Splenomegaly

What does this picture have to do with splenomegaly? Send me an email - if you're right, I'll buy you a coke.

The spleen is the 3rd most underappreciated internal organ.

If you need a reminder on how to perform any of the maneuvers below, send me an email/page and we can go through it.

Does my patient have splenomegaly? Tough to say... this is not the most reliable exam, however if your pre-test probability is somewhat high (>10%), then the exam can be quite helpful.

Castell's Sign: The patient is supine. Locate the intersection between the left anterior axillary line (*note* not mid axillary line) and the last intercostal space. Perucuss. It should be resonant. When your patient breaths in and holds, this should stay resonant. If you hear dullness then voila - probably splenomegaly(Sensitivity 82%; Specificity 83%).

Palpation: Either use your hand, or the patients hand for counter-pressure in the left flank. Palpate from the RLQ diagonally up, inching incrementally as the patient exhales. Try rolling the patient into the right lateral decubitus if you do not feel anything and repeat your palpation(Sensitivity ~60%; Specificity ~90%). Feel for the splenic notch.

Middleton's Hooking Maneuver: Approach the patient from the left side, with the patient supine and their fist under the left costovertebral angle, hook your fingers under the left costal margin. Ask the patient to breath in, and feel for the spleen tip (sensitivity ~60%; Specificity ~90%).

Others: Nixon's percussion method is helpful, but Traube's space is pretty useless. I can show you these whenever you like.

Here is a link to the sentinel JAMA article on splenomegaly.

Stevens-Johnson Syndrome

Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis are about as bad a drug reaction as you can get. These are basically the same condition, with TEN being on the more serious side of the spectrum compared to SJS

SJS: Less than 10% of body surface area is affected. Mucous membranes virtually always involved. macules which are erythematous or purpuric morph into epidermal necrosis and sloughing.

TEN: See above, except >30% of the body is involved. Diffuse erythema is also seen in this condition.

Both: Associated with systemic symptoms like fever, malaise, anorexia. Bullae may form on the skin prior to sloughing.

What causes it? Mostly in response to medications. Classic drugs include allopurinol, sulfa medications, penicillins, anti-seizure drugs (dilantin, carbamazepine), and some NSAIDS.

How do your manage these cases? 1. early recognition, 2. discontinuation of offending agent, 3. supportive care, 4. wound care management, including eyes and mouth, 5. monitor for fluid/electrolyte disturbances, 6. debridement. The use of IVIG or steroids is controversial. Many patients require specialist care in a burn unit.

How does the picture above relate to this topic? send me an email...if you're right I'll give you a prize.

Terry's Nails and Lindsay's Nails

Today we looked at nails a bit more closely. We examined the white part of the nail, termed the Lunula, which is Latin for 'little moon'. This normally occupies a very small proportion of your nail.






Lindsay's Nails: also termed "half-and-half" nails. As the name implies, the lunula extends to roughly 50% of the nail. It is classically described in chronic kidney disease.

Terry's Nails: The lunula extends to >75% of the nail. This is described in liver diseases, CHF, diabetes, and hyperthyroidism.

• A good review of nail changes in systemic diseases can be found at this link.
• Here is one of the best articles on Terry's Nails, published in The Lancet in 1984.

Interstitial Lung Disease

Today we saw some really interesting cases including:

*Ascites: check out a previous post with evidence-based physical exam findings here.

*Clubbing: also discussed, with evidence-based exam findings at this link.

One patient we saw had terrible interstitial lung disease. These are confusing conditions. Here's an approach as to the underlying etiology:

Systemic Diseases: ILD is found commonly in Sarcoidosis and Collegen Vascular Diseases - the prototype being Scleroderma. Remember, ILD is not so common in SLE or Rheumatoid arthritis.

Exposures: these are classically divided into organic and inorganic exposures:

1. Organic: aka "Hypersensitiviy Pneumonitis". Think about bird fanciers lung, cheese washers lung, farmers lung, and many many more.
2. Inorganic: aka "Pneumoconiosis". This includes dust from asbestos, silica, and coal.

Drugs: Exposure to alkylating chemotherapeutic agents (eg. bleomycin), but also commonly used drugs like amiodarone, methotrexate, nitrofurantoin, and sulfa medications are also implicated in ILD.

