Surgical treatment of a 180° thoracolumbar fixed kyphosis in a young achondroplastic patient: a one-stage “in situ” combined fusion and spinal cord translocation

European Spine Journal

J. C. Aurégan²  T. Odent²  M. Zerah¹  C. Glorion²  J. P. Padovani² 

[1] Department of Pediatric Neurosurgery, Hôpital Necker-Enfants-Malades, Assistance Publique-Hôpitaux de Paris, University Paris V, 161 rue de Sèvres, 75743 Paris Cedex 15, France;Department of Pediatric Orthopaedic Surgery, Hôpital Necker-Enfants-Malades, Assistance Publique-Hôpitaux de Paris, University Paris V, 161 rue de Sèvres, 75743 Paris Cedex 15, France

关键词: Achondroplasia;    Thoracolumbar kyphosis;    Spinal stenosis;   

DOI  :  10.1007/s00586-010-1530-7

学科分类：骨科学

来源: Springer

【 摘 要 】

An achondroplastic patient with a thoracolumbar kyphosis was first seen at the age of 16 at our institution. He was 102 cm tall when standing, 65 cm tall when sitting and 27 kg in weight. Past medical history was unremarkable. The family history was negative for achondroplasia. He had regular follow-up with a general practitioner in his country (North Africa). His only concern was the aesthetic implication of his deformity. At this time, his physical examination was normal except for loss of the Achillean and patellar reflexes in the lower limbs. He had no sphincter disturbance or pyramidal signs. The radiographs showed a 180° thoracolumbar kyphosis with no reduction on bolster test. The frontal and sagittal balances were normal with an odontoid process plumb line passing through the L5–S1 disc. The MRI did not reveal an abnormal spinal cord signal or nerve root compression. At our department conference it was decided to observe him every 6 months. At each consultation, a physical examination was done followed by frontal and sagittal X-rays. No physical or radiological worsening was noticed in the subsequent 2 years. At 2 years, he developed a progressive neurogenic claudication of the lower limb appearing at after 500 m of walking. At rest, the neurological exam was unchanged. No apparent worsening of the deformity was noticed in the frontal and sagittal X-rays. A new MRI revealed an abnormal central spinal cord signal in front of the apex of the kyphosis associated with a narrow spinal canal without vertebral wedging or disc protrusion. With regard to this progressive neurological worsening, a surgical treatment was decided to prevent neurological worsening.

【 授权许可】

Unknown