SAEM Clinical Images Series: Retrobulbar Spot Sign

vision

A 59-year-old male with no known past medical history other than an incidental abdominal aortic aneurysm presented with sudden onset, painless vision loss in his left eye. The patient was watching TV two days prior when he saw a “brightness” in his left eye and then progressive blurriness until his vision faded away, all occurring within the span of a minute. At the time of presentation, he only sees a speck of light from that eye. He denied associated pain, flashes, floaters, jaw claudication, the sensation of a “curtain falling”, prior vision problems, or a history of blood clots.

Eyes: Eyelids and lashes normal. Visual acuity: 20/30 OD, Light Perception OS. EOMI. PERRL. OD visual fields intact. Afferent Pupillary Defect OD. Normal conjunctiva. IOP 16 OD, 14 OS. Otherwise CN 3-12 intact.

Complete blood count (CBC): Within normal limits

Basic metabolic panel: Creatine 1.3 (unknown baseline)

ESR: Unmarkable

Central Retinal Artery Occlusion (CRAO) is an ocular emergency that presents as acute painless monocular vision, caused by ischemia and infarction to the retina via thromboembolic disease to the central retinal artery. It requires immediate consultation with ophthalmology as well as neurology as it is considered a stroke equivalent.

The case described above and several previously published case studies highlight the utility of POCUS in identifying CRAO via the retrobulbar spot sign (RBSS) within the optic nerve in a rapid, non-invasive manner that can be done prior to waiting for dilation for a fundoscopy exam. This has the potential to expedite consultations with specialty teams and treatment.

Several studies also reveal the potential of POCUS to predict the etiology of CRAO (arterio-arterial embolization vs cardio-embolic vs vasculitis) and thus to predict the success of thrombolytic treatment in CRAO. In a prospective monocenter study of 46 patients with ophthalmologically confirmed CRAO, embolism from large artery atherosclerosis (LAA, i.e. carotids or aortic arch) was the etiology in 27 patients, cardioembolic in 10 patients, vasculitis in 5 patients, and unknown in 4 patients. Out of the LAA patients, 59% had RBSS compared with only 20% in cardioembolic and 0% in the vasculitis patients. Within the 11 patients that underwent thrombolysis, statistically significant visual improvement occurred in all 4 patients with RBSS negative CRAO, while the 7 patients with RBSS positive CRAO had persistent visual impairment with persistent occlusion of their arteries. This study concludes that their results support the hypothesis that RBSS is seen due to calcium deposits that will not be dissolved with thrombolysis. Another small single-center German study points out the utility of seeing RBSS as 100% specific for an embolic cause of CRA, excluding temporal arteritis from the differential.

Take-Home Points

  • POCUS can guide us in diagnosing a patient with painless vision loss prior to more time-consuming fundoscopy exam.
  • Stroke workup for CRAO is necessary, and don’t forget about secondary prevention/risk stratification which must be part of the management.
  • RBSS may predict poor response to systemic thrombolysis.

  • Ertl M, Altmann M, Torka E, Helbig H, Bogdahn U, Gamulescu A, Schlachetzki F. The retrobulbar “spot sign” as a discriminator between vasculitic and thrombo-embolic affections of the retinal blood supply. Ultraschall Med. 2012 Dec;33(7):E263-E267. doi: 10.1055/s-0032-1312925. Epub 2012 Sep 21. PMID: 23023446.
  • Nedelmann, Matt et al. “Retrobulbar Spot Sign Predicts Thrombolytic Treatment Effects and Etiology in Central Retinal Artery Occlusion” American Heart Association (AHA). Stroke. 2015;46:2322–2324 https://doi.org/10.1161/STROKEAHA.115.009839
  • Smith, Austin T et al. “Using the Retrobulbar Spot Sign to Assist in Diagnosis and Management of Central Retinal Artery Occlusions.” Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in Medicine vol. 39,1 (2020): 197-202. doi:10.1002/jum.15073

