Hot Seat #45: 4yo F with edema
Posted on: October 20, 2014, by : Jason Woods MD
by Jason Woods, Children’s National
with Pavan Zaveri, Children’s National
The Case
4 year-old no PMH p/w with concern for “allergic reaction”. She awoke this morning with swelling and a red rash involving bilateral eyelids. +pruritis. Denies stridor, wheezing, emesis. Has no know allergic history and no new exposures. Her mother called her PCP this morning who recommended PO diphenhydramine, but after no improvement with 2 doses, she was directed to the ED.
Mother then mentions multiple other sx: For the last two weeks she has been complaining of abdominal pain and fatigue. This has progressed so that she is requesting not to be sent to school, and while she attends soccer practice, she is now sitting on the sideline during practice because “I’m too tired”. Mother feels that she is now short of breath anytime she climbs the stairs. Mother also notes that the patient’s abdomen appears distended, and beginning 3-4 days ago she began refusing to bend at the waist to put on her shoes because “it hurts my belly”.
ROS: Denies fevers, recent illnesses, sick contacts, travel, dietary changes, new animal exposures, or known weight changes. Denies nausea, vomiting, diarrhea. Denies muscle weakness, difficulty with gait, changes in balance or vision. Denies palpitations or chest pain.
FHx: Negative
Physical exam: VS T 37.2, HR 132, RR 28, Sats 100%, BP 122/68
Gen: well appearing, happy and conversant
HEENT: eyelids bilaterally swollen, erythematous, blanching rash across bilateral eyelids which extends onto nasal bridge and cheeks. OP clear, MMM
Resp: Lungs CTAB, no respiratory distress, no stridor
CV: mild tachycardia, regular rate, no m/r/g. Pulses 2+ x 4 extremities
Abd: notable abdominal distension, mild TTP diffuse. Spleen and liver edges not palpable, liver span percusses at 12 cm
MSK: 1+ non-pitting edema of bilateral lower legs to the mid shin. Refused to bend and touch toes due to worsening of her abdominal pain
Back: non-tender
Neuro: CNII-XII intact. Able to get out of bed and ambulate normally. Romberg negative. Tandem gait intact. Strength 5/5 BUE, and BLE with the exception of 4/5 hip flexion bilaterally. Sensation grossly intact.
Initial chest X-ray shows a normal cardiac silhouette and no pulmonary edema or pleural effusions. EKG is normal for age. Abdominal X-ray shows non-obstructive bowel gas pattern and no other abnormalities.
Labs were drawn and are pending.
Questions for you:
Interim Update:
Initial screening labs return as follows
CBC: WBC 7.17, H/H 11/33, Platelets 323
Chemistry: Electrolytes including Ca, Mg, Phos all normal.
LFT: Significant for AST 331, ALT 139, Bilirubin 0.1, Albumin 2.9, total protein 6.5
TSH and T4 normal
Coags normal
ESR 24, CRP <0.1
U/A negative including no protein
How would you approach this case? Please share your opinions by clicking on “What do you think?” below.
Rash on face and hip flexion weakness (difficulty climbing stairs) are very characteristic of dermatomyositis. Were there any periungual telangiectasias or scaly erythematous eruptions overlying the knuckles, elbows, and knees? If I could order onlhy one test, I’d get a CPK (not CPK isoenzymes!).
Dewesh is way smarter than I am. I’d still be working through a differential for anasarca without proteinuria. Obviously, a good dietary history, growth and development would be helpful, kwashiorkor can occur in the first world after all, especially with fad diets imposed on picky four year olds. This sounds neither allergic, nor of renal origin unless the initial urinalysis is spurious.
I remember a patient I had during internship with intestinal lymphangiectasia presenting in a similar fashion ( sorry for the anecdotal reference). In any case, admission would depend on the patient’s fluid status, pain scale, and a probable ultrasound to rule out tumors or portal venous issues.
If she looks well, no reason necessarily to keep her in house amidst ED68 and Ebolahysteria…
I’m going to put a bottle of scotch on the fact this is not dermatomyositis. Seems too young (i feel like they’re usually in the 8-12yo ballpark) and i’ve never seen one with anarsarca or tachycardia. And they’re not usually tender as is described, they just can’t put on shirt or brush teeth or other signs of proximal muscle weakness….not pain. And AST>ALT makes me think of hepatocellular injury rather than muscular injury. heliotrope rash isn’t itchy. neither are Gottron’s knuckle papules (i may have butchered the name of this).
