3 year old boy presents with left ankle swelling and pain x 2-3 days.
Ankle swelling initially started about 2 months ago while in Ghana visiting family. At the time he developed a small rash on the right leg (which resolved), as well as the left ankle swelling. Ankle was not painful, and swelling had spontaneously resolved about a month ago. He had had a extensive outpatient evaluation (labs and plain Xrays) by ID and Rheum at CNMC prior to symptom resolution. All tests were negative except some nonspecific soft tissue calcification seen on XR.
No fever in the last few weeks. He had a cough that resolved about a month ago. No history of trauma. Normal appetite and activity. No recent weight loss.
On exam:
T 36.7 P 149 R 22 BP 120/74 Pox 99%
Gen- Alert and interactive, no distress.
Normal cardiac and pulmonary exam. No murmur, rub or gallop. Good perfusion.
No hepatosplenomegaly. No significant lymphadenopathy on exam.
Abdomen soft, non-tender, normal bowel sounds.
Musculoskeletal exam: Left Ankle: swollen non-mobile mass. Not fluctuant. No erythema. Very tender to touch. Range of movement of ankle limited by pain. No sensory loss. Good perfusion of distal foot. Unable to walk due to pain.
All other joints without swelling or tenderness and with normal range of motion.
Skin: Right medial thigh with oval hyper-pigmented macular lesion, not erythematous, not painful.
Questions for you (Click on "vote" to record your answers and see what others said)
His new ankle X-ray is here:
The patient has received Toradol and can now walk but with a significant limp. The patient has a readily available PMD and appropriately concerned parents. Orthopedics does not think anything needs to be done tonight. Assume your laboratory evaluation thus far is normal.
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that looks like more than calcinosis....! terrible looking XR. if the patient is focally tender over the bony abnormality and can't walk, and you don't really understand why....i would be concerned. I wouldn't even consult the ortho resident, i would just call the attending and have a discussion while he/she looks at the XR. shared mental model for a patient that clearly nobody knows what's wrong with. need to discuss advantages of a more expedited in-patient workup versus outpatient MRI and surgical consultation, etc. they see stuff like this in ortho clinic all the time, the question now is what is worse and how acute is it. if it's acutely worse and focally tender, then it needs to be addressed now to look for signs of enlarging mass, infection, etc.
ReplyDeleteThis looks like an interesting case. I wonder what happens.
ReplyDeleteHmmmm, I agree that this thing in the ankle looks like more than a little calcification. It looks like he's growing another talus or something! When I googled "hyperpigmented rash and ankle calcification" I got a lot about dermatomyositis. The thing on his thigh doesn't sound typical of a dermatomyositis rash, though. And with his inflammatory markers all being normal (or were they?), that diagnosis is even less likely. The fact that it's so painful is concerning. Bony tumor? Infection? Nothing fits nicely but I feel like he needs more of a definitive plan before sending him home. I might call ID again, or maybe even heme onc? Kind of stumped on this one-good case!
ReplyDeleteI am also concerned about this kid -- there are more than enough reasons why I would like to keep him - persistent pain, he can't really walk for goodness sake, and there is a crazy looking thing-a-ma-jig in his ankle!! What exactly would I expect a PMD to do --motrin and send back to the ED if that did't work ?? Follow up there just seems like passing the buck just b/c I don't know what this is. I would like some better imaging to figure this out. Admit for MRI and pain control. Agree with Dave - would discuss with Ortho attending directly. Since Theresa used google as a medical search, I assume I can admit I used Wikepedia and came up with the following.
ReplyDeleteTumoral calcinosis - rare condition in which there is calcium deposition in the soft tissue in periarticular location (i.e. around joints). The accumulations are outside the joint capsule. The name indicates calcinosis (calcium deposition) which resembles tumor (like a new growth). They are not true neoplasms - they don't have dividing cells. They are just deposition of inorganic calcium with serum extrudate. Children and adolescents (6 to 25 years) are the most commonly affected. The symptom that the accumulations cause is not pain but swelling around joints. They have propensity to enlarge progressively and ulcerete the overlying skin and extrude. They are most common around shoulders, hips and elbows. Laboratory evaluation reveal normal serum calcium levels and hyperphosphatemia. Rarely ALP (alkaline phosphatase - an enzyme active at sites of bone formation) may be elevated. Treatment is normalization of serum phosphate levels and resection of lesion. Surgical removal should be complete and if part of it is left, there is inevitable recurrence." I'd bet on that as my diagnosis. So in that case, would check CMP (maybe with a fractionated Alk Phos as well?) Funky case -- thanks a lot Naomi!
I am sooo hoping I am on the hot seat because this is a tropical disease. Alas, probably not.
ReplyDeleteThe first time I read this through, I was thinking of a differential for sub-acute monoarticular arthritis. But on re-read, the problem is the non-mobile painful mass anterior to the ankle that limits movement, with calcified mass on Xray. The exam helps me quickly dismiss a bacterial abscess because there is no erythema or fluctuance to make me think we should incise and drain. I also see that he is tachycardic and hypertensive for age; so this needs to be repeated before knowing if there is significance to these vitals.
So now the differential I am focused on is the cause of calcinosis with intermittent swelling and pain. What causes calcium to deposit in the skin? Some I knew and some I looked up:
- trauma: blood can calcify in the process of reabsorbing; foreign body such as teeth left behind – these are easy to figure out by history. Not certain why pain/swelling would be intermittent other than reinjury.
- hypercalcemia: finding an abnormally high calcium would only be the beginning of a work-up. The differential would include oncologic causes (NHL, neuroblastoma), endocrinologic causes (hyper PTH), medications (Vit A or D excess, others such as lithium), and chronic renal failure. Clinically, hyperCa presents with fatigue and muscle weakness, bradycardia and sometimes hypertension—so not likely if his exam is otherwise normal.
- as Joelle found, there is tumoral calcinosis: the calcification accumulates near the joint capsule, with normal labs except for sometimes hyperphos. This is a familial disease and usually in more than one place.
- dermatologic disease: scleroderma can cause calcinosis as part of CREST syndrome (calcinosis, Raynaud’s, esophageal dysmotility, sclerodactyly, telangiectasia). Scleroderma is obviously much more common in adulthood, but there are children diagnosed with this autoimmune disease. A careful skin exam and questions about Raynaud’s will help. Not sure if bolded hyperpigmented macule on thigh is significant in pointing to derm diagnosis.
- infections: specific tropical diseases leave calcifications- onchocerciasis (river blindness- not likely with short trip to Ghana); guinea worm (checked Carter Center website and no cases of indigenous guinea worm in Ghana since May 2010- yeah eradication program); cystocercosis (cysts and calcifications found in brain and wouldn’t find their way to anterior ankle). Granulomatous diseases such as TB also need calcifications, but of LNs. Still a possibility given trip to Ghana, brief rash on his right leg (site of infestation), and intermittent nature (cysts dying with skin becoming tender and inflamed).
So, given this differential, I would do the following:
1. check bmp, Ca, phos, PTH—more testing only if these are abnormal
2. discuss with radiologist their differential for this calcified mass. I don’t think other imaging studies are indicated.
3. Agree with previous comments about discussion with Ortho about their thoughts.
4. ask Derm for their input, since a few of these diagnoses fall under their specific expertise. I would not keep him in the ED for this, nor would I admit the child. But I would communicate with the Derm resident to have him scheduled for clinic.