///2009 Abstract Details
2009 Abstract Details2018-05-01T17:45:11+00:00

Cesarean delivery in a Parturient with Klippel-Feil Syndrome Type III

Abstract Number: 204
Abstract Type: Case Report/Case Series

Eileen R Manabat M.D.1 ; Suzanne L. Huffnagle D.O.,F.A.O.C.A.2; H. Jane Huffnagle D.O.,F.A.O.C.A.3; Hsu George M.D.4; Grodecki Wlodzimierz M.D.5; Elia Elia M.D.6

INTRODUCTION: Klippel and Feil described a patient with a short, fused neck, and low posterior hairline in 1912 (1). Feil developed a classification system based on the site and extent of spinal fusion.

CASE REPORT: A 38 year old primapara (411", 52 kg) with Type III Klippel-Feil syndrome presented at term for elective cesarean delivery. Her physical exam revealed a short neck, short stature, low posterior hairline, Mallampati II airway, and limited neck flexion/ extension. She was kyphoscoliotic with narrow interspinous spaces and restricted thoracolumbar motion. Pulmonary function testing revealed a restrictive ventilatory defect and severely reduced carbon monoxide diffusing capacity, indicating loss of effective alveolar capillary interface. TTE was normal. MRI of the pelvis identified multiple segmentation vertebral body anomalies involving the thoracolumbar spine with block, butterfly, and hemi vertebra. The conus was approximately at the level of the diaphragm with an enlarged thecal sac. Our anesthetic plan was a CSE using a reduced dose of intrathecal local anesthetic. After multiple attempts, only an epidural was able to be placed at the L4-5 interspace and the patient delivered a healthy baby girl weighing 3210 grams.

DISCUSSION: Our patient presented with the physical characteristics(short, webbed neck, short stature, low dorsal hairline, limited neck motion, thoracolumbar spinal abnormalities) of Type III Klippel-Feil syndrome. The etiology is unknown but is thought to occur from failure of normal segmentation of cervical somites during the 3rd- 8th week of gestation (2). The most common associated orthopedic condition is scoliosis; progression beyond 55 degrees may cause severe respiratory compromise. Asynchronous respiratory movements, decreased lung compliance, and a restrictive pattern of respiratory dysfunction are common. A thorough preoperative work-up and multidisciplinary approach to manage these patients is imperative. We decided upon a CSE anesthetic with general anesthesia (GA) as back up. Concerns with GA include securing a potentially difficult airway and positioning problems, as these patients are at increased risk of cervical spinal cord injury and neurologic damage during laryngoscopy and positioning(3). Continuous epidural anesthesia allows slow titration of the anesthetic level and preservation of spontaneous ventilation, but improper catheter placement and unequal distribution of local may lead to inadequate or unilateral block. Utilizing CSE with a reduced dose of intrathecal local anesthetic would provide a reliable, dense block. Since our spinal was unsuccessful, careful dosing of the epidural catheter provided satisfactory anesthesia.

REFERENCES:

1. Klippel M, Feil A. Clinical orthopaedics and related research. 1975;109:3-8.

2. Singh M, Prasad R, Jacob R. Anaesthetic Indian J Anaesth 2005;49:511-14.

3. Naguib M, Farag H, Ibrahim AW. Can Anaesth Soc J 1986;33:66-70.

SOAP 2009