by Elizabeth Quaal Hines, Johns Hopkins
with Rasha Sawaya, Children's National, on the Hot Seat
Case edited by Emily Willner, Children's National
The Case
4 month-old female infant is brought in by mom for vomiting. Patient is full term with history of transposition of the great arteries with intact ventricular septum s/p repair on DOL 10 with complicated post-op course including ECMO. She had been in a long-term care facility until 11 days ago for “feeding and growing”, and transition to PO feeds with 24 kcal/oz formula. Per mom, she has had two days of non-bloody, non-bilious emesis that occurs after feeds. No diarrhea. Mom reports that while the baby seems hungry, she does not finish her bottles. Per mother, patient is sleepier than usual though does wake and interact. Only 3 wet diapers yesterday (baseline is 6 per day). The visiting nurse referred the baby in for evaluation today.
ROS: No fevers. No rashes. No cough or congestion. No heavy breathing. No sweating. No seizure like activity. No color change or cyanosis.
Medications: Aspirin, Propranolol, Keppra, Prevacid, Erythromycin, Albuterol and Flovent.
Exam: T 37.0 | HR 130 | RR 28 | BP 87/42 | POx 100% RA | 4.7kg (5.14kg at discharge from facility)
General. Quiet infant lying in mother’s arms. Cries with exam, but consolable. Decreased activity.
HEENT. NCAT. Flat fontanel. PERRL, clear sclera. TMs appear normal. Dry lips and sticky mucous membranes, clear oropharynx. Neck supple.
CV. Sternal scar, well healed. Regular rhythm. Normal S1S2, soft systolic ejection murmur. No gallop. Peripheral pulses 2+
PULM. Easy WOB and CTAB.
ABD. S/NT/ND with NABS. No palpable hepatomegaly.
GU. Normal female genitalia.
SKIN. Thickened doughy skin over abdomen. No rashes or lesions.
You order labs and consider your next management steps.
Questions for you (please answer the questions associated with your PEM experience):
You have ordered the above fluids. While waiting for the remainder of yoru studies, you receive a call about a critical lab. The BMP returns as follows:
Na 176 K 6.1 Cl 132 HCO3 24 BUN 62 Cr 0.7 Glu 89
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About the Hot Seat. Hot Seat cases are written by PEM fellows at Children's National, Inova Fairfax, and Johns Hopkins. The Hot Seat Attending is a selected faculty member who comments on the case without knowing the outcome. Emily Willner is the faculty mentor for the series.
I hope everyone made the diagnosis of hypernatremia before the critical lab results were revealed. Decreased activity and "doughy" skin together in an infant is pathognomonic for hypernatremia until proven otherwise. This is a classed pediatrics board-type question.
ReplyDeleteAs far as approach, the money is in asking the caregiver/s to demonstrate how they actually mix the formula -- not just asking how they do it, but to demonstrate it. Cases of extreme hypernatremia in infants on concentrated formulas are often due to improper mixing and excess solute load, or they are a form of Munchausen by proxy in which salt is deliberately added to formula. I'd also want to watch the infant feed.
Urine electrolytes (sodium concentration and Osms) would be key to differentiating the different common causes of hypernatremia in infants. In excess solute load, urine Na and Osms will both be high. In hypernatremic dehydration (from lack of sufficient free water intake), urine Na will be low but Osms will be high. In diabetes insipidus, uine Na and Osms will both be low.
Re: FLUID MANAGEMENT IN CARDIAC KIDS....
ReplyDeleteThe idea of "not giving a cardiac kid 20ml/kg" is silly. Keep in mind, most of the cardiac lesions require preload and volume so I would heir on the side of giving fluid rather than restricting it. The best way to know is looking at the child's medicines. Unless the child is on obvious afterload reduction (diuretics, calcium blockers, etc)........then odds are that fluid will help and not hurt the child. Most single ventricles and pHTN kids NEED fluid. Repaired transposition should not be an LV outflow problem so to withhold fluid in this child because there is a "cardiac lesion" is criminal. Give FLUID ! you can always take it away with Lasix or respiratory support. If the child has a dilated cardiomyopathy or critical CHF....then it's another story.
With blood products it's sometimes good to be cautious and give squidly amounts like 5ml/kg.......there is a risk for sludging and stroke if correcting too fast or in the setting of hyperleukocytosis or critical anemia.
I totally agree with Dewesh on evaluating for DI and getting a good dietary history for excess sodium load. Usually diarrhea causes hypernatremia not vomiting. So that's a little suspicious to me.....vomiting and hypernatremia make me think of intracranial things and DI rather than routine hypernatremic hypovolemic dehydration.
It's worth thinking about structural things. I know situs inversus is associated with dextrocardia, so I don't think it's associated with TGA....but may be worth considering a structural reason for the vomiting. or things like pancreatitis as a surgical complication.
Agree with the two gentlemen above on initial management:
ReplyDelete1st 10 minutes: line, 20/kg bolus push pull, labs including stat VBG with lytes, portable CXR, EKG, call cardiology down with echo machine. You know you've got a kid with severe dehydration just with VS/wt and even before laying hands on the pt and seeing the doughy/dry look - >10% weight loss from prior weight, given that he should have continued to gain another 0.22 kg in his 11 days out (5.26 --> 4.7).
