Intraocular Lens Exchange: Indications, Comparative Outcomes by Technique, and Complications.

Purpose: To describe the indications, outcomes, and complications associated with intraocular lens (IOL) exchange. Patients and Methods: To determine the relative frequency of postoperative complications between techniques for all patients undergoing IOL exchange from May 1, 2014 through August 31, 2020. Results: IOL exchange was performed in 511 eyes of 489 patients (59.7% men; mean age: 67.0 ± 13.9 years, median time from cataract procedure to IOL exchange: 47.5 months). Mean uncorrected visual acuity significantly improved from 20/192 Snellen equivalent (logMAR 0.981) preoperatively to 20/61 (logMAR 0.487) at last follow-up (P < 0.001). Overall, 384 eyes (78.7%) met their desired refractive outcome within ±1.0 diopter (D). The most frequent complication was cystoid macular edema (CME) (n=39, 7.6%). Iris-sutured technique was associated with significantly greater frequency of subsequent IOL dislocation (10.3%) than 4-point scleral sutured (0%, P = 0.002), anterior chamber IOL (ACIOL, 1.5%, P = 0.01), and 2-point scleral sutured (0%, P = 0.03) techniques. Yamane scleral-fixation technique was associated with significantly greater frequency of developing IOL tilt (11.8%) than ACIOL (0%, P = 0.002), 4-point scleral sutured (1.1%, P = 0.01), 2-point scleral sutured (0%, P = 0.04), and iris-sutured (0%, P = 0.04) techniques. Conclusion: IOL exchange significantly improved uncorrected visual acuity and more than three-quarters of eyes met the refractive goal. Certain techniques were associated with complications, including subsequent dislocation associated with iris-sutured technique and IOL tilt associated with Yamane scleral-fixation technique. This information may help guide surgeons in deciding between procedural techniques for individual patients during IOL exchange preoperative planning.

Trends in Sports-Related Ocular Trauma in United States Emergency Departments from 2010 to 2019: Multi-Center Cross-Sectional Study.

IMPORTANCE: Traumatic eye injury is one of the leading causes of visual impairment in the United States, but there is limited information available in the literature about ocular trauma related to sports. OBJECTIVE: To evaluate the primary ocular diagnosis, type of sport, seasonal prevalence, and injury severity following ocular sports-related trauma. DESIGN: Cross-sectional study. SETTING: United States (US) hospital emergency departments (ED). PARTICIPANTS: Patients with sports-related ocular injuries in the National Electronic Injury Surveillance System All Injury Program (NEISS-AIP) database from January 1, 2010 to December 31, 2019. MAIN OUTCOME AND MEASURE: To identify and compare the type of sport, primary diagnosis of ocular injury, and injury severity. RESULTS: For 4,671 sports-related ocular injuries identified, the mean age of injury was 19.4±15.2 years (median: 15.0 years, 79% male). The largest proportion of injuries occurred in the 12-17 years of age cohort (38.6%), occurring during summer months (P< .001). The most common sports associated with eye injury were basketball (37.8%), baseball (13.8%),, and football (12.3%). There were 3,214 injuries (68.8%) deemed 'minor' anterior segment injuries, 359 injuries (7.7%) deemed 'major' anterior segment injuries, 31 injuries (0.7%) deemed 'minor' posterior segment injuries, 77 injuries (1.7%) deemed 'major' posterior segment injuries, and 990 injuries (21.2%) of unknown severity. Basketball-related ocular trauma had a greater frequency of corneal/scleral abrasions than baseball (P < .001), soccer (P < .001), tennis (P = .03), softball (P = .001), and paintball (P = .02). Baseball-related ocular trauma had a greater frequency of contusions than basketball (P < .001), football (P < .001), soccer (P < .001), volleyball (P< .001). Paintball and soccer were more associated with 'major' anterior and posterior ocular injuries than basketball (P < .001, for both). CONCLUSION AND RELEVANCE: Sports-related trauma remains a highly prevalent cause of eye-related visits to the emergency room, particularly in young male adolescents in the spring and summertime. While most diagnoses were deemed 'minor' injuries with basketball associated with corneal/scleral abrasions and baseball with contusions, paintball, and soccer were more significantly associated with 'major' anterior and posterior ocular injuries.

Clinical Neuroimaging of Photophobia in Individuals With Chronic Ocular Surface Pain.

