Digital Ischemia Research Articles

0800 Dozing off is a problem, as is a toe falling off - Methylphenidate induced Raynaud’s phenomenon

Abstract Introduction Narcolepsy is a clinical syndrome characterized by a constellation of symptoms including excessive daytime sleepiness, cataplexy, sleep paralysis and sleep hallucinations. Stimulants are commonly used to treat hypersomolence associated with narcolepsy. Common adverse reactions reviewed with patients prior to initiation of amphetamines include decreased appetite, nausea, xerostomia, headache, insomnia, tachycardia and hypertension. A relatively rare concern with stimulants is development of peripheral vasculopathy including Raynaud’s phenomenon. Report of Cases: A 25-year-old female with past medical history of anxiety, depression and obstructive sleep apnea in childhood was treated with tonsillectomy and adenoidectomy. Over the years, she continued to have persistent hypersomnolence, auditory hypnogogic hallucinations with no symptoms of sleep paralysis or cataplexy. She had a polysomnography (PSG) along with multiple sleep latency test (MSLT). PSG did not show evidence of sleep apnea with an AHI of 0.96 per hour. MSLT confirmed the diagnosis of Narcolepsy, Type 2, with a mean sleep onset latency of 3.6 minutes and five sleep-onset REM periods (SOREMPs). She was started on 400mg daily of Modafinil and 20mg daily of Methylphenidate. After a month of using methylphenidate, she noticed purplish discoloration of her digits, sometimes with exposure to cold, other times with no obvious triggers, consistent with development of Raynaud’s phenomenon. The proposed mechanism is that use of methylphenidate causes excessive release of catecholamines due to inhibition of the reuptake of dopamine and norepinephrine, leading to peripheral vasoconstriction. As the patient’s hypersomnolence persisted, she was started on sodium oxybate and the dosage of methylphenidate was decreased to 10mg daily and eventually discontinued. With reduction in the dosage and discontinuation of methylphenidate, symptoms of Raynaud’s phenomenon improved. Conclusion Dose-related peripheral vasculopathy including Raynaud’s phenomenon has been reported in several case reports with the use of methylphenidate. Awareness of this relatively rare adverse effect is imperative among sleep physicians as it could cause significant delay in the diagnosis, management of Raynaud’s phenomenon and its complications including critical digital ischemia and gangrene. Dose adjustments and discontinuation of methylphenidate should be considered in the treatment course of the patients with such concern. Support (If Any)

The clinical relevance of Raynaud's phenomenon symptom characteristics in systemic sclerosis.

Raynaud's phenomenon (RP) is a cardinal feature of systemic sclerosis (SSc) and manifests with pain, digital colour change, sensory symptoms, and impaired function. SSc-RP is exacerbated by cold exposure (RP 'attacks') but many patients report persistent symptoms of background digital ischaemia. The aim of our study was to examine the significance of RP with digital colour change with or without symptoms, and persistent colour change in between attacks. Patients with SSc responses were obtained from the Patient Survey of experiences of Raynaud's Phenomenon (PASRAP). We enquired about symptoms associated with Raynaud's attacks, and persistent symptoms in between attacks. Data were analysed as descriptive statistics with appropriate parametric/non-parametric testing. Relevant PASRAP survey question data from 747 evaluable SSc patients from across three continents were analysed. Isolated colour change was rare (29/484, 6%). Digital ulcers were more common in SSc-RP associated with other sensory symptoms (42.1% vs. 24.1%, P=0.057) and more readily treated with phosphodiesterase-type 5 inhibitors (22.5% vs. 10.3%%, P=0.124). Over one-third of patients (n=92/239, 38%) reported persistent colour change in between Raynaud's attacks. Patients with persistent colour change were more likely to have pulmonary arterial hypertension (15.2% vs. 7.5%, P=0.057) and be treated with calcium channel blockers (54.3% vs. 39.0%, P=0.021). SSc-RP with colour change and other symptoms and/or or persistent decolourisation in between attacks were more likely to have vascular complications of SSc and be treated with vascular therapies. Future research should explore the judicious use of vascular therapies as a potential form of disease modification in SSc. Key Points • Isolated colour change without other symptoms is rare in SSc patients. • SSc patients often identify persistent symptoms in between attacks of RP. • SSc-RP with colour change and other symptoms, or persistent decolourisation, may have greater disease severity and be treated with vascular therapies.

Severe digital ischemia as an unrecognized manifestation in patients with antisynthetase autoantibodies: Case series and systematic literature review.

