January 2008

A 14-year-old girl with poorly controlled insulin-dependent diabetes mellitus (IDDM) was admitted from the emergency room to the pediatric intensive care unit (PICU) for treatment of diabetic ketoacidosis (DKA).

Her presenting symptoms included vomiting with dehydration and confusion. Even though she was afebrile on admission, she may have been febrile at home, but her parents did not take her temperature. In addition to lab findings consistent with DKA, her admitting white blood cell count was 48,000 with 75% granulocytes. Within 24 hours, she was transferred to the pediatric ward for further care, when she began complaining of a sore throat.

Her past medical history is significant only for having IDDM since she was 5, with multiple admissions for poor control, and many clinic visits each year for minor illnesses. She has had no surgeries or admissions for any other medical reasons. Her immunizations are up to date. Her family and social history is unremarkable. There have been no known sick contacts, but she attends public school, and does not know if any of her friends have been ill recently.

Examination on arrival to the PICU revealed tachycardia and tachypnea, consistent with dehydration, but no fever. She remained afebrile throughout her hospital stay. In the PICU, she had depressed mental status that resolved with fluids. No other positive findings were reported. Examination of her throat upon arrival to the ward revealed inflammation of the uvula with what appeared to be exudate, as shown in figure 1. The uvula was swabbed for a rapid strep test, which was negative, and cultures for bacteria and fungi are pending.

What is the most likely cause of this diagnosis?

1. Zygomycosis
2. Haemophilus influenzae type b
3. Group A Streptococcus
4. Candida albicans

Answer

The most common cause of Uvulitis is C, group A streptococcus (GAS).

Very few papers have been published on uvulitis, but it seems clear that group A streptococcus leads the list in all. This is likely to be because there is usually associated streptococcal pharyngitis (figure 2) when uvulitis is seen. However, in the days when infections with Haemophilus influenzae type b (Hib) was a common problem, uvulitis could be seen associated with epiglottitis. In fact, the presence of uvulitis in and of itself was considered reason to investigate for the possible diagnosis of epiglottitis. Since the virtual disappearance of Hib due to the widespread use of Hib vaccine and subsequent herd immunity, epiglottitis in children has almost disappeared, making that choice very unlikely.

The fact that this patient has IDDM that is poorly controlled with frequent admissions for the management of ketoacidosis places the child at increased risk for infectious complications of diabetes. These may be common infections with more severe or prolonged courses.

Additionally, there is a well-recognized association of DKA and mucormycosis, or more accurately, zygomycosis, for reasons that appear to have something to do with the hyperglycemic and/or acidotic state, as this life-threatening infection tends to occur in diabetics only when they are out of control. It almost always involves the perinasal sinuses, rapidly spreading in every direction, as shown in figures 3 to 5, an adolescent with DKA from the collection of Basil Williams, DO, in New York. I have not read of zygomycosis involving the uvula, but I suppose it could happen in a severely advanced case that involves the entire palate.

Lastly, in 1991, Krober and Weir reported two cases of Candida uvulitis in normal children, where Candida albicans was recovered from the surface cultures, with negative GAS and Hib cultures. Therefore, it may be possible for Candida to cause uvulitis, but just not very likely.

In the case presented, all cultures were negative. However, it was strongly believed that the patient received some antibiotics prior to admission, even though no proof could be found in the record. With some prior treatment, a minor strep infection might be difficult to prove. Then again it may have been due to something else altogether. We’ll never know.

To read more about uvulitis, I would recommend Kotloff and Wald, Uvulitis in Children, The Pediatric Infectious Disease Journal, (1983; 392-392). It’s an oldie, but a goodie.

Upcoming meeting

I just wanted to make you all aware of a great meeting coming up March 10-13, 2008 in Honolulu. It is the annual Uniformed Services Pediatric Seminar. It is an excellent AAP-endorsed educational opportunity, with at least 24 hours of CME, in a truly outstanding location, at an affordable price. It will be held at the Waikiki Beach Marriott Resort, and the meeting announcement can be found at www.aap.org/profed/2008/USPS.pdf.

This group always puts on a superb general pediatric meeting. Plus, it is a great way to meet some of the best pediatricians in the country. I will be there as an attendee, not as a speaker. So, you won’t have to listen to me. One more reason to attend. Hope to see you there.