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• Incomplete Kawasaki disease – Not to be forgotten
  Anjum Gandhi
  Published on: 9 January 2022

• Published on: 9 January 2022

  Incomplete Kawasaki disease – Not to be forgotten

  • Anjum Gandhi, SpR in Paediatric Cardiology
  • Other Contributors:
    • Dirk G. Wilson

  Dear Editor,

  Drs Sinha and Balakumar have provided a reminder of BCG scar reactivation as a diagnostic marker of Kawasaki disease (KD). This potentially serious disease has no definitive diagnostic test and it is not unusual for the diagnosis to be delayed. The authors also alluded to incomplete KD, of which there is increasing recognition and which may be associated with a higher chance of cardiac complications....

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  Dear Editor,

  Drs Sinha and Balakumar have provided a reminder of BCG scar reactivation as a diagnostic marker of Kawasaki disease (KD). This potentially serious disease has no definitive diagnostic test and it is not unusual for the diagnosis to be delayed. The authors also alluded to incomplete KD, of which there is increasing recognition and which may be associated with a higher chance of cardiac complications.[1]

  We recently reviewed the cases of KD presenting in South Wales and found an alarming increase in the number of patients developing coronary artery aneurysms in comparison to a similar review done in 2001. In 2001 the Paediatric Cardiac Unit at the University Hospital of Wales presented a ten-year audit of Kawasaki Disease. We reported 57 cases in ten years and only 3 patients had coronary artery aneurysms. Since that audit was undertaken we are aware of 27 more cases of Kawasaki Disease in the last four years. The incidence of the disease therefore does not appear to have changed, however we are now seeing a higher incidence of coronary artery abnormalities (observed in 10 of the 27 cases). This includes both medium-sized and giant coronary artery aneurysms. Remarkably, in one infant the diagnosis of KD was made retrospectively when an echocardiogram for an unrelated reason showed giant coronary artery aneurysms.

  Although the reason for this apparent increase in the incidence of coronary artery abnormalities remains unclear it highlights the importance of considering the diagnosis of KD in any infant or child with high grade fever for 5 or more days. This is especially true in younger infants who are at a higher risk of developing coronary artery aneurysms and who may present with fever and few if any of the cardinal clinical features of KD. In fact the latest guidelines by the American Heart Association recommend that echocardiography should be considered in any infant aged less than 6 months with fever of 7 days or more in duration and laboratory evidence of systemic inflammation.[2]

  A variety of helpful diagnostic clues have been recognized over the last few years. Notable is the presence of hydrops of gall bladder in 15% of the cases which can be picked up easily on an abdominal ultrasound.[3] Arthritis and arthralgia generally involving multiple small joints in the first week and large weight bearing joints later can occur. Up to a third of these patients have gastrointestinal symptoms such as diarrhea, vomiting and abdominal pain and rarely KD may present as acute surgical abdomen.[4] Transient unilateral facial nerve palsy and high frequency sensori-neural hearing loss may also occur.[5]

  The report by Drs Sinha and Balakumar is a timely reminder to be vigilant for this potentially dangerous disease with protean manifestations.

  References

  1. Witt MT, Minnich LL, Bohnsack JF, et al. Kawasaki disease: more patients are being diagnosed who do not meet American Heart Association criteria. Pediatrics 1997; 104:e10.

  2. Newburger J, Takahashi M, Gerber M, et al. Diagnosis, treatment and long-term management of Kawasaki disease. Circulation 2004; 110: 2747- 71.

  3. Suddleson EA, Reid B, Woolley MM, Takahashi M. Hydrops of the gallbladder associated with Kawasaki syndrome. J Pediatr Surg. 1987; 22: 956-959.

  4. Zulian F, Falcini F, Zancan L, et al. Acute surgical abdomen as presenting manifestation of Kawasaki disease. J Pediatr. 2003; 142: 731- 735.

  5. Knott PD, Orloff LA, Harris JP, et al. Kawasaki Disease Multicenter Hearing Loss Study Group. Sensorineural hearing loss and Kawasaki disease: a prospective study. Am J Otolaryngol. 2001; 22: 343- 348.

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  Conflict of Interest:
  None declared.

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