Abstract Background Hutchinson–Gilford progeria syndrome (HGPS) is an exceedingly rare genetic disorder characterized by accelerated aging and profound vascular fragility. While cerebrovascular accidents are common in this population, traumatic epidural hematomas (EDH) are clinical rarities, with only two pediatric cases previously documented. Currently, it is estimated that fewer than 200 children with HGPS are living worldwide. The primary objective of this report is to present a comprehensive multidisciplinary management strategy for traumatic EDH in a 15-year-old HGPS patient, highlighting the critical interplay between progerin-induced vascular stiffening, reduced intracranial compliance, and the formidable anesthetic challenges encountered in the oldest reported survivor of this pathology. Case presentation A 15-year-old female with HGPS presented with a right frontoparietal EDH following a ground-level fall. Initial Glasgow Coma Scale (GCS) was 14 but declined to 12 within 4 h. Cranial computed tomography (CT) revealed a large EDH measuring 22 mm at maximum thickness, causing a 6 mm midline shift. Due to severe glottic sclerosis and anatomical distortion, multiple orotracheal and fiberoptic (2.8 mm) intubation attempts failed. An emergency percutaneous tracheostomy was performed to secure the airway. Intraoperative findings revealed active bleeding from a coronal suture diastasis, which was managed with bone wax and dural tenting sutures. The patient achieved full neurological recovery and was discharged on postoperative day 10. Conclusion Managing HGPS patients requires a high index of clinical suspicion for intracranial injury even after minor trauma, as progerin-induced vasculopathy accelerates hematoma expansion. Standard airway techniques can fail due to progressive laryngeal changes, necessitating early consideration of surgical airways. Prompt multidisciplinary intervention and meticulous neurosurgical technique are essential to mitigate risks associated with extreme vascular fragility and complex anatomy in this high-risk population.
Ozmarasali et al. (Fri,) studied this question.