• Journal of Dental Anesthesia and Pain Medicine
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• v.19 no.5
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• pp.307-312
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• 2019

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.

• Clinics in Shoulder and Elbow
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• v.10 no.1
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• pp.146-149
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• 2007

Congenital undescended scapula is congenital structural abnormality which affects only one side usually. Scapula located higher than the usual and rotating deformity that inferior angle to medial side, superior angle to lateral side is common. This report presents one case of the surgical therapy of a sprengel deformity patient who passed an optimal operation period with age $3{\sim}7$years old, and includes brief review of the literature. 7 years old boy whose chief complaint was the limitation of left scapular-thoracic movement and he had an omovertebral bone bridge and periscapular muscle atrophy. There was improvement of motion ranges and cosmetic problems after surgical treatment.

• Clinical and Experimental Pediatrics
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• v.50 no.3
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• pp.311-315
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• 2007

This is the first case of a de novo balanced translocation 46, XY, t(3;17)(p12.2;q25) associated with multiple musculoskeletal abnormalities, including Sprengel's deformity (congenital undescended scapula to be reported). This translocation has not been described previously with this congenital anomaly in Korea.