Indian J Pediatr DOI 10.1007/s12098-015-1763-x
SCIENTIFIC LETTER
Retinal Vasculitis in Kawasaki Disease Sikha Agarwal 1 & Samyak Mulkutkar 2 & Deepti Suri 1 & Surjit Singh 1 & Amod Gupta 2
Received: 18 December 2014 / Accepted: 2 April 2015 # Dr. K C Chaudhuri Foundation 2015
To the Editor: While anterior uveitis is a common ophthalmologic finding in Kawasaki Disease (KD) [1], retinal vasculitis is distinctly uncommon. We report a 4-y-old boy with KD who developed diminution of vision in the subacute phase of illness and was found to have retinal vasculitis. A 4-y-old boy presented with history of fever 1 ½ mo back. In the first week, he had developed maculo-papular rash, bilateral conjunctival congestion, red tongue and cracking of lips, with edema over dorsum of hands and feet. In the second week, parents noticed periungual peeling of the skin. Fever subsided in next 15 d but in third week of illness there was Fig. 1 Right (a) and left (b) fundus photos showing optic disc pallor and sclerosed retinal vessels
* Surjit Singh [email protected] 1
Department of Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh 160012, India
2
Department of Ophthalmology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
diminution of vision – gradual, painless and progressive. He was administered three pulses of intravenous methylprednisolone (30 mg/kg/d) for suspected retinal vasculitis in association with KD and referred to our institute. He had developed Beau’s lines by this time. Ophthalmological examination here showed a normal pupillary reaction. Visual acuity was finger counting at 1 m. The anterior chamber was normal and intraocular pressure was not elevated. Fundus examination showed bilateral pale optic discs and extensive sheathing and sclerosis of all retinal vessels, suggestive of healed retinal vasculitis (Fig. 1a and b). Investigations showed a normal C-reactive
Indian J Pediatr
protein (5 mg/L) and platelet counts (3.3×105/mm3) with elevated erythrocyte sedimentation rate (32 mm 1st h). Varicella-Zoster IgM, HIV ELISA, Widal, Weil-Felix, tuberculin test and anti-nuclear antibody test were non-contributory. Echocardiography revealed mild ectasia of the left main coronary artery (LMCA - 3.5 mm). A clinical diagnosis of KD with healed retinal vasculitis was made. The child was given intravenous infliximab (5 mg/kg) and tapering doses of oral prednisolone. He showed gradual recovery. At 9 mo of follow up he was noted to have vitreous hemorrhage necessitating treatment with intravitreal anti-vascular endothelial growth factor A (Bevacizumab). At one year follow-up he still has significantly impaired visual acuity (counting fingers at 2 m). A myriad of ophthalmologic manifestations have been reported in KD. These include anterior uveitis, disciform keratitis, chorioretinitis, and global inflammatory response [2]. Retinal vasculitis has only occasionally been reported. A delay in diagnosis can be vision threatening as seen in the index
case. Both pediatricians and ophthalmologists must be aware of these protean manifestations so that early diagnosis and treatment can be instituted and serious consequences prevented.
Conflict of Interest None. Source of Funding None.
References 1. 2.
Burns JC, Joffe L, Sargent RA, Glode MP. Anterior uveitis associated with Kawasaki syndrome. Pediatr Infect Dis. 1985;4:258–61. Grouteau E, Debuisson C, Brochard K, et al. Severe global inflammatory involvement of ocular segments and optic disc swelling in a 12-year-old girl with Kawasaki disease. Eur J Ophthalmol. 2011;21: 112–4.
SCIENTIFIC LETTER
Retinal Vasculitis in Kawasaki Disease Sikha Agarwal 1 & Samyak Mulkutkar 2 & Deepti Suri 1 & Surjit Singh 1 & Amod Gupta 2
Received: 18 December 2014 / Accepted: 2 April 2015 # Dr. K C Chaudhuri Foundation 2015
To the Editor: While anterior uveitis is a common ophthalmologic finding in Kawasaki Disease (KD) [1], retinal vasculitis is distinctly uncommon. We report a 4-y-old boy with KD who developed diminution of vision in the subacute phase of illness and was found to have retinal vasculitis. A 4-y-old boy presented with history of fever 1 ½ mo back. In the first week, he had developed maculo-papular rash, bilateral conjunctival congestion, red tongue and cracking of lips, with edema over dorsum of hands and feet. In the second week, parents noticed periungual peeling of the skin. Fever subsided in next 15 d but in third week of illness there was Fig. 1 Right (a) and left (b) fundus photos showing optic disc pallor and sclerosed retinal vessels
* Surjit Singh [email protected] 1
Department of Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh 160012, India
2
Department of Ophthalmology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
diminution of vision – gradual, painless and progressive. He was administered three pulses of intravenous methylprednisolone (30 mg/kg/d) for suspected retinal vasculitis in association with KD and referred to our institute. He had developed Beau’s lines by this time. Ophthalmological examination here showed a normal pupillary reaction. Visual acuity was finger counting at 1 m. The anterior chamber was normal and intraocular pressure was not elevated. Fundus examination showed bilateral pale optic discs and extensive sheathing and sclerosis of all retinal vessels, suggestive of healed retinal vasculitis (Fig. 1a and b). Investigations showed a normal C-reactive
Indian J Pediatr
protein (5 mg/L) and platelet counts (3.3×105/mm3) with elevated erythrocyte sedimentation rate (32 mm 1st h). Varicella-Zoster IgM, HIV ELISA, Widal, Weil-Felix, tuberculin test and anti-nuclear antibody test were non-contributory. Echocardiography revealed mild ectasia of the left main coronary artery (LMCA - 3.5 mm). A clinical diagnosis of KD with healed retinal vasculitis was made. The child was given intravenous infliximab (5 mg/kg) and tapering doses of oral prednisolone. He showed gradual recovery. At 9 mo of follow up he was noted to have vitreous hemorrhage necessitating treatment with intravitreal anti-vascular endothelial growth factor A (Bevacizumab). At one year follow-up he still has significantly impaired visual acuity (counting fingers at 2 m). A myriad of ophthalmologic manifestations have been reported in KD. These include anterior uveitis, disciform keratitis, chorioretinitis, and global inflammatory response [2]. Retinal vasculitis has only occasionally been reported. A delay in diagnosis can be vision threatening as seen in the index
case. Both pediatricians and ophthalmologists must be aware of these protean manifestations so that early diagnosis and treatment can be instituted and serious consequences prevented.
Conflict of Interest None. Source of Funding None.
References 1. 2.
Burns JC, Joffe L, Sargent RA, Glode MP. Anterior uveitis associated with Kawasaki syndrome. Pediatr Infect Dis. 1985;4:258–61. Grouteau E, Debuisson C, Brochard K, et al. Severe global inflammatory involvement of ocular segments and optic disc swelling in a 12-year-old girl with Kawasaki disease. Eur J Ophthalmol. 2011;21: 112–4.
