This case is brought to you courtesy of ASK SNIFF . We are very grateful to the family of the young boy for consenting to the video being made publicly available. We hope it will be helpful for all health care professionals who deal with children.
Presenting Complaint
A 3 year old boy presents to an emergency department (ED) with a fever.
His parents describe him has having been very miserable from the start of the illness. Following a visit to his GP, he was prescribed penicillin but developed a widespread rash. He returned and an allergy was suspected. His antibiotics were switched to Clarithromycin but there was no improvement in his symptoms. 4 days into his illness he started complaining of pain in his right foot and his parents noticed he had been walking with a limp. Today they were worried he might be dehydrated as he had developed dry lips.
Initial Features
This is the child as he presents:
What additional information would you like in the history?
His parents report he has now had six days of fevers ranging between 38-39 degrees. There has been no history of foreign travel and no obvious contact with other infectious diseases.
What additional clinical information would you like?
In addition to the video footage:
Heart Rate: 162 Saturations: 97% Temperature: 39.2
Heart sounds were normal with no murmur audible. Chest and abdominal examinations were also unremarkable.
He had a right sided palpable cervical lymph node 1.5cm in size but no axillary lymphadenopathy. Both feet were oedematous, right more than left and he was reluctant to weight bear on his right. There was no localised tenderness and no other swollen joints.
Diagnosis and Management
What is/are potential differential diagnoses?
Septicaemia
Septic Arthritis
Streptococcal or Staphylococcal Related Disease
Stevens-Johnson Syndrome
Juvenile Arthritides
and…..
How would you manage this child?
There were concerns that this child may have Kawasaki’s disease. The decision was made to cover for sepsis with intravenous Ceftriaxone initially and within a few hours of presentation he was commenced on intravenous immunoglobulin and high dose aspirin. His initial echocardiogram was normal.
His symptoms resolved over 3-4 days and he was discharged home on low dose aspirin. He remained well and a repeat echocardiogram at 2 weeks and 5 weeks post start of treatment remained normal with no signs of coronary artery disease and no other features of vasculitis. Therefore Aspirin therapy was discontinued after 5 weeks treatment.
Learning
What are the key features?
Kawasaki’s disease (KD) is a condition in which a health care professional can have a real impact on a child’s long term health outcomes. It is thought to be an abnormal host response to a yet to be identified antigen. If you recognise and are able to treat within 10 days you can reduce the incidence of coronary artery aneurysm from 25% to 5% [1,2]. While the overall mortality in KD is low (0.2%-3%) [3-6] given the risk of death from thrombosis as a result of aneurysm formation [1] it is a disease process where complications are avoidable as a result of early recognition.
The diagnostic features of KD[1,7]
Fever (typically >39ºC) for at least four days and at least four of the following features
- Bilateral non-exudative bulbar conjunctival injection
- Polymorphous exanthema (no vesicles or crusts)
- Changes of extremities:
Acute- erythema of palms + soles, oedema of hands + feet.
Subacute- periungual peeling of fingers and toes)
- Changes to lips and oral cavity (red, fissured lips, strawberry tongue, erythema of oropharyngeal mucosa, without exudates)
- Cervical lymphadenopathy (≥1.5 cm, usually unilateral, rare in infants)
An important feature of KD is nearly universally children are extremely miserable. Although this is not a diagnostic criterion the condition should be considered in any child who seems unduly upset and very difficult to console.
The majority of children with KD are younger than 5 years of age (the peak incidence being at 18-24 months). Beware patients aged less than 3 months, or more than 5 years as although uncommon they have a greater incidence of aneurysm formation [7,8].
The dose of immunoglobulin and aspirin should be given according to local protocol. Suggested doses are 2g/kg immunoglobulin and 30-50mg/kg aspirin [7]
Why is it a difficult diagnosis?
