Repeated Anesthetic Management for a Patient With Klippel-Feil Syndrome

Abstract

Klippel-Feil syndrome (KFS) is a rare disease characterized by a classic triad comprising a short neck, a low posterior hairline, and restricted motion of the neck due to fused cervical vertebrae. We report repeated anesthetic management for orthognathic surgeries for a KFS patient with micrognathia. Because KFS can be associated with a number of other anomalies, we therefore performed a careful preoperative evaluation to exclude them. The patient had an extremely small mandible, significant retrognathia, and severe limitation of cervical mobility due to cervical vertebral fusion. As difficult intubation was predicted, awake nasal endotracheal intubation with a fiberoptic bronchoscope was our first choice for gaining control of the patient's airway. Moreover, the possibility of respiratory distress due to postoperative laryngeal edema was considered because of the surgeries on the mandible. In the operating room, tracheotomy equipment was always kept ready if a perioperative surgical airway control was required. Three orthognathic surgeries and their associated anesthetics were completed without a fatal outcome, although once the patient was transferred to the intensive care unit for precautionary postoperative airway management and observation. Careful preoperative examination and preparation for difficult airway management are important for KFS patients with micrognathia.

Klippel-Feil Syndrome Associated with Renal and Cardiac Anomalies in an Infant: A Case Report

“…Thorough cervical spine evaluation is essential to avoid potential spinal cord injury stemming from laryngoscopy, intubation, intraoperative positioning, and head manipulation that may increase the risk of craniovertebral dislocation and atlantoaxial subluxation. 8 - 10 Furthermore, proper counselling of the parents about the course, outcome, and treatment options helps to dispel the stigma surrounding this deformity.…”

Anaesthetic considerations in paediatric klippel feil syndrome- A case report

“…They have cervical instability and are at high risk for spinal cord injury during laryngoscopy, intubation and positioning for surgery. 4 In 1988, Daum and Jones 5 suggested that the most prudent and effective way is an awake fibreoptic intubation. The nasal route is preferred since the tongue is out of the way and the patient cannot bite down on the tube or scope.…”

Otolaryngologic Manifestations of Klippel-Feil Syndrome in Children

“…Cervical spine instability, hypermobility, and symptomatic stenosis at the interspaces between the fused segments may develop. There have also been multiple reports of mandibular abnormalities in patients with KFS including retrognathia, 33 duplication of the mandibular rami, [34][35][36] and extra-articular ankylosis of the temporomandibular joint. 37 Progressive cervical instability or neurological compromise attributable to cervical instability is usually managed with surgical stabilization.…”

“…Cervical spine instability, hypermobility, and symptomatic stenosis at the interspaces between the fused segments may develop. There have also been multiple reports of mandibular abnormalities in patients with KFS including retrognathia, duplication of the mandibular rami, and extra-articular ankylosis of the temporomandibular joint …”