Anesthetic Implications of an Obstetric Patient with Blue Rubber Bleb Nevus Syndrome

Abstract

Blue rubber bleb nevus syndrome, a syndrome of multifocal venous malformations, has been reported rarely during pregnancy. This syndrome has been associated with airway lesions in some patients and neuraxial abnormalities in other patients. We report the anesthetic and obstetric management of a patient with an extensive distribution of both airway and neuraxial lesions.

A multidisciplinary approach to caring for a pregnant patient with blue rubber bleb nevus syndrome: A case report

“…Literature on the management of BRBNS in pregnancy is rare. Documented complications of BRBNS in pregnant patients have included involvement of the vaginal vault prohibiting a vaginal delivery [ 5 ], airway and neuraxial compromise with contraindications to epidural anesthesia [ 6 ], pulmonary embolism [ 7 ], severe GI bleeding requiring laparotomy and small bowel resection [ 8 ], acute intestinal intussusception complicated by postoperative disseminated intravascular coagulation [ 9 ], anterior abdominal wall involvement resulting in hemorrhage during cesarean delivery [ 17 ], and hemorrhage and consumptive coagulopathy of the affected neonate [ 10 ]. Because of the breadth of potential complications associated with BRBNS, a multidisciplinary approach to prenatal and intrapartum care is necessary.…”

“…While neurological care was not necessary for this patient, Galey et al consulted neurosurgery for further evaluation of a patient who had spinal involvement from BRBNS. Neurological complications may be seen in patients when BRBNS manifests neuraxially and in the central nervous system, necessitating consultation with a neurosurgeon [ 6 ]. Social services should be involved as needed to assist with any potential social barriers to care.…”

“…While the exact etiology of BRBNS is not clear, most cases occur sporadically, while some familial cases with autosomal dominant inheritance have been noted [ [2] , [3] , [4] ]. Approximately 200 cases of BRBNS have been published in the literature with a very small subset documented among pregnant women [ [5] , [6] , [7] , [8] , [9] , [10] , [11] , [12] , [13] , [14] , [15] , [16] ]. Reports of BRBNS in pregnant women have described the presence of VMs in several locations, such as the GI tract, central nervous system, airway, spinal epidural space, vaginal vault, and anterior abdominal wall, creating obstetrical concerns that require consultation with physicians of numerous specialties.…”

Anesthetic considerations for blue rubber bleb nevus syndrome: a case report

“…Surgical resection of the gastrointestinal tract is the choice of treatment for bleeding from venous malformations [2], and it requires general anesthesia. In general, anesthetic management in patients with BRBNS is challenging because of vascular malformations, especially in the airway and spinal canal [3][4][5]. Here, we report the case of a patient with BRBNS who underwent open abdominal surgery with successful general and epidural anesthesia.…”

“…With regard to airway evaluation, focused history taking and physical examination are essential, and use of a fiber optic bronchoscope is reasonable. Oral intubation is feasible, and video laryngoscope is useful for safe intubation [4,5,7]. Epidural anesthesia can be safely managed by paying attention to the possibilities of spinal canal lesions.…”