By |2024-01-28T21:19:20-08:00Jan 29, 2024|Ophthalmology, SAEM Clinical Images, Ultrasound|

SAEM Clinical Images Series: A Rare Case of Purpura

An 88-year-old female presented to the ER with a chief complaint of cough, vague abdominal pain, and a rash. The patient stated that she was started on Cipro eyedrops 1 or 2 days prior to presentation for a possible eye infection. A day prior to presentation she developed a purple purpuric rash on her lower extremities that gradually progressed up her legs, and was present on her buttocks thighs, and lower legs. It was not on her palms or soles. She had no mucous membrane involvement. She lives alone. The nursing home called EMS given the patient’s severe and progressive rash and the fact that the patient was feeling unwell. She had no fever, vomiting, foreign travel, other new drug exposure, or other complaints.

GI: Abdomen is mildly diffusely tender without guarding or rebound.

Skin: There are scattered petechiae and purpura on her lower extremities, thighs, and buttocks. They are somewhat raised, non-blanching, not itchy, and non-tender. They are most prominent on her buttocks and dependent areas of her body.

WBC: 7.8 with normal differential

Platelets: 423

Comprehensive metabolic panel (CMP): normal kidney function and electrolytes

ESR: 125 mm/hour

CRP: 89 mg/L

Urinalysis (UA): >9,8000 bacteria, nitrite positive

This patient’s history and physical are consistent with Henoch-Schönlein purpura (IgA Vasculitis).

Common triggers include infection, drugs, and autoimmune.

Take-Home Points

  • Consider IgA vasculitis, even in an older patient.
  • Ciprofloxacin has been documented as a cause of IgA vasculitis.
  • Steroids and NSAIDs are the treatment of choice, and this condition usually improves with time.

  • Gamboa F, Rivera JM, Gómez Mateos JM, Gomez-Gras E. Ciprofloxacin-induced Henoch-Schönlein purpura. Ann Pharmacother. 1995 Jan;29(1):84. doi: 10.1177/106002809502900119. PMID: 7711355.
  • Gkoufa A, Sakellariou S, Katsoulas N, Georgakopoulou VE, Lazaris A, Cholongitas E. Henoch-Schönlein purpura associated with ciprofloxacin. Dermatol Ther. 2021 Jan;34(1):e14591. doi: 10.1111/dth.14591. Epub 2020 Dec 3. PMID: 33244823.

SAEM Clinical Images Series: Fever with Rash

eschar

A 40-year-old male, tailor by occupation, was brought to the Emergency Department with complaints of high-grade fever for the past 11 days. Fever was documented to be 102°F and was not associated with any chills or rigors. The patient also complained of shortness of breath for one week associated with a dry cough, as well as an altered sensorium for one day. The patient during his hospital stay developed ARDS and was on mechanical ventilation for 20 days. He was then extubated and discharged after 27 days.

Skin: Multiple eschars on knee, foot, and lower chest.

Complete Blood Count: WBC 31,000; Plt 12,000

BUN: 215 mmol/L

Creatinine: 2.5 mmol/L

Liver Function Tests: AST 192 IU/L; ALP 591 IU/L

Blood PCR for Scrub Typhus was found to be positive.

Scrub typhus is often diagnosed clinically based on exposure to endemic regions and its characteristic eschar, which usually appears on the lower extremities, axillae, or genital region. [1,2] Still, diagnosis can be tricky, and similar eschars can be caused by spider bites, Mediterranean spotted fever, Queensland tick typhus, African tick-bite fever, and anthrax. [3] Scrub typhus is a potentially fatal mite-borne rickettsial infection caused by Orientia tsutsugamushi. It is endemic to the Asia–Pacific region, which has an estimated 1 million instances per year. Those affected may have headaches, myalgias, hearing loss, and rash, in addition to fever. Encephalitis, hepatitis, and pulmonary and cardiac involvement can occur. [1,2]

Early empiric treatment with Doxycycline is life-saving.