Severe digital ischemia, including digital ulcers and gangrene, is considered rare in patients with antisynthetase antibodies. This study aimed to elucidate the clinical features of antisynthetase-positive patients complicated with digital ulcers and/or gangrene using a systematic literature review and case series in a single-center cohort. A systematic literature review was conducted to identify reports describing antisynthetase-positive cases with digital ulcers and/or gangrene. Our cohort of consecutive patients with antisynthetase antibodies was stratified by the history of severe digital ischemia. Demographic and clinical features and outcomes in patients with severe digital ischemia identified in the systematic literature review and our cohort were compared with those in patients without severe digital ischemia in our cohort. The systematic literature review revealed 12 antisynthetase-positive patients with severe digital ischemia from one case series and eight case reports. Seven (7%) of 100 patients with antisynthetase antibodies in our cohort had a record of severe digital ischemia. Severe digital ischemia was often found at presentation and was associated with the classification of systemic sclerosis with or without myositis overlap. Clinical features associated with severe digital ischemia in antisynthetase-positive patients included Raynaud's phenomenon (p < 0.001), digital pitting scars (p = 0.001), and nailfold capillary abnormality (p = 0.02). Outcomes of severe digital ischemia were generally favorable with vasodilators. Severe digital ischemia is an overlooked complication in antisynthetase-positive patients. Antisynthetase antibodies should be measured in patients presenting with digital ulcers or gangrene, especially in those with systemic sclerosis phenotype and features associated with antisynthetase antibodies in the absence of systemic sclerosis-specific autoantibodies.

P045 Octopus Toes?

Abstract Background/Aims Takotsubo cardiomyopathy is a relatively rare condition. Its name derives from the distinctive apical ballooning seen on cardiac angiography, which resembles the shape of takotsubo, a Japanese octopus trap. The clinical presentation is similar to acute coronary syndrome (ACS). Ischaemic ECG changes and elevated serum cardiac biomarkers are seen despite normal coronary artery angiography. Methods: History A 72-year-old female presented with a five-day history of constant bilateral foot pain and toe discolouration, associated with reduced sensation. She had a past history of hypertension and Raynaud’s phenomenon. She had recently been admitted with an alcohol withdrawal seizure and had recent severe stressful life events. She denied chest pain or dyspnoea and was a non-smoker. Her medications were ramipril and thiamine. Examination: Both feet were warm to touch proximal to the toes, with tenderness and diminished sensation in the distal feet. The toes were dusky but pedal pulses were palpable with good biphasic Doppler signal. Blood pressure was 117/105 mmHg on admission, with normal nail folds and heart sound, and no splinter haemorrhages were seen. Results: Investigations Blood parameters were CRP 60 mg/L, eGFR 47 mL/min, troponin &amp;gt;25,000 ng/L, and negative RF, ANA, ANCA, MPO, PR3, C3, C4. ECG showed lateral ST-segment elevation but normal coronary vessels were seen on coronary angiography. Echocardiogram revealed hypokinetic apical region and mid-anterior wall, reduced ejection fraction 35-40 % with apical ballooning, consistent with Takotsubo cardiomyopathy. Urine catecholamines, normetadrenaline, metadrenaline and 3-methoxytyramine levels were normal when measured eight days after the initial presentation. Management Daily Iloprost was given for five days with aspirin, furosemide, spironolactone and ivabradine, but no significant improvement was observed in the ischaemic feet. Her foot pain gradually subsided but the toes remained necrotic. Over the next six months, her toes mummified and auto-amputated. She has had no further ischaemic events. Conclusion Takotsubo cardiomyopathy accounts for 1-2% of troponin positive suspected ACS. It is most common in older females. The cause is not clear, but the predominant hypothesis is that a catecholamine surge leads to microvascular spasm and myocardial stunning. Raised catecholamine levels at presentation have been documented in patients with takotsubo cardiomyopathy, and the syndrome has been described in patients exposed to exogenous catecholamines. Digital ischaemia has been described in phaeochromocytoma. We postulate that our patient’s background of Raynaud’s phenomenon predisposed her to digital ischaemia during a period of catecholamine excess, which led to takotsubo cardiomyopathy. To our knowledge, this phenomenon has not previously been described. Given the profound effect of the presumed catecholamine surge on cardiac function in takotsubo cardiomyopathy and the high prevalence of Raynaud's phenomenon in the general population, it is perhaps surprising that ischaemia in digits and other organs is not more commonly observed. Disclosure K. Yeap: None. C. Badu-Boateng: None. M. Lloyd: None.

