Abstract
Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu syndrome, isa rare, autosomal-dominant, vascular dysplasia syndrome associated with genetic mutations in the binding protein of transforming growth factor ss.1Key clinical manifestations of this disease include vascular pathologies involving the skin, nose, brain, lung, and gastrointestinaltract.(2)Within the HHT population, the overall prevalence of brain arteriovenous malformations(AVMs) is approximately 10%, and there is a significant association between brain AVMs andHHT1mutations.(3)The rate of brain AVM hemorrhage among individuals with HHT is approximately half the observed rate of sporadic lesions or 1% per year.(4)Among pediatric patients with HHT, expressivity of cerebrovascular lesions is relatively high, with a prevalence of 25% atthetimeofdiagnosis.(5)A patient in her early childhood received a diagnosis of pericallosal arteriovenous fistula after presenting with facial venous ectasia and pulsatile jugular venous distension. The patient underwent complete endovascular obliteration of this lesion. Fourteen months later, a partial recurrence was found, as well as progression of a previously identified Spetzler-Martin grade II right frontal AVM. A discrete ipsilateral right frontal micro-AVM in the premotor area was also diagnosed. The patient underwent a right-sided 2-piece pterional-bifrontal craniotomy for sequential resection of the large right frontal AVM, the previously coiled reconstituted pericallosal arteriovenous fistula, and the right frontal micro-AVM. The postoperative angiography findings showed no residual cerebrovascular malformations. The patientrecovered well without neurological deficit. The patient's guardians provided written informedconsent for the procedure. The video is used with permission from Barrow Neurological Institute.