Take-Home Points

  • Consider Scrub Typhus in a patient presenting with eschars.
  • Early empiric treatment with Doxycycline is life-saving.
  • Botelho-Nevers E, Raoult D. Fever of unknown origin due to rickettsioses. Infect Dis Clin North Am. 2007 Dec;21(4):997-1011, ix. doi: 10.1016/j.idc.2007.08.002. PMID: 18061086.
  • Hendershot EF, Sexton DJ. Scrub typhus and rickettsial diseases in international travelers: a review. Curr Infect Dis Rep. 2009 Jan;11(1):66-72. doi: 10.1007/s11908-009-0010-x. PMID: 19094827.
  • Shiao CC, Lin SY. Eschar: a clue to scrub typhus. CMAJ. 2011 Oct 18;183(15):E1152. doi: 10.1503/cmaj.101929. Epub 2011 Sep 12. PMID: 21911554; PMCID: PMC3193135.

SAEM Clinical Images Series: Post-Vaccination Rash

hypersensitivity

A 42-year old Bengali man with a history of hyperlipidemia presented to the Emergency Department with facial swelling, diffuse rash, renal insufficiency and proteinuria after receiving his COVID-19 vaccine (Moderna) booster dose. There were no adverse events with the first two doses of the vaccine except for mild transient sore throat and cough after the 2nd dose. Within a few hours after the booster dose, the patient noted a pruritic rash initially on his scalp, that then spread to his torso associated with facial swelling, fever, and chills. He presented to his primary care physician three days later. At that time, laboratory workup showed proteinuria, elevated C-reactive protein (65.2), and an elevated serum creatinine (2.84 mg/dl). He was advised to go to the Emergency Department.

General: He was in no distress; his vital signs were normal.

Skin: While the facial swelling had improved, the rash had progressed to involve the entire body. There were multiple skin lesions with raised borders, and central clearing (Figures 1 and 2); no mucosal involvement was noted.

The rest of his physical exam including lung, cardiac, gastrointestinal, and neurological examinations were normal.

Laboratory workup in the ED revealed resolution of proteinuria with serum creatinine returning to normal baseline value (0.89 mg/dl).

The patient’s rash is a classic erythema multiforme (EM) rash. The mRNA COVID-19 vaccine is a lipid nano particle-encapsulated, nucleoside-modified mRNA vaccine that encodes the perfusion spike glycoprotein of the SARS-CoV-2 virus. Local reactions include mild to moderate pain at the injection site, and systemic effects including fatigue, fever, and headache, commonly appearing within 2-5 days after the second dose. Erythema multiforme has been reported as a cutaneous reaction after the COVID-19 mRNA vaccine. As per the vaccine adverse event reporting system (VAERS) from the Centers for Disease Control and Prevention, to date, there have been 284 reported cases of EM after the Moderna COVID-19 vaccine and 500 cases reported after the Pfizer vaccine. The exact pathogenesis of EM after the vaccine is unclear. This delayed hypersensitivity reaction is likely from sensitization to a vaccine component. It appears to be a T-cell mediated response making CD4+ helper T-1 cells, release of gamma-interferon, and then recruitment of auto-reactive T-cells. It should be differentiated from immediate IgE-mediated hypersensitivity reactions such as flushing, urticaria, angioedema, and hypotension that usually appear within minutes of administering the vaccine.

While immediate hypersensitivity is a contraindication for further doses, erythema multiforme and other such delayed manifestations should not discourage the use of additional COVID-19 mRNA doses if appropriate.

Take-Home Points

  • Erythema multiforme is a delayed hypersensitive reaction that may occur after COVID-19 mRNA vaccine.
  • This type of delayed hypersensitivity reaction, likely from sensitization to vaccine component, is not a contraindication to further COVID-19 boosters.