Botulinum Toxin A for the Treatment of Sympathomimetic Pressor-Induced Digital Hand Ischemia in the Critically Ill Intensive Care Unit Patient.

Vasopressor-induced ischemia of the hand, while relatively rare, is a severe complication in critically ill intensive care unit (ICU) patients requiring high concentrations of sympathomimetic pressors and often results in digit necrosis and amputation. Currently, there are no widely accepted approaches for treating this cause of peripheral digital ischemia. Case reports have demonstrated that reducing the concentration of vasopressors that patients are given may reverse the progression of ischemic events prior to necrosis. While this approach is at odds with the principle of "life over limb," it demonstrates that digit necrosis can be reversed, resulting in improved outcomes. Here, we present a therapeutic strategy for treating digital limb ischemia in the septic ICU patient without the need to lower systemic vasopressor dose by using locally injected botulinum toxin A into ischemic hands.

Change in calcinosis over 1 year using the scleroderma clinical trials consortium radiologic scoring system for calcinosis of the hands in patients with systemic sclerosis

IntroductionCalcinosis cutis is a debilitating complication of systemic sclerosis (SSc). We previously developed a radiographic scoring system to assess severity of calcinosis affecting the hands in patients with SSc. We sought to further validate our radiographic scoring system to assess for change over 1 year and to identify factors associated with improvement or progression. Materials and methodsBaseline and 1-year antero-posterior hand radiographs were obtained in 39 SSc patients with calcinosis prospectively enrolled at 6 centers within the US, Canada, Mexico and Australia. Two readers (one radiologist and one rheumatologist) scored all radiographs using the calcinosis scoring system and a 5-point Likert scale (1 = A lot better, 2 = A little better, 3=No change, 4 = A little worse, 5 = A lot worse) on follow-up. By maximizing the Kappa coefficient of agreement between grouped Likert scale (better/no change/worse) and the percentage of change of calcinosis in the radiographic scoring system, we defined progressive calcinosis as >25% increase in score from baseline at 1-year, stable calcinosis as change in score between -25% to 25%, and improvement of calcinosis as decrease in score by >25%. Nineteen SSc patients from an independent cohort were used for validation. ResultsInter-rater reliability of the calcinosis scoring system was high with intra-class correlation coefficient of 0.93 (0.89–0.95). The median percentage of change from baseline to 1 year was 12.8% (range -89.3 to 290.2%). Sixteen patients (41%) experienced progression of calcinosis over 1 year; 18 (46%) remained stable; and 5 (13%) had improvement. Patients with progressive calcinosis had lower T-score on bone densitometry (-3.3 vs -1.7, p = 0.044) and higher prevalence of loss of digital pulp on physical exam (56% vs 22%, p = 0.027), with a trend towards lower baseline modified Rodnan skin score (mRSS) (3.8 vs. 5.9, p = 0.057), than patients who did not progress. Patients who experienced improvement in calcinosis had lower prevalence of digital pitting scars (20% vs 71%, p = 0.047) than patients whose calcinosis did not improve. In multivariable analysis, loss of digital pulp remained a predictor of calcinosis progression (OR 5.8, p = 0.023, CI 1.27 - 26.36). In the validation cohort, 2 (11%) patients improved, 10 (53%) remained stable, and 7 (37%) progressed. ConclusionsWe confirmed the excellent inter-rater reliability of our radiographic calcinosis scoring system and demonstrated its usefulness to detect change over time. Approximately 40% of patients experienced progression of calcinosis over 1 year. Loss of digital pulp was predictive of progressive calcinosis providing further evidence that digital ischemia contributes to the progression of calcinosis.

Small vessel vasculitis and dry gangrene secondary to combined CTLA-4 and PD-1 blockade in malignant mesothelioma

BackgroundMalignant pleural mesothelioma (MPM) is a rare and aggressive tumour with an overall poor prognosis. In October 2020, first line treatment with the PD-1 antagonist nivolumab and the CTLA-4 antagonist ipilimumab for unresectable disease was FDA approved—the first approved treatment regime since 2004. Interim analyses from the phase 3 CHECKMATE-743 study shows improvements in overall survival. Skin-related toxicities are the most commonly reported any-grade treatment-related adverse event from combined nivolumab and ipilimumab therapy.Case presentationHere we report a case of a 35-year-old white male who developed digital ischaemia secondary to small vessel vasculitis after receiving PD-1 and CTLA-4 blockade therapy for MPM. His progressive ischaemia became gangrenous, and he required multi-speciality input and treatment with prednisolone, prostacyclin, mycophenolate mofetil and hydroxychloroquine.ConclusionsOur case highlights the importance of early detection, intervention, and a multispecialty approach to managing such complications in order to minimise the associated morbidity and mortality.

