Anesthetic management of a parturient with VACTERL association undergoing Cesarean delivery

Can J Anesth/J Can Anesth (2013) 60:570–576 DOI 10.1007/s12630-013-9919-5 CASE REPORTS / CASE SERIES Anesthetic management of a parturient with VACTERL association undergoing Cesarean delivery Prise en charge anesthésique d’une parturiente atteinte du syndrome de VACTERL subissant un accouchement par césarienne Gillian Hilton, MBChB • Frederick Mihm, MD • Alexander Butwick, MBBS Received: 14 January 2013 / Accepted: 13 March 2013 / Published online: 22 March 2013 Ó Canadian Anesthesiologists’ Society 2013 Abstract Purpose We present the anesthetic management of a parturient with VACTERL association undergoing combined regional and general anesthesia for Cesarean delivery. Defined as a syndrome, VACTERL association comprises at least three of the following abnormalities: vertebral, anal atresia, cardiac, tracheoesophageal, renal, and limb. Clinical features The patient’s anatomic abnormalities and comorbidities comprised severe cervicothoracic scoliosis, kyphoscoliosis, congenitally fused ribs, and severe restrictive lung disease. She had a Mallampati class 3 airway, a right laterally flexed neck, and reduced mandibular protrusion. We performed a lumbar spine ultrasound for epidural placement which was used to provide peri- and postoperative analgesia. Due to the anticipated difficult tracheal intubation, the patient underwent an awake fibreoptic intubation and subsequently received general anesthesia. The patient’s trachea was extubated on the first postoperative day, and she received adequate post-Cesarean epidural analgesia. Conclusion This case highlights the challenges that anesthesiologists face when managing parturients at extremely high risk for perioperative anesthetic morbidity Author contributions Gillian Hilton, Frederick Mihm, and Alexander Butwick contributed to the preparation of the manuscript. G. Hilton, MBChB (&)
F. Mihm, MD
A. Butwick, MBBS Department of Anesthesia, Stanford University School of Medicine, 300 Pasteur Drive, Rm H3580, Stanford, CA 94305, USA e-mail: 123 due to the presence of severe pre-existing disease, anticipated difficult airway, and major spinal abnormalities complicating neuraxial anesthesia. We used a combined general and epidural anesthetic approach to control ventilation, provide effective postoperative analgesia, and reduce the risk of anesthetic-related perioperative morbidity. An individualized approach should be considered for the anesthetic management of high-risk pregnant patients with complex and multiple medical and surgical morbidities undergoing labour and delivery. Résumé Objectif Nous pre´sentons la prise en charge anesthe´sique d’une parturiente souffrant du syndrome de VACTERL et subissant un accouchement par ce´sarienne sous anesthe´sie re´gionale et ge´ne´rale combine´e. Le syndrome de VACTERL, ou association VACTERL, comprend au moins trois des anomalies suivantes : anomalie verte´brale, imperforation de l’anus, anomalie cardiaque, trache´o-œsophagienne, re´nale, ou des membres. Éléments cliniques Les anomalies anatomiques et les comorbidite´s de la patiente comprenaient une grave scoliose cervico-thoracique, une cypho-scoliose, des côtes fusionne´es de façon conge´nitale, et une maladie pulmonaire restrictive grave. L’e´valuation de ses voies ae´riennes a re´ve´le´ un score de Mallampati de 3, son cou pre´sentait une flexion late´rale droite et elle pre´sentait une protrusion mandibulaire re´duite. Nous avons re´alise´ une e´chographie de la colonne lombaire pour positionner la pe´ridurale qui a e´te´ utilise´e pour l’analge´sie pe´ri- et postope´ratoire. En pre´vision d’une intubation trache´ale Parturient with VACTERL association Fig. 1 Photograph showing the patient with VACTERL association (vertebral defects, anal atresia, cardiac defects, tracheoesophageal abnormalities, renal abnormalities, and limb abnormalities) highlighting the gross anatomical abnormalities with emphasis on the head and neck to torso position difficile, la patiente a subi une intubation fibroscopique e´veille´e et l’anesthe´sie ge´ne´rale a e´te´ re´alise´e subse´quemment. La trache´e de la patiente a e´te´ extube´e le premier jour postope´ratoire, et elle a reçu une analge´sie pe´ridurale post-ce´sarienne adapte´e. Conclusion Ce cas souligne les de´fis auxquels font face les anesthe´siologistes lorsqu’ils prennent en charge des parturientes courant un risque extreˆmement e´leve´ de morbidite´ anesthe´sique pe´riope´ratoire en raison de la pre´sence de maladies graves pre´existantes, de voies ae´riennes anticipe´es comme difficiles, et d’anomalies majeures de la colonne, compliquant l’anesthe´sie neuraxiale. Nous avons utilise´ une approche anesthe´sique ge´ne´rale et pe´ridurale combine´e afin de contrôler la ventilation, de fournir une analge´sie postope´ratoire efficace, et de re´duire le risque de morbidite´ pe´riope´ratoire lie´e à l’anesthe´sie. Une approche personnalise´e doit eˆtre envisage´e pour la prise en charge anesthe´sique des patientes enceintes à risque e´leve´ pre´sentant des morbidite´s me´dico-chirurgicales à la fois complexes et nombreuses et devant accoucher. The acronym, VACTERL, is used to denote the following abnormalities associated with the disorder: vertebral defects, anal atresia, cardiac defects, tracheoesophageal abnormalities, renal abnormalities, and limb abnormalities. This multisystem disorder is rare, as the estimated incidence of VACTERL association varies from 1/10,000 - 1/40,000 571 live-born infants.1 The diagnosis of VACTERL association requires the presence of at least three of these abnormalities without other major congenital abnormalities.2 The exact cause of this disorder is unknown, as no clear genetic or environmental etiologic factors have been previously identified; thus, a high degree of clinical and causal heterogeneity is suspected.1 Patients with VACTERL association pose important challenges for anesthesiologists. Neuraxial block placement and/or airway management for endotracheal intubation may prove to be difficult in patients with major skeletal and/or spinal deformities. In addition, these patients may have severe restrictive lung disease secondary to scoliosis, which may negatively impact the adequacy of oxygenation and ventilation during neuraxial or general anesthesia. The physiological effects of pregnancy may increase the severity and complexity of these anatomic and physiologic comorbidities. We describe the successful anesthetic management of a parturient with VACTERL association who underwent a combined neuraxial and general anesthesia technique for a scheduled Cesarean delivery. The patient provided written informed consent for publication of this case report. Case report A 20-yr-old parturient G1P0 (height = 1.3 m; weight = 36 kg) with a singleton pregnancy at 39 weeks gestation was scheduled for primary Cesarean delivery. The patient had severe skeletal abnormalities related to VACTERL association, namely, cervical-thoracolumbar sco (...truncated)