Kawasaki disease (KD) is a type of acute systemic vasculitis that mainly affects infants. It is associated with various complications, some of which also involve the eye, such as uveitis. Herein, we report a rare case of KD-related retinal vasculitis. A 7-year-old boy with a 5 day history of fever was admitted to a general hospital. Despite immediate treatment with i.v. cefotaxime, the fever did not subside. Two days after admission, immunoglobulin (IVIG, 2 g/kg) was given, followed by prednisolone (2 mg/kg) due to suspected incomplete KD. The patient was transferred to the present hospital 5 days after admission to the previous hospital, however, due to heart failure and hypoxia caused by pleural effusion. Moreover, he presented with persistent high fever, hyperemia, trunk erythema, edema of the fingertips, elevated white blood cell count (15 700/μL) and C-reactive protein 18.38 mg/dL. Therefore, IVIG (2 g/kg) was readministered, but no response was noted. Alternatively, the i.v. cefotaxime and vancomycin initiated at admission to the present hospital was continued until blood and cerebrospinal fluid cultures were confirmed to be negative. The cause of the fever was still unclear; subsequently, an ophthalmologist was consulted despite the absence of visual symptoms. Fundus examination indicated bilateral retinal hemorrhage and vitreous haze, sheathing and exudates around the retinal vessels, vascular meandering, and vitiligo due to bilateral retinal vasculitis (Fig. 1a,b). Furthermore, visual acuity was impaired (logMAR 0.1 and 0.4 in the right and left eyes, respectively). Notably, examination of the whole blood for Epstein–Barr virus, cytomegalovirus and Herpes simplex virus DNA was negative. Finally, a clinical diagnosis of retinal vasculitis with KD was established. Based on these findings and to avoid the progression of visual impairment, the patient was treated with a single infusion of infliximab (5 mg/kg). Subsequently, visual acuity gradually improved to 0.8 and 1.2 in both eyes at days 21 and 42, respectively. Additionally, fundus examination conducted on day 42 of the follow-up period indicated complete resolution of retinal vasculitis (Fig. 1c,d). Compatible with the diagnosis of KD, desquamation was observed on the patient's fingertips. Sequential echocardiography showed no dilatation of the coronary arteries. He was discharged 34 days after admission to the present hospital. At 1 year follow up, visual acuity had been maintained at a normal value. In this study we report a case of KD complicated with retinal vasculitis that caused transient impairment of visual acuity. Anterior uveitis is a common ophthalmologic complication associated with KD,1 but the posterior involvement of KD such as retinal vasculitis is extremely rare. To the best of our knowledge, there are only three reports of long-term visual impairment due to KD: the first reporting a 9-year-old girl with bilateral blindness due to KD;2 the second reporting a 4-year-old male patient with unilateral vision loss due to retinal and vitreous exudation;3 and the third reporting a 4-year-old male patient with visual impairment due to retinal vasculitis.4 In most cases, including the present case, visual impairment was transient. Furthermore, i.v. infliximab effectively prevented the progression of visual impairment in the present patient. Agarwal et al. reported the use of infliximab 20 days after the onset of illness in a 4-year-old boy with KD complicated with retinal vasculitis and impaired visual acuity. In that case, however, visual acuity had not recovered at 1 year follow up.4 This suggests that the early use of infliximab is effective to prevent the progression of retinal vasculitis and further ischemic changes. Recently, Chen et al. reported that the retinal venules of patients with KD at least 2 years after acute illness were larger in size than those of control participants after adjusting for traditional cardiovascular risk factors.5 Furthermore, an increase in the size of retinal venules was observed in patients with coronary artery abnormalities compared with control patients. Retinal vasculitis appears to be a relatively common complication of KD, suggesting a tight correlation between the severity of KD and ophthalmological findings. Moreover, retinal vasculitis is caused not only by other systemic disorders, including vascular Behcet and sarcoidosis, but also by infectious diseases, such as tuberculosis. Although vasculitis of the retinal vessels is an extremely rare manifestation, especially in children, this differential diagnosis should be considered. In conclusion, although retinal vasculitis is rarely associated with KD, consulting an ophthalmologist is necessary. A severe manifestation of this complication may result in unfavorable ophthalmic sequela. The early use of infliximab may be important to prevent visual impairment. We thank the patient and his parents for their support. The authors declare no conflict of interest. E.S. prepared the manuscript. T.K., S.S., and M.H. treated the patient and revised the manuscript. Y.K. supervised the entire drafting of the manuscript. All authors have read and approved the final manuscript.
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Mar 9, 2022
Amanda Ricciuto + 4
Open Access