WALANT in hand surgery: Ischemic complications and their medicolegal implications in France: A systematic review of the literature and of French legal databases

WALANT (Wide Awake Local Anesthesia No Tourniquet) presents a theoretical risk of digital ischemia due to the presence of epinephrine, associated to the local anesthetic. For this reason, in France, the market authorization prohibits the use of epinephrine in digital extremities. The main objective of the present study was to assess the risk of ischemic complications reported in literature, and then to analyze the medicolegal implications in France. A systematic literature review was performed by three independent readers, using the PubMed and Embase databases. Also, declarations of claims and legal proceedings between 2007 and 2020 in France were examined in the official national Légifrance and Doctrine databases. Eight of the 424 articles retrieved were selected. Only 3 cases of digital necrosis following local anesthesia with adrenalized lidocaine were reported. Adrenalized xylocaine may be considered in case of peripheral microcirculation disorder. From a medicolegal point of view, no complaints or medicolegal implications were associated with WALANT in France. It seems that the market authorization for adrenalized local anesthesia could be extended to use in the digital extremities. However, the lack of medical and legal data calls for caution. We therefore recommend the use of an institutional protocol specifying the cases of overdose and the patient's pathway, and training for practitioners wishing to use this technique.

WALANT: Perceptions, approaches, and contraindications in a tertiary hand surgery unit

Wide awake local anesthetic no tourniquet (WALANT) is gaining popularity amongst hand surgeons. Digital adrenaline use has been shown to be safe in multiple studies and the misconception forbidding it is receding. Phentolamine has been shown to safely reverse the effects of adrenaline should the feared complication of digital ischemia occur. A survey was circulated to 40 specialist practitioners who regularly perform hand procedures at a major tertiary plastic and hand surgery unit. Knowledge and understanding of WALANT, onset and duration of adrenaline effects and reversal was assessed. Whilst the majority of respondents (80%) recognized digital adrenaline use as safe, only 65% were aware of the delay until adrenaline takes full effect. Similarly, only 25% of respondents were aware of the duration of effect of adrenaline. Half of respondents were aware that phentolamine is the established reversal agent for adrenaline with only 20% knowing the correct dose. Given the lack of clinician knowledge surrounding adrenaline and its reversal, we feel that to safely undertake WALANT surgery at our Unit a WALANT protocol must be implemented. Drawing on the successes in the airline industry, a variety of safety frameworks have been established to deliver targeted education for prevention and eventual management of predictable risks. We plan to develop a checklist style protocol targeting the knowledge gaps raised in the survey. This will educate and equip all practitioners working with adrenaline with the knowledge to safely manage complications should they occur. Level of evidenceLevel 5 (UK Oxford Centre for Evidence based Medicine (CEBM) Levels of Evidence).

The interplay of inflammation and coagulation in COVID-19 Patients receiving extracorporeal membrane oxygenation support.

Bleeding and thrombosis are common complications during Extracorporeal Membrane Oxygenation (ECMO) support for COVID-19 patients. We sought to examine the relationship between inflammatory status, coagulation effects, and observed bleeding and thrombosis in patients receiving venovenous (VV) ECMO for COVID-19 respiratory failure. Cross-sectional cohort study. Quaternary care institution. The study period from April 1, 2020, to January 1, 2021, we included all patients with confirmed COVID-19 who received VV ECMO support. None. Thirty-two patients were supported with VV ECMO during the study period, and 17 patients (53%) survived to hospital discharge. The ECMO nonsurvivors mean lactate dehydrogenase (LDH) levels were markedly elevated in comparison to survivors (1046 u/L [IQR = 509, 1305] vs 489 u/L [385658], p = 0.003). Platelet/fibrinogen dysfunction, as reflected by the low Maximum Amplitude (MA) on viscoelastic testing, was worse in nonsurvivors (65.25mm [60.68, 67.67] vs 74.80mm [73.10, 78.40], p = 0.01). Time-group interaction for the first seven days of ECMO support, showed significantly lower platelet count in the nonsurvivors (140 k/ul [103, 170] vs 189.5 k/ul [ 146, 315], p < 0.001) and higher D-dimer in (21 μg/mL [13, 21] vs 14 μg/mL [3, 21], p < 0.001) in comparison to the survivors. Finally, we found profound statistically significant correlations between the clinical markers of inflammation and markers of coagulation in the nonsurvivors group. The ECMO nonsurvivors experienced higher rate of bleeding (73.3% vs 35.3%, p = 0.03), digital ischemia (46.7% vs 11.8%, p = 0.02), acute renal failure (60% vs 11.8%, p = 0.01) and bloodstream infection (60% vs 23.5%, p = 0.03). The correlation between inflammation and coagulation in the nonsurvivors supported with VV ECMO could indicate dysregulated inflammatory response and worse clinical outcomes.

