Crouzon's Syndrome with Life-Threatening Ear Bleed: Ruptured Jugular Vein Diverticulum Treated by Endovascular...

Area of Science:

• Neurology
• Vascular Surgery
• Otolaryngology

Background:

• Crouzon's syndrome is a common syndromic craniosynostosis.
• Jugular venous diverticulum is a rare but serious complication.
• Previous literature has not described life-threatening ear bleeds from this condition.

Observation:

• A 24-year-old woman with Crouzon's syndrome presented with hearing loss and severe ear bleeding.
• Computed tomography revealed a jugular venous diverticulum adjacent to the external auditory canal.
• This finding suggests a link between the syndrome, diverticulum, and bleeding episodes.

Findings:

• Ruptured jugular venous diverticulum is a previously undescribed cause of severe otorrhagia in Crouzon's syndrome.
• Endovascular embolization successfully managed the bleeding, avoiding high-risk open surgery.
• This case highlights a new clinical presentation and treatment approach for syndromic craniosynostosis complications.

Implications:

• Endovascular treatment should be considered for jugular venous diverticulum in syndromic craniosynostosis.
• This approach may reduce surgical risks, improve outcomes, and prevent mortality.
• Further research into the pathophysiology and management of such vascular anomalies is warranted.