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G385 An unusual presentation of atypical kawasaki disease in a 6 year old child: a case report

Introduction Kawasaki disease (KD) is an acute systemic idiopathic inflammatory disorder, typically affecting children between 6 months and 4 years old. In developed countries it is the leading cause of acquired heart disease and is the second commonest vasculitis of childhood.1 Prompt recognition and diagnosis is imperative given the risk of serious cardiac complications without treatment. Case report A 6 year old female presented to A and E with 4 day history of generally feeling unwell, photophobia and neck stiffness. 4 days prior she had been discharged from A and E with suspected viral illness. Past medical history included failure to thrive. Examination noted the patient as irritable and photophobic with a blanching erythematous rash and neck stiffness. Blood tests on admission: CRP 172, WCC 14.1. During admission ophthalmology review was inconclusive due to difficult examination. She had a CT head and lumbar puncture which were normal. She was treated with intravenous ceftriaxone and aciclovir. By day 7 her lethargy had mildly improved but she continued to have ongoing temperatures and severe photophobia (unable to open her eyes). Her CRP remained elevated with low albumin and rising platelet counts. Her CSF cultures, viral PCR and viral throat swabs were all negative prompting further investigations. Echocardiogram performed at day 8 identified dilated coronaries with Z scores>4 for both right and left coronaries (no aneurysms/effusions). This was discussed with an infectious disease unit and a diagnosis of atypical KD agreed. She was commenced on high dose aspirin and intravenous immunoglobulin therapy after which her photophobia and fever gradually improved with coronary measurements improving over the next few months. Discussion KD typically presents with a persistent high grade fever plus a minimum of 4 of: bilateral conjunctivitis, mucous membrane changes, cervical lymphadenopathy, polymorphous rash and extremity desquamation. This patient presented with photophobia and neck stiffness with no evidence of lymphadenopathy or changes in extremity. Photophobia and neck stiffness can be unusual presentations of KD as seen in this case. Children with severe photophobia and high grade temperatures should therefore be assessed for Kawasaki’s disease if the neurological investigations are normal. References Eleftheriou D, Levin M, Shingadia D, Tulloh R, Klein N, Brogan P. Management of Kawasaki disease. Archives of Disease in Childhood [Internet]. 2013;99(1):74–83. Freeman A, Shulman S. Issues in the diagnosis of Kawasaki disease. Progress in Pediatric Cardiology [Internet]. 2004 [cited 17 September 2018];19(2):123–128.