Children with Down syndrome no longer require screening for atlantoaxial instability but radiography is appropriate when any symptoms develop. Children found to have atlantoaxial subluxation are at risk for spinal cord compression, which may lead to symptoms such as brisk tendon reflexes, upgoing plantar response, and bladder or bowel dysfunction. Surgery is required for stabilization.

Other neurologic complications associated with trisomy 21 but unrelated to atlantoaxial instability include generalized hypotonia, epilepsy, cognitive impairment, mood disorders, stroke (due to cardiac issues), and vision and hearing impairment.

Reference:

Bull MJ; Committee on Genetics. Health supervision for children with Down syndrome. Pediatrics. 2011;128(2):393-406.

This patient was suffering from a nasopharyngeal carcinoma that had metastasized to the lymph nodes in the sternocleidomastoid area.

In general, unilateral referred otalgia or persistent serous otitis media in an adult is a nasopharyngeal cancer (not a retropharyngeal cancer) until proven otherwise.

Other rules of thumb with regards to neck masses are:

1. Lateral neck masses are more likely to be malignant.
2. 80% of non-thyroid neck masses in adults are neoplasms, of which 80% are malignant (80-80 rule).

The undiagnosed cancer patient will often not connect pertinent symptoms of a neoplasm together. Thus the clinician should perform and document a more general exam (beyond the patient's immediately symptomatic area) and review of systems when confronted with a mass that may be a tumor. A minimum HEENT review would include a cranial nerve exam and questioning about otalgia, hoarseness, odynophagia, dysphagia, stridor, globus phenomena, and new speech problems. The status of neck lymphnodes should also be remarked upon. The level at which an abnormal node occurs is in and of itself a clue to the location of the primary tumor.

Hoarseness in particular is an easily ignored complaint that may be the only presenting sign of a laryngeal or tracheal malignancy. Any hoarseness that has lasted for more than two weeks requires investigating or referral.

Pearl: Globus phenomenon refers to the sensation of a mass in the throat.

References:
Marx: Rosen's Emergency Medicine, Concepts and Clinical Practice, 6th ed., 2006

Robert Dolan, MD, Associate Professor, Department of Otolaryngology, Boston University Medical Center, Case Study.

Additional Literature:
Molecular Biology of Head and Neck Cancer: Risks and Pathways. Stadler ME - Hematol Oncol Clin North Am - December, 2008; 22(6); 1099-1124

Doxycycline, amoxicillin, and cefuroxime are the preferred oral antibiotics for the treatment of Lyme disease. Ceftriaxone is the preferred parenteral antibiotic. The length of treatment for patients with Lyme disease depends on the type of manifestation (see Table). 

Reference:

Wormser GP, Dattwyler RJ, Shapiro ED, et al. The clinical assessment, treatment, and prevention of lyme disease, human granulocytic anaplasmosis, and babesiosis: clinical practice guidelines by the Infectious Diseases Society of America. Clin Infect Dis. 2006;43(9):1089-1134.

Educational Objective:

Differentiate sinusitis from viral upper respiratory infection.

Key Point:

The diagnosis of sinusitis is clinical and based on the duration and severity of symptoms or worsening symptoms after an initial period of improvement.

Explanation:

Viral URIs can be difficult to differentiate from sinusitis. Most URIs take 6 to 10 days to improve, and, if fever is present, it is usually mild and at the beginning of the illness. Sinusitis should be suspected if symptoms last more than 10 days, are severe and accompanied by fever, or if symptoms worsen after a period of initial improvement. Imaging such as CT is not needed for uncomplicated sinusitis and would only serve to confirm the presence of inflammation in uncomplicated URI. Nasal cultures are unlikely to be helpful as they are oversensitive and unlikely to change the choice of therapy. In children without risk factors for resistant infection, amoxicillin/clavulanate is likely to be effective.

References:

Cherry JD, et al. Rhinosinusitis (chapter 14). In: Cherry J, Demmler-Harrison GJ. Feigin and Cherry’s Textbook of Pediatric Infectious Diseases. 8th ed., 2018:137-143.

Pappas D, Hendley O. Sinusitis (chapter 380). In: Kliegman RM, et al. Nelson Textbook of Pediatrics. 20th ed., 2016:2014-2017.

Wald ER, Applegate KE, Bordley C, et al. Clinical practice guideline for the diagnosis and management of acute bacterial sinusitis in children aged 1 to 18 years. Pediatrics. 2013;132:e262.

Educational Objective:

Discuss initial treatment of necrotizing infections.

Key Point:

Surgery with tissue biopsy and culture are key EARLY steps to selecting the definitive antibiotics. However, a reasonable empiric antibiotic regimen is clindamycin (stops staphylococcal toxin production) plus a broad-spectrum beta-lactam such as ampicillin-sulbactam plus vancomycin (to cover for MRSA)

Explanation:

This patient presents with clinical manifestations of necrotizing soft tissue infection. In 80% of cases, these rapidly fatal infections are caused by a direct extension from a skin lesion. Type I is polymicrobial (aerobes and anaerobes; crepitance oten repsent) and is more common in diabetics and immunocompromised individuals (such as the above patient).

Type II is caused by a single organism (most commonly group A streptococci; less commonly MRSA), occur in any age group and in patients who are overall healthy.

Broad-spectrum intravenous antibiotic therapy should always be initiated as soon as this diagnosis is suspected. Clindamycin (stops staphylococcal toxin production) plus a broad-spectrum beta-lactam such as ampicillin-sulbactam plus vancomycin (to cover for MRSA) is a reasonable regimen suggested in the 2018 edition of Rosen.

The Infectious Diseases Society of America (IDSA) most current guidelines (last published in 2014) suggest a slightly different regimen, which is another reasonable empiric choice:

(1) vancomycin or linezolid plus

(2a) either piperacillin-tazobactam or

(2b) a carbapenem or

(2c) ceftriaxone plus metronidazole.

This later regimen is also discussed in the 2020 assigned life-long learning and self-assessment (LLSA) reading of the American Board of Emergency medicine (ABEM). It lacks the clindamycin that specifically stops the toxin of staphylococcal and streptococcal species.

Changes in the overlying skin may not reflect the degree of underlying infection, and clinical suspicion is important to diagnose the condition early.

Necrotizing fasciitis has a rapidly progressive course. Early surgical intervention is a key step to reducing mortality.

References:

Pallin DJ. Skin Infections. (Chapter 129) In: Walls R, et al. Rosen’s Emergency Medicine: Concepts and Clinical Practice. 9th ed., 2018: 1719 - 1722.e2.

Stevens DL, Bryant AE. Necrotizing soft-tissue infections. N Engl J Med 2017 Dec;377(23): 2253-65.

Stevens DL, Bisno AL, Chambers HF, Dellinger EP, Goldstein EJ, Gorbach SL, et al. Practice guidelines for the diagnosis and management of skin and soft tissue infections: 2014 update by the Infectious Diseases Society of America. Clin Infect Dis. 2014 Jul 15. 59 (2):e10-52

Kiat HJ, En Natalie YH, Fatimah L. Necrotizing Fasciitis: How Reliable are the Cutaneous Signs? J Emerg Trauma Shock. 2017;10(4):205-210.