Introduction
Kawasaki disease is an acute, self-limited systemic vasculitis that predominantly affects medium- and small-sized arteries in infants and young children. Although coronary artery aneurysms remain the hallmark vascular complication, extra-coronary arterial lesions occur in up to 2–5 % of severe casesReference McCrindle, Rowley and Newburger 1 and may produce long-term sequelae. Peripheral vascular sequelae are rarely reported but can cause lasting morbidity.Reference Chang, Lin, Peng and Tsai 2
We report an unusually severe infantile Kawasaki disease caseReference Bajolle, Jurzak, Cohen and Boudjemline 3 complicated by bilateral axillary aneurysm development, whose divergent evolution led to asymmetric limb perfusion: early right-sided occlusion causing limb hypoplasia, and late left-sided aneurysmal pulsatility resulting in unilateral digital clubbing over a 13-year follow-up.
Initial case presentation and management
The patient presented at 1.5 months of age (2011) with severe Kawasaki disease manifesting as prolonged fever, rash, and cheilitis. Laboratory studies showed inflammatory anaemia (haemoglobin 7 g/dL), thrombocytopenia, leukocytosis, and elevated C-reactive protein. Despite early administration of intravenous immunoglobulin (IVIG) on days 2 and 6 of fever, persistent fever, and inflammation prompted escalation of immunomodulatory therapy with corticosteroid pulses, exchange transfusion, and infliximab.
Vascular imaging revealed aneurysmal involvement of both coronary and peripheral arteries. Coronary aneurysms included 10-mm dilations of the proximal right coronary artery, circumflex artery, and proximal left anterior descending artery. Peripheral aneurysms were identified in the coeliac trunk, proximal renal arteries, superior mesenteric artery, and intercostal arteries. Notably, both axillary arteries exhibited large aneurysms measuring up to 20 mm (Supplementary Figure S1). On the right, local compression secondary to rupture of the aneurysmal sac led to trophic and sensory-motor deficits, prompting endovascular exclusion of the aneurysm with a covered stent. The left axillary aneurysm remained patent and pulsatile, with an intact vascular wall.
Short- and long-term vascular course
Despite resolution of inflammation and continuous anticoagulation (warfarin + aspirin), the patient developed a circumflex coronary occlusion with mild left-ventricular dysfunction (LVEF 50 %) during the first year.
Axillary aneurysm evolution was monitored annually using invasive angiography, MRI, or CT imaging. At one year, angiography demonstrated complete right-axillary stent occlusion with well-developed collateral circulation and no clinical signs of hypoperfusion. In contrast, the left axillary aneurysm remained unchanged. All other peripheral aneurysms had regressed. Over childhood, a mild but progressive right-arm length discrepancy emerged, reaching 2.5 cm at 12 years, with preserved grip strength and global motor function.
At age 12 (January 2024), the patient developed unilateral digital clubbing of the left hand (Figure 1). MRI angiography in 2019 and 2023 (Figure 2) showed stable right-sided occlusion with collaterals and gradual mural thrombosis of the left aneurysm while maintaining pulsatility (Figure 2).
Serial photographs of the patient’s hands at 12 years (January 2024, top) and 13 years (September 2025, bottom). The right hand shows stable hypoplasia, and the left shows persistent unilateral clubbing.
Serial magnetic resonance angiography (MRA) of the thorax at two time points (2019 and 2023). Coronal contrast-enhanced MRA views showing the bilateral axillary regions. Left panel (2019): Complete occlusion of the right axillary artery stent (*), with well-developed collateral circulation supplying the right upper limb. The left axillary aneurysm (**) remains patent with preserved lumen and thin mural thrombus. Right panel (2023): Stable appearance of the right-sided occlusion (*). The left axillary aneurysm (**) demonstrates progressive mural thrombosis with relative reduction in lumen size.
