Ramos Juan A, Shettar Shashank S, James Christopher F
Department of Anesthesiology, University of Florida Health, Gainesville, Florida, EUA.
Department of Anesthesiology, University of Florida Health-Jacksonville, Jacksonville, Florida, EUA.
Braz J Anesthesiol. 2018 Mar-Apr;68(2):205-208. doi: 10.1016/j.bjan.2016.05.002. Epub 2017 Jan 9.
The term VACTERL is an acronym for an association of congenital malformations: including vertebral, anal, cardiac, tracheo-esophageal, renal and limb anomalies. VACTERL anomalies pose a formidable challenge to anesthesiologists. We describe the anesthetic management of a parturient with VACTERL association, who underwent neuraxial analgesia for labor and vaginal delivery.
A 23 year old primigravida at 39 weeks gestation presented in labor at 4 cm cervical dilatation, completely effaced, requesting labor analgesia. Past medical history included VACTERL association with an imperforate anus and a partial endocardial cushion defect, both repaired in early childhood. She also had significant dorso-lumbar scoliosis with an extra lumbar vertebra. An MRI performed at 14 years age revealed the above findings with no spinal cord abnormalities. With a normal neurologic exam, a combined spinal epidural technique was performed. Despite significant scoliosis, the epidural space was identified at approximately the L3–L4 interspace at a depth of 5 cm. Spinal Fentanyl 25 mcg was administered followed by continuous patient-controlled epidural analgesia. The patient experienced excellent pain relief throughout her labor, and had an uneventful vaginal delivery 5 h after epidural placement.
The rarity of VACTERL association in the obstetric population with its extensive anomalies mandates a multidisciplinary approach in the prenatal period as it can pose major challenges to all health care providers, including airway, ventilatory, cardiac and neuraxial problems. This is the first reported case of a successful and safe neuraxial technique in a laboring patient with the VACTERL association with albeit limited vertebral and spinal cord anomalies.
术语VACTERL是一种先天性畸形关联的首字母缩写词,包括脊柱、肛门、心脏、气管食管、肾脏和肢体异常。VACTERL异常给麻醉医生带来了巨大挑战。我们描述了一位患有VACTERL关联的产妇的麻醉管理情况,该产妇接受了分娩和阴道分娩的椎管内镇痛。
一名23岁初产妇,孕39周,宫颈扩张4cm,宫颈完全消失,前来要求分娩镇痛。既往病史包括VACTERL关联,伴有肛门闭锁和部分心内膜垫缺损,均在幼儿期修复。她还患有严重的背腰脊柱侧弯,有一个额外的腰椎椎体。14岁时进行的MRI检查显示了上述发现,无脊髓异常。神经系统检查正常,采用联合脊髓硬膜外技术。尽管存在严重的脊柱侧弯,但在深度5cm处大约L3-L4间隙处确定了硬膜外腔。给予脊髓芬太尼25μg,随后进行持续的患者自控硬膜外镇痛。患者在整个分娩过程中疼痛缓解良好,硬膜外置管5小时后顺利阴道分娩。
产科人群中VACTERL关联的罕见性及其广泛的异常情况要求在产前采取多学科方法,因为它可能给所有医疗保健提供者带来重大挑战,包括气道、通气、心脏和椎管内问题。这是第一例报道的在患有VACTERL关联且脊柱和脊髓异常有限的分娩患者中成功且安全的椎管内技术病例。