Blue Rubber Bleb Nevus Syndrome Treatment & Management

  • Author: Basil S Cherpelis, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Apr 9, 2010
 

Medical Care

No systemic therapy is currently considered a standard of care for blue rubber bleb nevus syndrome (BRBNS). One report of blue rubber bleb nevus syndrome 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] Another report describes long-term subcutaneous octreotide therapy as successful as decreasing GI bleeding in blue rubber bleb nevus syndrome.[13]

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Surgical Care

Surgical treatment of cutaneous lesions in blue rubber bleb nevus syndrome (BRBNS) is as follows:

  • Cutaneous lesions do not need to be treated, unless they are cosmetically unacceptable or functionally troublesome. Malignant transformation of blue rubber bleb nevus syndrome 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.

Surgical treatment of extracutaneous lesions in blue rubber bleb nevus syndrome is as follows:

  • 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.[14] 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.[15]
  • In one report, an aggressive surgical approach to the treatment of blue rubber bleb nevus syndrome 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.[16]
  • Multiple hemangiomas of the terminal ileum and colon were treated endoscopically in an adult female with the argon plasma coagulator.[17]
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Consultations

  • Patients with blue rubber bleb nevus syndrome (BRBNS) 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.
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Contributor Information and Disclosures
Author

Basil S Cherpelis, MD  Assistant Professor, Chief of Dermatologic Surgery, Department of Dermatology and Cutaneous Surgery, Assistant Professor, Department of Oncologic Sciences, University of South Florida; Consulting Staff, Moffitt Cancer Center, Tampa General Hospital and James A Haley Veterans Affairs Medical Center

Basil S Cherpelis, MD is a member of the following medical societies: American Academy of Dermatology, American College of Mohs Micrographic Surgery and Cutaneous Oncology, American Society for Dermatologic Surgery, and Association of Professors of Dermatology

Disclosure: Nothing to disclose.

Coauthor(s)

Neil Alan Fenske, MD  Chairman, Department of Dermatology and Cutaneous Surgery, Professor, Department of Dermatology and Cutaneous Surgery, Department of Pathology and Cell Biology, Department of Oncologic Sciences, University of South Florida College of Medicine

Disclosure: Dermik Honoraria Speaking and teaching; Amgen Honoraria Speaking and teaching; Graceway Pharmaceuticals Honoraria Speaking and teaching; Abbott Honoraria Speaking and teaching

Specialty Editor Board

Bernice R Krafchik, MBChB, FRCPC  Professor Emeritus, Department of Pediatrics, Section of Dermatology, University of Toronto

Bernice R Krafchik, MBChB, FRCPC is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, Canadian Medical Association, College of Physicians and Surgeons of Ontario, Royal College of Physicians and Surgeons of Canada, and Society for Pediatric Dermatology

Disclosure: Nothing to disclose.

Richard P Vinson, MD  Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Van Perry, MD  Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas Health Science Center

Van Perry, MD is a member of the following medical societies: American Academy of Dermatology and American Society for Laser Medicine and Surgery

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References

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