Vogt Konayagi Harada syndrome in an 8-year-old patient

*Correspondence: [e-mail].

Abstract

Case Presentation: An 8-year-old male patient. He was born via cesarean section due to preeclampsia at 37 weeks without perinatal complications. At 3 months of age, he started experiencing focal epilepsy. He exhibited delayed neuropsychomotor development, sitting without support at 11 months, walking at 1 year and 6 months, and speaking simple phrases at 2 years. At the age of 5, he developed bilateral ocular hyperemia without a history of ocular trauma. Initially treated as conjunctivitis, with no improvement. After 6 months, glaucoma and uveitis were identified, leading to bilateral amaurosis. During the same period, the mother noticed the appearance of diffuse hypochromic patches, mainly on the upper and lower limbs. These patches were painless, non-pruritic, and showed no growth or worsening of appearance. The patient's mother also reported low auditory acuity and frequent headaches, with no other signs of meningism. Cranial magnetic resonance imaging revealed signs of bilateral hippocampal malrotation, as well as some FLAIR hyperintensity foci in the white matter of the cerebral hemispheres, which were nonspecific and could represent gliosis and/or chronic vascular changes.

Discussion: Vogt-Koyanagi-Harada syndrome is a rare, multisystemic inflammatory disease (representing 2.5% of uveitis cases in Brazil) with an autoimmune etiology targeting proteins of melanocyte membranes. It affects pigmentary structures such as the retina, meninges, inner ear, skin, and central nervous system. It predominantly affects women in their fourth decade of life (median age of 34 years) with dark or mixed skin. The syndrome is characterized by bilateral panuveitis, accompanied by neuroaditive findings that may be temporary, and dermatological manifestations occurring after the onset of ocular or neural involvement.

Final Comments: The occurrence of Vogt-Koyanagi-Harada syndrome is rare, especially in males and in childhood. In this case, the patient presents cerebral malformation with epilepsy, which are not well-described diagnostic criteria but may be related to the syndrome and its pathophysiology. Given the peculiarity and rarity of this case, we consider this report to be relevant.

Publication History

Article published online:
12 May 2025