PURPOSE: To examine neural mechanisms underlying photophobia in individuals with chronic ocular surface pain by using functional magnetic resonance imaging (fMRI). DESIGN: Cross-sectional case/control analysis. METHODS: A total of 16 individuals from the Miami Veterans Affairs eye clinic underwent comprehensive ocular surface evaluations and were surveyed for ocular surface symptoms. Case patients included patients who reported chronic ocular surface pain symptoms and light sensitivity at least most of the time over 1 week. Controls included persons without chronic ocular surface pain who reported no or minimal light sensitivity. All patients viewed light stimuli during 2 fMRI scans, one before and one after topical anesthetic instillation, and rated their level of pain intensity to the stimulus at the end of each scan. Areas of brain activation in response to light stimuli presentation were correlated with pain responses and examined post- vs pre-anesthesia. RESULTS: Case patients (n = 8) reported higher pain intensity ratings than controls (n = 8) in response to light stimuli during fMRI. Case patient ratings correlated more with light-evoked activation in pain-related areas within the trigeminal brainstem, primary somatosensory cortex (S1), anterior mid-cingulate cortex (aMCC), and insula than in controls. Topical anesthesia led to varying responses in pain ratings among case patients as well as decreased light-evoked activation in S1 and aMCC. CONCLUSIONS: The trigeminal nociceptive system may contribute to photophobia in individuals with chronic ocular surface pain. We demonstrate modulation of cortical structures in this pathway with topically applied anesthetic to the eyes. Further understanding of modulatory interactions that govern ocular surface pain and photophobia is critical for developing effective, precision-based therapies.

Case-Control Study Examining the Composition of the Gut Microbiome in Individuals With and Without Immune-Mediated Dry Eye.

PURPOSE: Gut microbiome alterations have been associated with various autoimmune diseases. There are limited data, however, on relationships between gut dysbiosis and immune-related dry eye (DE). Our aim was to compare the gut microbiome composition of individuals with early and late markers of Sjögren syndrome (SS) with controls without DE. METHODS: We compared 20 individuals with positive early markers [antisalivary protein 1 (SP1), antiparotid secretory protein (PSP), anticarbonic anhydrase 6 (CA6) IgG, IgA, and IgM, n = 19)], or late markers (anti-Ro/SS-A and anti-La/SS-B, n = 1) of SS with no comorbid autoimmune diagnoses and 20 age-matched and sex-matched controls. Collected stool samples underwent deep RNA sequencing. The main outcomes measured included gut microbiome composition and diversity. RESULTS: A total of 20 cases [Dry Eye Questionnaire-5 15.2 ± 3.4, Ocular Surface Disease Index 55.1 ± 22.8, and Schirmer 7.1 ± 5.2 mm] were compared with 20 controls (Dry Eye Questionnaire-5 4.8 ± 3.8, Ocular Surface Disease Index 14.2 ± 12.3, and Schirmer 20.4 ± 9.2 mm). No differences were observed in α-diversity (P = 0.97) or overall community structure (P = 0.62). Between groups, 32 species were differentially abundant (P < 0.01). Among cases, 27 were relatively more abundant, including 10 Lactobacillus and 4 Bifidobacterium species. A relative depletion of 5 species was found in cases compared with controls, notably Fusobacterium varium and Prevotella stercorea. CONCLUSIONS: Differences in gut microbiome composition were found in individuals with mostly early markers of SS compared with controls. However, their clinical significance to DE manifestations remains unclear. Further studies are needed to elucidate the role of gut dysbiosis on immune dysregulation and disease activity in the various forms of immune-mediated DE.

Recurrent Hiatal Hernia Resulting in Rightward Mediastinal Shift: Diagnostics in Cardiology and Clinical Pearls.

On radiographic imaging, the finding of a right-sided heart location can be due to multiple etiologies and may be congenital or acquired. We present the case of a 71-year-old male with a self-reported past medical history of hiatal hernia and previously diagnosed dextrocardia. The patient experienced cardiovascular intervention following an ST-elevation myocardial infarction. In the cardiac workup, a low-voltage normal electrocardiogram confirmed dextroposition of the heart due to significant herniation of gastric contents into the thoracic cavity. This gentleman had presumably been diagnosed with dextrocardia, a right-left reversal of heart anatomy and electrophysiology, based on imaging and incomplete workup. Dextroposition refers to a rightward shift of the mediastinum with no changes in orientation of cardiac anatomy, and therefore unchanged directional orientation of conduction. This is an important distinction from dextrocardia, a mirror-image reversal of the cardiac chambers and heart location in the chest wall, such as that due to congenital ciliary dysfunction. A sliding hernia is an uncommon cause of the rightward mediastinal shift, with few such cases documented in the literature, and cardiovascular manifestations of hiatal hernias are discussed. This case exemplifies the role of an electrocardiogram in distinguishing between dextrocardia and dextroposition for accurate diagnosis and management.

