Hot Seat Denouement #32: 2 month-old with new onset cheek swelling

by Fareed Saleh, Children’s National
with Jennifer Chapman, Children’s National, on the Hot Seat

The Case

An almost 3 month-old boy with new left cheek swelling without a clear precipitant. The swelling is mildly tender without erythema. For a complete case presentation, click here. This was another challenging case, which is reflected in the insightful comments by the faculty and the answers to our multiple choice questions. What would you do if this was your patient? Read on to see if you agree and find out what happened.

Here’s how you answered our questions
Would you perform further imaging during this ER visit?
Less than 3 years PEM experience (n=14)

More than 3 years PEM experience (n=6)

Most people thought that no further imaging was needed. In contrast to those with more than 3 years PEM experience, the under 3 years PEM experience group had some votes for MRI or CT. One person in the under 3 years group said, “I am not sure at this point. I would want info from further labs.” Fair answer. It's a tough spot to be in. One of the more than 3 years group said, “Call plastics or surgery for consult on best further imaging. Also the attending radiologist.” Perhaps someone else has seen something like this?
 
Following your imaging decision, what is your next management step?
Less than 3 years PEM experience (n=11)

More than 3 years PEM experience(n=6)

This was a mixed bag. The less than 3 years EPM experience group was mostly split between consulting ENT or the “None” option with a few wanting to wait for imaging results or getting a CBC and blood culture. The more than three years experience group was evenly split between labs, ENT, and the “none” option. I think some of the "none" people explain their reasoning below.

Clinical Reasoning
On gathering more data 
“It's great to make sure the swelling doesn't cross the angle of the mandible as you'd see with partotitis.” -Dave Mathison, Children’s National 

“I would start by asking radiology what differential the US findings (of "free fluid within the soft tissues of the cheek with some small debris, no clear sepatations to suggest abscess, no discernable mass") are most consistent with.” -Dewesh Agrawal, Children’s National 

“I guess I'd send coags because it's a boy and theoretically this could be the first presentation of hemophilia if he bled into his cheek. but I've never heard of that.” -Dave Mathison, Children’s National 

On what’s the differential for this unusual presentation 
“For me, it depends on how much you trust your history. TRUE history (less than 6 hours onset): More likely: salivary gland cyst, blocked salivary duct (stone, infection, mumps), bleed/hematoma, fracture.” -Paul Mullan, Children’s National 

“There aren't that many things that cause pain. Fat necrosis does not. Urticaria does not. To me, pain equals: broken bone or clot or infection or contusion.” -Dave Mathison, Children’s National 

“Less likely: tumor (name your -oma), infection (abscess, buccal cellulitis, parotiditis; unlikely given not red or hot, but re-examination might find one of these esp as very young infants not always manifesting the rubor/tumor/dolor/calor).” -Paul Mullan, Children’s National

From the Hot Seat 
by Jennifer Chapman, Children’s National

To summarize, this is a healthy FT 3mo with 6 hours of one-sided cheek swelling. Possibilities include infection, trauma, congenital anomaly and malignancy. In order:

Infection: even before my time, Hflu caused a classic cellulitis in the cheek from hematogenous spread. The baby’s incomplete immunization against Hflu is his major risk factor for this infection- not race or gender. What supports infection is the relatively rapid onset and the tenderness, while the lack of redness and lack of fever go against it. Other infections: abscess related to a break in the skin and cellulitis related to odontogenic infection- neither is well supported by the physical exam.

Trauma: a not very traumatic cause of this type of exam in older children is popsicle panniculitis- the cold from the popsicle resting inside the cheek injures the fat and causes an inflammatory change. Nothing needs to be done after establishing the history of cold exposure. As for other trauma leading to swelling, the exam doesn’t demonstrate signs of bruising or skin breaks that would make me think of an injury to the cheek.

Congenital anomaly: there are a variety of congenital anomalies that could cause swelling of the cheek, including a cyst within the soft tissue or related to the parotid gland, an adenoma of the parotid gland, a lymphatic abnormality, a hemangioma, probably many others. What would speak against these is the rapidity of onset of the swelling, though sometimes cystic abnormalities do suddenly enlarge with infection or trauma.

Malignancy: rhabdomyosarcomas can arise from any muscle, with the face/neck being a common site (I think the pelvis is most common). But this is much more common in older children/adolescents and I wouldn’t think of it as readily in a 3mo.

How does the US help? The free fluid makes cellulitis and solid tumors less likely. Abscess still not as likely given the exam. So the US leads more towards a congenital anomaly. 

Plan: have to commit to whether I think this is an infection- since this is the most serious concern in this young infant. On my bold day, I would not do bloodwork and not admit. I would ask ENT for their input on what might cause this isolated, noninfectious fluid collection. I would also involve the PMD around follow-up and recommended imaging/ENT follow-up. On a worried day, I would get a cbc and BCx and admit for observation, with discussion with the Hospitalist around not starting antibiotics unless the baby develops a fever and around what further imaging might help. In practice, I might phone-a-friend and get another PEM physician’s input.

The Denouement
by Fareed Saleh, Children’s National
The subsequent work-up included further imaging (MRI orbits/face) noted for 'large, heterogenous, lobulated 4 cm mass in the right facial soft tissues. Mass most likely represents a venolymphatic malformation. Also, extensive inflammatory changes surrounding the mass which could be related to recent hemorrhage or superimposed infection.' 

See below for representative US and MRI images

Labs drawn included:
CBC noted for leukocytosis (17k), stable H/H (10.2/30.5), platelets of 328k. MCV 64.5.
PT/PTT/INR (14.4/106.3/1.18).
BMP and CRP both were wnl.
Aerobic blood culture was negative.

Of note, after repeated questioning the mother stated her other son from a different father had Hemophilia A that presented with spontaneous bleeding into his scrotum.

Patient was admitted with ENT consulted but deferred surgical intervention. Hematology consulted and levels for Factor VIII (less than 1%) and Factor IX (wnl) were drawn. He was diagnosed with hemophilia A and started on Factor VIII replacement therapy. He was subsequently discharged without incident.

Editor's Note: This is a nice example of medical school hematology in real life. This patient has Hemophilia A, which is a deficiency of Factor VIII. This resulted in an abnormal PTT but relatively normal PT/INR. (Kudos to Dr. Caleb Ward for knowing these facts off of the top of his head when asked during a shift in the ER). For those of you who want the trauma of seeing the coagulation cascade, a good explanation of how it fits into hemophilia can be found here.

The Hot Seat is a recurrent online case series aimed at facilitating asynchronous sharing of PEM knowledge and experience. Faculty are placed on The Hot Seat and publish their opinions without knowing the case or its outcomes. Cases are based on real cases and written by PEM fellows at Children's National in DC, Johns Hopkins in MD, and INOVA Fairfax in VA to highlight teaching points. Emily Willner is the faculty advisor for the Hot Seat.