Senior Report 5.15

Case Presentation by Dr. Shereaf Walid

CHIEF COMPLAINT(S): Chest pain.

HISTORY OF PRESENT ILLNESS:

A 33-year-old obese African American female presents with right-sided pleuritic chest pain that started yesterday afternoon suddenly while she was walking at work.  She works a clerical job where she sits down at a desk for the majority of the day; however, she says she does not stay seated for long intervals, as her work requires her to do a lot of walking around the office.  The pleuritic chest pain is maximum at end inspiration.  She feels the pain on the right side of her chest around the anterior axillary line of the chest wall.  The pain radiates to her neck, below her axilla, and in the lateral right rib/chest wall area.  The pain seems worst on her right side below the axilla in the mid-axillary line near the costal margin.  The pain is reproducible by taking a deep breath in, and since it started, she has been taking shallow breaths in to reduce the pain.  The discomfort is also pruritic, but she notes no rash.  She appears to be splinting upon deep inspiration.  She denies any leg swelling or pain and no recent surgery.  No fevers or dyspnea on exertion.  She does not take any oral contraceptive pills.  She has had no trauma to the chest wall.  This pain episode is not associated with any nausea, vomiting or diaphoresis.  She has never experienced pain of this character in the past.  No personal history of hypercoagulable disorders, or family history of blood clots.

REVIEW OF SYSTEMS:

CONSTITUTIONAL:  No recent weight loss.

EYES:  No double vision, itching, pain or discharge.

ENMT:  No ear infection, epistaxis or current dental problems.

CARDIOVASCULAR:  No palpitations.

RESPIRATORY:  Right-sided chest pain that is pleuritic since yesterday.

GASTROINTESTINAL:  No recurrent pain, diarrhea or bloody stools.

GENITOURINARY:  No burning or frequency on urination.

MUSCULOSKELETAL:  No muscle pain or weakness.

NEURO:  No loss of consciousness, speech or balance problems.

SKIN:  No rashes.

PAST MEDICAL, FAMILY, AND(OR) SOCIAL HISTORY:

PRIMARY CARE PHYSICIAN:  Dr. XXXXX

PAST MEDICAL HISTORY:  Negative for hypertension, diabetes or hypercholesterolemia.

PAST SURGICAL HISTORY:  Tubal ligation.

MEDICATIONS:  None.

ALLERGIES:  Shellfish.

LAST MENSTRUAL PERIOD:  Two weeks ago.

FAMILY HISTORY:  Hypertension, diabetes.

SOCIAL HISTORY:  Positive for tobacco use and occasional alcohol use, negative for any illicit or IV drug use.

EXAMINATION OF ORGAN SYSTEMS-BODY AREAS:

VITAL SIGNS:  On presentation blood pressure 154/79, heart rate is 92, respiratory rate is 20, oral temperature is 36.9, SaO2 100% on room air.

CONSTITUTIONAL:  Obese female in no apparent distress but anxious.  She is taking shallow quick breaths.  Her respiratory rate seems to be only mildly elevated, but for the most part she is sitting comfortably and she speaks in complete sentences.  No tripoding.

PSYCHIATRIC:  A and O x3.  Judgment is sound.

HEAD:  Normocephalic, atraumatic.  No tenderness to palpation.

EYES:  Pupils are equal, round and reactive to light and accommodation.  Extraocular movements are grossly intact.  No conjunctival pallor.  Sclerae anicteric.

ENMT:  Mucous membranes are moist.

NECK:  Supple.  No JVD.  No carotid bruits.  No lymphadenopathy.

CARDIOVASCULAR:  S1, S2.  Regular rate and rhythm.  No murmurs, rubs or gallops.

RESPIRATORY:  Clear to auscultation bilaterally.  No wheezes, rhonchi or rales.  Decreased air entry due to shallow respirations.  No tenderness anywhere over the chest wall.  No palpable crepitus over the chest wall or the neck.

GASTROINTESTINAL:  Abdomen is soft, nontender, nondistended.

MUSCULOSKELETAL:  Strength is 5/5 proximally and distally in both the upper and lower extremities.  No palpable cords in the lower extremities.  Gastrocnemius diameter is equal bilaterally.  No swelling.  No dependent edema.  2+ dorsalis pedis pulses bilaterally.

SKIN:  No acute rashes or lesions.

NEURO:  Normal speech and normal gait.

An ECG and Chest X-Ray were obtained and are shown below:

Question 1:

What is the most specific chest X-ray finding seen in patients with diagnosed pulmonary embolism?

a.   cardiomegaly

b.   elevated hemidiaphragm

c.   hampton’s hump

d.   plate-like atelectasis

e.   small pleural effusion

Question 2:

What is the patient’s Well’s score for pulmonary embolism?

a.  0

b.  1

c.  2

d.  3

e.  4

Question 3:

Which of the following interventions is the most reasonable next step for this patient?

a.  cat scan of the thorax with intravenous contrast

b.  draw blood cultures and start the patient on intravenous antibiotics for community acquired pneumonia

c.  obtain a d-dimer assay

d.  start the patient on PO antibiotics, an incentive spirometer and arrange close outpatient follow-up

e.  ventilation-perfusion (V/Q) scan due to the patient’s shellfish allergy, which puts her at risk for an allergic reaction to iodinated radiocontrast dye

 

 

Answers:

1.  c

2.  d

3.  c

 

This case is of an actual patient seen in the ED.  The patient has a pulmonary embolism.  This patient presents with no traditional risk factors for PE, although several studies quote obesity as an indirect risk factor due to relative immobility and increased risk for lower extremity DVT.  She has significant pleuritic chest pain and splinting, yet her oxygen saturation is 100%.  An ECG and CXR are indicated in the ED as initial screening tests in evaluating a patient with suspected pulmonary embolism due to being relatively inexpensive and accessible, and to assess for other potential causes.  The American College of Radiology recommends chest radiography as the most appropriate study for ruling out other causes of chest pain in patients with suspected pulmonary embolism.

