Progressive double major scoliotic curve with concurrent lumbosacral spondylolisthesis in a skeletally immature patient with Marfan syndrome treated with anterior scoliosis correction.

STUDY DESIGN

Case report (review of patient records, imaging, and pulmonary function tests) and literature review.

OBJECTIVES

To describe the case of a skeletally immature patient with Marfan syndrome who underwent anterior scoliosis correction (ASC) and muscle-sparing posterior far lateral interbody fusion (FLIF) in a two-stage procedure to correct progressive severe double major scoliosis and spondylolisthesis. Patients with Marfan syndrome suffer from rapidly progressive scoliosis and spondylolisthesis. Operative treatment has typically been limited to PSF, but newer techniques may be less invasive and provide more spine motion.

METHODS

A 12-year-old girl with Marfan syndrome, spondylolisthesis, and severe progressive scoliosis underwent a two-stage procedure to achieve correction. Muscle-sparing posterior FLIF of the spondylolisthesis from L4-S1 was initially performed, followed 1 week later by ASC from right T4-T11 and left T11-L3 using an anterior screw/cord construct.

RESULTS

Follow-up from the index procedures for the spondylolisthesis and scoliosis is 35 months. No significant complications occurred in perioperative and postoperative follow-up periods. At the 13-month follow-up, the double major scoliosis showed continued curve correction via growth modulation and overcorrection of the lumbar to - 13°. A revision lengthening procedure of the anterior cord from T11-L3 was performed. An asymptomatic elevated hemidiaphragm was discovered at 6 weeks postoperation, which was believed to be secondary to retraction neuropraxia and subsequently improved. At 21 months postlengthening and 35 months postindex procedure, she is skeletally mature and the curves have maintained correction in both the coronal and sagittal planes without any further complications.

CONCLUSIONS

Anterior scoliosis correction of both a thoracic and lumbar curve combined with an L4-S1 PSF was effective for this patient and may be promising for patients with Marfan syndrome, progressive scoliosis, and spondylolisthesis. Overcorrection can be planned for and easily corrected by inserting a new cord of a different length.