Indirect bypass for revascularization in Hutchinson-Gilford progeria syndrome: an illustrative case.

Abstract

Purpose: Hutchinson-Gilford progeria syndrome (HGPS) is a rare genetic disorder characterized by premature aging. Cerebral hypoperfusion and stroke have been described in HGPS however revascularization techniques have not been reported from a neurosurgical perspective in HGPS patients.

Methods: We present a case of a pediatric patient with HGPS who underwent cerebral bypass along with a review of the literature.

Results: Cerebral hypoperfusion and resulting ischemic strokes have been reported in children with HGPS, as early as 4 years old. Medical treatment, including antiplatelet agents, following standard ischemic stroke guidelines has been commonly reported. There is a paucity of literature regarding surgical management of cerebral hypoperfusion in HGPS patients. As an illustrative case we report a 4-year-old boy with HGPS who presented with acute left ischemic stroke from ipsilateral internal carotid artery stenosis and arteriopathy. He underwent encephaloduroarteriosynangiosis for indirect cerebral artery bypass, with neoangiogenesis on follow up imaging and with good clinical status. Wound healing in this patient became a challenging complication, with multidisciplinary input required for successful resolution.

Conclusion: Children with HGPS can present with cerebral hypoperfusion due to cerebral flow deficits and indirect bypass can be cautiously considered as an option to restore cerebral blood flow. Neoangiogenesis and collateralization can occur in patients with HGPS following indirect bypass, though tissue fragility in this population makes them at high risk for wound healing issues. We discuss preoperative and perioperative considerations, as well as medical and surgical maneuvers undertaken to achieve successful healing.