Centre for Spinal Studies and Surgery, Queens Medical Centre, Derby Road, Nottingham, UK.
Eur Spine J. 2012 Jun;21(6):1043-9. doi: 10.1007/s00586-011-2032-y. Epub 2011 Nov 3.
Congenital spinal vertebral anomalies may present with deformity resulting in congenital scoliosis and kyphosis. This leads to abnormal spinal growth. The latter when combined with associated rib fusions may impair normal thoracic cage development and resultant pulmonary hypoplasia. Most congenital scoliosis can be detected in utero by ultrasound scan or recognized in the neonatal period, but a few spinal defects can remain undetected.
In this Grand Round, we present the case of a 7-year-old girl with a severe scoliosis and thoracic insufficiency syndrome (TIS). 3D CT reconstruction imaging demonstrated a mixed picture of fusion and segmentation abnormalities. A marked kyphoscoliosis was demonstrated at the thoraco-lumbar junction. Via a left thoracotomy, anterior excision of intervertebral discs was performed together with, interbody fusion, and in situ stabilisation of the kyphosis with double allograft (femur) strut grafts.
This article highlights the features of congenital kypho-scoliosis and TIS. The difficulties of treating kyphosis when combined with TIS are discussed together with the limitations of current surgical techniques.
先天性脊柱椎体异常可表现为畸形，导致先天性脊柱侧凸和后凸。这会导致脊柱生长异常。后者与肋骨融合结合时，可能会损害正常的胸廓发育和由此导致的肺发育不全。大多数先天性脊柱侧凸可以通过超声扫描在子宫内检测到，或在新生儿期识别，但少数脊柱缺陷可能未被发现。
在本次大查房中，我们介绍了一例 7 岁女孩患有严重脊柱侧凸和胸壁发育不全综合征（TIS）的病例。3D CT 重建成像显示融合和分割异常的混合图像。在胸腰椎交界处显示出明显的脊柱后凸侧凸。通过左开胸术，进行了椎间盘的前路切除，同时进行了椎间融合，并通过双同种异体（股骨）支撑移植物原位稳定后凸。
本文强调了先天性脊柱后凸和 TIS 的特征。讨论了在 TIS 合并后凸时治疗后凸的困难以及当前手术技术的局限性。
