along with proliferative vitreoretinopathy and tractional retinal detachment. CASE PRESENTATION: A 33-year-old man was injured during a traffic accident and had diffuse brain swelling and intraocular hemorrhage. Poor vision in both eyes was noted after the patient regained consciousness. B-scan ultrasonography showed extensive vitreous opacity with a posterior vitreous detachment and without obvious retinal detachment. Vitrectomy was performed in both eyes five months after the accident. After clearing up the vitreous opacity, a peculiar pigmented mushroom-like mass lesion was noted in the posterior pole and had severe adhesion to the underneath optic disc. Extensive multilayered peripapillary epiretinal membrane was found covering the posterior pole and led to tractional retinal detachment around the macula. The mass was presumed to be an organized vitreous hemorrhage originated from the optic disc. The extensive and adherent epiretinal membrane together with the mass lesion were removed as much as possible and silicon oil was injected for tamponade. However, in the right eye, the retina redetached under silicon oil, whereas in the left eye, his vision improved to 20/100. CONCLUSIONS: Terson syndrome usually has a favorable prognosis but may be complicated by proliferative vitreoretinopathy and tractional retinal detachment. Careful monitoring is warranted and early vitrectomy should be considered in cases suspecting additional pathologies.

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.

A Patient With Type 1 Diabetes and Acute Rhinosinusitis.

A 41-year-old with type 1 diabetes had generalized weakness, muffled voice, and slurred speech. Neck computed tomography showed soft-tissue gas in the nasopharynx and prevertebral fascia; examination of sinus mucosal samples identified numerous broad, nonseptate right-angled hyphae and fruiting bodies. What is the diagnosis and what would you do next?

Rapid progression of orbital abscess requiring lateral canthotomy in a pediatric patient.

Subperiosteal abscess (SPA) is a rare complication of acute sinusitis in children that may develop rapidly. In this case report, we describe an 11 year-old boy who presented with a large SPA 2 days after being diagnosed with conjunctivitis. The patient required emergent lateral canthotomy and cantholysis (LCC), IV antibiotics, and emergent surgery. It is crucial that emergency physicians be able to identify and treat this vision-threatening complication.

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.