with Jamil Madati, Children's National, on the Hot Seat
The Case
He was seen at OSH hospital ED #1 that night where he had normal radiographs of his right wrist and forearm, a negative duplex ultrasound of the right upper extremity and a normal CBC, ESR and CRP. He was discharged with a dx of arm sprain.
He was seen at OSH ED # 2 the next day after pain and swelling spread to his forearm. WBC, ESR and CRP were again normal. He followed up at an orthopedic clinic the next day and a bone scan was performed which also was negative. Lyme titers pending. He presents today due to worsening pain from the right wrist to shoulder in a band like pattern, unimproved on ibuprofen. He brings CDs containing the reports of the duplex and bone scan as well as previous lab results.
ROS: No fevers. No tick bites, He reports seeing a circular rash over his lateral R arm 3 days ago that is no longer present. No vision changes, muscle weakness, neurological deficits, shortness of breath or abdominal pain. He reports a headache since yesterday. PMHx remarkable only for L tibial osteomyelitis in 2007, treated at CNMC and as an outpatient.
Vitals: Temp: 37.2, Pulse 92, RR 20, BP 125/79 SpO2 99 %RA
General: Alert, cooperative. Well-nourished and well perfused, drinking apple juice.
Skin: Warm. Dry. No rash
Cardiovascular: Normal cardiovascular exam and perfusion. Extremity pulses equal.
Respiratory: Lungs are clear, normal effort
Gastrointestinal: Soft. Nontender. Non distended. No organomegaly.
Musculoskeletal: Patient is holding R UE in the most comfortable position; internally rotated and adducted. Reports acute generalized pain (of equal intensity) to light touch from wrist to shoulder. Sensation is intact. Strength is intact, though decreased due to pain. Normal flexion at the elbow and normal external rotation and abduction of the shoulder. The RUE (including wrist) is not visibly swollen, no pitting edema, no erythema.
Neurological: CN II-XII intact, discs on fundoscopy are sharp. No focal neurological deficit.
Lymphatics: No lymphadenopathy
Questions for you (click on "vote" to see what others said)
Some lab tests come back: WBC 6.1, Hb 13.9, Hct 38.5 Plt 203, ESR 8, CRP 0.07, CMP normal. If you sent other labs, they are pending. X-rays of the elbow, forearm and humerus show no abnormality. The patient is now asleep and appears comfortable but still complains of pain when you touch his arm. His mother is getting anxious that they have been waiting too long in the ED and needs to get to work tomorrow.
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My first decision tree is if it's swollen. Because localized swelling in this distribution really makes me think about either venous obstruction or lymphedema. The differential for lymphedema is large (parasite, auto-immune, neoplastic, etc) but has a characteristic appearance that's not described here. if it's painful and swollen, add in a soft tissue infection (cellulitis, pyomyositis, nec fasc, etc). And an ischemic proces like compartment syndrome. But it sounds like it's NOT swollen, so that takes me down a different path.
ReplyDeleteSo now we're talking about diffuse pain without swelling. I think our job as ED docs is to rule out arterial obstruction/claudication, neoplasms, and localized infections. But these things are usually obvious on exam. Add in a arterial US here but everything else is negative.
So then my next decision is deciding what specialist should follow this up? So it helps to look for any signs of systemic disease such as rash, joint pain, etc to point towards something auto-immune or rheumatologic. Localized Scleroderma?
If the rest of the ROS is negative, then it's not rheum. So i move on to ortho, neuro, or psych. We're basically talking about either pain out-of-proportion to exam findings or allodynia. Allodynia is pain from a light touch, which makes me think a patient is somatisizing (e.g., full of shit). If the pain is out-of-proportion and constant, then i think of either a nerve impingement or complex regional pain syndrome. With CRPS, there is usually some autonomic dysfunction with it such as temperature instability or decreased pulses. And i think you can see signs of bone uptake irregularity on bone scan (not sure here). But with nerve impingement, patients can have pain and hold the arm in an unusual position like in this case. So my money is on some form of spinal root impingement or thoracic outlet syndrome. The history of bike riding to me points to thoracic outlet syndrome.
Doctor Doctor, give me the news ?
Can't put it more eloquently. Doubt infectious etiology if all inflammatory markers are negative although Lyme is consistently inconsistent. Rash could be a red herring or Lyme, VERY EARLY so labs have not had a chance to bump.
ReplyDeleteLocalised pain in the wrist, without significant swelling, could also point to tendinous injury and tunnel syndromes, cubital for example. Might necessitate an ultrasound to evaluate "safely", or an EMG if there is impingement. Would be odd to have significant cervical impingement minus a motor deficit.
Thanks Kaynan for the interesting case. This patient presents with persistent symptoms that point towards both a neurologic (arm pain, numbness and tingling) and possible vascular cause (arm/wrist swelling). He has had a pretty extensive (xrays, labs x2, bone scans, dopplers), and thus far, a negative work up at the previous EDs and now presents to our institution with the same symptoms. If a patient keeps returning to an ED with the same complaints, and I’m the 3rd guy seeing him, I generally take a step back and look through everything in the H&P with a fine toothed comb. Symptoms started after a bike ride (which from personal experience involves all kinds of aches and pains from being in a specific position for prolonged periods of times) implying that symptoms might be positional in nature. Pain seems to be his most persistent symptom and from the PE appears to extend from his wrist to his shoulder and is exacerbated by light touch (hyperesthesia). If the pain involves the entire arm, one would think that the nerves involved would be at/or proximal from the brachial plexus, however there does not seem to be any specific dermatomal distribution pattern to his pain (sparing the hand, stopping at the shoulder). He has both sensation and strength “intact” which is also reassuring for any focal spinal cord or nerve root lesion. The swelling (which appears intermittent in nature, since none noted on exam) is also worrisome. On initial thinking, unilateral arm swelling is concerning for DVT or clot somewhere in the upper extremity resulting in venous congestion leading to arm swelling. But patient had dopplers of the RUE that were negative. I would have liked to know if the patient had noted any color or temp changes in his upper extremity in relation to his symptoms (pallor or coolness associated with the swelling, pain, numbness/tingling). If it’s not a clot then I wonder if there is some intermittent claudication of the subclavian artery leading to the patient’s symptoms. Thoracic Outlet Syndrome (TOS) is the diagnosis that comes to mind resulting from changes in neck/arm positions that cause compression of nerves and/or blood vessels leading to the upper extremity symptoms much like the ones our patient exhibits. Common causes in pediatrics are congenital upper rib abnormalities, trauma and repetitive/overuse injuries. The work up for TOS would include EMG studies, musculoskeletal US, MRI/MRA of upper extremity. The other diagnosis that can present similarly (hyperesthesia, pain, swelling, numbness/tingling) is Reflex Sympathetic Dystropy (complex regional pain syndrome). Not enough space to discuss this in detail, but suffice to say that this is a diagnosis that requires many specialists (Rheumatology, Neurology, Psychiatry, Pain specialists) to assist in making the diagnosis and initiating treatment. Not sure what to make of rash on arm 3 days ago??? Lyme disease shouldn’t cause arm swelling (unless arthritis) or arm numbness. With that said, I suspect that repeating some of the same labs from previous ED visits would be of low utility. The patient’s symptoms would urge me to obtain further imaging of the UE along the lines of MRI/MRA looking for vascular cause of symptoms and/or anatomic compression of nerves and vessels of the brachial plexus. Given that this is his 3rd visit for the same, I would probably admit for further work up and pain management but he is stable, could also work up as an outpatient if PCP is willing to facilitate this.
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