Carotid cavernous fistula in a young adult with Bannayan-Riley-Ruvalcaba syndrome

Authors

• Anirudh Mukhopadhyay, LSU Health Sciences Center - New OrleansFollow
• Cammille C. Go, LSU Health Sciences Center - New OrleansFollow
• Wayne J. Wortmann, LSU Health Sciences Center - New OrleansFollow
• Sara J. Coulon, Ochsner Medical Center, New Orleans, LA

Document Type

Article

Publication Date

5-11-2026

Publication Title

Orbit

Abstract

Direct carotid-cavernous fistulas (CCFs) are an uncommon but recognized vascular complication of inherited connective tissue disorders. We report a direct CCF in a 22-year-old patient with Bannayan-Riley-Ruvalcaba syndrome (BRRS), a rare disorder within the phosphatase and tensin homolog (PTEN) hamartoma tumor syndrome (PHTS) spectrum. The patient, diagnosed with BRRS at age two based on the classic triad of macrocephaly, abdominal lipomas, and genital lentiginous macules, presented with acute unilateral eyelid swelling and chemosis in the absence of trauma, visual deficits, or afferent pupillary abnormalities. Imaging revealed a direct CCF, which was successfully treated with endovascular embolization. This case expands the vascular phenotype of BRRS and highlights the importance of considering CCF in PTEN-related disorders, even in the absence of trauma.

Rights

© 2026 Taylor & Francis Group, LLC

Recommended Citation

Mukhopadhyay, Anirudh; Go, Cammille C.; Wortmann, Wayne J.; and Coulon, Sara J., "Carotid cavernous fistula in a young adult with Bannayan-Riley-Ruvalcaba syndrome" (2026). School of Medicine Faculty Publications. 4709.
https://digitalscholar.lsuhsc.edu/som_facpubs/4709
10.1080/01676830.2026.2660372