A primary care observational study of KD found a delay of greater 10 days between initial presentation and subsequent hospital admission for 7% of children [9]. Children presenting with persistent fever and non-specific signs of rash and oral-mucosal changes are relatively common so you must think KD in order to diagnose it. Although the 5-day threshold is a useful discriminator there is evidence of atypical presentations and often the time span is missed because of incomplete histories. There is support for an approach in which KD is considered before 5 days of fever if the other 5 criteria are present, coronary artery aneurysms are detected or there is an unexplained persistence of inflammatory markers in the context of suspicion of KD [3,6].
Safety netting children who have prolonged fever is absolutely vital as parents might be falsely reassured that their child has a simple viral illness and delay seeking advice when symptoms persist or new signs appear.
What you may not know
Incomplete Kawasaki disease—fever but fewer than four of the other diagnostic criteria (bilateral conjunctival injection, polymorphous exanthema, changes to the extremities or lips and oral cavity or both)—represents 15-20% of cases [10]
A microbiologically confirmed infection is also present in a third of patients with Kawasaki disease and should not preclude the diagnosis [11]
Live vaccines (such as measles, mumps, and rubella) should be delayed for 11 months after treatment with intravenous immunoglobulin [10]
Outcome
After initial treatment our young boy started to make an improvement
Acknowledgements
A huge thanks to the family for agreeing to universal publication of this case. We are indebted to them for being able to demonstrate the key features of Kawasaki’s Disease. The video footage was obtained by Dr. Mariyum Hyrapetian who contributed to the production of this short case and we are grateful for her support as well as that of Whittington Hospital who allowed filming to take place. This video was taken as part of the ASK SNIFF research programme. ASK SNIFF (Acutely Sick Kid Safety Netting Interventions For Families) aims to to develop safety netting interventions for families to use to determine when to seek help for an acutely sick child.
References
- Newburger JW, Takahashi M, Gerber MA, Gewitz MH, Tani LY, Burns JC, et al Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Pediatrics 2004:114:1708-33.
- Tsuda E, Hamaoka K, Suzuki H, Sakazaki H, Murakami Y, Nakagawa M, et al. A survey of the 3-decade outcome for patients with giant aneurysms caused by Kawasaki disease. Am Heart J 2014;167:249-58
- Newburger JW, Takahashi M, Gerber MA, et al. Diagnosis, treatment, and longterm management of Kawasaki disease. Circulation 2004;110:2747–71
- Burns JC, Shike H, Gordon JB, et al. Sequelae of Kawasaki disease in adolescents and young adults. J Am Coll Cardiol 1996;2:253–7
- Belay ED, Maddox RA, Holman RC, et al. Kawasaki syndrome and risk factors for coronary artery abnormalities: United States, 1994–2003. Pediatr Infect Dis 2006;25:245–9
- Brogan PA, Bose A, Burgner D, et al. Kawasaki disease: an evidence based approach to diagnosis, treatment, and proposals for future research. Arch Dis Child 2002;86:286–90.
- Eleftheriou D, Levin M,Shingadia D,Tulloh R,Klein N,Brogan P Management of Kawasaki disease Arch Dis Child 2014;99:74–83 [Open Access]
- Harnden A, Alves B, Sheikh A. Rising incidence of Kawasaki disease in England: analysis of hospital admission data. BMJ 2002;324:1424–5.
- Moore A, Harnden A and Mayon-White R Recognising Kawasaki disease in UK primary care: a descriptive study using the Clinical Practice Research Datalink British Journal of General Practice 2014; 64(625) e477-e48
- Harnden A, Tulloh A, Burgner D. Easily Missed? Kawasaki Disease BMJ 2014;349:g533
- Benseler SM, McCrindle BW, Silverman ED, Tyrrell PN, Wong J, Yeung RS. Infections and Kawasaki disease: implications for coronary artery outcome. Pediatrics 2005;116:e760-6
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Excellent case and video, thank you. It might be nice to remove the title from the top of the video to preserve the mystery for the questions.
What a great spot and brilliant suggestion – have amended the title of clip. Thank you!