Paediatric Intracranial Dolichoectasia — A Rare Case

Male, 6

Intracranial Arterial Dolichoectasia

Headache, convulsions, "hot head", visual disturbances.

Anterior circulation arteries appear elongated & widened — Rt ICA diameter 3.8 mm, Lt ICA 3.9 mm, Rt M1 3.8 mm, Lt M1 3.8 mm, Rt A1 3.3 mm, Lt A1 3.3 mm. Tortuous branches are noted at M2 segment of right MCA. Focal widening is noted in left Sylvian fissure around origin of M2 segment of left MCA (encephalomalacia). Basilar artery measures 4.7 mm, which is normal.

IADE is defined as abnormal dilation & elongation of intracranial arteries. It may represent the end-stage condition of arterial damage due to various pathologies. By itself a rare occurrence, IADE has been mostly (80%) reported involving the vertebrobasilar system. Involvement of the anterior circulation is uncommon, and IADE at paediatric age is a very rare & unique presentation, with <10 cases reported worldwide so far.

Subtle change in vessel diameters may often be overlooked.