Patient satisfaction following wide awake local anaesthetic no tourniquet hand surgery

ABSTRACT BACKGROUND: Wide awake local anaesthetic no tourniquet (WALANT) hand surgery is a rapidly growing technique for hand surgery whereby a lignocaine/adrenaline/bicarbonate mixture is injected into the hand or fingers where the procedure is to be carried out METHODS: This was a retrospective study with prospective recall analysing satisfaction of patients who underwent WALANT hand surgery at our academic hospital in the first year of its inception. Data collection included a questionnaire to analyse demographics, comparison to dental procedures, subjective and objective experience of the procedure, overall experience, expectations, pain and surgical outcome RESULTS: We included 80 procedures in 67 patients; 87% would prefer WALANT in the future, and 87% would recommend WALANT to friends or family. For 79% of patients (who had dental procedures before), the pain was less or the same as a dental procedure, and 70% of patients said the experience was better than expected. Average pain scores were 3.89/10 during local anaesthetic injection, 1.25/10 during the procedure and 5.20/10 postoperatively; with postoperative pain starting at an average of nine hours. Eighty-five per cent of conditions were cured at follow-up and no cases of digital ischaemia or infection were noted CONCLUSION: Our study suggests WALANT hand surgery is a safe, effective and satisfactory method of performing hand surgery in the South African context Level of evidence: Level 4 Keywords: WALANT, hand surgery, local anaesthetic, carpal tunnel, de Quervain's, ganglion, patient satisfaction

An Unusual Case of Late-onset Systemic Lupus Erythematosus Presenting with Digital Gangrene

Digital gangrene is an uncommon initial manifestation of systemic lupus erythematosus (SLE) and rarely in late-onset disease. This case presents a 50-year-old woman who developed digital gangrene and was subsequently diagnosed with SLE. Early treatment with immunosuppression and anticoagulation halted the progression of the digital ischemia. This case highlights that late-onset SLE, often described as having a more benign and insidious course, can also present with catastrophic limb-threatening manifestations.

Magnetic Resonance Angiography of the Hand Vasculature in Patients With Systemic Sclerosis and Systemic Lupus Erythematosus.

When patients with systemic sclerosis (SSc) and systemic lupus erythematosus (SLE) develop digital ischemia, conventional angiography (CA) is traditionally used to assess hand vasculature. Recently, Chang et al described an angiographic classification system for patients with SSc. Conventional angiography uses intravascular contrast agents that are nephrotoxic and vasoconstrictive. Owing to these limitations, this study assesses the use of contrast-enhanced magnetic resonance angiography (MRA) as an alternative to CA to evaluate hand vasculature in patients with digital ischemia. This retrospective case series reports on 38 contrast-enhanced MRAs of hand vasculature from 30 symptomatic patients with SSc (N = 21) or SLE (N = 9). The radial and ulnar arteries (RA, UA) and the superficial and deep palmar arches were evaluated at standard reference points both quantitatively and qualitatively for their diameter, patency, and Chang classification. In SSc MRAs (n = 26), the UA was significantly smaller than the RA and was occluded in 46%. In SLE MRAs (n = 12), the UA and RA had no difference in diameter and the UA was occluded in 25%. In SSc, the most common Chang classification was type 2 (UA involvement) in 44%. In SLE, the most common Chang classification was type 4 (UA and RA involvement) in 45%, with 18% classified as type 2. Contrast-enhanced MRA used to assess hand vasculature in SSc patients with digital ischemia shows similar patterns of vascular involvement as previously demonstrated by CA. While vascular involvement in SSc predominantly involves the UA, the RA is also frequently involved in SLE.