At 13 years (September 2025), clinical reevaluation confirmed stable right-sided hypoplasia and persistent left-hand clubbing, with preserved strength and perfusion. Linear measurements showed a consistent 5 % reduction in limb length on the right (e.g., hand 18 cm versus 19 cm on the left; shoulder-to-fingertip 71 versus 72 cm). Concomitantly performed nailfold capillaroscopy demonstrated normal capillary density (8–10 per field) with two focal rarefactions (digits 3D and 4D), no architectural disorganisation, megacapillaries, or haemorrhage—indicating absence of structural microangiopathy.
Discussion
This report presents an unusual case involving two distinct manifestations of chronic arterial hypoperfusion in severe infantile Kawasaki disease. While both slowed limb growth and digital clubbing are associated with perfusion anomalies, the mechanisms, timing, and severity of arterial obstruction led to divergent outcomes in this patient.
Hypoplasia of the right limb likely resulted from occlusion during the rapid post-natal growth phase. Similar aftereffects, especially those related to catheter use, have been reported following early-onset arterial thrombosis in neonates.Reference Rizzi, Kroiss, Kretschmar, Forster, Brotschi and Albisetti 4 Longitudinal bone development can be restricted without overt ischaemia by chronic yet compensated hypoperfusion.
Left-sided unilateral clubbing appeared much later, indicating a long-term low-grade vascular mechanism. Clubbing reflects increased capillary density and connective-tissue proliferation, mediated by VEGF and PDGF pathways.Reference Spicknall, Zirwas and English 5 Unilateral cases are linked to low-flow malformationsReference García-Souto, López-Gutiérrez, Narváez-Moreno, Fernández-Pineda and Bernabéu-Wittel 6 and chronic regional hypoxia.Reference Janaszak-Jasiecka, Siekierzycka, Płoska, Dobrucki and Kalinowski 7 In aneurysmal vessels, geometrical modification results in perturbed WindkesselReference Hussein, Brunozzi, Shakur, Ismail, Charbel and Alaraj 8 function, producing oscillatory distal perfusion which may modify regional signalling. Additionally, progressive mural thrombosis may create micro-embolic or low-shear stress conditions that activate local VEGF expression.Reference García-Souto, López-Gutiérrez, Narváez-Moreno, Fernández-Pineda and Bernabéu-Wittel 6
The preserved capillaroscopic architecture and density (observed in 2025) support the hypothesis of a localised haemodynamic mechanism rather than persistent systemic vasculitis. Paediatric studies report temporary or modest microvascular changes, typically during the acute or subacute stages, in 20–40 % of Kawasaki disease survivors.Reference Sedaghat, Raeeskarami and Tahghighi 9 , Reference Konda, Jindal, Nadig, Banday, Vinay and Singh 10
Early and sustained anticoagulation likely prevented acute ischaemic injury, yet this case underscores that chronic arterial remodelling may have long-term morphologic effects. This supports the need for thorough vascular screening beyond the coronary arteries in severe Kawasaki disease, particularly in high-risk groups such as infants < 6 months and those with severe vasculitis.Reference Chang, Lin, Peng and Tsai 2 Patient clustering studies suggest that some individuals may be genetically predisposed to severe vascular manifestations.Reference Wang, Shimizu and Bainto 11 Genetic variants in inflammatory-regulatory pathways (ITPKC, CASP3) may modulate susceptibility.Reference Onouchi 12
Conclusion
This 13-year follow-up documents the first reported combination of contralateral limb hypoplasia and unilateral digital clubbing, secondary to asymmetric axillary perfusion in Kawasaki disease. Serial imaging, morphometric data, and capillaroscopy confirm the persistence of asymmetry without diffuse microangiopathy. This example emphasises the necessity of systematic peripheral imaging and microvascular monitoring in cases of severe and atypical Kawasaki disease.
Supplementary material
The supplementary material for this article can be found at https://doi.org/10.1017/S1047951125111050.
Financial support
This research received no specific grant from any funding agency, commercial or not-for-profit sectors.
Competing interests
None.
Ethical standard
Written informed consent was obtained from the patient’s legal guardian for publication of this case report and accompanying images.