Relationships between activated dendritic cells and dry eye symptoms and signs.

PURPOSE: To examine whether "activated" dendritic cells (aDCs) could serve as a biomarker of systemic immune disorders in individuals with dry eye (DE) symptoms. Secondarily, to examine the impact of a topical anti-inflammatory agent on aDC number. METHODS: Retrospective analysis was conducted to identify individuals with DE symptoms who had in-vivo confocal microscopy (IVCM) imaging between October 2018 and July 2020 at the Miami Veterans Hospital. aDCs were manually quantified based on morphology. Receiver operating curve (ROC) analysis examined relationships between aDC number and systemic immune disease status. Individuals were then grouped by aDC number (≥2 versus <2) and demographics and DE parameters were examined. Paired t-test was performed to evaluated aDC number pre-vs post-initiation of an anti-inflammatory agent. RESULTS: 128 individuals were included. Their mean age was 57.1 ± 15.0 years; 71.1% were male, 53.1% self-identified as White and 24.2% as Hispanic. The mean number of aDCs in the central cornea was 1.28 ± 2.16 cells/image. The presence of ≥2 aDCs had a sensitivity of 60% and specificity of 77% for the diagnosis of a systemic immune disorder. Individuals with ≥2 aDCs were more likely to self-identify as Black, have Secondary Sjögren's, and have higher nerve fiber area and fractal dimension. In 12 individuals, aDC number decreased from 2.69 ± 2.36 to 0.58 ± 0.73 cells/image after initiation of an anti-inflammatory agent, p = 0.01. CONCLUSIONS: The presence of ≥2 aDCs in the central cornea suggests a systemic immune disorder in individuals with DE symptoms. Topical anti-inflammatory therapy can reduce the number of aDCs in the central cornea.

Multifocal Post-Traumatic Myositis Ossificans Circumscripta in a Young Male Following a Motor Vehicle Accident: A Review of Imaging and Clinical Presentation.

Traumatic myositis ossificans (MO) circumscripta is an uncommon nonhereditary pathophysiological result of muscular trauma that is detected by radiographic imaging three to four weeks following initial trauma. It is responsible for great global morbidity, with symptoms of prolonged pain, diminished flexibility, and stiffness. There is frequently a delay in diagnosis due to the generalized symptoms and varying radiographic presentation. The goal of therapy is to rule out serious complications (such as soft tissue sarcoma) and to restore strength and range of motion (ROM) as soon as possible. Here we detail the case of a 32-year-old male with a delayed diagnosis of MO who presented to the hospital with left lower extremity pain and swelling following a motor vehicle accident (MVA) that occurred one month prior.

Long-Term Trigeminal Nerve Stimulation as a Treatment for Ocular Pain.

OBJECTIVES: Ocular pain symptoms (e.g., hypersensitivity to light and wind, "burning" sensations) can be debilitating and difficult to treat. Neuromodulatory therapies targeting sensory trigeminal and central pain pathways may help treat chronic ocular pain refractory to traditional therapies. The current study evaluates the long-term effects of a trigeminal neurostimulator (TNS) on ocular pain. MATERIALS AND METHODS: Retrospective review of 18 individuals at the Miami Veterans Affairs Eye Clinic with chronic, severe ocular pain who were prescribed and used TNS at home for ≥3 months. The primary outcome measures were 1) ocular symptom intensity over a 24-hour recall period (dryness, pain, light sensitivity, wind sensitivity, burning; rated on 0-10 scales) captured pre-TNS and at monthly follow-up intervals and 2) side effects. The frequency and duration of TNS was a secondary outcome measure. RESULTS: The mean age of the population (n = 18) was 57.5 years (range, 34-85 years) with a male majority (67%). Two individuals discontinued use due to lack of efficacy and one due to confounding health issues. Initial mean weekly frequency of TNS use was 3.7 ± 1.9 sessions of 25.8 min at month 1 and 2.7 ± 2.3 sessions of 28.0 min at month 6. At six months, pain intensity (↓ 31.4%), light sensitivity (↓ 36.3%), wind sensitivity (↓ 32.6%), and burning sensation (↓ 53.9%) were all decreased compared to baseline (p < 0.01 for all); greater decreases in ocular pain were noted in individuals with migraine (n = 10) than those without migraine (n = 8). No significant change was noted in mean dryness scores. Fifteen subjects experienced sedation with TNS use, persisting throughout the follow-up visits. No other adverse effects were communicated by any subjects. CONCLUSION: Our study suggests TNS is a safe, adjunctive treatment option in individuals with severe, chronic ocular pain. Individuals demonstrated gradual, continual improvement in pain symptoms over time within a multimodal approach.