  • Su JR, Haber P, Ng CS, Marquez PL, Dores GM, Perez-Vilar S, Cano MV. Erythema multiforme, Stevens Johnson syndrome, and toxic epidermal necrolysis reported after vaccination, 1999-2017. Vaccine. 2020 Feb 11;38(7):1746-1752. doi: 10.1016/j.vaccine.2019.12.028. Epub 2019 Dec 20. PMID: 31870573; PMCID: PMC7008074.
  • Vaccine Adverse Events Reporting System [Internet]. CDC. 2022. Available from: https://vaers.hhs.gov/data.html.

SAEM Clinical Images Series: A Lethal Combination of Skin and Lung Findings

dermatomyositis

A 49-year-old female with a past medical history of recurrent diverticulitis initially presented with one month of shortness of breath and a minor nonproductive cough for which she was started on doxycycline by her primary care provider. She then developed a rash on her chest, upper back, and face. Antibiotics were switched to amoxicillin and azithromycin. She underwent a brief admission of six days for shortness of breath but did not have an oxygen requirement at that time. She was evaluated by pulmonology (evaluated for cocci, unknown results), and then discharged. She then presented again to the ED with two weeks of worsening shortness of breath, intermittent fevers (Tmax 101°F), nausea/vomiting, fatigue, and arthralgias.

Vitals: BP 100/66; HR 128; Temp 37.2 °C (99 °F); Resp 44; SpO2 84%; BMI 28.25 kg/m2; Wt 79.4 kg (175 lb); Ht 1.676 m (5′ 6″)

General: NAD

Cardiovascular: Tachycardia, no m/r/g

Lungs: Coarse breath sounds at bases bilaterally, tachypneic

Abdomen: Soft, non-distended

Skin: Heliotrope rash to face (violaceous, erythematous rash to eyelids and nasolabial fold), shawl sign (erythematous patches to chest and upper back), shallow ulcers to tongue and lower inner lip, tender papules involving palms and lateral fingers bilaterally, and faint erythema of proximal nail fold

White blood cell (WBC) count: No leukocytosis

ESR: Elevated

LDH: Elevated

CK: Within normal limits

CXR: Bilateral infiltrates

CTPE: Negative for PE, but with scattered areas of ground glass and consolidative opacities throughout both lungs.

If emergency medicine physicians consider MDA5 Dermatomyositis (MDA5 DM) with rapidly progressive interstitial lung disease (RP-ILD) on their differential for patients presenting with skin and pulmonary symptoms, this can result in more rapid diagnosis and aggressive treatment.

This patient was admitted requiring 40 L HFNC, then two days later required intubation for severe ARDS and was placed on VV-ECMO the same day. Her hospital course was complicated by tachyarrhythmias requiring cardioversion, and Takostubo physiology. She was found to be MDA-5 antibody positive and ultimately expired while waiting for a lung transplant.

Take-Home Points

  • Critical actions in approaching ED patients with dermatological physical exam findings (even in the absence of known rheumatological history) with progressive pulmonary symptoms should include early consideration of dermatomyositis, serologic testing, early rheumatology and pulmonology consults, and early consideration of ECMO as a bridge to response to immunotherapy or lung transplant
  • Beginning these critical actions with first patient contact in the ED will only help improve patient outcomes throughout hospitalization.
  • Huang K, Levy RD, Avina-Zubieta JA. Successful lung transplant in rapid progressive interstitial lung disease associated with anti-melanoma differentiation associated gene 5. Rheumatology (Oxford). 2020 Aug 1;59(8):2161-2163. doi: 10.1093/rheumatology/keaa032. PMID: 32068868.
  • Koga T, Fujikawa K, Horai Y, Okada A, Kawashiri SY, Iwamoto N, Suzuki T, Nakashima Y, Tamai M, Arima K, Yamasaki S, Nakamura H, Origuchi T, Hamaguchi Y, Fujimoto M, Ishimatsu Y, Mukae H, Kuwana M, Kohno S, Eguchi K, Aoyagi K, Kawakami A. The diagnostic utility of anti-melanoma differentiation-associated gene 5 antibody testing for predicting the prognosis of Japanese patients with DM. Rheumatology (Oxford). 2012 Jul;51(7):1278-84. doi: 10.1093/rheumatology/ker518. Epub 2012 Feb 29. PMID: 22378718.
  • Moghadam-Kia S, Oddis CV, Sato S, Kuwana M, Aggarwal R. Anti-Melanoma Differentiation-Associated Gene 5 Is Associated With Rapidly Progressive Lung Disease and Poor Survival in US Patients With Amyopathic and Myopathic Dermatomyositis. Arthritis Care Res (Hoboken). 2016 May;68(5):689-94. doi: 10.1002/acr.22728. PMID: 26414240; PMCID: PMC4864500.

