Atypical Moyamoya Disease Associated with Midaortic Syndrome

We report a case of a 4-year-old boy who presented with moyamoya disease associated with midaortic syndrome. He had been treated for severe persistent hypertension until he suffered multiple episodes of seizure and cerebral ischemic attack. Cerebral angiography showed bilateral terminal internal carotid artery stenosis. Angiographic survey showed severe stenosis of abdominal aorta and bilateral proximal renal arteries. This is a very rare report of moyamoya disease with midaortic syndrome.

Fetal congenital midaortic syndrome with unilateral renal artery stenosis prenatally presenting with polyhydramnios and postpartum as hyponatremic hypertensive syndrome

The midaortic syndrome (MAS) is a rare anomaly, characterised by narrowing of the distal aorta and its major branches. The most common symptom is severe arterial hypertension. The combination of hyponatremia, polyuria and renovascular hypertension caused by a unilateral renal artery stenosis is described as hyponatremic hypertensive syndrome. We report a case of MAS with unilateral renal artery stenosis in a preterm female neonate. A pregnant woman at 34 weeks of gestation was referred with fast growing abdominal circumference and pain. The ultrasound revealed severe polyhydramnios and fetal myocardial hypertrophy. Within the first 48 hours of the neonatal period, the diagnosis of MAS was made. We conclude that symptomatic MAS, caused by unilateral renal artery stenosis, resulting in increased renin–angiotensin–aldosterone system activity and subsequent polyuria of the non-stenotic kidney, lead to clinically significant polyhydramnios.