Over the years many amazing cases have been presented during the PEMPix presentation at the American Academy of Pediatrics’ annual national Conference and Exhibition during the Section on Emergency Medicine Sessions. PEMPix Classic is a new featured series on PEMBlog that will highlight some of those classic cases.
A six year old female with initial onset of right sided neck mass six months prior to presentation presents to the Emergency Department. At onset she was initially treated with a course of amoxicillin. It got better (for about two months) but then recurred and was treated with clindamycin and eventually an incision and drainage was performed. It was felt that the clindamycin had failed, and she was given a course of trimethoprim/sulfamethoxazole following the I&D. The wound culture was negative.
On the day of presentation to the Emergency Department her parents note that the swelling has again returned. They deny fevers or systemic symptoms (including no chronic cough, night sweats or weight loss). She has not been on any more antibiotics since completing the trimethoprim/sulfamethoxazole more than a month ago. There has been no drainage from the area, and parents note that the area around the I&D never quite healed, with a scab persistently present. They deny any significant exposures to HIV, known TB patients or kittens/cats.
Her physical exam is notable for a well appearing young lady with a nontender, erythematous fluctuant mass in the right submandibular region.
What is the diagnosis?
A. Cat-Scratch Disease
B. Cervicofacial actinomycosis
C. MRSA cervical adenitis
E. Branchial cleft cyst
A chest X-Ray showed right hilar lymphadenopathy suggestive of possible granulomatous infection. Ultrasound of the neck revealed suppurative right sided submandibular lymphadenopathy (phlegm). The quantiferon gold test was positive, but gastric aspirates x4 were negative for acid fast bacilli.
Fine needle aspiration of the mass was subsequently performed and the pathologist noted few stainable bacilli with necrotizing granulomatous lymphadenitis suspicious for mycobacterial infection. She was started on treatment with azithromycin to cover atypical mycobacteria (i.e. Mycobacterium avium complex) which was thought to be the most likely cause of infection in this age group. She was also treated with a combination of rifampin, isoniazid, pyrazinamide, and ethambutol (RIPE) therapy with B6 supplementation to cover Mycobacterium tuberculosis due to positive Quantiferon-TB Gold test. Six and a half weeks later, the surgical specimen wound culture from fine needle aspiration grew Mycobacterium tuberculosis complex which is extremely rare in this age group.
Peripheral tuberculous lymphadenitis accounts for ∼10% of tuberculosis cases in the United States. The peak age range is 30–40 years, with a predominance of foreign birth noted, especially East Asian. Peripheral tuberculous lymphadenitis—previously termed “scrofula”—is a unique manifestation of disease due to organisms of the Mycobacterium tuberculosis complex.
Patients with scrofulas present with a >1–2 month history of slowly progressive, painless swelling of a single group of cervical lymph nodes. Cervical chain involvement is most common and is reported in 45%–70% of cases, with 12%–26% in the supraclavicular region. The median lymph node size is 3 cm, but nodes may be up to 8–10 cm in diameter. Unilateral involvement of 1–3 nodes has been noted in 85% of cases. Concomitant pulmonary tuberculosis is reported in 18%–42% of patients, with higher rates among HIV-positive patients than among HIV-negative patients. Fine needle aspiration has emerged as a first-line diagnostic technique and it is safer, less invasive, and more practical than biopsy. Culture remains the gold standard for diagnosis, but may take 2–4 weeks to yield results.
The Infectious Disease Society of America (IDSA) recommends 6 months of the following treatment for lymphadenitis caused by drug-susceptible organisms: isoniazid, rifampin, pyrazinamide, and ethambutol for 2 months, followed by isoniazid and rifampin for another 4 months. Keep in mind that Mycobacterium tuberculosis lymphadenitis is very uncommon in this age group.
Atypical mycobacterium infections more commonly manifest as chronic cervicofacial lymphadenitis in pediatric patients aged 2-5 years old because they are found naturally in soil and water environments and they commonly place non-sterile objects from the environment in their mouths.