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Tenleighs troops Platform: Raise awareness to spondylocostal dysostosis, klippel — feil syndrome, and thoracic insufficiency syndrome.
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Spondylocostal Dysostosis, Klippel-Feil, & Thoracic Insufficiency Syndrome:
a.k.a. Jarcho-Levin Syndrome.
- A rare congenital condition.
- Multiple segmentation defects of the vertebrae (M-SDV) along with rib abnormalities.
- Congenital scoliosis.
- Short-trunk compared to height with a short neck.
- Congenital condition
- Abnormal fusions of two or more in the cervical vertebrae
- Low hairline at the back of the neck.
- Limited range of motion in the neck.
Spondylocostal Dysostosis & Thoracic Insufficiency Syndrome Treatment.
The Vertical Expandable Prosthetic Titanium Rib (VEPTR):
The initial procedure consists of one or more opening wedge thoracotomies and insertion of one or more VEPTR devices, one of which may include a spinal distraction hook (hybrid VEPTR device). The thoracostomies of the hemithorax are expanded maximally at the initial procedure and the devices are placed to stabilize the correction. Repeated surgeries to lengthen the VEPTR are performed on an outpatient basis at appropriate intervals to further correct deformities of the thorax and spine.Campbell RM. , Smith MD, Mayes TC, Mangos JA, Willey-Cour and, DB, Surber, J. The treatment of thoracic insufficiency syndrome associated with scoliosis and fused ribs, Presented at the American Association of Orthopaedic Surgery, March 2000, Orlando, FL.
The Titanium Rib Project:
Dr. Smith & Dr. Campbell’s worked together for 13 years to perfect the VEPTR and the surgical technique. The VEPTR rod finally gained FDA approval in September 2004, becoming the first major advancement in spine instrumentation treatment since Dr. Harrington of Houston, Texas, created the Harrington Rod in 1962.
Conditions that the VEPTR has been used to treat:
- Spondylocostal dysostosis
- spondylothoracic dysostosis
- Jeunes Syndrome
- Congenital scoliosis
- Missing or fused ribs
- Missing or malformed vertebra