SAEM Clinical Images Series: Enigmatic Traumatic Hip Pain

hip

An 84-year-old female presented with a chief complaint of right hip pain after a fall 12 hours prior to presentation. The patient reported a history of falls resulting in shoulder, rib, and left hip fractures in the past. The patient stated that upon getting out of bed, she took 4-5 steps, lost her balance, and fell backward onto the bedroom floor. She denied loss of consciousness. She denied syncope or vertigo before the fall. She was unable to bear weight due to a 7/10 intensity pain on the anterior medial aspect of her right thigh that was worse with movement.

Vitals: 37.8°C; BP 138/92; HR 94; RR 18; SpO2 98% on room air; BMI 24

General: A&Ox4, anxious, in moderate distress.

HEENT: Normocephalic, atraumatic, PERRLA, EOM’s intact.

Cardiac: RRR w/out m/r/g, pulses 2+ in all extremities.

Respiratory: BBS, CTA.

Abdomen: BS+, nondistended, nontender.

MSK: No gross deformities appreciated, right hip with limited flexion and extension due to pain. Tender to palpation superior, anterior medial aspect of the right thigh. Full range of motion of the right knee, ankle, and left lower extremity.

Complete blood count (CBC): Within normal limits

Comprehensive metabolic panel (CMP): Within normal limits

AP Pelvis Radiograph (Figure 1): “Osteopenia with no acute fractures or dislocation”

Occult femur fracture

Occult fractures are defined as fractures that cannot be detected by standard radiographic examination until weeks after the injury either due to lack of displacement or limitations of the imaging study. Occult femur fractures account for 2-10% of total hip fractures and have an associated one-year mortality of 14-16% even when surgically repaired within two days. Delayed recognition coupled with patient immobility may lead to complications such as pulmonary emboli that have been shown to increase one-year mortality by up to 30%.

Magnetic Resonance Imaging

In our case, computed tomography with 3D reconstruction (Figures 2,3) revealed a non-displaced intertrochanteric fracture with involvement of the greater and lesser trochanters. As CT scanning is usually more readily available than MRI, it may be the first additional imaging choice when radiographs are normal. A normal CT scan, however, especially in patients with osteopenia is considered insufficient to rule out an occult fracture. In a 7-year retrospective analysis at a regional trauma center, 57.4% of cases were diagnosed by MRI and 14.6% were diagnosed by CT scan within the first 24 hours. Of the remaining portion, a final diagnosis was made 72 hours after presentation with CT scan (39% of false negative cases) or MRI (61% of false negative cases).

Take-Home Points

  • Occult fractures are an important differential when standard imaging modalities do not correlate with physical exam findings.
  • Occult fractures can be missed on X-rays and CT scans, delaying definitive treatment. Delayed diagnosis can result in further complications and increased mortality and morbidity.
  • MRI is considered the gold standard for identifying occult fractures.
  • Deleanu B, Prejbeanu R, Tsiridis E, Vermesan D, Crisan D, Haragus H, Predescu V, Birsasteanu F. Occult fractures of the proximal femur: imaging diagnosis and management of 82 cases in a regional trauma center. World J Emerg Surg. 2015 Nov 18;10:55. doi: 10.1186/s13017-015-0049-y. PMID: 26587053; PMCID: PMC4652353.
  • Jonathan Grammer, Michael Sternberg. Occult femur fracture. Visual Journal of Emergency Medicine. Volume 14, 2019, Pages 15-16, ISSN 2405-4690

