Calcified Tuberculous Cervical Lymphadenitis (Scrofula)

Clinical Presentation Asymptomatic Radiologic Findings AP (Fig. A) and lateral (Fig. B) views of cervical spine demonstrate multiple variable-sized partially calcified nodular opacities with an “egg-shell” pattern of calcification. Note the central lucency. The lung apices are clear.     Diagnosis: Calcified Tuberculous Cervical Lymphadenitis (Scrofula)

Differential Diagnosis

Granulomatous Infection or Inflammatory Processes of other Etiologies

Discussion

Tuberculous lymphadenitis or scrofula (after the Latin word “scrofa” for sow’s milk) was first described in Medieval times. Centuries latter discovered that the infection was caused by the ingestion of infected cow’s milk and most commonly from infection with Mycobacterium bovis.

Affected patients presented with swollen “glands” in the neck. For centuries, people believed that Kings had been given divine powers from God to rid people of this “curse” and that the simple touch by their royal hand would cure those affected. Large pompous ceremonies were often carried out during their “cleansing process”. In addition to the affected person and the King, a choir, ladies and gentlemen of the court, and a troop or two of Beefeaters participated in the ceremony. In fact, Charles II, King of England, Scotland, Ireland, one of the cleverest but most controversial of English Kings, is credited with having “Touched” more than 92,000 affected persons during his reign.

Etiology

Cervical lymphadenitis is the most common form of extra-pulmonary Tuberculosis.

Tuberculous infection of the neck may be caused by:

• Mycobacterium tuberculosis
• Mycobacterium scrofulaceum (children)
• Mycobacterium avium complex (children)

Present day tuberculous cervical lymphadenitis is more commonly caused by inhalation rather than ingestion. The bacterium spreads throughout the body and affects the cervical and supraclavicular lymph node chain.

Clinical Findings

Affected patients often present with “Rubbery” enlargement of cervical lymph nodes. There may be concomitant involvement of the lymph nodes in the supraclavicular fossa, axillae, and groin. The nodes may then ulcerate producing chronic draining sinuses and abscesses. Cervical lymphadenitis was relatively rare pre-AIDS epidemic. Since the HIV-AIDS epidemic, there has been a resurgence of both localized and systemic forms of tuberculosis. Mycobacterium scrofulaceum accounts for approximately 2% of mycobacterial infections in AIDS patients but also affects minorities, women, and children.

Immunocompetent Adults:

• May present with swollen lymph nodes
• Unilateral
• Cervical node distribution
• Along upper border sternocleidomastoid muscle
• Calcified nodes often incidental finding
• Usually no active pulmonary disease

Immunocompetent Children:

• Present with painless, swollen, rubbery lymph nodes
• Unilateral
• Cervical node distribution
• Usually have concomitant active pulmonary disease

Immunocompromised (HIV +) Children:

• Multifocal disease
• Higher prevalence of systemic spread (active pulmonary disease)
• Most commonly affected nodes along upper border sternocleidomastoid muscle

Differential Diagnosis Cervical Lymphadenitis-Immunocompetent Patients

• Mycobacterium tuberculosis
• Atypical mycobacterium
• Various gram (+) bacteria
• Bartonella henselae (cat scratch)
• Lymphoma
• Branchial cleft cysts (cystic hygromas)

Differential Diagnosis Cervical Lymphadenitis-Immunocompromised (HIV +)

• HIV adenitis
• Mycobacterium tuberculosis
• Various gram (+) bacteria
• Atypical mycobacterium
• Bartonella henselae (cat scratch)
• Lymphoma
• Branchial cleft cysts (cystic hygromas)

Treatment

Immunocompetent

• Minimum six months anti-microbial therapy
• 3-4 anti-tuberculosis drugs
• Isoniazid and Rifampin
• Pyrazinamide added during first two months

Immunocompromised

• Complete surgical excision
• Minimize potential for fistula formation
• Minimum six months anti-microbial therapy
• 3-4 anti-tuberculosis drugs
• Isoniazid and Rifampin
• Pyrazinamide added during first two months

Selected Readings

1. Jawahar MS. Scrofula Revisited: An Update on the Diagnosis and Management of Tuberculosis if Superficial Lymph Nodes. Indian J Pediatr 2000; 67(2): S28-33.
2. Kafle P, Kolade V. Scrofula. J Hosp Med 2010, 5(7): E3.
3. Khan R, Harris SH, Verma AK, et al. Cervical Lymphadenopathy: Scrofula Revisited. J Laryngol Otol 2009; 123(7): 764-767.