Dry eye: why artificial tears are not always the answer.

Dry eye disease (DED) is a multifactorial disease that manifests in patients with a variety of symptoms and signs such as ocular pain, visual issues, rapid tear evaporation and/or decreased tear production. It is a global health problem and is the leading cause of optometry and ophthalmology clinic visits. The mainstay therapy for DED is artificial tears (ATs), which mimics tears and improves tear stability and properties. ATs have been found to improve symptoms and signs of disease in all DED subtypes, including aqueous deficient DED and evaporative DED. However, given the heterogeneity of DED, it is not surprising that ATs are not effective in all patients. When AT fails to relieve symptoms and/or signs of DED, it is critical to identify the underlying contributors to disease and escalate therapy appropriately. This includes underlying systemic diseases, meibomian gland dysfunction, anatomical abnormalities and neuropathic dysfunction. Thus, this review will discuss the benefits and limitations of ATs and review conditions when escalation of therapy should be considered in DED.

Disseminated Mycobacterium avium Intracellulare Infection With Concurrent Small Bowel Obstruction: Case, Pathophysiology, and Clinical Considerations.

Mycobacterium avium intracellulare (MAI) is an opportunistic infection that typically manifests itself as pulmonary infection. In immunocompromised patients, however, MAI can uncommonly cause disseminated disease and diffuse gastrointestinal involvement. Small bowel obstruction with concurrent MAI infection is rarely documented in literature. Here, a 60-year-old female with a past medical history significant for a gastrointestinal stromal tumor, two small bowel obstructions, and a bowel perforation repair presented to the emergency department with sharp abdominal pain due to a small bowel obstruction. Cultures obtained from the laparoscopic release of small bowel obstruction confirmed the presence of MAI. An antibiotic course of ethambutol, azithromycin, and rifampin was initiated and continued upon transfer to a long-term acute care facility. We describe this case to highlight the possibility of MAI infection in patients with postoperative abdominal pain resulting from small bowel obstruction, review the underlying pathophysiology, and discuss its epidemiology.

Milk-Alkali Syndrome as a Cause of Hypercalcemia in a Gentleman With Acute Kidney Injury and Excessive Antacid Intake.

Among the pertinent differentials for hypercalcemia, milk-alkali syndrome remains a diagnosis of exclusion following a thorough workup of other severe causes. However, several key signs may increase a clinician's index of suspicion for possible milk-alkali syndrome, including a prolonged history of antacid ingestion. Milk-alkali syndrome commonly presents with a classic triad: hypercalcemia, metabolic alkalosis, and acute kidney injury. The diagnostic workup should include evaluation of both serum and urine calcium levels, serum phosphate levels, and other hormones (parathyroid hormone, vitamin D). In the case of a confirmed diagnosis of milk-alkali syndrome, rapid correction of calcium levels is of utmost importance. We present the case and workup of an individual presenting to the emergency room with hypercalcemia, acute kidney injury, and several key systemic symptoms. Given a significant history of antacid overuse, and a thorough diagnostic workup, a diagnosis of milk-alkali syndrome was made and the patient was treated accordingly, making a full recovery. We review this rare case and important clinical pearls regarding milk-alkali syndrome.

Photophobia: shared pathophysiology underlying dry eye disease, migraine and traumatic brain injury leading to central neuroplasticity of the trigeminothalamic pathway.

