Giant tumefactive perivascular spaces that expanded and became symptomatic 14 years after initial surgery

Perivascular spaces (PVSs) or Virchow-Robin spaces in the brain are pial-lined interstitial fluid (ISF)-filled structures surrounding the penetrating arteries and arterioles. These spaces appear as 1- to 2-mm in diameter, round, oval, or curvilinear smooth-walled structures on magnetic resonance imaging (MRI). Typical PVSs are asymptomatic. Occasionally, they become enlarged and cause specific clinical manifestations that depend on location and the degree of tissue compression. In this case, they are referred to as giant tumefactive PVSs. To our knowledge, there have been no reported cases in which giant PVSs increased remarkably in number and size during both the natural course and postoperative course. We describe a rare progression of giant tumefactive PVSs 14 years after initial surgery.

On first admission at age 17, endoscopic ventriculocystostomy and third ventriculostomy were performed to relieve hydrocephalus caused by cysts compressing the cerebral aqueduct. Fourteen years later, the multicystic lesion reappeared with an increase in both cyst number and size. The patient showed no hydrocephalus but presented with oculomotor and trochlear nerve palsies, which were caused by a mass effect on the midbrain. Endoscopic ventriculocystostomy was performed and symptoms improved.

This is the first case report in which giant PVSs increased significantly in number and size.