The ECG provided in this case shows normal sinus rhythm.  At triage the patients HR was 92.  The most common ECG finding in patients with confirmed pulmonary embolism is sinus tachycardia.  It is important to note that this patient’s heart rate is usually in the 70s, this data was not provided in the case, but was ascertained by reviewing the electronic medical record.  Hence, her relative tachycardia is an important sign to recognize and consider in formulating a differential diagnosis. The finding of S1 Q3 T3 is nonspecific and insensitive in the absence of clinical suspicion for pulmonary embolism. Classic findings of right heart strain and acute cor pulmonale include tall, peaked P waves in lead II (P pulmonale); right axis deviation; right bundle-branch block; presence of an S1 Q3 T3 pattern; or atrial fibrillation. Unfortunately, only 20% of patients with proven pulmonary embolism have any of these classic electrocardiographic abnormalities. If electrocardiographic abnormalities are present, they may be suggestive of pulmonary embolism, but the absence of such abnormalities has no significant predictive value.  Some studies have shown that T wave inversions in anterior precordial and inferior leads is the most specific ECG finding in patients with confirmed pulmonary embolism.

The Chest X-ray shown in this case was extremely valuable in this patient’s evaluation and is often under-appreciated and under-utilized.  It was initially read as negative by radiology, however, if you examine closely there are findings that are suggestive of pulmonary embolism in the correct clinical context.  Although CXR cannot establish the diagnosis of PE, it has several useful roles in patients suspected of having a PE.  When examining radiographs, the following findings should be sought.

First, the classic finding is a relatively normal chest radiograph in a patient in “dire straights”.  However, this “classic” teaching is not true, as observations from the PIOPED and PIOPED II studies and several others have shown that a normal CXR is found in only 12-18% of patients with confirmed pulmonary embolism.  So keep in mind that an apparent “normal” CXR is suggestive of PE in that it can lower the suspicion of other disorders such as pneumothorax, pulmonary edema or pneumonia.  Next, there are radiographic abnormalities seen in some cases of PE, although these are non-specific, such as small pleural effusion, plate-like atelectasis, or elevation of a hemidiaphragm.  Occasionally, there are radiographic findings that are characteristic of PE, although they may be subtle and difficult to identify with certainty.  Occlusion of a large pulmonary artery can produce localized oligemia (which looks like diminished lung markings in the region supplied by that vessel) and the occluded pulmonary artery may be dilated proximally due to a large intraluminal thrombus and then taper abruptly (the knuckle sign).  Seen together, localized oligemia and a dilated pulmonary artery with abrupt cut-off are termed the Westermark sign.  Sometimes, you’ll see dilation of the contralateral pulmonary artery because the affected pulmonary artery contains an embolism that is so massive that nearly all the blood flow is directed to the contralateral pulmonary artery.  This is called a “reversed Westermark sign”.

You should note that the chest x-ray in this case is a poor inspiratory film.  Never overlook this as studies that you may be basing your findings on will exclude inadequate films in their data analysis, hence the utility of searching for specific or sensitive findings of pathology may fail before you have even started looking.  With that in mind, if you look closely at the right costophrenic angle on the PA view of the CXR, you’ll notice that the angle is blunted when compared to the left side.  There is clearly a hazy opacity there.  Additionally, there appears to be an elevated right hemidiaphragm, a non-specific finding that is sometimes found in cases of PE, but is neither sensitive nor specific.  It should be noted that the right hemidiaphragm is normally elevated relative to the left due to the liver and this can make the distinction between normal and abnormal difficult.  In this case, the elevated right hemidiaphragm is easier to appreciate on the lateral view, as with most cases that show this finding.  Now after assessing the lateral CXR, you can follow the left and right hemidiaphragmatic lines back to the posterior rib margins near their insertion sites.  You can also clearly see the costophrenic sulcus made by these structures posteriorly.  The “haziness” seen on the PA view is probably anteriorly located, as suggested by the lateral view, or you could argue that its location is obscured (again, this is equivocal possibly due to the inadequate film).  One thing you can definitely conclude is that the haziness seen on the PA view is not a pleural effusion, or else it would have blunted the most dependent area of the lung, the posterior costophrenic sulcus.  This should raise the suspicion of a “Hampton’s Hump.”

In some cases of PE, there is an area of focal airspace filling.  This represents focal intraparenchymal hemorrhage due to ischemia or infarction of lung tissue that occurs distal to a large embolus.  The area of hemorrhage is typically located at the lung periphery and appears as a wedge-shaped pleural-based opacity with its apex pointing toward the lung hilum.  This has been termed “reversible infarction” because it clears rapidly over several days, reducing in size progressively without residual scarring.  This focal area is called a Hampton’s Hump and should be suspected in this patient.  Also note that the elevated right hemidiaphragm is much easier to appreciate on the lateral CXR.  Finally, non-specific findings radiographic findings in PE are:  “normal”, small pleural effusion, plate-like atelectasis and elevated hemidiaphragm.  Specific abnormalities are the Westermark sign and Hampton’s hump, hence the answer to question 1 is “c”.