Idiopathic: This is a strange category with bizarre acronyms. It doesn't help that there are old and new acronyms, and that both are used frequently. The diagnosis is usually based on histology.

1. Usual Interstitial Pneumonia (UIP), also known as Interstitial Pulmonary Fibrosis (IPF) - this is perhaps the most common idiopathic ILD.
2. Acute Interstitial Pneumonia (AIP), also known as the 'Hamman-Rich Syndrome'. Just like IPF above, but more rapid.
3. Cryptogenic Organising Pneumonia (COP), but if the underlying etiology is known, you will hear it called Bronchiolitis Obliterans and Organising Pneumonia (BOOP). There is lots of granulation tissue in the small airways here.
4. Others: Desquamative Interstitial Pneumonia (DIP), Lymphocytic Interstitial Pneumonia (LIP), Non-specific Interstitial Pneumonia (NSIP)....oy vey...

Other Rare Causes of ILD: Alveolar hemorrhage, Pulmonary Alveolar Proteinosis, and my personal favorite, Pulmonary Infiltrates with Eosinophilia (PIE). mmmmmh.....pie. And Lymphangioleiomyomatosis (LAM).

Mimickers of ILD: Pneumocystis Carini Pneumonia, Lymphangitic carcinomatosis, and once in a while, good ol' congestive heart failure.

Links: A good review of Idiopathic Pulmonary Fibrosis can be found at this link.

Clubbing

Common causes of Clubbing:

• Gastrointestinal: liver disease, inflammatory bowel diseases, celiac disease, malignancy
• Pulmonary: malignancy, suppurative (empyema, abscess), Cystic Fibrosis, TB, bronchiectasis, many fibrosing conditions (like IPF), AVM's
• Cardiac: endocarditis, cyanotic heart diseases
• Other: hyperthyroidism, alpha 1 antitrypsin, sarcoid, lymphoma

Physical Examination for clubbing:

• Look for changes in the nail shape, like increased PA and lateral diameter, and a "bird-beaking" or "drumstick" appearance to the nail.
• Palpate a spongy texture to the nailbed.
• "Phalangeal Depth Ratio": See the picture below in Frame C. Normally the finger height at the DIP should be greater than at the distal phalanx. In clubbing, the height at the distal phalanx is greater than the at the DIP.
• Angles: See the picture below in Frame B: the Profile angle (also known as Lovibonds angle) is increased greater than 180 degrees in clubbed individuals - this is angle ABC below; and the Hyponychial angle is greater than 190 degrees in clubbing as well - this is angle ABD below.
• Shamroth Sign: See the picture below. This is an indirect measurement of the Profile angle (aka Lovibonds angle). When you put two fingers together at the terminal phalanx, you should see a diamond shape. This is lost in clubbed individuals.

What is the evidence for all this?

• JAMA has a good article in their Rational Clinical Exam Series at this link.
• There is a recent paper specific to the utility of Shamroth's sign in JAMA here as well.
• Bottom line: the Phalangeal Depth Ratio and Shamroth Sign are the more useful findings.

(Image is from the JAMA Rational Clinical Exam series; 2001, 286, 341-47 at this link)

Edema

Today we saw of case of severe non-pitting edema, and a case of what appeared to be cellulitis, but in fact was radiation-induced skin changes.

An approach to Edema is below.

Here is a link to diseases which may masquerade as cellulitis from the Annals of Internal Medicine.


Edema is a common finding in clinics and on the wards. It is usually found in dependent areas like the ankles and legs, but may be seen in the sacrum of supine individuals.

Pitting vs Non-Pitting: to have 'pitting' edema, a dent is left in the skin after pressure is applied over a bony prominence. How long do you have to press? Some say 5 seconds, however many experienced clinicians agree that 15-20 seconds is more appropriate.

• Non-Pitting: Suggests lymphedema, or rarely hypothyroidism (pretebial myxedema).
  