BACKGROUND: Photophobia is a potentially debilitating symptom often found in dry eye disease (DE), migraine and traumatic brain injury (TBI). METHODS: We conducted a review of the literature via a PubMed search of English language articles with a focus on how photophobia may relate to a shared pathophysiology across DE, migraine and TBI. RESULTS: DE, migraine and TBI are common conditions in the general population, are often comorbid, and share photophobia as a symptom. Across the three conditions, neural dysregulation of peripheral and central nervous system components is implicated in photophobia in various animal models and in humans. Enhanced activity of the neuropeptide calcitonin gene-related peptide (CGRP) is closely linked to photophobia. Current therapies for photophobia include glasses which shield the eyes from specific wavelengths, botulinum toxin, and inhibition of CGRP and its receptor. Many individuals have persistent photophobia despite the use of these therapies, and thus, development of new therapies is needed. CONCLUSIONS: The presence of photophobia in DE, migraine and TBI suggests shared trigeminothalamic pathophysiologic mechanisms, as explained by central neuroplasticity and hypersensitivity mediated by neuropeptide CGRP. Treatment strategies which target neural pathways (ie, oral neuromodulators, transcutaneous nerve stimulation) should be considered in patients with persistent photophobia, specifically in individuals with DE whose symptoms are not controlled with traditional therapies.

How Should Corneal Nerves be Incorporated Into the Diagnosis and Management of Dry Eye?

PURPOSE: a)Confocal microscopy and aethesiometry have allowed clinicians to assess the structural and functional integrity of corneal nerves in health and disease. This review summarizes literature on nerves in dry eye disease (DED) and discusses how this data can be applied to DED diagnosis and treatment. RECENT FINDINGS: b)Subjects with DED have a heterogenous symptom and sign profile along with variability in nerve structure and function. Most studies have reported lower nerve density and sensitivity in aqueous tear deficiency, while findings are more inconsistent for other DED subtypes. Examining nerve status, along with profiling symptoms and signs of disease, can help categorize subjects into disease phenotypes (structural and functional patterns) that exist under the umbrella of DED. This, in turn, can guide therapeutic decision-making. SUMMARY: c)Due to the heterogeneity in symptoms and signs of DED, corneal nerve evaluations can be valuable for categorizing individuals into disease sub-types and for guiding clinical decision making.

Corneal Nerve Abnormalities in Ocular and Systemic Diseases.

The trigeminal nerve gives rise to the corneal subbasal nerve system, which plays a crucial role in sensations of touch, pain, and temperature and in ocular healing processes. Technological advancements in instruments, in particular in vivo confocal microscopy and aethesiometry, have allowed for the structural and functional evaluation of corneal nerves in health and disease. Through application of these technologies in humans and animal models, structural and functional abnormalities have been detected in several ocular and systemic disorders, including dry eye disease (DED), glaucoma, migraine, and fibromyalgia. However, studies across a number of conditions have found that structural abnormalities do not always relate to functional abnormalities. This review will discuss instruments used to evaluate corneal nerves and summarize data on nerve abnormalities in a number of ocular and systemic conditions. Furthermore, it will discuss potential treatments that can alleviate the main manifestations of nerve dysfunction, namely ocular surface pain and persistent epithelial defects.

Digital Screen Use and Dry Eye: A Review.

Prolonged and continuous daily use of digital screens, or visual display terminals (VDTs), has become the norm in occupational, educational, and recreational settings. An increased global dependence on VDTs has led to a rise in associated visual complaints, including eye strain, ocular dryness, burning, blurred vision, and irritation, to name a few. The principal causes for VDT-associated visual discomfort are abnormalities with oculomotor/vergence systems and dry eye (DE). This review focuses on the latter, as advances in research have identified symptomology and ocular surface parameters that are shared between prolonged VDT users and DE, particularly the evaporative subtype. Several mechanisms have been implicated in VDT-associated DE, including blink anomalies, damaging light emission from modern devices, and inflammatory changes. The presence of preexisting DE has also been explored as an inciting and exacerbating factor. We review the associations between digital screens and DE, mechanisms of damage, and therapeutic options, hoping to raise awareness of this entity with the goal of reducing the global morbidity and economic impact of screen-associated visual disability.

Abdominal Supracervical Hysterectomy With Bilateral Salpingo-Oophorectomy as the Surgical Approach for a 22-Week Uterus With Incidental Endometrial Polyp Focal Atypia.