 

Now after obtaining the ECG and CXR the most reasonable next step in the patient work-up should be to evaluate the patient’s pre-test probability for a pulmonary embolism. Evidence-based literature supports the practice of determining the clinical probability of pulmonary embolism before proceeding with testing. One study assessed the performance of 4 clinical decision rules in addition to d-dimer testing to exclude acute PE. All 4 rules – Wells rule, simplified Wells rule, revised Geneva score, and simplified revised Geneva score, showed similar performance for excluding acute PE when combined with a normal d-dimer result.  Several studies have validated the use of the Wells criteria for pulmonary embolism in the ED setting.  The Wells scoring system is as follows:

Hence, based on this scoring system what would your score be?  There are no clinical signs of DVT.  On exam, the patient’s gastrocnemius diameter is equal without palpable cords, something that should be clearly documented in all cases of suspected pulmonary embolism.  The heart rate is less than 100 bpm.  There is no history of immobilization.  Please note that to qualify for “history of immobilization” according to Wells, and Jeff Kline’s PERC score, one must have uninterrupted immobility in a knees-flexed position for 6 consecutive hours.  So if the patient gets up to stretch their legs half way through a trans-Atlantic flight there is no history of immobilization as per these scoring systems.  However, these scoring systems are not, under any circumstance, designed to replace clinical “gestault”, but to supplement it.  In other words, never abandon your clinical suspicion in lieu of a clinical scoring system.  The patient has no objectively diagnosed PE or DVT.  There is no history of hemoptysis or active malignancy.  Also, note that prior malignancy is not a risk factor (another point of confusion).  The Wells score includes malignancy with treatment within 6 months because only active malignancy is a risk factor, not remission.  Finally, is PE the #1 diagnosis or equally likely?  If you answer no for this then your Wells score is zero.  If you answer yes then it is 3.  My Wells score on this patient was 3.  If you are not suspecting PE in this patient, then what is the more likely diagnosis?  Hence, the answer to question 2 is “d”.  More on this later if you answered zero.

Upon calculating a Wells score of 3, we now have to decide what to do with this data.  In a seminal study by Jeff Kline he used a test threshold formula to derive the test threshold for PE to be 1.8% (this is essentially the derivation of the PERC rule).  What this means is that when the pre-test probability for PE is less than 1.8% then pursuing testing is more likely to do more harm than good (ie. the false positives and potential harm inflicted by treatment outweighs treatment of true positives).  During grand rounds I will discuss the concept of test threshold and treatment threshold more thoroughly.  For now, recognize that for every test there is a test threshold, below which, testing does more harm than good.  And, on the other end of the spectrum, there is a treatment threshold, above which testing does more harm than good (ie. your pre-test probability is so high that a negative d-dimer has poor negative predictive value and you should go straight to CT/ treatment).  The patients that fall between the test threshold and the treatment threshold are the ones where d-dimer should be used.

There are 3 studies that I will comment on at this point, two of which are cited on MD-calc (the website from which the above figure was obtained).  The first is “Excluding pulmonary embolism at the bedside without diagnostic imaging: management of patients with suspected pulmonary embolism presenting to the ED by using a simple clinical model and d-dimer.”  Wells PS et al.  Ann Intern Med. 2001.  In this study, low risk patients, defined as Wells score of 0-2 had a 1.3% risk of pulmonary embolism, hence, they were below the testing threshold which means that testing is more likely to do harm than good.  The second study is “Prospective validation of Wells Criteria in the evaluation of patients with suspected pulmonary embolism.”  Wolf SJ et al.  Ann Emerg Med. 2004.  In this study, low risk patients, also defined as Wells score of 0-2 had a 1.7% risk of pulmonary embolism, also below the testing threshold indicating that testing is more likely to do harm than good.  Finally, in one of the most impressive studies on PE decision rules, “Effectiveness of managing suspected PE using an algorithm combining clinical probability, d-dimer testing, and CT.” Christopher Study Investigators. JAMA, 2006, in a large ED patient population this impact study tested the validity of using d-dimer on patients with Wells scores of 3 or 4 and found it to be safe.  Remarkably, 6 months later, there were less adverse outcomes in using d-dimer in Wells ≤ 4 than CT scan.  Additionally, 15% of the patients studied were inpatient, a population known to be at higher risk than those seen in the ED, and the data, in the context of PE/DVT studies was compelling.  This study used a simplified decision tree: if Wells ≤ 4 use d-dimer; if > 4 use CT.  For more details, read the article.

The mistake often made with d-dimer is that it’s used on the wrong population (ie. those below the testing threshold = Wells 0-2).  If you’re doing this, you are practicing dangerous medicine.  What the Christopher study showed was that our collective “gestault” is greatly skewed and we teach the application of d-dimer grossly incorrectly.  If you look at the table for Wells score for PE, if a patient presents with flu-like symptoms but they have a history of DVT, hemoptysis and are currently being treated for cancer, then their Wells score is 4 and you should order a d-dimer.  Let me repeat this validated point: a patient with history of DVT, hemoptysis and active cancer should still get a D-dimer.  And here is the take home point:  this is what d-dimer was designed for.  There are large, validated studies that show that doing less is safer than doing more.  Additionally, from a medico-legal standpoint, it is my belief and practice that the Christopher study should be mentioned in your dictation or macro for PE and is so easily defensible as to be a powerful enough deterrent to keep litigation at bay.  In other words, a prosecuting attorney will be hard pressed to take a case with this referenced in the patient’s chart.  Let’s never forget that we don’t practice medicine for attorneys, we practice for patients.  However, as we all know, we don’t practice in a vacuum.

