Spinal and pelvic parameters in Marfan's syndrome and their relevance to surgical planning

• Published in: Journal of bone and joint surgery. British volume Vol. 88; no. 4; pp. 515 - 519
• Main Authors: GARREAU DE LOUBRESSE, C, MULLINS, M. M, MOURA, B, MARMORAT, J.-L, PIRIOU, P, JUDET, T
• Format: Journal Article
• Language: English
• Published: London British Editorial Society of Bone and Joint Surgery 01.04.2006; British Editorial Society of Bone & Joint Surgery
• Edition: British volume
• Subjects: Adolescent; Adult; Biological and medical sciences; Diseases of the osteoarticular system; Female; Humans; Kyphosis - diagnostic imaging; Kyphosis - etiology; Lordosis - diagnostic imaging; Lordosis - etiology; Lumbar Vertebrae - diagnostic imaging; Male; Marfan Syndrome - complications; Marfan Syndrome - diagnostic imaging; Medical sciences; Middle Aged; Pelvic Bones - diagnostic imaging; Radiography; Regression Analysis; Retrospective Studies; Sacrum - diagnostic imaging; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Scoliosis - diagnostic imaging; Scoliosis - etiology; Spine - diagnostic imaging; Thoracic Vertebrae - diagnostic imaging; Connective tissue disease; Skin disease; Treatment; Elastic tissue disease; Spine; Surgery; Orthopedics; Systemic disease; Planning; Marfan syndrome; Genetic disease
• ISSN: 0301-620X; 2049-4394; 2044-5377; 2049-4408
• DOI: 10.1302/0301-620X.88B4.17034

Spinal deformities are a common feature of Marfan's syndrome and can be a significant cause of morbidity. The morphology of the scoliosis associated with this condition was previously described by Sponseller, but no correlation with the pelvic parameters has been seen. We performed a retrospective radiological study of 58 patients with scoliosis, secondary to Marfan's syndrome and related the findings in the thoracolumbar spine to the pelvic parameters, including pelvic version (tilt), pelvic incidence and sacral slope. Our results showed marked abnormalities in the pelvic values compared with those found in the unaffected population, with increased retroversion of the pelvis in particular. In addition we found a close correlation between the different patterns of pelvic parameters and scoliosis morphology. We found that pelvic abnormalities may partially dictate the spinal disorders seen in Marfan's syndrome. Our results supplement the well-established Sponseller classification, as well as stressing the importance of considering the orientation of the pelvis when planning surgery.