Posterior spinal fusion for severe kyphoscoliosis in a Loeys-Dietz syndrome patient with a large syringomyelia |
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| Affiliation: | 1. Department of Orthopaedic Surgery, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, Nagano 390-8621, Japan;2. Department of Neurosurgery, Shinshu University Hospital, Matsumoto, Japan;3. Center for Medical Genetics, Shinshu University Hospital, Matsumoto, Japan;4. Department of Spinal Surgery, Kameda Medical Center, Kamogawa, Japan;1. Department of Experimental Medicine, University of Perugia, Perugia, Italy;2. Neurologist, Perugia, Italy;3. Department of Philosophy, Social Sciences and Education, University of Perugia, Italy;4. Department of Medicine, University of Perugia, Perugia, Italy;5. Degree Course in Speech and Language Therapy, University of Perugia, Perugia, Italy;6. Department of Neuroscience, “Santa Maria” Hospital, Terni, Italy;1. Department of Cardiovascular Surgery, Oita University Hospital, Oita, Japan;2. Department of Clinical Engineering, Oita University Faculty of Medicine, Oita, Japan;1. Department of Respiratory Medicine, Kyoto University, Kyoto, Japan;2. Department of Respiratory Medicine, Nara Medical University, Nara, Japan;3. Department of Bioscience and Genetics, National Cerebral and Cardiovascular Center Research Institute, Osaka, Japan;4. Department of Medical Genetics, Sakakibara Heart Institute, Tokyo, Japan;5. Department of Respirology, Kyoto Prefectural University of Medicine (North Medical Center), Kyoto, Japan;1. Department of Neurosurgery, Stanford University, Stanford, CA, United States;2. Department of Neurosurgery, University Hospital Zurich & Clinical Neuroscience Center, University of Zurich, Switzerland;3. Department of Medicine, Division of Oncology, Stanford University, Stanford, CA, United States;4. Department of Pathology, Stanford University, Stanford, CA, United States;1. Department of Radiation Oncology, Kaiser Los Angeles Medical Center, 4950 Sunset Blvd, Los Angeles, CA 90027, USA;2. Department of Interventional Radiology, Kaiser Los Angeles Medical Center, 4867 Sunset Blvd, Los Angeles, CA 90028, USA;3. Department of Neurological Surgery, Kaiser Los Angeles Medical Center, 1505 N Edgemont St, Los Angeles, CA 90027, USA;4. Department of Neurological Surgery, Keck School of Medicine of USC, 1200 N State St, Suite 3300, Los Angeles, CA 90033, USA |
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| Abstract: | Spinal deformity is a characteristic feature of Loeys-Dietz syndrome (LDS). Surgical correction in LDS is indicated when the deformity is progressive to avoid neurological deficits, respiratory impairment, and back pain. However, few reports exist on the surgical treatment of spinal deformity in LDS, and no therapeutic standards have been established. We described the clinical and radiological outcomes of a patient with LDS receiving surgery for severe kyphoscoliosis.A 21-year-old male patient with LDS underwent posterior spinal fusion with an all-pedicle screw construct from T10 to L5 for a preoperative main curve Cobb angle of 70 degrees and kyphotic angle of 49 degrees. The postoperative Cobb angle of the main curve and kyphotic angle improved to 36 and 8 degrees, respectively. Correction surgery was performed with frequent motor evoked potential testing, taking care not to cause motor paralysis. Ameliorated low back pain and improvements in clinical questionnaire scores were noted at 21 months after surgery. No perioperative complications were reported.Based on the present case, posterior spinal fusion represents a good correction option for severe spinal deformity in LDS with syringomyelia. Careful preoperative examination and treatment for neurovascular and neurological lesions is advised to prevent severe complications. |
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| Keywords: | Posterior spinal fusion Loeys-Dietz syndrome Annuloaortic ectasia Syringomyelia Kyphoscoliosis Radiological findings Clinical findings |
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