Finally, the answer to question 3 is “c”.  Initially, my Wells score was zero (was I right?).  After reviewing the CXR, initially read by radiology as negative, I noted a Hampton’s hump (specific, relatively), not a pleural effusion, and an elevated right hemidiaphragm (non-specific).  Since the patient’s clinical presentation did not fit pneumonia, I concluded that this was more likely a Hampton’s hump than a pneumonic infiltrate.  I re-calculated her Wells score to be 3, ordered a d-dimer, electrolytes and gave her 1L of normal saline for renal protection in case the d-dimer came back positive.  Should I have just ordered a serum creatinine?  As an aside, regarding the allergic cross-reaction of shellfish and radio-iodinated contrast dye, the literature has shown that this is a fallacy.  To date, I have not found any literature to support this and there is a robust amount of literature to refute this correlation.  Please feel free to read the following review,  “The relationship of radiocontrast, iodine, and seafood allergies: a medical myth exposed.”  Schabelman E, Witting M. J Emerg Med. 2010 Nov; 39(5):701-7.

The d-dimer came back elevated at 1.33. CT scan – PE protocol was ordered and was reported by radiology as, “positive for pulmonary emboli in the superior segment of the right lower lobe pulmonary artery with associated pulmonary infarction and in a posterior basilar branch of the right lower lobe pulmonary artery.  There is also a triangle shaped airspace opacity in the anterior aspect of the right upper lobe without definite associated pulmonary embolus.  This finding could represent a focal area of atelectasis.”  The lung window of the CT scan is found below, clearly showing the associated pulmonary infarct, or Hampton’s hump.

 

 

“Therefore, at this point we sent a set of coagulation studies on the patient and started the patient on fondaparinux and called to have the patient admitted.  Results of the CT PE protocol were explained to the patient and the reason for admission has also been explained to her.  She is obviously agreeable to the necessity of this admission.”  In the near future, the validity of the last sentence of the quoted dictation will be challenged, but for now all confirmed pulmonary embolisms are to be admitted.  Warfarin was also appropriately started in the ED and the patient was discharged within 48 hours.  Outpatient follow-up was arranged and a repeat CXR showed resolution of the pulmonary infarct with anticoagulation rather than antibiotics further confirming the suspicion of a Hampton’s hump.  Six months out the patient had no recurrences and has made lifestyle modifications.  As of one month ago the patient continues to do well and, as per her routine, frequently flexes her gastrocnemius muscles while working at her desk.

 

Intern Report 5.14

Case Presentation by Dr. Jamie Kenney

HPI: 5 year old male presents to the ED complaining of a rash.  According to his parents he recently returned home from visiting his grandparents in Tennessee and has been complaining of “not feeling well” for the 4 days.  He has had a decreased appetite with nausea and vomited 3 times yesterday.  He is also complaining of feeling “hot” but his parents did not take his temperature at home.  They first noticed the rash yesterday evening that began on his hands and feet and has since spread to his abdomen.  Rash does not itch, no blistering or pus formation.  He was given Tylenol at home with no relief.  Patient is also complaining of a severe headache 9/10, pulsating, does not radiate to neck.  No one else in the family has similar symptoms.

Review of Systems:

Constitutional: Positive for “feeling hot” and fatigued

Head: Negative for neck pain, positive for headache

Eyes: Negative for redness, pain or discharge

ENT: Negative for runny nose, ear pain or sore throat

Cardiovascular: Negative for palpitations or chest pain

Lungs: Negative for shortness of breath or cough

Gastrointestinal: Positive for nausea/vomiting and abdominal pain

Musculoskeletal: Positive for body aches and swelling, negative for joint deformities

Skin: Positive for rash, no ulcers or lesions

Neurologic: Negative for numbness or tingling

Past Family, Medical and Social History

Medical Hx: Asthma

Surgical Hx: None

Medications: Albuterol

Allergies: NKDA

Examination of Organ Systems and Body Areas

VS: BP 90/56 HR115 RR14 Temp 40.1 Pulse ox 98% on room air

General: Patient is well nourished, in mild distress.  He is lying on the stretcher in the fetal position and appears uncomfortable.

HEENT: NC/AT, EOMI, PERRLA, non-icteric, neck supple no meningeal signs

Mouth: Dry mucus membranes, no lesions or vesicles

Cardiovascular: S1/S2, tachycardic, no m/r/g

Respiratory: CTABL, no wheezing or rhonci

Gastrointestinal:  Hyper-active bowel sounds, TTP diffusely, hepatomegaly, no masses, no rebounding or guarding

Musculoskeletal: NROM, TTP over all four extremities.  No cyanosis, clubbing.  Subtle non-pitting edema of lower extremities.

Skin: Diffuse palpable purpura located over all four extremities including the palms and soles.  Rash is non-blanching, non-vesicular and symmetric.  (See Images Below)

Neurologic:  Patient is awake, oriented x 3.  Not interactive but responds appropriately to all questions.  Strength 5/5 in upper extremities, 4/5 in lower extremities. Normal finger to nose and heel to shin.  Reflexes +2 and symmetric bilaterally.  Gait was normal.

Laboratory Values

WBC: 8 Hgb: 10.2 Hct: 35 Plt: 90

Na: 124 K 3.8 HCO2: 24 Cl: 102 BUN 18 Cr 0.9

INR: 2.0  PT: 18 PTT: 60

AST: 100 ALT: 180

IMAGES:

 

 

QUESTIONS:

1) What is the Diagnosis?

2) When does the rash typically appear?

