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Anesthetic Considerations in a nulliparous patient with Klippel-Feil Syndrome
Abstract Number: RF7B10-251
Abstract Type: Case Report Case Series
Klippel-Feil syndrome describes a triad of low hairline, short neck, and restricted neck motion. In addition to this triad, many patients have severe lumbar scoliosis and cervical spine immobility which can lead to unsuccessful neuroaxial analgesia and difficult airway. These features present significant challenges to anesthesiologists caring for obstetric patients where neuroaxial analgesia is commonly used and in a patient population who otherwise is already considered a difficult airway.
Our patient is a 25-year-old nulliparous female, with a past medical history significant for Klippel-Feil syndrome and repaired double outlet right ventricle, who presented for induction of labor at 39 weeks. Physical exam findings were significant for characteristic short neck, limited neck extension and rotation, and moderate scoliosis with palpable bony landmarks. She had full strength in all four extremities without evidence of clonus. Cervical x-rays demonstrated several abnormalities including incomplete fusion at multiple levels. Lumbar x-rays were not available.
Given the complexity of the case, a multidisciplinary team convened and a plan to proceed with epidural placement was made to afford the patient the best possible outcome. Two attempts with a 17-gauge Touhy needle were made at L3-L4. On the first attempt, the patient reported right lower extremity paresthesia and the needle was immediately withdrawn. The second attempt was made slightly more cephalad and to the left of the palpated midline within the same space. Loss of resistance was achieved at 9 cm without complication. Adequate analgesia was achieved for the duration of labor.
There are many factors to consider when deciding on an anesthetic plan in a parturient with Klippel-Feil syndrome. Both neuroaxial and general anesthesia can be problematic in this patient population. Given that our patient had only moderate scoliosis we decided attempting to place an epidural would be in the patient’s best interest. If successful, the epidural could be dosed to provide adequate surgical anesthesia should she require a cesarean section. This would avoid general anesthesia in a patient with a potentially difficult airway.
Epidural placement in any patient with scoliosis is more technically challenging. Additionally, the extent and distribution of local anesthetic spread is variable in these patients. However, risks and benefits must be weighed accordingly as was done with our patient. Attempts for neuroaxial analgesia in high risk patients should be considered when reasonable.
1) Hensinger RN, Lang JE, MacEwen GD. Klippel-Feil syndrome; a constellation of associated anomalies. J Bone Joint Surg Am. 1974 Sep;56(6):1246-53.
2) Kavanagh T, Jee R, Kilpatrick N, Douglas J. Elective cesarean delivery in a parturient with Klippel–Feil syndrome. International journal of obstetric anesthesia. 2013 Nov 1;22(4):343-8.