The case sounds like CHF with liver congestion, or hepato/portal obstruction. or something in liver parenchyma causing congestion but not dysfunction.
If the kid is edematous and has hypoalbuminemia, he’s either losing protein or not making it. Sending a stool alpha antitrypsin can look for GI losses but i would be more concerned about him making albumin in the setting of his elevated transaminases. Helps to get a prealbumin to look at nutritional stores. And yes to the CK only because my bottle of scotch depends on it. but hard to have synthetic dysfunction with normal coags…hmmm. i guess he could just have low albumin from chronic disease but that doesn’t help us much.
In the ED, I’m all for the abodminal US with doppler to look at portal vein system and for hepatocellular mass/injury and to quantify ascites. And I’d do a bedside US of heart to look for dilated cardiomyopathy which would fit everything except the normal cardiac silhouette. it would be weird for liver dysfunction and ascites to cause orthopnea in the absence of pleural effusions or cardiac dysfunction. Sending a BNP comes back quickly and can be helpful or at least a nice reason to convince your cardiologists to do a formal echo despite the normal EKG and CXR.
The other classic finding that mimics “facial swelling” is SVC syndrome from mediastinal mass, although this could also occur from something affecting preload which is more the reason to get an echo if everything else fails. and pruritis can occur from cholangitis but unlikely with bili 0.1.
i think our job in the ED is often to r/o the worst, so i would r/o intra-abdominal mass, hepato-portal problem, and cardiomyopathy……..
Hmmm, now how do I say anything more intelligent than Dave. He nailed every point that came across my mind with to me all concern emanating from an intra-abdominal mass like Wilm’s or the such. Ruling that out will be key….then depending on wellness can affect admission and further workup.
However, looking back at the case, I wouldn’t rule out dermatomyositis so easily, given that abdominal pain can definitely be upper thigh/hip discomfort from the weakness and the rash screams it out. Let’s get that CK.
Now, this is a hot seat, so we ought to think of more common things rather than the serious badness, when these tests come back normal.
Going through all systems/processes like a med student (But when so many systems are involved, breaking it down may help find the unifying diagnosis)
Allergic – unlikely given timeframe
Cardiac – possible still despite normal ECG/CXR with unusual causes of heart failure (cardiac non-compaction, etc.)
GI: Hepatic disease (acquired or congenital – possible with complications leading to portal hypertension, swelling, abd pain)
Hem: slightly low H/H, unable to find a diagnosis under this category to explain it all….maybe some unusual thrombotic disease….
Infectious: can explain some things like a hepatitis, less likely the lower leg swelling, maybe the rash.
Neuro: unlikely
Onc: Abdominal mass: Wilm’s, neuroblastoma, other abd mass with related paraneoplastic processes
Pulm: clear lungs, normal CXR makes it unlikely, but cannot exclude something pulm causing the orthopnea/dyspnea, possibly from a paraneoplastic process
Renal: Unlikely with normal UA, but check out kidneys on US given LE swelling.
Systemic diseases: Always can throw in Rheum diseases here with lupus, systemic-onset JIA (less likely without fever), though normal CRP and minimally elevated ESR make less likely.
Now to move the case in a different direction….all pediatricians are not equal. If everything returns normal, consider discussing with pediatrician the possibility to continue the evaluation outpatient as mentioned above, though also keeping in mind the family’s willingness to follow-up….Sometimes we get a hold of the PMD, often we do not, each case is a judgement call as to how definitive the follow-up seems. Further multiple outpatient diagnostics/therapeutics can be quite a challenge to coordinate and a nightmare of running around for the family missing school/work and torn between with whom to engage to understand the why and how for each test ordered….Admission can easily absolve the parent of the inordinate effort to coordinate often their own work with the scheduled tests and schooling where an admission brings it all in a short time and is a clear excuse from work 🙂
I will stop my overnight rambling there and look forward to the discussion on Thursday and the denouement later.
Sounds like you owe me a bottle of scotch!