Clinical Update on Vasopressors and Titration Strategies.

Clinical Update on Vasopressors and Titration Strategies.

Digital gangrene as an unusual paraneoplastic manifestation of hodgkins lymphoma\u2014a rare case report

BackgroundHodgkin’s lymphoma presenting with digital ischemia and gangrene is a rare manifestation. Paraneoplastic manifestations are rare in Hodgkin’s lymphoma but can occur in the form of paraneoplastic cerebellar degeneration (PCD) and dermatomyositis/polymyositis. This case report adds an exceptional presentation of Hodgkin's lymphoma as digital ischemia and gangrene.Case presentationWe report a case of a 60-year-old male patient who presented with fever, cough, shortness of breath, the pain in the right middle finger. On examination bluish-black discoloration of the right middle finger, left middle finger, ring finger, and generalized lymphadenopathy was noted. On further evaluation, he was found to have anemia, eosinophilia, and severe thrombocytopenia with a normal coagulation profile and negative rheumatological workup. Arterial Doppler of both upper limbs showed the normal study. He was diagnosed to have Hodgkin’s lymphoma on the lymph node and bone marrow biopsy. He was started on chemotherapy with partial improvement in symptoms and was lost to follow-up after 2 cycles.ConclusionsDigital ischemia can be a rare paraneoplastic manifestation of Hodgkin’s lymphoma.

TWO CASES OF ARTERIAL THROMBUS IN COVID-19 INFECTION

TOPIC: Critical Care TYPE: Fellow Case Reports INTRODUCTION: Several studies have observed thrombotic complications in COVID-19 patients with rates as high as 30%. We present two COVID-19 cases of arterial and venous thrombotic complications. CASE PRESENTATION: A 64-year-old African American female was admitted for worsening respiratory failure due to COVID-19. HIT panel was obtained due to worsening thrombocytopenia, as the patient was on heparin for venous thrombosis prophylaxis, which was negative. Duplex of the lower extremities performed on hospital day 12 showed an acute non-occlusive deep vein thrombosis of the left common femoral vein, for which therapeutic heparin was initiated. On hospital day 25, the patient was found to have right digit ischemia with no flow to the right ulnar artery secondary to an occlusive arterial thrombus. Hematology was consulted for severe thrombocytopenia and considered to treat empirically for ITP with IVIG;however, the patient continued to decompensate and the family elected for comfort care measures. The patient expired on hospital day 30.Our second case report is a 64-year-old African American male presented from a local prison with worsening shortness of breath and was found to have COVID-19. Shortly after arrival from the ED to ICU, the patient went into cardiac arrest, ACLS was initiated and ROSC was achieved. The patient was in torsades and Vfib rhythm. He underwent left heart catheterization on hospital day 1 and was found to have 100% occlusion of obtuse marginal and underwent successful percutaneous coronary intervention with a drug-eluting stent to OM2. On hospital day 8, the patient was found to have digit ischemia of the right first through third digits. Arterial and venous duplex showed thrombotic occlusion of the right radial artery from the mid-forearm to the wrist and an acute deep vein thrombus of the left subclavian and axillary veins. DISCUSSION: COVID-19 can be associated with arterial and venous thrombosis as highlighted by our two cases. Even though the pathophysiology of thromboembolic complications in COVID-19 is not reported in the literature, it is implicated due to inflammation, hypoxia, immobilization, and DIC. In a study done by Klok et al in COVID-19 ICU patients, 27% of the study population had CTPE/Ultrasonography confirmed venous thromboembolism and 3.7% had arterial thrombosis compared to 12% of venous thrombosis in the ICU. The mechanism for hyper-coagulability in COVID infection is not known, it has been linked to the inflammatory response rather than specific properties of the virus. This association of excess inflammation and COVID-19 with subsequent activation of coagulation has been implicated in the pathogenesis of thrombosis. CONCLUSIONS: Intermediate to therapeutic anticoagulation may be necessary to prevent venous and arterial thromboembolic events in ICU patients admitted with COVID-19 given their excessive inflammatory state. REFERENCE #1: Connors JM, Levy JH. COVID-19 and its implications for thrombosis and anticoagulation. Blood, The Journal of the American Society of Hematology. 2020 Jun 4;135(23):2033-40. REFERENCE #2: Leisman DE, Deutschman CS, Legrand M. Facing COVID-19 in the ICU: vascular dysfunction, thrombosis, and dysregulated inflammation. Intensive Care Medicine. 2020 Apr 28:1-4. REFERENCE #3: Middeldorp S, Coppens M, van Haaps TF, Foppen M, Vlaar AP, Muller MC, Bouman CC, Beenen LF, Kootte RS, Heijmans J, Smits LP. Incidence of venous thromboembolism in hospitalized patients with COVID-19. Journal of Thrombosis and Haemostasis. 2020 May 5. DISCLOSURES: No relevant relationships by Sarenthia Epps, source=Web Response No relevant relationships by Harmeen Goraya, source=Web Response No relevant relationships by Raga Deepak Reddy Palagiri, source=Web Response

