BACKGROUND: Spinal deformities associated with neurofibromatosis type 1 (NF1) often have an early onset. These curves frequently develop dysplastic features. Rapid progression is common, and is often difficult to control with casting or bracing. Spinal fusion at a young age can potentially interfere with chest and trunk growth. Growing rods (GRs) have been used in early-onset scoliosis (EOS) effectively. The purpose of this study was to evaluate GR use in NF1. METHODS: Retrospective data collection was performed from a multicenter EOS database with additional patients from our own institute. Each patient had a genetic diagnosis of NF1 and was treated with GR. Results were compared with reported results of GR in EOS in the literature. RESULTS: Fourteen patients from 5 centers underwent a total of 71 procedures with an average follow-up of 54 months. Mean age at surgery was 6.8 years. Means of initial and final curves were 74 and 36 degrees, respectively (51% correction). Spine grew at an average of 39 mm (11.2 mm per year). Implant-related complications were the most common (8/14, 57%), including failure of proximal construct (5/14), rod breakage (2/14), and prominent implants (1/14). There was no significant difference between screws and hooks as proximal anchors (Fischer test). Two patients had deep infection that needed debridement. CONCLUSIONS: This retrospective pooled data study is the first report on the treatment of early-onset NF1 scoliosis with GRs. The use of GRs in these patients effectively controls the spinal deformity and facilitates growth of the spine. The complications were no greater than those seen in other conditions causing EOS. Failure of proximal anchors was found to be the most common complication. LEVEL OF EVIDENCE: Level IV-retrospective case series.
BACKGROUND:Spinal deformities associated with neurofibromatosis type 1 (NF1) often have an early onset. These curves frequently develop dysplastic features. Rapid progression is common, and is often difficult to control with casting or bracing. Spinal fusion at a young age can potentially interfere with chest and trunk growth. Growing rods (GRs) have been used in early-onset scoliosis (EOS) effectively. The purpose of this study was to evaluate GR use in NF1. METHODS: Retrospective data collection was performed from a multicenter EOS database with additional patients from our own institute. Each patient had a genetic diagnosis of NF1 and was treated with GR. Results were compared with reported results of GR in EOS in the literature. RESULTS: Fourteen patients from 5 centers underwent a total of 71 procedures with an average follow-up of 54 months. Mean age at surgery was 6.8 years. Means of initial and final curves were 74 and 36 degrees, respectively (51% correction). Spine grew at an average of 39 mm (11.2 mm per year). Implant-related complications were the most common (8/14, 57%), including failure of proximal construct (5/14), rod breakage (2/14), and prominent implants (1/14). There was no significant difference between screws and hooks as proximal anchors (Fischer test). Two patients had deep infection that needed debridement. CONCLUSIONS: This retrospective pooled data study is the first report on the treatment of early-onset NF1 scoliosis with GRs. The use of GRs in these patients effectively controls the spinal deformity and facilitates growth of the spine. The complications were no greater than those seen in other conditions causing EOS. Failure of proximal anchors was found to be the most common complication. LEVEL OF EVIDENCE: Level IV-retrospective case series.
Authors: Kiril V Mladenov; Alexander Simon Spiro; Kara Leigh Krajewski; Ralf Stücker; Philip Kunkel Journal: Childs Nerv Syst Date: 2020-07-01 Impact factor: 1.475
Authors: Johanna Uthoff; Jared Larson; Takashi S Sato; Emily Hammond; Kimberly E Schroeder; Frank Rohret; Christopher S Rogers; Dawn E Quelle; Benjamin W Darbro; Rajesh Khanna; Jill M Weimer; David K Meyerholz; Jessica C Sieren Journal: Sci Rep Date: 2020-03-19 Impact factor: 4.996