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The blue rubber bleb nevus syndrome
The Journal of Otolaryngology
|October 1, 1981
Summary
Blue rubber bleb nevus syndrome (BRBNS), or Bean syndrome, involves skin and organ hemangiomas. This report details a rare supraglottic mass in a 16-year-old female, the first such otolaryngologic case.
Area of Science:
- Medicine
- Dermatology
- Otolaryngology
Background:
- Blue rubber bleb nevus syndrome (BRBNS), also known as Bean syndrome, is a rare congenital disorder.
- It is characterized by multiple, globe-shaped, cavernous venous malformations (hemangiomas) affecting the skin and internal organs, particularly the gastrointestinal tract.
Observation:
- A 16-year-old Caucasian female presented with a supraglottic mass.
- This mass was identified as a manifestation of BRBNS.
- The supraglottic location is an unusual presentation for this syndrome.
Findings:
- The case highlights the potential for BRBNS to manifest in the head and neck region.
- Surgical intervention was deemed appropriate for the supraglottic mass.
- This represents the first reported case of BRBNS presenting with a supraglottic mass in otolaryngologic literature.
Implications:
- This case expands the known spectrum of BRBNS manifestations.
- It underscores the importance of considering BRBNS in the differential diagnosis of supraglottic masses.
- Early diagnosis and surgical management can potentially prevent severe complications such as hemorrhage and airway obstruction.