A RAPIDLY PROGRESSIVE AND RARE ILLNESS: AUTOANTIBODIES AGAINST MELANOMA DIFFERENTIATION-ASSOCIATED PROTEIN 5 (ANTI-MDA5): AMYOPATHIC DERMATOMYOSITIS WITH PROGRESSIVE INTERSTITIAL LUNG DISEASE THAT DEVELOPED AFTER COVID-19 VACCINE

TOPIC: Critical Care TYPE: Global Case Reports INTRODUCTION: Anti-MDA5 dermatomyositis is a subtype of dermatomyositis characterized by a high risk of rapidly progressive interstitial lung disease. It is characterized by fever, progressive shortness of breath, joint swelling and skin ulcerations. (1) There is invariable hyper-ferritinemia and radiographically there is a pattern of diffuse bilateral ground glass opacities. Complications with spontaneous pneumothorax and pneumomediastinum are not uncommon. Diagnosis is confirmed by biopsy as well as serum antibodies against MDA5. A known trigger is viral infection. (2) CASE PRESENTATION: We present the case of a 58-year-old man who was admitted to the Intensive Care Unit on account of respiratory failure and shock. He had a progressive Illness that started 4 days after receiving a novel mRNA COVID-19 Vaccine. He developed bilateral extremities edema, oral sores, worsening fatigue and dyspnea on exertion. He failed outpatient treatment for empiric pneumonia as well as prednisone for vaccine reaction. He presented to the hospital with fever, progressive dyspnea and fatigue. On physical exam he had oral blisters, digital tip ischemia and ulceration. His vital signs were pertinent for a SpO2 of 90% on ventilator support. A chest CT revealed diffuse and sub-pleural ground-glass opacities.There was an extensive negative infectious work up from serum, urine and BAL samples. These included negative SARS COV2 Antigen and SARS-Cov2 IgG. His inflammatory markers were pertinent for a positive ANA with titer of 1:320, Ferritin of 2,143 mcg/L, LDH of 1,105 U/L and CRP of 2.7mg/dL. Based on this information the differential for amyopathic dermatomyositis with progressive interstitial lung disease was considered. A skin biopsy of his index finger confirmed the diagnosis. His therapeutic interventions through his hospitalization included a trial of stress dose steroids, cyclophosphamide, IVIG, colchicine and tacrolimus. His ICU course was complicated by pneumomediastinum, pneumothorax and pericardial effusion. These were managed by placing a chest tube thoracotomy and pericardial drain respectively. His plan of care was changed to comfort measures after he had a drastic and prolonged desaturation episode without recovery. DISCUSSION: For conditions that lead to cytokine storm with subsequent development of ARDS or multi-organ dysfunction;there is a progressive clinical deterioration and high risk of death. We had insufficient evidence to definitively demonstrate a direct correlation between the vaccination and the patient's illness. CONCLUSIONS: In conditions that lead to Cytokine storm with multi-organ dysfunction targeting timely control of this inflammatory cascade via immunomodulation, cytokine antagonist and reduction of inflammatory cell infiltration in particular early in the process could lead to a reduction in mortality. REFERENCE #1: Fiorentino D, Chung L, Zwerner J, Rosen A, Casciola-Rosen L. The mucocutaneous and systemic phenotype of dermatomyositis patients with antibodies to MDA5 (CADM-140): a retrospective study. J Am Acad Dermatol. 2011;65(1):25-34. doi:10.1016/j.jaad.2010.09.016 REFERENCE #2: Sakamoto N., Ishimoto H., Nakashima S. Clinical features of anti-MDA5 antibody-positive rapidly progressive interstitial lung disease without signs of dermatomyositis. Intern Med. 2019;58(6):837–841. doi: 10.2169/internalmedicine. 1516-18. REFERENCE #3: Margherita Giannini,1 Mickael Ohana,2 Benoit Nespola,3 Giovanni Zanframundo,4,5 Bernard Geny,1,6 and Alain Meyer1,6,7 Similarities between COVID-19 and anti-MDA5 syndrome: what can we learn for better care? Eur Respir J. 2020 Sep;56(3): 2001618. Published online 2020 Sep DISCLOSURES: No relevant relationships by Ashlie Arthur, source=Web Response No relevant relationships by Leonel Carrasco, source=Web Response no disclosure on file for Chad Case;No relevant relationships by Juan Gaitan Rueda, source=Web Response

