eMedicine Specialties > Dermatology > Diseases of the Vessels
Blue Rubber Bleb Nevus Syndrome: Treatment & Medication
Updated: Oct 23, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
No systemic therapy is currently considered a standard of care for blue rubber bleb nevus syndrome (BRBNS). One report indicated the successful use of interferon-beta to treat the manifestations of disseminated intravascular coagulation in one patient with disseminated skin and GI venous malformations.12
Surgical Care
- Cutaneous lesions
- Cutaneous lesions do not need to be treated, unless they are cosmetically unacceptable or functionally troublesome. Malignant transformation of BRBNS lesions has not been reported.
- Methods to remove lesions include electrodesiccation and curettage, liquid nitrogen, and excision. These modalities often lead to recurrence or healing with a hypertrophic scar. Successful treatment without scarring has been reported with the use of the carbon dioxide laser. Treatment of 225 lesions without recurrence was reported with the use of the carbon dioxide laser.
- The Medscape Dermatologic Surgery Resource Center may be of interest.
- Extracutaneous lesions
- Bleeding from GI lesions usually is managed conservatively with iron supplementation and blood transfusions when necessary. Endoscopic coagulation or removal is an effective modality for repeated bleeding. Experience with endoscopic sclerotherapy suggests that it is ineffective and complicated by the development of ulcerations and strictures. Endoscopic laser (Nd:YAG) photocoagulation has been used successfully for lesions in the alimentary tract.13 When traditional methods fail and the vascular lesions are confined to a segment of the GI tract, resection of the involved segment of gut may be indicated. This approach should be used with caution because recurrences may occur after excision. Diffuse and scattered lesions or those where operation is not feasible should be treated conservatively.
- Orthopedic lesions are managed with orthotics and supportive measures, unless impaired function necessitates surgery.
- An 8-month old infant with bleeding hemangiomas of the rectum and colon was successfully treated with argon plasma coagulation. This treatment resulted in long-term success.14
- In one report, an aggressive surgical approach to treatment was attempted in 10 patients. GI venous malformations were identified endoscopically and then treated by wedge resection, polypectomy, suture ligation, segmental bowel resection, or band ligation. Only one patient developed recurrent GI bleeding.15
Consultations
- Patients should be referred to a gastroenterologist for management of GI manifestations as needed.
- Referral to an orthopedist can assist in the care of patients with bone or joint involvement.
- Symptomatic patients should be referred to the appropriate specialist depending on the organ system involved.
- In cases with familial occurrence, patients should be referred for genetic counseling.
More on Blue Rubber Bleb Nevus Syndrome |
| Overview: Blue Rubber Bleb Nevus Syndrome |
| Differential Diagnoses & Workup: Blue Rubber Bleb Nevus Syndrome |
Treatment & Medication: Blue Rubber Bleb Nevus Syndrome |
| Follow-up: Blue Rubber Bleb Nevus Syndrome |
| Multimedia: Blue Rubber Bleb Nevus Syndrome |
| References |
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References
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Further Reading
Keywords
blue rubber bleb nevus syndrome, BRBNS, Bean syndrome
Treatment & Medication: Blue Rubber Bleb Nevus Syndrome