  1. Within 24hrs of exposure
  2. Immediately after the fever breaks
  3. One to two weeks after exposure
  4. On the third to fifth febrile day

3) Treatment for this patient consists of:

  1. Doxycycline PO 2.2mg/kg Q12 hrs
  2. Vancomycin/Cefepime pharmacy to dose
  3. Doxycycline IV 100mg BID
  4. Ampicillin/Sublactam IV 3g Q6hrs

DISCUSSION 

This patient has Rocky Mountain Spotted Fever (RMSF).  RMSF is defined as an acute, febrile, systemic tick-borne illness caused by Rickettsia rickettsii that can be found in North, South and Central America.  Within the United States the most frequent occurrences of RMSF occur in Oklahoma, North Carolina, South Carolina, Tennessee and Pennsylvania (hence the hint with Blue Ridge Mountains).  The history of RMSF dates back to the late 1800’s and was known by the White settlers as “black measles.”  Within the past decade the number of reported cases has more than tripled, especially in suburban areas.  RMSF ranges in clinical severity from a subclinical illness to a fulminant disease with vascular collapse.  There are approximately 40 deaths per year in the United States with the most at risk age groups being children younger than 10 years and adults over 60 years old.

Rickettsia rickettsii are obligate intracellular bacteria that can invade both the nucleus as well as the cytoplasm.  Ticks are the vectors for RMSF with the American Dog Tick, Dermacentor variabilis and the Rocky Mountain Wood Tick, Dermacentor andersoni being the most common species within the United States.  Ticks will feed on any warm blooded mammal transporting the organism from animals to humans.  After R. rickettsii enters the host the organisms it will invade and multiply in the vascular endothelial cells.  They continue to invade into deeper tissues disrupting vessel walls and infecting vascular smooth muscle.  The organisms are able to move from cell to cell by actin-based motility.  Their invasion causes the release of tissue plasminogen activator and von Willebrand factor further leading to microhemorrhage, microthrombus formation, and increased vascular permeability.  These extensive vascular lesions are what cause most of the features associated with RMSF.  Increased small-vessel permeability results in hypotension, edema and increased extravascular fluid space.

Clinical features of RMSF are initially vague and it is hard to make the diagnoses without a high index of suspicion.  The “common” triad is fever, rash and known tick bite.  However it is rare that emergency physicians will have all three and only 3-18% of all cases actually have the triad.  Early symptoms include sudden onset of fever, severe headache, nausea, vomiting and myalgias.  The rash, present in 88% of patients with RMSF is the most distinctive feature of the disease.  It normally appears on the 3rd to 5th febrile day.  Vasculitis and changes in vessel permeability cause the early features of the rash and subsequent petechial and hemorrhagic lesions are secondary to thrombocytopenia.  The rash typically starts on the wrist and ankles then begins to spread centripetally to the trunk within 6-12 hours.  One unique feature of the rash is its presence on the palms and soles (please refer to pictures above).  Initally the rash consists of small red macules that worsen when a warm compress is applied.  After 2-3 days it becomes maculopapular and no longer blanches with pressure.  Without the appropriate treatment the lesions will coalesce to form large areas of ecchymosis that eventually slough off and form indolent ulcers.

RMSF has the potential to spread rapidly throughout the body if not treated properly.  As R. rickettsii continues to invade the vasculature it can lead to some life threatening conditions such as myocarditis, arrythmias, interstitial pneumonitis, hepatosplenomegaly and several neurologic manifestations ranging from headache to seizures and coma.

Diagnosis in the emergency department relies heavily on physicians being aware of the prominence of the disease within their region.  Clinic evidence is the mainstay of diagnosis within the ED.  Initial laboratory testing may show some non-specific abnormalities such as hyponatremia, prolonged PT/PTT, thrompocytopenia and elevation of LFTs if hepatomegaly is present.  A definitive diagnosis requires positive results on one or more of the following tests: serologic serum antibody titer reactive with R. rickettsii, skin biopsy or direct isolations and identification of the organism in a cell culture.

Treatment consists of supportive care and antibiotic therapy.  Antibiotic therapy is most effective when given within the early stages of the disease; when the rash first appears or prior to that.  Doxycycline is regarded as the therapeutic agent of choice.  The dosing for adults is 100mg BID PO/IV and for children (<45kg) 2.2mg/kg PO Q12hrs.  Chloramphenicol should be used for patient whom are known to be allergic to tetracyclines and for pregnant women unless they are near term.  Therapy should last for 7-10 days or until the patient is afebrile for 2-5 days.  Steroids are a controversial topic and are currently not recommended.  Without appropriate antibiotic treatment fatality rates rise to 25% from 5%.

All cases of RMSF should be reported to the CDC.

References:  Rosen’s Emergency Medicine 7th Edition, Tintinalli’s Emergency Medicine 7th Edition and www.dermatlas.jhmi.edu (pictures)

Senior Report 5.13

Case Presentation by Dr. Aimee Nefcy

CHIEF COMPLAINT:

“I feel so weak.”

