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Education And Debate

Lesson of the Week: Delayed diagnosis of Kawasaki disease presenting with massive lymphadenopathy and airway obstruction

BMJ 1996; 312 doi: https://doi.org/10.1136/bmj.312.7044.1471 (Published 08 June 1996) Cite this as: BMJ 1996;312:1471
  1. David Burgner, registrara,
  2. Marino Festa, registrarb,
  3. David Isaacs, headc
  1. a Department of Microbiology, Royal Alexandra Hospital for Children, Westmead, NSW 2145, Australia
  2. b Department of Intensive Care, Royal Alexandra Hospital for Children
  3. c Department of Immunology and Infectious Diseases, Royal Alexandra Hospital for Children
  1. Correspondence to: Dr Isaacs.
  • Accepted 18 March 1996

Kawasaki disease is an acute, self limiting febrile illness of infants and young children. In children under 5 years of age the incidence varies from about 3-6 per 100 000 in Europe1 to 90 per 100 000 in Japan.2 It causes coronary artery lesions in about 20% of untreated patients, up to 2% of whom die.3 Diagnosis is based on the presence of a prolonged fever (of more than five days duration) plus four of the following five signs: conjunctival injection, changes in the mucous membranes, changes in the peripheries (erythema of the palms and soles and swelling of the hands and feet), rash, and lymphadenopathy. The clinical features may be supplemented by non-specific laboratory investigations—for example, thrombocytosis and an increased erythrocyte sedimentation rate—but there is no specific diagnostic test. Prompt diagnosis permits early treatment with intravenous immunoglobulin, which reduces the incidence of coronary artery lesions by up to two thirds.4 5

Lymphadenopathy is the least constant of the diagnostic features of Kawasaki disease, occurring in 50-75% of patients; the other clinical criteria occur in about 90%.3 We describe a case of Kawasaki disease in which massive lymphadenopathy was the overriding clinical feature, resulting in upper airway obstruction and delayed diagnosis.

Case report

A previously well and fully immunised 4 year old white boy presented with a 10 day history of sore throat and fever that was unresponsive to oral antibiotics. Two days earlier he had developed a painful swelling in the right side of his neck which continued to enlarge. Initial examination showed that he was feverish, with a painful torticollis. He had large red tonsils, and the right tonsil was displaced medially. There was a large (5 x 3 cm), tender, and erythematous swelling in the posterior triangle of the right side of the neck. His respiratory function was not compromised, and none of the other features of Kawasaki disease was evident.

An initial full blood count showed moderate leucocytosis (16.4 x 109/l) and normal numbers of platelets. Cervical lymphadenitis was diagnosed and he was treated with intravenous ampicillin and flucloxacillin. Over the next 24 hours he remained unwell and feverish, and the cervical mass increased, with erythema and oedema spreading caudally to the chest wall. The antibiotic regimen was changed to cefotaxime, metronidazole, and penicillin to cover a possible polymicrobial fasciitis.

Respiratory embarrassment and increasing dysphagia necessitated intubation and ventilation. A fine needle aspiration of the neck mass gave only scanty return, which was sterile on bacterial culture. A cervical computed tomogram showed no focal collection but pronounced lymphadenopathy (fig 1). On the third day of admission rash, palmar erythema, non-purulent conjunctivitis, palmar erythema, a scalded skin appearance of his buttocks, together with pronounced thrombocytosis (537 x 109/l) and leucocytosis (26.1 x 109/l) developed. Kawasaki disease was diagnosed clinically and he was given intravenous immunoglobulin (2 g/kg) and low dose aspirin (2 mg/kg). His fever immediately subsided. His initial echocardiogram was normal and he was extubated uneventfully. Three days later his palmar and groin rash desquamated. A repeat echocardiogram at six weeks was normal. An antistreptolysin O titre taken two weeks into his illness was negative (< 150 IU/ml: normal range < 200 IU/ml).

Fig 1
Fig 1

Transverse cervical computed tomogram showing pronounced lymphadenopathy and oedema.

Comment

This child eventually fulfilled the diagnostic criteria for Kawasaki disease; the negative culture and antistreptolysin O titre made a diagnosis of streptococcal or staphylococcal lymphadenitis less likely. The presentation of Kawasaki disease with predominantly upper airway symptoms is rare but well recognised. Adenopathy occurs more commonly in older children and may reflect greater immunological maturity.6 In one large series 11 of 450 patients with Kawasaki disease presented with cervical lymphadenitis as the primary complaint.6 The neck nodes were large and often erythematous and tender, although pronounced cellulitis, as seen in our patient, was not described. The diagnosis of Kawasaki disease was delayed in most of the children; three developed coronary artery lesions. None required intubation. The cervical lymphadenopathy and fever resolved promptly in those given intravenous immunoglobulin. Kawasaki disease may also present with upper airway inflammation7 or mimic a retropharyngeal abscess.8

In the United States Kawasaki disease is the leading cause of acquired heart disease in childhood.3 In most cases cardiac lesions can be prevented by prompt diagnosis and treatment. Not all of the diagnostic features of Kawasaki disease may be obvious on presentation, and one particular sign, such as lymphadenopathy, may predominate. The diagnosis of Kawasaki disease, however, should always be considered in a young child with unexplained and prolonged fever, and careful and repeated examination is needed as the classical features of Kawasaki disease may only become evident over time.

Footnotes

  • Funding None.

  • Conflict of interest None.

References

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