An Approach to Bilateral Leg Edema:

• Cardiogenic: think about right-sided heart failure or constrictive pericarditis
• Venous Obstruction: should be above the level of iliac veins, or bilateral distal venous thrombus
• Hepatic: when the synthetic function declines, decreased protein production like in late cirrhosis
• Other GI: protein losing enteropathies, malnutrition
• Kidney: nephrotic syndrome
• Endocrine: Cushing's syndrome - high cortisol levels increase renal sodium absorption. Also remember hypothyroidism (pretibial myxedema)
• Drugs: calcium channel blockers, OCP, corticosteroids, minoxidil
• Pregnancy: an planned (or unplanned?) mass pressing on IVC

Unilateral Edema: this may be caused by

• Venous disease: such as DVT, thrombophlebitis, damaged venous valves after infection or clot.
• Lymphatic disease or disruption: often lymph nodes are removed during surgery (eg for breast cancer). Infection is a common cause of LN damage, such as in recurrent cellulitis or lymphatic filariasis. You may see unilateral edema from LN disease with certain malignancies or radiation-induced damage as well.

Aortic Stenosis Redux

(rainbow trout pictured left)

Today we saw a case of severe aortic stenosis (valve area 0.6 cm).


We have discussed AS in detail before, and you can check out this link for more information on the physical exam.

Can you detect moderate to severe AS at the bedside? Absolutely. Some smart people from Toronto came up with a very helpful clinical prediction rule. The published paper from JGIM is at this link.

This is how it works:

• If there is no murmur over the right clavicle, moderate to severe AS is essentially ruled out.
• If there is a murmur radiating over the right clavicle with 0-2 associated findings, then the Likelihood Ratio for moderate to severe AS is 1.8, and in this study reflected about a 20% chance.
• If there is a murmur radiating to the right carotid with 3-4 associated findings, then moderate to severe AS is essentially ruled in (Likelihood ratio of 40)

So what are the "Associated Findings"?

• Reduced S2
• Reduced carotid volume
• Slow carotid upstroke
• Murmur loudest in the 2nd right intercostal space

Guillain-Barré syndrome

Guillain-Barre Syndrome (GBS) is an umbrella term to describe a few syndromes of immune-mediated demyelinating polyneuropathy.





This is commonly manifested by:

• Motor findings: typically weakness starts in the legs. It may ascend rather quickly and affect respiratory muscles. Facial muscles are also commonly involved. Reflexes are severely diminished to absent.


• Sensory changes: found usually in extremities. Typically mild decreases to light touch, pain, temperature. Pain in the lower extremities and back are also a common feature.


• Autonomic findings: patients may present with tachycardia, bradycardia, hypotension, urinary retention.

These symptoms may start abruptly and develop over a period of hours, days, to a couple of weeks.

A related variant: the Miller-Fischer Syndrome: This is under the same umbrella as GBS and is manifested by 1. Ophthalmoplegia, 2. Ataxia, 3. Areflexia. Patients will also usually have peripheral weakness.

Diagnosis: You will hear the term "Albuminocytologic Dissociation". This means that in the CSF there are elevated protein levels with normal WBC counts. This is a classic feature of GBS. Nerve conduction studies will also help clinch the diagnosis. MRI will often show enhancement of nerve roots.

Treatment: with plasma exchange or IVIG therapy. This is to eliminate or incapacitate auto-antibodies to schwann cells. Patients must be watched closely for possible respiratory muscle compromise. Roughly 80% will make a full recovery.

Risk Factors: Autoantibodies may form after exposure/infection:

• Campylobacter
• EBV/CMV
• HIV
• Hodgkin's Disease
• Influenza vaccine? If real, likely a tiny risk.

A few good links:

• When to suspect impending respiratory compromise in GBS.
• Evidence for treatment of GBS.

Mitral Regurgitation

Mitral Regurgitation is pretty common. when you hear this murmur and are trying to determine the underlying cause, think about the individual components of the mitral valve and particular disease states which might affect them. Let's start at the annulus and work our way down....

1. Annulus: This may be dilated from cardiomyopathies, or calcified in diseases like rheumatic fever or chronic renal insufficiency.
2. Leaflets: The mitral leaflets can fail in a number of disease states including infectious endocarditis (acute or chronic), rheumatic fever, autoimmune diseases (SLE, scleroderma), myxomatous degeneration (MVP), Connective Tissue Diseases like Marfan's syndrome, or with congenital anomalies.
3. Chordae: These can be damaged or rupture under ischemic, infected, or traumatic conditions, and in rheumatic heart disease.
4. Papillary Muscles: These muscles can rupture after trauma or infarct. They become 'dysfunctional' in ischemic conditions or when the LV becomes dilated (myopathy or aneurysm). Papillary muscles can also become infected, and rarely can be infiltrated with amyloid deposits or granuloma (eg. sarcoid).