HISTORY OF PRESENT ILLNESS:

This 20-year-old male comes into the emergency department brought in by his mother.  She states that he was at St. John’s Hospital 1 week ago and he went there because he was having a hard time breathing associated with chest pain, which has been going on for about 2 weeks.  They thought he was having an asthma exacerbation.  She has some paperwork, which showed that he had a CT scan for PE that was negative and he had laboratory testing which apparently ruled out lupus.  This was done because he had a rash across his face which they told her was from lupus.  She says that he has just not been getting better.  She noticed that he is complaining of profound weakness and pain in his chest, back, shoulders and thighs, which is kind of an achy, constant, non-pleuritic pain in his muscles.  He is complaining of having no strength.  They discharged him home on a medrol dose pack, which she has been giving to him appropriately.  She says nothing seems to be helping him.  He has only been getting worse and now he is complaining of so much of a hard time breathing that she is just sure that he is having an asthma flare-up, so that is why she brought him to the emergency department at this time.  His shortness of breath is constant and non-exertional, without exacerbating or relieving factors.  The patient himself is not providing any history at this time, except to say that he is too weak to talk or cooperate.

REVIEW OF SYSTEMS:

CONSTITUTIONAL:  Negative for fever and positive for chills.

EYES:  Negative for redness or discharge.

ENT:  Negative for runny nose or sore throat.

CARDIOVASCULAR:  Positive for chest pain and dizziness.

RESPIRATORY:  Positive for shortness of breath as noted above and negative for cough.

GASTROINTESTINAL:  Negative for vomiting or diarrhea.

GENITOURINARY:  Negative for changes in urination or dysuria.

MUSCULOSKELETAL:  Positive for diffuse muscle aches and negative for swelling or deformity.

SKIN:  Negative for lesions and is positive for rash around his eyes for 2 months.

NEUROLOGIC:  Negative for numbness and positive for global weakness.

PAST MEDICAL, FAMILY, AND SOCIAL HISTORY:

PAST MEDICAL HISTORY:  Mom reports that he had asthma as a child, but has not had to use an inhaler in many years.

PAST SURGICAL HISTORY:  No surgeries.

MEDICATIONS:  Prednisone and a multivitamin as prescribed by St. John’s 1 week ago.

ALLERGIES:  NO KNOWN DRUG ALLERGIES.

FAMILY HISTORY:  Significant for asthma and allergies.

SOCIAL HISTORY:  The patient used to smoke cigarettes.  He smokes marijuana occasionally.  He denies any current tobacco or alcohol use.

 

EXAMINATION OF ORGAN SYSTEMS-BODY AREAS:

VITAL SIGNS:  Blood pressure is 116/80, heart rate 115, respiratory rate 26, temperature 36.2, pulse ox 99% on room air.

GENERAL:  This is a well-nourished, well-developed African-American male appearing his stated age.  He is in no acute distress.  He is lying on the stretcher not moving with his eyes closed and his arms at his sides, and appears ill and fatigued.

PSYCHIATRIC:  The patient is sleepy, but answers questions appropriately.  He is oriented x3.  He responds to voice.  He is basically refusing to speak or cooperate with the exam, saying over and over “I’m too weak,” despite encouragement from staff and mother; history was obtained from his mother.

HEENT:  Head is normocephalic, atraumatic.  Pupils were equal, round, and reactive to light.  Extraocular muscles were intact.  There is no conjunctival pallor or scleral icterus.  The patient did have diffuse patchy hyperpigmented rash in the periorbital area and involving the lower portion of his forehead, above both eyes, and sparing the eyelids and the nose but extending down his cheeks laterally on both sides.  It was irregular and asymmetric.  It was not erythematous.  It was flat and nonpalpable.  No other abnormalities were noted.

MOUTH:  Mucous membranes moist without signs of oral ulcerations.

THROAT:  No erythema or exudates.

NECK:  Supple with no lymphadenopathy or thyromegaly.

CARDIOVASCULAR:  Regular rhythm.  Tachycardia.  S1 and S2 heard.  No murmurs, rubs, or gallops.  Peripheral pulses were 2+ and symmetric bilaterally.

RESPIRATORY:  The patient was having mild tachypnea with rapid shallow breathing.  He was making a poor respiratory effort.  He was not using accessory muscles or having any retractions.  On auscultation he had decreased air entry bilaterally.  There were no wheezes, crackles, or rhonchi.  Again, he had poor effort.

GASTROINTESTINAL:  Positive bowel sounds.  Abdomen is soft, nontender, nondistended.  No masses or organomegaly.

MUSCULOSKELETAL:  Limbs were atraumatic and nontender.  No cyanosis, clubbing, or edema.

SKIN:  Warm and dry without any lesions.  There were no other rashes except as noted above.  Of note, the skin of his hands was very rough and dry.

NEUROLOGIC:  There was no facial asymmetry or dysarthria.  Muscle strength was 4/5 distally in all four extremities, but was 3/5 proximally.  The patient was unwilling or unable to lift up his arms at the shoulder, but was able to move his arms at the elbow and able to move his feet and bend his legs, but was unable to lift his legs against gravity.  He was so profoundly weak that he was unable to even use the railings of the stretcher to sit up and we actually had to hold him up, which was quite difficult as he was very heavy and floppy.  Sensation was intact to light touch throughout all four extremities and was symmetric bilaterally.  The patient was unable to participate with testing for pronator drift or finger-to-nose.  He had 1+ reflexes that were symmetric bilaterally.  He had diffuse hypotonia of his muscles with no rigidity or clonus. Remainder of the neurologic exam could not be performed as the patient really was not cooperating.

Laboratory values

Na 141

K 3.9

Cl 103

HCO3 27

AG 11

Glucose 49

BUN 16

Cr 1.0

Ca 9.6

Mg 2.2

Phos 2.3

ALT 39

AST 53

CPK 447

WBC 4.1

Hgb 14.3

Hcrt 41.6

PLT 244

Albumin 3.9

TSH 2.563

CRP <2.90

ESR 34

ANA Negative

ENA Panel Negative

U/A Tr Ket, Tr Prot, SpGrav 1.020, otherwise negative

CXR: unremarkable.

