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Anesthetic Considerations in an Obstetric Patient with Klippel-Feil Syndrome
Abstract Number: FCA-193
Abstract Type: Case Report Case Series
Introduction: Klippel-Feil Syndrome (KFS) is a disorder characterized by the congenital fusion of cervical vertebrae. The most common signs are short neck, low posterior hairline and restricted mobility of the upper spine. Patients may also have CV, pulmonary and renal abnormalities with scoliosis and rib anomalies (1,2).
Case: A 27 year old G2P0010 presented at 25 weeks for anesthetic planning prior to delivery. She had a known history of KFS diagnosed at birth, cleft palate with subsequent repair, jaw surgery and low back pain. On physical exam her height was 147 cm and weight 56 kg (BMI of 25 kg/m2). Her airway exam was significant for a Mallampati Class IV airway with short thyromental distance and limited mouth opening. She reported a history of a failed oral intubation attempt that was successfully converted to a nasal intubation. Ultrasound examination of her lumbar spine did not predict difficulty with neuraxial placement. A plan of care was formulated at this visit to include labor epidural for vaginal delivery versus combined spinal epidural with reduced spinal local anesthetic for a cesarean delivery (CD). Awake fiberoptic intubation was discussed during this visit as patient history included a known difficult airway.
She presented to L&D at 37 weeks for induction of labor for gestational hypertension. A labor epidural placed in 2 attempts provided satisfactory analgesia. She progressed to complete dilation at zero station and pushed for 3 hours before arrest of descent necessitated CD. Her epidural was dosed with 2% Lidocaine and adequate surgical anesthesia was achieved. The CD was uncomplicated and patient tolerated delivery well. APGARS were 1 and 7 and neonatal weight was 3140 grams.
Discussion: Previous KFS case reports are limited and describe frequent inadequacy of neuraxial anesthesia requiring IV narcotic supplementation or GA (1,2). In this case neuraxial anesthesia was adequate for both labor analgesia and surgical anesthesia; likely due to the mild severity of scoliosis in our patient (2). Case reports have discussed airway topicalization prior to neuraxial placement when intubation may be required. When planning for delivery each potential strategy poses risks that must be balanced for individual patients. Ultrasound guidance can aid in assessing likelihood of successful neuraxial placement.
Klippel-Feil Syndrome Information Page. National Institute of Neurological Disorders and Stroke. https://www.ninds.nih.gov/Disorders/All-Disorders/Klippel-Feil-Syndrome-Information-Page. Published June 13, 2018. Accessed January 7, 2019.
Kavanagh T, Jee R, Kilpatrick N, Douglas J. Elective cesarean delivery in a parturient with Klippel–Feil syndrome. International Journal of Obstetric Anesthesia. 2013;22(4):343-348. doi:10.1016/j.ijoa.2013.06.005.