Clinical and Radiographic Outcomes Following Complex Spine Reconstruction in Neurofibromatosis with Severe Spinal...

Neurofibromatosis (NF) associated spine deformity is uncommon and can be debilitating to patients. Surgery for deformity correction is associated with improved neurological status and quality of life, however, the surgical correction of the spinal deformity can be challenging can be and associated with high complication rates. We present a series of NF patients with curves >100° or treated with a vertebral column resections (VCR).

Spine deformity correction surgery in NF patients with curves >100° or indicated for VCR have higher rates of postsurgical complications.

Twelve consecutive NF patients from a multi-centre study group who underwent complex spine reconstruction as a result of NF associated deformity (>100° or treated by VCR) were enrolled in the study. Analysis included demographic, clinical, pr-operative, intraoperative, and postoperative complications. scoliosis research society (SRS)-22 scores were used to compare pre-and 2-years postoperative functional outcomes.

Mean age and body mass index were 14 ± 2.9 and 19.8 ± 4.7 kg/m², respectively. Of the twelve patients enrolled, six patients had severe kyphoscoliosis, two patients presented with kyphosis, and four with scoliosis. Halo-gravity traction was used in 75% (9/12) of patients. Mean preoperative coronal and sagittal Cobb angles were 99.7 ± 22.5 and 99.1 ± 35.4, respectively. Seven patients had VCR and five had posterior column osteotomies. At 2-years postoperatively, mean coronal and sagittal Cobb angles were 44.9 ± 17.5 and 62.7 ± 21.7, respectively. Two patients with preoperative motor deficits improved at 1 year follow-up and 1 (8.3%) patient had new neurologic deficit postoperatively but recovered within a year follow-up. About 16.70% (2/12) had post-op pulmonary complications (atelectasis and pleural effusion) and 16.7% (2/12) patients required a revision surgery for implant related complications. There were no pseudarthrosis, visual complications (blindness) or deaths in the study cohort. health related quality of life measures, including SRS-22 scores demonstrated significant improvement at 2-years follow-up.

Complex reconstruction resulted in significant curve improvement in both coronal and sagittal planes in NF-associated spine deformity. There were low of postsurgical complications. SRS outcomes improved in all patients after 2 year f/u.