| Autor: |
Mat Johar, Fatimah, Wan Sulaiman, Wan Azman, Mat Saad, Arman Zaharil, Basiron, Normala, Sahid, Nik Amin |
| Zdroj: |
International Journal of Surgery Case Reports; 2020, Vol. 72, p202-206, 5p |
| Abstrakt: |
• BRBNS also known as Bean's Syndrome are atypical type of vascular malformation which uncommon. • The key features of this syndrome is characterized by multiple cutaneous, soft tissue and gastrointestinal tract venous malformation. • Current treatment modality for this rare disease is based on the severity of the organ involvement. It may involve minimaly invasive technique or extensive surgical resection. Blue Rubber Bleb Nevus Syndrome (BRBNS) also known as Bean's Syndrome is an atypical type of vascular malformation. To date, around 200 cases have been reported world-wide. In view of its low incidence rate, clinicians might misdiagnose and under treat. The key features of this syndrome are characterized by multiple cutaneous, soft tissue and gastrointestinal tract venous malformations. We report the first case of Blue Rubber Bleb Nevus Syndrome in Malaysia, a 23 years old Malay girl who suffers from multiple cutaneous venous malformation and gastrointestinal bleeding episodes. The typical morbidity for this syndrome is symptomatic anemia due to secondary iron deficiency due to the gastrointestinal venous malformation bleeding. In managing the gastrointestinal bleeding, it mainly depends on the severity of gastrointestinal bleeding, some may resolve spontaneously, while the others may be needing blood transfusion, and some may require GIT resections. As for cutaneous lesions, normally it is innocuous depending on the region and size. Large or problematic cutaneous venous malformation might benefit from sclerotherapy or excision. Multidisciplinary approach is crucial in managing BRBNS case due to its complexity and the spectrum of multiple organ involvement to ensure the best outcome to the patient. [ABSTRACT FROM AUTHOR] |
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