For his hypoglycemia, the patient received an ampoule of D50, after which his Accucheck improved to 99, but he did not experience any improvement in his symptoms of weakness and shortness of breath.

Questions:

1)     Based on the information above, what is the patient’s most likely diagnosis?

2)     What diagnostic test would be most helpful in confirming the diagnosis?

  • a) Complement levels
  • b) CT scan of the chest
  • c) EKG
  • d) Muscle biopsy
  • e) The diagnosis is clinical

3)     What is the initial treatment of choice for this condition?

  • a) Glucocorticoids
  • b) Hydroxychloroquine
  • c) Immunosuppressants
  • d) IVIG
  • e) Plasmapheresis

ANSWERS:

1) Dermatomyositis

2) Muscle biopsy

3) Glucocorticoids

  

DISCUSSION:

Dermatomyositis in the juvenile form classically affects 5-15yo children with greater prevalence in girls.  The clinical presentation is dominated by symmetric proximal muscle weakness and accompanied by skin manifestations including the characteristic heliotrope rash (pictured below) or less commonly a malar rash, as well as shawl-distribution erythema of the upper back and chest which is photo-sensitive, periungual abnormalities, “mechanic’s hands,” psoriasiform scalp changes, and linear violaceous streaks on the trunk called flagellate erythema.  Gottron’s papules are flat erythematous papules on the extensor surfaces of the fingers.  Calcinosis cutis, deposition of calcium in the skin, is common in the juvenile form but rare in adults.  Other non-cutaneous manifestations include malaise, low-grade fever, anorexia, weight loss, fatigue, headache, myalgias and arthralgias, and dysphagia.  The adult form primarily affects ages 40-50 years but can occur in any age, and is associated with underlying malignancy.

More serious manifestations of dermatomyositis are common.  Interstitial lung disease occurs in 10% of cases, and may require invasive respiratory support, which may also be required for severe diaphragmatic and chest wall muscle weakness causing respiratory insufficiency.  Signs of respiratory compromise such as hypoxia on room air should prompt arterial blood gas analysis and testing by respiratory staff for measurement of negative inspiratory force, peak flow, and forced vital capacity.  Cardiac manifestations include myocarditis which may lead to elevated troponins but usually does not cause clinically significant heart failure.  Esophageal involvement may lead to severe dysphagia and even aspiration.

Polymyositis is differentiated from dermatomyositis by the lack of cutaneous manifestations, and muscle findings – including pulmonary, cardiac, and esophageal effects – and treatment options remain the same in both diseases.

Diagnosis of dermatomyositis is based on the presence of classic clinical findings, supported by elevated muscle enzymes, and confirmed with muscle biopsy showing atrophy and vascular C5b-9 deposition in the perimysial blood vessels.  EMG can also be useful for ruling out other pathology such as myotonic dystrophy, and typical findings in DM are increased membrane irritability, spontaneous fibrillations, abnormal motor potentials, and complex repetitive discharges.

The treatment of dermatomyositis primarily consists of high-dose steroid therapy, which may be oral for outpatient treatment or may be intravenous for more serious clinical manifestations of weakness or lung involvement.  Patients who are poorly responsive to glucocorticoids may require more aggressive therapy such as IVIG, immunosuppressant drugs (methotrexate, cyclosporine, azathioprine), or in anecdotal cases, hydroxychloroquine.  Plasmapheresis has no role in the treatment of dermatomyositis.

OUTCOME:

Rheumatology was consulted while the patient was in the Harper ED, and he was admitted to the service of his private PMD.  Dermatology, ID, neurology, and general surgery (for muscle biopsy) also followed him on the floor.  All services were in agreement as to the likely diagnosis of dermatomyositis, and although lupus was also a consideration it was felt to be less likely as he did not have significant arthralgias, photosensitivity, or oral ulcerations.  His facial rash was felt to be atypical of DM, and derm was concerned it was similar to that of systemic amyloidosis, so serum protein electrophoresis was performed, but was found to be normal.  A high-resolution chest CT was performed to rule out pulmonary involvement, and was negative.  RT performed testing showing an FVC of 4.1 L and an NIF of -80 cmH2O (both normal).  He was started on 60mg of oral prednisone daily, after which he experienced dramatic improvement.  He was discharged on HD#4 with instructions to continue the same dose of prednisone for at least 2-4 weeks and was given appropriate follow-up with all involved services.  Muscle biopsy results later demonstrated atrophy and other findings confirming the diagnosis of DM.  EMG results were not found in the EMR.

Senior Report 5.12

Case Presentation by Dr. Bao Dang

CC: “I think I’m having a miscarriage”

HPI: A 23-year-old G3P1011 patient states that she took a home pregnancy test two weeks ago that was positive.  Her last menstrual period was about 8 weeks ago.  She has been having vaginal bleeding for 7 days.  The bleeding started getting heavier over the last 3 days.  Today she noticed that there were large clots.  She’s had a similar presentation with her previous abortion and thinks that she may be having another one.  She complains of abdominal pain that is crampy and located in the suprapubic region without radiation, exacerbating or relieving factors.  Pt has not yet seen a physician for her pregnancy or vaginal bleeding.  No fevers, chills, nausea, vomiting, dysuria, or diarrhea.

PAST MEDICAL HISTORY:  asthma.

PAST SURGICAL HISTORY:  Cesarean section.

MEDICATIONS:  albuterol metered dose inhaler.

ALLERGIES:  NO KNOWN DRUG ALLERGIES.

