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Kawasaki disease with profound systemic vascular involvements: An insightful pediatric case
Naila Nadeem1, Muhammad Nadeem Ahmad1, Muhammad Haseeb Malik2
1Department of Radiology The Aga Khan University Hospital Karachi Pakistan.
Insights
This case report details a rare instance of Kawasaki disease (KD) in an infant with extensive vascular involvement beyond coronary arteries. Early multidisciplinary management and treatment were crucial for patient stabilization and favorable outcomes.
Area of Science:
- Pediatric Cardiology
- Rheumatology
- Vascular Medicine
Background:
- Kawasaki disease (KD) is a multisystem inflammatory syndrome affecting small to medium vessels in young children.
- Coronary artery aneurysms (CAAs) are the most significant complication of KD.
- The spectrum of vascular involvement in KD can extend beyond coronary arteries.
Observation:
- A 2-month-old infant presented with an extremely rare and extensive vascular involvement in Kawasaki disease.
- Aneurysmal dilatations were observed in the internal carotid arteries, descending aorta, intercostal arteries, celiac artery, superior mesenteric artery, and renal arteries.
- The infant also developed pseudomonas sepsis, posing additional management challenges.
Findings:
- A multidisciplinary approach combined with early treatment using intravenous immunoglobulin (IVIG) and dexamethasone was effective.
- Despite severe coronary dilation and sepsis, the patient was stabilized and discharged after 11 days.
- This case highlights the importance of recognizing a broader spectrum of vascular involvement in KD.
Implications:
- Early recognition and prompt management are critical for favorable outcomes in complex Kawasaki disease cases.
- Multidisciplinary collaboration is essential for managing extensive vascular involvement and complications.
- Long-term follow-up, further research into screening tools, and management strategies for multi-organ involvement are necessary.
Key Clinical Message:
Kawasaki disease (KD), a self-limiting vasculitis, can present with a broader spectrum of vascular involvements, necessitating early recognition and prompt management. This case exemplifies the importance of involving multiple teams on board in managing complex presentations of KD. It also highlights the importance of close monitoring for the progression of the disease spectrum as well as family education to ensure favorable outcomes. The case also emphasizes the importance of long-term follow-up and further research to understand the prognosis, early screening tools, and possible complications due to multi-organ involvement in KD along with their management strategies.
Abstract:
Kawasaki disease (KD) is a multisystem vascular inflammatory syndrome, which predominantly affects the small and medium vessels in children within the age group of less than 5 years. The most threatened complication is the development of coronary artery aneurysms (CAAs). We present an extremely rare case of KD in a 2-month, 21-day-old male infant with extensive vascular involvement, expanding the disease spectrum beyond the involvement of coronary arteries. These included aneurysmal dilatations of both internal carotid arteries, the descending aorta, bilateral multilevel intercostal arteries, coeliac artery, superior mesenteric artery, and both renal arteries. Implementing a multidisciplinary approach with early treatment via intravenous immunoglobulin (IVIG) and dexamethasone proved to be most effective in the patient's management. Despite unique challenges such as severe coronary dilation and pseudomonas sepsis during the special care, the patient was stabilized and discharged after 11 days of hospital stay, highlighting the importance of early prompt management and a centered approach to evaluate in a broader spectrum. This case emphasizes the importance of long-term follow-up and further research to understand the prognosis, early screening tools, and possible complications due to multi-organ involvement in KD along with their management strategies.
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