Congenital scoliosis \3- Quo vadis?

Abstract

Congenital spinal vertebral anomalies can present as scoliosis or kyphosis or both. The worldwide prevalence of the vertebral anomalies is 0.5-1 per 1000 live births. Vertebral anomalies can range from hemi vertebrae (HV) which may be single or multiple, vertebral bar with or without HV, block vertebrae, wedge shaped or butterfly vertebrae. Seventy per cent of congenital vertebral anomalies result in progressive deformities. The risk factors for progression include: type of defect, site of defect (junctional regions) and patient's age at the time of diagnosis. The key to success in managing these spinal deformities is early diagnosis and anticipation of progression. One must intervene surgically to halt the progression of deformity and prevent further complications associated with progressive deformity. Planning for surgery includes a preoperative MRI scan to rule out spinal anomalies such as diastematomyelia. The goals of surgical treatment for congenital spinal deformity are to achieve a straight growing spine, a normal standing sagittal profile, and a short fusion segment. The options of surgery include in situ fusion, convex hemi epiphysiodesis and hemi vertebra excision. These basic surgical procedures can be combined with curve correction, instrumentation and short segment fusion. Most surgeons prefer posterior (only) surgery for uncomplicated HV excision and short segment fusion. These surgical procedures can be performed through posterior, anterior or combined approaches. The advocates of combined approaches suggest greater deformity correction possibilities with reduced incidence of pseudoarthrosis and minimize crankshaft phenomenon. We recommend posterior surgery for curves involving only an element of kyphosis or modest deformity, whereas combined anterior and posterior approach is indicated for large or lordotic deformities. In the last decade, the use of growing rods and vertebral expandable prosthetic titanium rib has improved the armamentarium of the spinal surgeon in dealing with certain difficult congenital spinal deformities. The goal of growing rod treatment is to provide simultaneous deformity correction and allow for continued spinal growth. Once maximal spinal growth has been achieved, definitive fusion and instrumentation is performed.

Intrauterine phenotypic features associated with 16p11.2 recurrent microdeletions

“…Vertebral malformations are common birth defects among newborns with 16p11.2 recurrent microdeletion. The malformations occurred in different regions of the spine, including the thoracic spine (64%), the thoracolumbar spine (20%), the lumbar spine (11%), and the lumbosacral spine (5%) . In our 5 fetuses with vertebral malformations, 4 fetuses had thoracolumbar spine malformations, and 1 fetus had a lumbar spine malformation.…”

“…The malformations occurred in different regions of the spine, including the thoracic spine (64%), the thoracolumbar spine (20%), the lumbar spine (11%), and the lumbosacral spine (5%). 22 In our 5 fetuses with vertebral malformations, 4 fetuses had thoracolumbar spine malformations, and 1 fetus had a lumbar spine malformation. The vertebral column is differentiated from the bilateral segmented somites during embryonic development.…”

Perioperative concerns in pediatric patients undergoing different types of scoliosis correction surgery: A retrospective observational study

“…The remaining spine is allowed to grow in a controlled fashion so that the child can achieve full height and thoracic capacity. [3]…”

“…On an average only 50% of the curve is corrected during the initial surgery, subsequently the rods are distracted by a centimeter or so every 6 months to a year to keep up with the child's growth. [3]…”

Congenital kypho-scoliosis: a case of thoracic insufficiency syndrome and the limitations of treatment

“…A key feature of congenital scoliosis is the presence of one or more abnormally formed vertebrae [1]. The prevalence of the vertebral anomalies is 0.5-1 per 1,000 live births [2].…”