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12-year old female patient with Marfan Syndrome (an inherited disorder that affects connective tissue — the fibers that support and anchor your organs and other structures in your body). The patient, who was seen during one of my missionary trips to the Dominican Republic with the “World Spine Organization” and the “Butterfly Foundation”, presented with spondyloptosis (defined as a complete anterior dislocation of the L5 vertebral body from sacrum at L5-S1) as well as sweeping congenital thoracic scoliosis with an 80 degree thoracic-lumbar curve and lordotic thoracic spine to compensate for the spondyloptosis.
The plan for this patient’s surgery was a T2 to pelvis fixation with an S1 transdiscal/ transvertebral screw to help stabilize the spondyloptosis.
The goal of this procedure was not to reduce the spondyloptosis, but to stabilize it – mainly because the patient was intact with no deficit – which consequently allowed for the correction of the curve.
The patient did well after one of the most challenging cases I performed in the Dominican Republic, but needed other procedures for limb shortening due to the Marfan’s Syndrome.
