Sir,

We illustrate the first reported case of hypertensive choroidopathy and retinopathy, including Elschnig spots, in a young, visually asymptomatic child with malignant hypertension.

Case description

A 3½ year old boy presented with cough, vomiting, lethargy, and malignant hypertension (245/169 mm Hg). Investigations revealed renal failure (creatinine 231 μmol/l; reference age range, 17–36 μmol/l), microangiopathic haemolytic anaemia, and thrombocytopenia. Examination revealed normal anterior segments, bilateral swollen optic discs, and subretinal exudation (Figures 1a and b), and multiple discrete pigmented lesions surrounded by a pale halo (Elschnig spots; Figure 1e). There was a right subfoveal scar and a left macular star with massive exudation.

Figure 1
figure 1

Photographs of right (a) and left (b) fundi at 3 weeks after presentation, showing bilateral optic disc oedema, subretinal exudation, right subfoveal scarring, left macular star exudate configuration, and Elschnig spots. Photographs of right (c) and left (d) fundi at 4 months after presentation and treatment, showing resolution of disc oedema, resorption of exudate, as well as residual Elschnig spots and peripapillary pigmentation. Elschnig spots in the mid-periphery, seen at 3 weeks (e) and 4 months (f) after presentation.

Full size image

After 3 weeks of treatment, his blood pressure was 120 mm Hg systolic. Visual acuity was 20/80 OU (Kay's pictures) with existing +3DS glasses (cycloplegic refraction, OD +5.50/−0.50 × 15, OS +5.50/−0.50 × 160). At 3 months later, creatinine was 50 μmol/l, spectacle-corrected acuity 20/25 OU and stereoacuity 170″. Optic disc oedema and subretinal exudate had mostly resolved. There was left residual peripapillary pigmentation and a contracted right subfoveal scar (Figures 1c and d). Elschnig spots appeared unchanged (Figure 1f). Despite extensive investigation, the cause of his hypertension remains unknown. Possible aetiologies include atypical haemolytic uraemic syndrome, or renal scarring from an episode of transient neonatal renal failure, which had resolved spontaneously.

Comment

Hypertension in young children is rare, and usually secondary to an identifiable disorder.1 Childhood hypertensive chorioretinal changes are even rarer. Reviewing 83 hypertensive children, Foster et al2 found only three with ophthalmoscopic abnormalities; these were mild, and they questioned the need for screening ophthalmoscopy in hypertensive children. However, Krause et al3 found swollen optic discs and hypertensive retinopathy in 5.7% of paediatric kidney transplant recipients.

Optic disc oedema and macular star exudate configurations are seen in neuroretinitis as well as malignant hypertension. Neuroretinitis though is usually unilateral, and is not associated with extremely elevated blood pressure. These fundus findings have been reported in children with severe hypertension secondary to underlying renal malformations4 and pheochromocytoma.5

Hypertensive choroidopathy is associated with acute severe hypertension (eg, pre-eclampsia and pheochromocytoma). Elschnig spots represent necrosis of choroidal arterioles and capillaries secondary to these acute rises in blood pressure, and were noted in rhesus monkeys within 24 h of the induction of malignant hypertension.6 To our knowledge, they have never been reported in a child of this age.

We describe a unique case of severe hypertensive chorioretinopathy in a young child. Screening hypertensive children involves close collaboration between paediatric nephrologists and ophthalmologists, because many of these children have underlying renal disorders.