• Title, Summary, Keyword: Klippel-Feil syndrome

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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, Rathna
    • 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.

Torticollis Management Using the Customized Soft Neck Collar in CATCH 22 Syndrome Combined with Klippel-Feil Anomaly: A Case Report

  • Moon, Myung-Hoon;Kim, Soo-Yeon
    • Journal of Interdisciplinary Genomics
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    • v.1 no.2
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    • pp.19-22
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    • 2019
  • CATCH 22 syndrome is rare genetic disease that has various manifestations. Cervical vertebral anomaly, such as Klippel-Feil anomaly, is frequently observed in the patients with CATCH22 syndrome. We present the case of an 11-year-old female patient with CATCH22 syndrome and Klippel-Feil anomaly who had been treated torticollis using the customized soft neck collar. During the patient's first visit to our clinic, she presented with low ear set, skull deformity, intellectual disability, and tilting of the head to the left by approximately 25 degrees. Imaging studies revealed multisegmental fusion and C3 hemivertebrae of the cervical spine and left thoracic scoliosis at T4 with 50 degrees of Cobb's angle. We instructed passive stretching and applied the customized soft neck collar we invented. The ipsilateral aspect of the neck collar is designed to provide vertical support between the clavicle and mandibular angle and is adjustable in height. The Velcro was attached to the neck collar at the point of contact with the ipsilesional mandibular angle, which provides negative sensory feedback, inducing her to tilt neck to the contralesional side. We applied the neck collar for 2 hours a day. After 1 year of treatment, her neck inclination angle improved from 25 to 10 degrees. Providing negative sensory feedback using the customized soft neck collar can be one of the treatment options of postural management in patients with torticollis in cases of CATCH 22 syndrome combined with Klippel-Feil anomaly.

Traumatic Hemiparesis Associated with Type III Klippel-Feil Syndrome

  • Park, Jin-Kyu;Huh, Han-Yong;Ryu, Kyeong-Sik;Park, Chun-Kun
    • Journal of Korean Neurosurgical Society
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    • v.42 no.2
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    • pp.145-148
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    • 2007
  • Klippel-Feil Syndrome (KFS) is a complex congenital syndrome of osseous and visceral anomalies. It is mainly associated with multi-level cervical spine fusion with hypermobile normal segments. Therefore, a patient with KFS can be at risk of severe neurological symptoms even after a minor trauma. We report a patient with type III KFS who developed a hemiparesis after a minor trauma and was successfully managed with operation.

A Case Report of Neck Pain Patient with Klippel-Feil Syndrome by Cervical Manipulation Treatment (Klippel-Feil 증후군 환자의 경추 도수치료 후 발생한 부작용 치험1례)

  • Lee, Jong-Ha;Kwon, Jeong-Gook;Keum, Dong-Ho
    • Journal of Korean Medicine Rehabilitation
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    • v.25 no.2
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    • pp.175-180
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    • 2015
  • The Klippel-Feil Syndrome (KFS) is characterized by congenital fusion of two or more vertebrae with hypermobile normal segment. According to this, a patient with KFS can be at risk of severe neurological symptoms after manipulation treatment. We had a KFS patient who suffered from neck pain and limited range of motion at cervical after manipulation treatment. The patient, 49-year-old woman was diagnosed as KFS through Cervical X-ray and MRI. The patient was treated by acupuncture therapy and SCENAR therapy. We measured neck pain by visual analog scale (VAS) and neck disability index (NDI) and checked range of motion at cervical before and after the treatments. After 8 times treatments, the patient's pain decreased and the range of motion increased. From this case, we can recognize the risk of Chuna manipulation treatment for KFS patient. So, we suggest that radiological examination is needed before cervical Chuna manipulation treatment to avoid adverse reactions.