SOCIAL HISTORY:  Positive for tobacco smoking; denies alcohol or illicit drug use.

SEXUAL HISTORY:  Positive PID years ago, no IUD use, no use of infertility treatment.  LMP 8 weeks ago

PE:

VITAL SIGNS:  BP131/78, HR 117, RR16, T36.2, P Ox 99% RA.

CONSTITUTIONAL:  The patient is awake, alert, and oriented x3, able to talk in full sentences and is comprehensible. Pt appears comfortable on her stretcher.

HEENT:  Conjunctivae are pink.  Mouth/throat – oral mucosa is moist.

PULMONARY:  Lung sounds are clear bilaterally.  No wheezes, rales, or rhonchi.

CARDIOVASCULAR:  S1 and S2 are present.  Tachycardic rate and regular rhythm.  No murmurs, rubs, or gallops.

GASTROINTESTINAL:  Normal bowel sounds. Abdomen is soft.  There is tenderness to palpation at the suprapubic area; however, no guarding or rebound tenderness.

GENITOURINARY:  Blood is seen within vaginal vault without clots.  The cervical os was closed.  No cervical motion tenderness.  Uterus midline, anteverted, normal size and shape, mobile, no masses, and non-tender.  No adnexal mass or tenderness.

Laboratory Studies:

β-HCG quantitative: 150

CBC: WBC 6.9, Hgb 12.5, Hct 37.0, Plts 370

UA: Nitrite & LE negative, Blood 3 +, RBC 10-20/HPF, WBC <5/HPF

Ultrasound:

Questions:

1. Which of the following poses the highest risk for ectopic pregnancy?

A.            history of previous pelvic inflammatory disease

B.            infertility treatment

C.            intrauterine device use

D.            previous ectopic pregnancy

E.            tubal ligation

2.  Which of the following conditions is most likely if the β-HCG level fails to double, or decreases in 48-hours?

A.            ectopic pregnancy

B.            heterotopic pregnancy

C.            non-viable intrauterine pregnancy

D.            viable intrauterine pregnancy

3.  Which of the following is associated with methotrexate use?

A.            an increase in abdominal pain is observed in about 30-60% of patients treated with methotrexate

B.            delayed tubal rupture occur in about 3-20%

C.            nausea, vomiting & diarrhea in about 5-20% of patients

D.            patient should be instructed to avoid vitamins containing folic acid and avoid sexual intercourse until β-HCG level has come back to baseline

E.            all of the above

Answers:
1. E.
Female patients of reproductive age who present with amenorrhea, abdominal pain and/or vaginal bleeding must have ectopic pregnancy in the differential. Ectopic pregnancy is defined as implantation of a fertilized ovum in any location other than the endometrial cavity. Up to 2% of reported pregnancies are ectopic. 95% of ectopic pregnancies implant in a fallopian tube, of which 80% implant in the ampulla, 12% in the isthmus, 5% in the fimbrae, and 2% at the junction of the uterus and fallopian tube. Other rare sites of implantation include abdominal cavity, ovary, and cervix.
All the above question responses increase the risk of ectopic pregnancy. A history of tubal ligation, however, poses the highest risk. About 10% of ectopic pregnancies occur in women with prior tubal ligation. Unless there is a confirmed hysterectomy, pregnancy cannot be excluded in a child-bearing-aged female.
2. A.
There is no combination of historical and physical findings that will definitively diagnose ectopic pregnancy. The diagnosis is made clinically by combining elements of the history and physical exam with ultrasound findings and a quantitative β-HCG. Urine β-HCG will screen for pregnancy. The quantitative β-HCG is used to guide the disposition of the patient. Trans-vaginal ultrasound (TVUS) should reliably detect an intrauterine pregnancy (IUP) if β-HCG level is above 1500-2000 mIU/mL. This level is called the discriminatory zone. Trans-abdominal ultrasound should be able to detect an IUP when β-HCG is above 6,500 mIU/mL. For patients with β-HCG above the discriminatory level and a TVUS demonstrating an IUP, ectopic can be reliably ruled out. Patients with β-HCG below the discriminatory zone should be instructed to follow-up in two days for a repeat quantitative level. The β-HCG should approximately double in 48 hours in a normal gestation. A TVUS should be obtained once the β-HCG level is above discriminatory zone.

3. E.
Ectopic pregnancy can be managed via pharmacological or surgical methods. Methotrexate is the primary agent used in pharmacological management. It is an analog of folic acid that will be taken up by rapidly dividing cells, such as those of the fetus, and disrupts DNA and RNA synthesis within those cells. The use of methotrexate for treatment should only be used in patients who meet the following criteria: patient would like to avoid surgery, have ultrasound findings that show no fluid outside the pelvis and adnexal mass less than 4.0 cm, available for weekly follow-up visits, and is hemodynamically stable. The surgical approach to treatment involves either a complete salpingectomy or tubal preserving strategy. A consultation with OB/Gyn is always needed.

Case Summary:
The patient’s ultrasound showed evidence concerning for a ruptured ectopic pregnancy. However, clinically the patient was stable. She did not show any signs of shock or instability often associated with ruptured ectopic pregnancy. OB/Gyn was consulted, and the patient was taken to the OR for emergent laparoscopic surgery. At surgery, a large amount of adhesions were demonstrated, but no evidence of an ectopic pregnancy. Adhesions were lysed and she was discharged home on post-operative day #1. Biopsy of the right adnexa showed no evidence of ectopic pregnancy. Adhesions were likely from previous PID. The patient likely had a spontaneous abortion of her pregnancy.