PRESENTATION OF CASE: 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.

DISCUSSION: 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.

METHODS: This retrospective study reviewed records of children operated from January 2001 to July 2019. Data regarding concurrent congenital anomalies, complications, type of surgery, etc., were collected from outpatient records, operative notes, and clinical photographs.

RESULTS: Twenty-eight children were treated, and 33 clefts (5 bilateral) were repaired. The male-to-female ratio was 1.3:1 (16 males and 12 females). Twenty-three patients had unilateral clefts (82.14%), with 14 right (60.86%) and 8 left (34.78%). Bilateral clefts were less common (17.86%). Twenty-three patients (82.14%) were Malay, 3 (10.71%) Chinese, 1 (3.57%) Indian, and 1 (3.57%) Cambodian. Eleven medical records were untraceable (discontinued due to duration of inactivity). There were 10 straight-line repairs, 5 Z-plasties, and 1 W-plasty performed; 3 cases did not detail the type of repair. One child required scar revision, and 1 had hypertrophic scarring requiring corticosteroid injection-no disturbances in speech or oral incompetency while eating were reported. Duration of follow-up ranged from 3 to 14 years.