On Exam:

• Inspection/Palpation: Apical impulse may be displaced to the left and is brisk and hyperdynamic.


• Heart Sounds: S1 may be soft, S2 may be widely split from early A2 closure. You may hear a loud P2 if pulmonary hypertension is present. S3 can be heard in volume overloaded states.


• Murmur: This is a holosystolic murmur that starts right after S2 (and may even obscure it). It is high-pitched, loudest at the apex, and radiates to the axilla. There is minimal respiratory variation - this may help you distinguish it from Tricuspid Regurgitation.


• Special Tests: The murmur is accentuated with maneuvers that increase afterload (eg. bilateral isometric hand grip or by transient arterial occlusion with a blood pressure cuff). The murmur will be diminished by decreasing preload - like going from a crouching position to standing, or with valsalva.
  

Good Links:

• Here is a good review on the Evaluation and Management of Mitral Regurgitation.
• This is the JAMA article for systolic murmurs.

Aortic Insufficiency

(a leaky valve, pictured left)

Aortic Insufficiency: probably the most eponymous physical exam scenario....

• Pathophisiology: increased stroke volume results in an abrupt distention of peripheral arteries and elevation in blood pressure. This is followed by regurgitation through the valve back into the LV, causing a rapid fall in pressure with quick collapse for the arteries and a low diastolic pressure. This accounts for the majority of physical exam findings.


• Inspection of the Precordium: the apex may be displaced laterally or inferiorly.


• Palpation: displaced cardiac apex, with a hyperdynamic pulse. You may feel a thrill near the sternal notch.


• Heart Sounds: S1 may be soft secondary to a longer PR interval. An S3 will be heard in volume overloaded states.


• Murmur: typically a high-pitched early diastolic murmur, decrescendo in nature. It is best heard with a patient sitting up and leaning forward at end exhalation. Classic teaching suggests that if the murmur is loudest at the Right Sternal Boarder it represents aortic root disease, and if the murmur is loudest at the Left Sternal Border it represents aortic valve disease.


• Other Murmurs you may hear: A systolic murmur at the base that may mimic aortic stenosis - this is usually just a murmur from the high volume and flow of blood through the aortic valve during systole. Why so much during systole? Because a large fraction is regurgitationg back into the LV during during diastole. Also, an Austin-Flint Murmur may be heard. As Dr. Davis pointed out, this is a diastolic murmur heard at the apex of the heart that may mimic mitral stenosis.
  

Etiology of Aortic Insufficiency:

• Aortic Valvular Disease: Rheumatic heart disease, endocarditis, bicuspid aortic valve, myxomatous degeneration, rheumatoid arthritis, Marfan's, ankylosing spondylitis.
• Aortic Root Disease: HTN, dissecting aortic aneurysm, aortitis (from syphilis, ankylosing spondylitis, IBD, reactive arthritis), rare connective tissue diseases like Ehlers Danlose syndrome.
  

What's with all the eponyms? These are cool to see, but largely useless as they are not sensitive or specific, and are mostly related to the wide pulse pressure.

• DeMusset's sign: head bobbing with each heart beat.
• Becker's sign: visible pulsations in the retinal arterioles.
• Mueller's sign: systolic pulsations of the uvula.
• Quincke's sign: capillary pulsations in the finger tips.
• Rosenbach's sign: systolic pulsations in the liver.
• Gerhard's sign: systolic pulsations in the spleen. Usually you need an enlarged spleen to feel.
• Duroziez's sign: a systolic and diastolic bruit heard over the femoral artery when it is partially compressed. Note that when the distal part of your stethoscope is pressed down, the diastolic component will be accentuated.
• Pistol Shot sound: heard over the femoral artery.
• Hill's sign: when BP at the popliteal region is greater than the brachial BP by more than 20 mmHg.
• Water-hammer pulse aka Corrigan's sign: with a patient lying down, press down to obliterate the radial pulse. Then slowly raise their arm up and voila! The pulse becomes palpable again with the same amount of applied pressure.

So, is there any evidence for all this?

A little bit. Some smart folks in Toronto wrote a good JAMA article that you can read at this link. AI can be ruled out with the absence of an early diastolic murmur, and ruled in with the presence of an early diastolic murmur - the caveat? These murmurs were heard by cardiologists. Perhaps mere mortals like ourselves will hear it too.