Background: Hirayama Disease (HD) is a rare
disorder consisting of insidious onset of unilateral weakness and atrophy of
the forearm and intrinsic hand muscles. Vein of Galen aneurysmal
malformations (VGAMs) are rare congenital cerebral vascular malformations,
consisting of high-flow arteriovenous shunting between a persistent median
prosencephalic vein and arterial feeders. Methods: 14
years old boy known for VGAM presented with left-sided HD. His cervical MRI
revealed enlarged epidural with anterior, left-ward displacement of the
posterior dura and spinal cord. He underwent surgical treatment by
laminotomies, along with tenting of an autologous duroplasty to the
overlying laminae. Results: We decided to combine
epidural venous plexus coagulation with posterior duraplasty and dural
fixation using tenting suture which led to a favorable clinical outcome has
not been previously proposed in the literature.
We hypothesize that in this context, an abnormal vasculature could also
predispose to posterior epidural venous plexus engorgement, anterior dural
displacement in cervical flexion, and microvascular changes in the anterior
spinal arterial circulation, leading to the progressive anterior horn cell
ischemia that lead to the clinical phenotype of HD.
Conclusions: The association between HD and VGAM
in this patient may provide clues with regard to the pathophysiology of
HD.
Cervical laminectomy and micro resection of the posterior venous plexus in Hirayama disease