BRBN syndrome is generally present from birth or childhood. the evolution may be complicated by gastrointestinal bleeding .
Blue rubber bleb nevus syndrome is a rare disorder that consists mainly of abnormal blood vessels affecting the gastrointestinal tract. It is even a rare cause of gastrointestinal hemorrhage during childhood. Blue rubber bleb nevus usually cause massive or occult gastrointestinal hemorrhage and iron deficiency anemia secondary to the bleeding episodes. It is even a rare cause of gastrointestinal hemorrhage during childhood. BRBNS is an important syndrome because of its potential for serious or fatal bleeding.
The bleeding is usually occult and chronic or may be acute in the form of hematemesis or melena. The management of the gastrointestinal lesions depends on the extent of involvement and severity of gastrointestinal bleeding. A conservative approach usually is recommended whenever the clinical features and bleeding episodes are mild. Blebs are nevi that measure more than 5 mm around. Composed of skin and large dilated blood vessels, the nevi do not disappear and are found on internal organs such as the stomach, liver, spleen, heart, bone, muscle, bladder, and vulva.
They are easily compressible and refill after compression. Occasionally, the nevi are painful. Ranging in size from millimeters to several centimeters, the nevi can number from a few to hundreds. Treatment of BRBNS will rely upon the severity, number, size, and location of the nevi. Skin lesions that are life-threatening can be safely removed by surgery, or laser therapy. Surgery can remove single lesions; however, the number may be too great to excise them all. Treatment methods that are less invasive than surgery use endoscopy to tie off bleeding nevi. Seizures can usually be controlled by medications. Physical therapy may improve paralysis.