A 49-year-old perimenopausal female presented with abnormal uterine bleeding (AUB) and chronic lower abdominal pain with associated urinary urgency. The patient elected to have an abdominal supracervical hysterectomy with bilateral salpingo-oophorectomy for a large, symptomatic fibroid uterus. Preoperative ultrasounds revealed a uterine size of 22 x 20 x 17 cm and a 15.9 x 13 x 9 x 9.2 cm subserosal fibroid occupying the majority of the fundus and body of the uterus. Under general anesthesia, abdominal supracervical hysterectomy and bilateral salpingo-oophorectomy with a midline vertical incision were completed. Pathology reported a uterus with multiple leiomyomata as well as endometrial polyps with focal atypical endometrial hyperplasia and squamous metaplasia. Overall, the uterine corpus with one attached adnexa weighed 3433 g and was 25.8 x 20.3 x 15cm. Choice of surgical approach in a hysterectomy depends upon clinical circumstances, the surgeon's technical expertise, and patient preference. Although minimally invasive hysterectomies via vaginal and laparoscopic approaches are now preferred due to decreased hospitalization stays and postoperative recovering times, individualized treatment plans for patients should be considered depending on uterine size and the possibility of not achieving adequate exposure, which may lead to complications. As this case presents, an abdominal hysterectomy is an important option for certain patients where the use of other approaches could pose significant risk.

Ocular Surface Pain: A Narrative Review.

Ocular surface pain is a frequent cause of visits to an eye care provider and has a substantial impact on healthcare cost, yet a complete understanding of its causative factors and tools for diagnostic workup are notably missing in many eye clinics. The cornea has the densest sensory innervation in the human body and has the potential to be a powerful producer of pain. Pain can manifest as a result of a noxious stimulus or disruption in the ocular surface anatomy (nociceptive pain), or it can result from abnormalities in the ocular surface neurosensory apparatus itself (neuropathic pain). Novel advances in neurobiology have sought to differentiate the two entities, particularly to identify when chronic dry eye symptomatology is driven by neuropathic ocular pain. In this review, we seek to provide an overview of the prevalence, physiologic factors, and management of ocular surface pain complaints.

Complicated Surgical Removal of an Adherent Port-a-Cath After 11 Years of Implantation.

A 65-year-old woman with a medical history significant for anal cancer was referred by her primary care physician for a port-a-cath removal. The port was placed prior to treatment of squamous cell carcinoma of the anus, 11 years prior to this scheduled removal. She received chemotherapy and radiation in accordance with the Nigro protocol, treating the anal cancer to complete resolution. During port removal, a fibrous capsule was dissected and the port was removed from the left upper breast border along with proximal portion of the catheter. Significant difficulty was found in removing the remaining catheter despite sustained traction and guidewire insertion. Fluoroscopy revealed an intravascular adhesion of the catheter tip in the superior vena cava, which could not be freed. In order to prevent vascular injury, the adhesed portion of the distal catheter was left in place with three large surgical clips placed distally. This case highlights the very rare complication of complete vascular adherence of the terminal catheter tip and extended port intracorporeal time as a risk factor for adhesion. This case also highlights the importance of timely permanent central venous catheter removal following completion of its intended regimen.

Rosai-Dorfman Disease and Unusual Local Invasive Presentation.

Rosai-Dorfman disease (RDD) is a rare medical condition with bilateral painless lymphadenopathy. We present the case of a young man diagnosed with a very unique presentation of Rosai-Dorfman disease. A 40-year-old African-American man presented with a firm, non-tender, progressive chest and neck mass appeared three months ago. Imaging of the neck demonstrated an 8.6-cm anterior neck subcutaneous soft tissue mass extending into the anterior mediastinum through the sternum with erosive changes in the sternum and the lesion is abutting the right common carotid artery and innominate vein and surrounds the medial aspect of the clavicles bilaterally. Ultrasound (US)-guided biopsy showed marked polytypic-appearing plasma cell proliferation associated with relatively prominent histiocytes with hemophagocytosis/emperipolesis and focal neutrophils. There were S100+ histiocytes; however, findings were not typical for RDD. As that biopsy was not diagnostic, incisional biopsy with adequate sampling was performed. Surgical pathology demonstrated a very abnormal infiltrate with prominent histiocytes including areas with the features of extranodal RDD. BRAF V600E immunohistochemistry (IHC) was negative. Modified radical neck dissection, proximal sternal resection and superior mediastinal nodal dissection surgery was recommended. However, the patient refused the procedure. Typical manifestations are lymphadenopathy with fever that our patient did not experience. Bone involvement happens in 5-10% of cases. There is not enough data about blood vessel invasion which make our case unique. Treatment plan is still controversial. Clinical monitoring is recommended if the symptoms are tolerable as regression has been reported in many cases (20-50%). Surgery is reserved for patients with vital organ involvement or extra-nodal disease.