To the Editor: Retinopathy of prematurity (ROP) causes visual morbidity in preterm neonates [1]. Moyamoya disease is a cerebrovascular etiology of stroke in children [2]. Here, a preterm infant who had severe ROP was subsequently diagnosed with Moyamoya disease during early infancy. This preterm (31+2 wk, 1130 g) male infant was vaginally delivered with normal APGAR. Baby had respiratory distress, seizures, and thrombocytopenia and required mechanical ventilation, antibiotics, anti-seizure medication, multiple PRBC, and platelet transfusion. The baby had CSF-culture-positive meningitis and Grade I-GMH. The respiratory support was gradually weaned off to room air by 45 d of age. Aggressive-ROP was found in both eyes and treated with intraocular anti-vascular endothelial growth factor at 26 d of age; subsequently required three times laser photocoagulation (60 d, 73 d and 80 d of age) and pars plana vitrectomy surgery (101 d of age).

After 2 mo, the child was readmitted for tonic-clonic seizures and limited movement in the left upper and lower limbs. Sepsis evaluation and serum electrolytes were normal. In MRI, diffusion restriction of the right middle cerebral artery (MCA) territory in DW and hyperintense on T2 and FLAIR sequences; and diffuse narrow calibre of the circle of Willis with thrombus of the right supraclinoid and cavernous segment of the internal carotid artery and proximal segment of the right MCA in angiogram were found. The baby was treated with anti-seizure medication and discharged on oral aspirin prophylaxis.

ROP is a disease caused by dysregulated angiogenesis and retinal neovascularization [1]. Surprisingly, despite early screening and aggressive interventions, ROP progressed to end-stage in this case. The steno-occlusive changes in the circle of Willis are characteristic of Moyamoya disease, which are rarely reported in early infancy [2, 3]. This case report, for the first time, depicts the possible association of severe ROP with Moyamoya disease, which could be explained by two disease spectrums of vascular etiology.