Update: Jennifer Chapman's Hot Seat response has been posted (under "Comments," after the question section)
by Rosemary Thomas-Mohtat
At UMC, you see a 2.5 yr old female with history of neurofibromatosis type 1, mild developmental delay, mild intermittent asthma, GERD (resolved), and milk allergy, coming in with 3 days of NBNB emesis. She had 3 episodes of emesis on the first day, none yesterday, and once today. She has had a low grade temp of 100 (3 days ago), decreased PO intake x 24 hrs, and decreased activity. ROS is negative. Specifically, there is no diarrhea, constipation, URI, SOB, dysuria, trauma.
Her NF1 is followed with MRIs. 6 months ago there was thickening of prechiasmatic optic nerves and increased signal in the cerebellum. Consistent with NF.
Exam:
T: 37.4, P: 119, R: 20, BP: 98/66 wt: 13kg
She is a tired-appearing toddler who doesn’t protest much with exam. Not smiling.
Exam is otherwise notable for baseline frontal bossing. Equal pupils briskly reactive, though she will not cooperate for fundoscopic exam.
There is axillary freckling and café au lait spots.
Abdomen is slightly distended without HSM, and nontender.
Neuro exam: Though she appears weak and tired, she can stand and has normal gait, with baseline toewalking. Moves all extremities. DTRs 2+ bilateral patella.
Cap refill <2 sec, distal pulses 2+
Questions for you (click on "vote" to submit your answers and see what others said):
What do you think? Is there something else you'd look for? How would you approach the management. Click on the Comments link below to add your thoughts. It is easiest to comment if you are logged into your gmail account.
See Jennifer Chapman's Hot Seat response below and come back later for the denouement.
NF1 is a slowly progressive disease. Most NF experts don't even recommend routine outpatient neuroimaging in these kids unless they have symptoms or complicating factors. Sometimes as ER docs, i think we jump on certain buzz words....but in this case i think that's an unnecessary bias. 4 episodes of vomiting over 3 days (with 1 in past 2 days) without new neuro findings indicates that the vomiting is likely from something else. and pretty underwhelming. sounds like she needs some oral rehydration and reassessment. fast track? :)
ReplyDeleteEh. I agree with Dave. NF-1 is not a rapidly progressive disease and this kid seems pretty ok - and very UMC-ish. If she tolerates PO, I would send her out with close f/u.
ReplyDeleteAlthough, in the back of my sleep-deprived brain, I do wonder if she can get abdominal tumors, too... could they be causing all this?
Too tired to think. And its 6:50am of an overnight shift (for real), so if this were my patient at this time, I would po trial her and then wait til the next doc came in to discuss with a fresher and clearer head. I have found this (stalling) to be a useful technique with things that make me uncomfortable in the middle of the night. So, trust your gut and its never wrong to observe a kid.
I agree with Dave and Sabah. Her head isn't on the top of my list assuming she improves. If I were feeling uber cautious (which at this moment I'm not feeling based on what's written), I MIGHT, obs her for a bit.
ReplyDeleteAsha
It is my turn to be on the hot seat, so I will offer my response, unclouded by the previous answers, though very similar…
ReplyDeleteTo remind myself, NF-1 is diagnosed with 2/7 criteria: 6+ hyperpigmented macules, neurofibromas, ax/inguinal freckling, optic glioma, 2+ Lisch nodules, skeletal abnormalities, 1st-degree relative with NF.
Now, looking at this child with vomiting, my questions are what are disease-specific concerns that cause vomiting and what are issues in otherwise healthy children that cause vomiting?
Disease-specific:
- vomiting from increased pressure from a brain tumor: while children with NF-1 do have an increased risk of brain tumors, these are most often optic nerve gliomas, which cause subtle visual field defects to complete vision loss. There is no evidence of a brain tumor by hx/exam.
- Vomiting from a hypertensive emergency: children with NF-1 do have an increased risk of pheochromocytomas. I checked her BP on the NHLBI table: she is at the 90th/95th %ile if small for age. I might recommend rechecking her BP in follow-up, but no more needs to be addressed now.
- Vomiting from abd obstruction: children with NF-1 can get sm bowel stromal tumors and neurofibromas. The description is not one of a child with obstruction: the frequency of emesis is decreasing; the abdomen is only mildly distended but otherwise normal; no abd pain described.
Causes of vomiting in an otherwise healthy child:
- UTI: maybe
- Viral illness
- Intussusception: she is on the older side unless she has a lead point. Not a hx that points to this: 3 days of symptoms that are improving, no mass/fullness on exam, no bloody stool (admittedly a late sign)
In ED, my thought is to offer child po trial. If she fails, consider a flat plate/upright or flat plate/left lateral decub to look for signs of obstruction.
As always, remind family what to return for: abdominal distension/significant pain, repeated vomiting/bilious vomiting, lethargy, signs of dehydration.
I missed the opportunity to comment before our teaching attending this week -- but if I may, I wanted to comment that I am particularly appreciative of the thought process(es) discussed by the attendings above. Wonderful trains(s) of thought. Thanks from Fellow J!! :-)
ReplyDelete