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Alexander Disease
Joseph Junewick, MD FACR
over 9 years ago
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Case Detail

Anatomy: Neck-Face
Joseph Junewick, MD FACR
Diagnostic Category: Infectious-Inflammatory
Created: over 8 years ago
Updated: over 8 years ago
Tags: PEDS
Modality/Study Types: CT CR
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4 year old male with refractory cervical adenopathy

Case Images



Clinical Notes

Positive PPD skin test.


CR – Soft tissue fullness with faint punctate calcifications in the left submandibular region.

CT – Multiple level IIA lymph nodes with punctate and coarse calcifications.


Scrofula refers to tuberculous nuchal adenitis. The usual presentation is an enlarging, non-tender neck mass that is unresponsive to conventional antibiotics. Mycobacterium tuberculosis accounts for 95% of scrofula cases in adults but only 8% of cases in children; the remainder is due to atypical Mycobacteria or nontuberculous mycobacterium (NTM).

NTM infections commonly present as cervical adenitis in immunocompetent children under 5 years of age. Systemic symptoms in NTM are often absent although occasional symptoms of low grade fever and slight tenderness are noted. In NTM, PPD testing ranges from negative to weakly positive. Culture and identification of the organisms may take up to 6 weeks. Immunocompromised patients have a higher prevalence of TB and extrapulmonary involvement of TB. TB in the head and neck represents approximately 15% of cases of extrapulmonary TB. PPD skin test is markedly positive with TB.

CT findings include heterogeneously enhancing adenopathy with contiguous low density necrotic ring-enhancing lesions. Stippled calcification may be present. Most commonly, adenopathy arises near the angle of the mandible or parotid space. Differential diagnostic considerations include neuroblastoma, papillary thyroid cancer metastases, fungal infection, and treated lymphoma.

Treatment of choice is excisional biopsy. Incision and drainage may lead to recurrent sinus tract formation. Long term double or triple drug regimens are often given. Response is gradual and may require up to 18 months to resolve.


Robson C, Hazra R, Barnes PD, et al. Nontuberculous Mycobacterial Infection of the Head and neck in Immunocompetent Children: CT and MR Findings. Am J Neuroradiol(1999); 20:1829-1835.

Burrill J, Williams C, Bain G, et al. Tuberculosis: A Radiologic Review. RadioGraphics (2007); 27:1255-1273


S. Andrew Hoff, MD

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