Blue rubber bleb nevus syndrome  is a rare disorder that consists mainly of abnormal blood vessels affecting the skin or internal organs – usually the gastrointestinal tract. The disease is characterized by the presence of fluid-filled blisters (blebs) as visible, circumscribed, chronic lesions (nevi). BRBNS is caused by somatic mutations in the TEK (TIE2) gene. It was described by William Bennett Bean in 1958. Presentation
BRBNS is a venous malformation, formerly, though incorrectly, thought to be related to the hemangioma. It sometimes causes serious bleeding. Lesions are most commonly found on the skin and in the small intestine and distal large bowel. The lesions can also be found in the central nervous system, liver, and muscles. It usually presents soon after birth or during early infancy. Causes
The cause of blue rubber bleb nevus syndrome is currently unknown. The syndrome is considered sporadic. A patient who is diagnosed with BRBNS likely has a family history of other multifocal venous malformations which are a symptom of the disease. Autosomal inheritance of BRBNS has been found in familial cases associated with chromosome 9p, but the majority of cases are sporadic. The disease correlates with an onset of GI complications. It is reported that GI bleeding is the most common cause of death in most cases. Diagnosis
Blue rubber bleb nevus syndrome is difficult to diagnose because of how rare the disease is. Diagnosis is usually based on the presence of cutaneous lesions with or without gastrointestinal bleeding and/or involvement of other organs.
It is also often used in combination with ketamine with minimal side effects. Assisted death in Canada
A lethal dose of propofol is used for medical assistance in dying in Canada to quickly induce deep coma and death, but rocuronium is always given—even when patient dies as a result of propofol injection. Delivery order of IV medication is as follows:Step 1: Midazolam 10–20 mg given as 2−4ml of 5 mg/ml preparation for pre-anesthesia, induces sleep in 1−2 minutes. Step 2: Lidocaine 40 mg given as 4ml of 1% preparation to reduce possible burning in peripheral vein caused by propofol. Pause to allow effect. Step 3: Propofol 1000 mg given as 100ml of 10 mg/ml preparation. Loss of consciousness occurs within 10 seconds and coma occurs in 1–2 minutes. Death may result from the propofol but rocuronium is still given. Step 4: Rocuronium 200 mg given as 20ml of 10 mg/ml preparation to induce respiratory arrest. Cardiac arrest subsequently follows about 5 minutes later due to hypoxia. COVID-19
In March 2021, the U.S. Food and Drug Administration (FDA) issued an emergency use authorization (EUA) for Propofol‐Lipuro 1% to maintain sedation via continuous infusion in people older than sixteen with suspected or confirmed COVID-19 who require mechanical ventilation in an intensive care unit ICU setting.