By |2023-12-23T10:25:21-08:00Dec 22, 2023|Orthopedic, Radiology, SAEM Clinical Images|

SAEM Clinical Images Series: Rapidly Spreading Rash

DRESS

A 19-year-old female with a past medical history of epilepsy presented to the emergency department for evaluation of rash and fever. Two days prior to presentation she began to experience fevers with a Tmax of 103°F. One day before presentation she developed a rash that began on her face and slowly spread down her body, now involving her palms. The patient endorsed associated pruritus and cervical lymphadenopathy with the rash. The patient specifically denied mucous membrane involvement (mouth, eyes, genitalia), vomiting, diarrhea, dysuria, hematuria, neck stiffness, cough, dyspnea, chest pain, or exposure to ticks or exotic animals. Of note, she reported that her dose of lamotrigine has been slowly uptitrated, most recently two days prior changing from 50 mg BID to 75 mg BID.

Vitals: T 39.6°C; HR 140; BP 102/66; RR 15; O2 sat 97% on RA

General: Alert and oriented, well-developed female in no acute distress

Cardiovascular: Tachycardia, regular rhythm

HEENT: Bilateral cervical lymphadenopathy; facial edema; conjunctiva clear; oral mucous membranes clear

Skin: Deeply erythematous/papules coalescing into plaques diffusely on the face, trunk, extremities; no pustules, purpura, or vesicles/bullae noted; no scales or desquamation

Complete Blood Count (CBC): WBC 9.84 (eosinophils 7%), Platelets 144

Alkaline phosphatase (ALP): 180

ALT: 378

AST: 228

C-reactive protein (CRP): 91

Urine protein: 11 mg/dL

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome. DRESS syndrome is an idiosyncratic, potentially fatal, adverse drug reaction to anticonvulsants, antimicrobials, antivirals, or allopurinol with a multifactorial pathogenesis. It has a delayed onset, typically within 2-8 weeks, after drug initiation. Clinical presentation can be diverse but is usually characterized by a diffuse skin rash, fever, lymphadenopathy, eosinophilia, and internal organ involvement (most commonly the liver). Incidence ranges from 1 in 1,000 to 1 in 10,000 with a mortality rate that can be as high as 10%, and is commonly related to fulminant hepatitis. Diagnosis can be extremely challenging due to the variability in clinical presentation. The regiSCAR diagnostic criteria is the most used diagnostic criteria and is based on a scoring system for possible, probable, or definite diagnosis.

Immediate discontinuation of the offending medication is critical. Symptoms may continue for several weeks after withdrawal of the inciting agent. The mainstay of treatment is systemic corticosteroids with tapering over a long period. Other immunosuppressants may be used in refractory cases. If DRESS syndrome occurs in the setting of an aromatic anticonvulsant, the patient should not be started on any other aromatic anticonvulsants due to their cross-reactivity.

Take-Home Points

  • DRESS syndrome is a severe, possible life-threatening adverse drug reaction that is frequently overlooked and missed because of its variable clinical presentation. Increasing familiarity with its clinical presentation is of utmost importance for recognition and treatment.
  • The inciting agent (aromatic anticonvulsant, antimicrobial, allopurinol, etc.) should be stopped immediately and should not be restarted at any time. Corticosteroids are the mainstay of treatment with gradual tapering over multiple months.
  • Cho YT, Yang CW, Chu CY. Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS): An Interplay among Drugs, Viruses, and Immune System. Int J Mol Sci. 2017 Jun 9;18(6):1243. doi: 10.3390/ijms18061243. PMID: 28598363; PMCID: PMC5486066.
  • Choudhary S, McLeod M, Torchia D, Romanelli P. Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) Syndrome. J Clin Aesthet Dermatol. 2013 Jun;6(6):31-7. PMID: 23882307; PMCID: PMC3718748.

By |2023-12-15T09:19:09-08:00Dec 18, 2023|Dermatology, SAEM Clinical Images|
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