We can easily resuscitate this child acutely and get the CICU to handle the rest of his Na correction, but we need to make sure that "it's not the heart." By physical, you're not seeing signs of LHF (crackles, wob, tachypnea, desats, LV heave at L MCL subcostal) or RHF (liver down, RV heave subxiphoid; rarely will you see edema so don't rely on that), so I'd say he needs fluid. He's post TGA - so he should have "normal anatomy" now as his atrial septostomy should have been closed and everything reattached nicely. Except - these TGA kids can have trouble at all of the "connection points" that were repaired - coronaries (can be low flow/stenosed as these were reconnected), aorta (can get AI), thromboses (virchow's triad = stasis + hypercoagulable state + endovascular integrity not perfect; he's got 3 of 3 right now). His physical exam is not suggesting AI, but an echo will confirm this and also look at the coronaries and check for thromboses. If he is having any of those cardiac problems, even with perfect formula mixing, he could go into a low cardiac output state --> low GI flow with vomiting --> low renal flow with resultant bump up in K, crt, Na, and bun --> low brain flow with lethargy, etc. Personally I'd ask them how they did the formula, but hold off on the formula demonstration for now but pass it on to the inpatient team to assure it's being done right. Let's say you do the demo, you find out and tell them they've done it wrong, and the child dies in the next couple hours during the resuscitation - the parents are always going to think the death was their fault; this education would need to happen if it's being done wrong, but the timing of it would need to be carefully considered and perhaps come from the CI team who has better/longer rapport with the family.
Oh, and put in a ICU bed.
First time on the hot seat, “hot” describes it well!
ReplyDeleteThis is how I would run through this case:
With that initial history and physical exam provided I notice the following: A 4 month old, not healthy child, s/p cardiac surgery with complications, still on cardiac medications (hence does not have a perfect cardiac function), who has vomiting, decreased activity, decreased urine output. On exam, VS wnl, definite weight loss, and signs of severe dehydration.
Note: 1st sign of dehydration or shock in kids is tachycardia, this kid is not, but she is on propranolol, which would mask that. I’m not sure what “doughy” skin means. It makes me think of severe decreased skin turgor that I have seen with severe dehydration and malnutrition in underdeveloped countries.
Missing information: what’s the capillary refill (I find it often changes to the worse, before any peripheral pulses changes), and type of feeds and mixing as suggested in above comments with a bit more details about the past 11 days.
What am I thinking at this point? This child is sick and needs immediate resuscitation. I’m not sure what caused this illness: is the vomiting a cause or a symptom, but either way she is severely dehydrated and not in cardiogenic shock (based on exam) and therefore I would say fluids first. I don’t think there is a harm in starting 20cc/kg NS bolus (child is not on meds for heart failure at this point) however I have learnt that everyone else in the ED is scared of this and arguing about 10 vs 20 is not usually fruitful and a waste of time. So I usually go ahead and start with 10cc/kg NS bolus, but given fast, over 15min (if need be faster), while I remain at the bedside if possible, assessing the respiratory status of the child, the liver, the perfusion and if all well, will give another 10cc/kg NS bolus immediately after… and continue as such. I found this puts up less of a fight and everyone is more comfortable with it, without it affecting my management.
Get a D-stick (hypoglycemia is more common that we think in dehydration) and i-stat lytes (if the child is as sick as described).
While all this is taking place, I would wonder, what is causing all this?
Vomiting I have learnt from the cardiologists, can be a sign of heart failure (decreased perfusion to the gut)
Vomiting can also be secondary to hypernatremia, dehydration, or hypernatremic dehydration. What caused the latter? A wrong formulation is possible, though with a home nurse, recent d/c from chronic facility, you would hope everyone at home knows how to mix the formula. Assumptions though I have learnt are our worst enemy in medicine! A new disease would be really unfair to this child, 4 months after recovering from TGA! And why now? I don’t think any of her current meds could be culprits.
This child can still have what any other child can:
Gastroenteritis (diarrhea will soon start), gastritis, a UTI (though no fevers) and pyloric stenosis (though it’s a late presentation); More severely volvulus (though abdomen is soft, not distended and vomiting non bilious), intussusception (I’ve seen it at this age, though the child does not seem to be in pain or discomfort).
So what work-up will I start while starting fluid resuscitation?
CXR, EKG to r/o signs of heart failure as they are not always present on physical exam alone (plus every cardiac kid gets these anytime they appear sick), BMP, UA/Ur Cx.
Finally, what will I do with that BMP?
To me, this still looks like hypernatremic dehydration: elevated Na, BUN, Creat (for age, usually it is 0.3-0.4 in infants). The vomiting could have started it, and in a cardiac child, generally not that healthy, and with poorer reserves, it could be enough to tip her off. I would go with my 1st instinct, and treat with more fluids. That would be the treatment of any hypernatremia at this point.
Also to remember: With this Na, she is at risk for seizures. Keep on monitors, have Non-rebreather and BMV at the bedside, and send to the PICU. You will not fix this Na fast enough for the floor!
Cheers!