Kawasaki disease is a necrotizing vasculitis featuring fever, erythema, conjunctivitis, and lymphadenopathy. Ocular manifestations in Kawasaki disease are commonly limited to anterior segment characterized by bilateral bulbar conjunctivitis without exudate, superficial punctate keratitis, uveitis, or vitreous opacities.
We found 6 literatures[3–7] describing posterior segment lesions in Kawasaki disease. But none of them progressed to retinal detachment. Only one literature described a patient lost vision in 1 eye due to retinal and vitreous exudation. We report a unique case of incomplete Kawasaki disease (atypical) with severe enophthalmos, cataracts, exudative retinal detachment, and retinal vasculitis.
2 Report of a case
An 11-year-old Asian girl presented with a 10-day history of fever (up to 40.5 °C), generalized rash, erythema of oral mucosa, cervical lymphadenopathy, bilateral bulbar conjunctivitis without exudate, and cervical lymphadenopathy. Laboratory results showed elevated white blood cell count with neutrophil predominance C-reaction protein, erythrocyte sedimentation rate, plantlet count, serum liver enzymes, and decreased albumin. Patient did not have strawberry tongue, erythema, and edema of the hands and feet. Electrocardiogram showed no abnormal findings. Incomplete (atypical) Kawasaki disease was suspected (Table 1).[1,8–11]
Patient was immediately treated with intravenous immunoglobulin (IVIG) for 10 days in a community hospital. Unfortunately, she responded poorly to the treatment, the fever persisted despite the infusion of immunoglobulin. Ten days later, she was transferred to our university hospital. We added high-dose corticosteroids to her treatment regimen.
Two days after we started her on intravenous dexamethasone (30 mg/d for 4 days and 15 mg/d for 5 days), the patient was referred to the ophthalmology clinic presenting with ophthalmodynia, photophobia, and enophthalmos. Visual acuity was HM/5 cm OU. Ocular B-scan showed severe reduction of ocular volume and bilateral chorioretinal folds. Axial length was 15.6 mm OD and 16.1 mm OS (Fig. 1C and D). Intraocular pressure (IOP) was extremely low in OU (unmeasurable). Ophthalmology examination showed conjunctival edema and injection without exudate, corneal edema with folds (Fig. 1A and B), lens opacity, 3+ aqueous flare, and 1+ keratin precipitates.
Fundus photography suggested retinal detachment, choroidal detachment, and extensive chorioretinal folds (Fig. 1E and D). A fluorescein fundus angiography was obtained 2 weeks later when the ocular inflammation slightly resolved, which revealed hyperfluorescence of the disc and fluorescein leakage (Fig. 1I and J).
Tobramycin dexamethasone eye drops and atropine eye drops were given. The fever and rash started to resolved gradually 4 days after the administration of dexamethasone. She was discharged after 15 days of hospitalization. At follow-ups, retinas gradually flattened and axial length recovered to normal. At 2-month follow-up, visual acuity recovered to 20/50 OU. IOP was normal in OU. Fundus photography revealed normal retinas (Fig. 2G and H). Ophthalmology examination showed posterior subcapsular opacities and normal retinas (Fig. 2C and D). At 24-month follow-up, visual acuity recovered to 20/25 OU (Fig. 2).
To the best of our knowledge, this is the first documented case of incomplete Kawasaki disease with severe reduction of ocular volume, cataracts, retinal vasculitis, and retinal detachment. We report this case wishing to raise the awareness of posterior segment involvement, especially retinal lesions in Kawasaki disease.
Our patient showed 2 features. She is an older child (10 years old) who didn’t present with all the typical symptoms of Kawasaki disease. Despite the correct diagnosis and treatment, her fever was poorly controlled. She had resistance to intravenous immunoglobulin. The devastating enophthalmos, ocular hypotony, and retinal detachment could very likely be the result of the prolonged course of systemic inflammation.
We learned 3 lessons from our practice. The local use of dexamethasone in the eye was effective in our patient. Surgical intervention might not be necessary, given the ocular inflammation could resolve itself as the systemic condition improves. Unnecessary surgeries might cause further damage. Whether the patient needs a surgery depends largely on the evaluation and judgment of the ophthalmologist. Monitoring the eye and the fundus routinely, while treating Kawasaki disease systemically, benefits patients with retinal lesions, and potentially prevents the ocular inflammation progress to vision loss.
The authors thank Mr. Dan Meng and Mr. Yongzhi Huang from Imaging Department, Sichuan University West China Hospital for the excellent work on fundus photography and fluorescein fundus angiography.
Conceptualization: Yunxia Gao, Ming Zhang.
Data curation: Yunxia Gao, Yifan Zhang, Xiaoyue Wang, Ming Zhang.
Formal analysis: Yunxia Gao, Yifan Zhang, Fang Lu.
Investigation: Yunxia Gao, Fang Lu, Ming Zhang.
Methodology: Fang Lu.
Project administration: Yifan Zhang, Ming Zhang.
Resources: Yunxia Gao, Xiaoyue Wang, Ming Zhang.
Supervision: Yunxia Gao, Fang Lu, Ming Zhang.
Writing – original draft: Yifan Zhang.
Writing – review and editing: Yunxia Gao, Yifan Zhang, Ming Zhang.
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