Is digital necrosis in COVID-19 caused by neutrophil extracellular traps: Potential therapeutic strategies

Some of the COVID-19 patients present with ischemic lesions of their finger and toes. Standard anticoagulant therapy is usually unsuccessful for the treatment of this unique presentation of COVID-19. In this review current evidence is presented, which supports the hypothesis that these necrotic lesions are primarily related to the formation of neutrophil extracellular traps is blood vessels. Also, currently available and potential pharmacological methods of the management of this unique thrombotic complication are discussed. Drugs that possibly could be used in COVID-19 patients suffering from acute ischemia of distal parts of the extremities particularly comprise DNase I and DNase1L3, which could directly dissolve these extracellular webs that are mostly composed of DNA. However, at the moment, none of these enzymes are registered for an intravascular administration in humans. Lactoferrin and dipyridamole are other pharmaceutical agents that could potentially be used for the treatment of neutrophil extracellular traps-evoked digital ischemia. These agents exhibit prophylactic activity against excessive formation of these extracellular structures. Such an experimental treatment should probably be accompanied by standard antithrombotic management with heparin. Open-label and then randomized trials are needed to confirm feasibility, safety and efficacy of the above-suggested management of critically ill COVID-19 patients.

Considerations When Performing Arthrodesis in the Scleroderma Hand.

Hand deformities secondary to scleroderma can limit activities of daily living and be associated with substantial disability. This study aimed to evaluate the outcomes following arthrodesis performed to treat digital contractures secondary to scleroderma. We performed a retrospective review of all patients with scleroderma who underwent arthrodesis by a single surgeon from 2015 to 2020. We collected demographic information, operative variables, and outcomes variables. Our primary outcome was occurrence of any postoperative complication, which we defined to include wound dehiscence, digital ischemia, malunion, nonunion, cellulitis, and osteomyelitis. We calculated descriptive statistics and performed all analyses at the joint level. We identified 9 patients who underwent arthrodesis of 19 joints. All patients were women with a mean age of 55.3 years. At the time of surgery, most patients were taking disease-modifying antirheumatic drugs (DMARDs). Kirschner wires (K-wires) were used in most cases (n = 18), 15 of which were removed uneventfully at an average of 4.8 months after surgery. With a mean follow-up time of 15.4 months, the overall complication rate was 5.3% (n = 1). This patient developed digital ischemia in 1 of 4 operative digits, which became gangrenous and required amputation. Our study suggests that arthrodesis can be performed safely in the scleroderma hand, even when patients are taking DMARDs. Given the uneventful K-wire removal in all joints and the high risk of exposure of buried hardware in this population, we recommend nonpermanent placement of K-wires. Hand surgeons may consider arthrodesis in the scleroderma hand before proceeding to revision amputation.

Presentation of the hypothenar hammer syndrome as a low incidence aneurysmal disorder of the ulnar artery.

Introduction and importanceThe hypothenar hammer syndrome (HHS) is either rare or underdiagnosed condition that arises from vascular insufficiency of the ulnar artery in the hand. The most common accused mechanism is repetitive blunt trauma to hypothenar region while activating the latter as a hammer to do the job.Case presentationA 48 year old woman worked as a master of university claimed from pain and numbness in her left hand concurrent with a pulsatile mass. The second case was a 48 year old man who was a mechanic engineer in automotive industry that claimed of a pain and pulsatile mass in his non-dominant left hand.Clinical discussionUlnar artery enters the hand from Guyon's canal where fixes to adjacent structure and is susceptible for injury. Aneurysmal formation is an outcome of arterial trauma which could result in distal necrosis if remains untreated.ConclusionThis study has revealed that even whether a single blunt trauma or writing with pen could lead to aneurysmal formation. It has also showed primary aneurysmal resection with no further surgical procedure is both safe and reliable if digital ischemia is not concurrently present.