J Dent Anesth Pain Med.  2019 Oct;19(5):307-312. 10.17245/jdapm.2019.19.5.307.

Anesthesia for elective bilateral sagittal slip osteotomy of the mandible and genioplasty in a young man with Klippel-Feil syndrome, Sprengel deformity, and mandibular prognathism

• Paramaswamy R

• KMID: 2461248
• DOI: http://doi.org/10.17245/jdapm.2019.19.5.307

Abstract

Klippel-Feil syndrome is characterized by congenital fusion of two or more cervical vertebrae, a low hair line at the back of the head, restricted neck mobility, and other congenital anomalies. We report a 16-year-old young man with Klippel-Feil syndrome, Sprengel deformity of the right scapula, thoracic kyphoscoliosis, and mandibular prognathism with an anterior open bite. He was treated with orthodontic treatment and maxillofacial surgery. An anticipated difficult airway due to a short neck with restricted neck movements and extrinsic restrictive lung disease due to severe thoracic kyphoscoliosis increased his anesthesia risk. Due to his deviated nasal septum and contralateral inferior turbinate hypertrophy, we chose awake fiber optic orotracheal intubation followed by submental intubation. Considering the cervical vertebral fusion, he was carefully positioned during surgery to avoid potential spinal injury. He recovered well and his postoperative course was uneventful.

Keyword

Klippel-Feil Syndrome; Kyphoscoliosis; Mandibular Prognathism; Orthognathic Surgery; Sprengel Deformity; Submental

MeSH Terms

Adolescent
Anesthesia*
Cervical Vertebrae
Congenital Abnormalities*
Female
Genioplasty*
Hair
Head
Humans
Hypertrophy
Intubation
Klippel-Feil Syndrome*
Lung Diseases
Mandible*
Nasal Septum
Neck
Open Bite
Orthognathic Surgery
Osteotomy*
Prognathism*
Scapula
Spinal Injuries
Surgery, Oral
Turbinates

Figure

• 

  Fig. 1 Side and back view of the same patient showing a low hair line at the back of the neck, restricted neck movements, Sprengel deformity of the right scapula, thoracic scoliosis, and mandibular prognathism.

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  Fig. 2 Radiograph of neck showing fusion of the upper cervical vertebra and an extensive anterior open bite.

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  Fig. 3 Radiograph of the chest and abdomen showing thoracic kyphoscoliosis with Cobb angle>35°.

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  Fig. 4 Coronal unenhanced computed tomography scan of the paranasal sinuses showing a leftward deviated nasal septum with compensatory inferior turbinate hypertrophy on the right.

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  Fig. 5 Oral submental intubation.

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