The growth-friendly surgical treatment of scoliosis in children with osteogenesis imperfecta using distraction-based ins
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CASE SERIES
The growth‑friendly surgical treatment of scoliosis in children with osteogenesis imperfecta using distraction‑based instrumentation Lawrence I. Karlin1 · Anna McClung2 · Charles E. Johnston2 · Amer Samdani3 · M. Timothy Hresko1 · Francisco Javier Perez‑Grueso4 · Michael Troy1 · Children’s Spine Study Group5 Received: 12 February 2020 / Accepted: 25 August 2020 © Scoliosis Research Society 2020
Abstract Purpose The study was undertaken to determine the feasibility of growth-friendly distraction-based surgery in children with OI. Methods Two multi-center databases were queried for children with OI who had undergone GR or VEPTR surgery. Inclusion criteria were a minimum 2-year follow-up and three lengthening procedures following the initial implantation. Details of the surgical techniques, surgical complications, and radiographic measurements of deformity correction, T1–T12 and T1–S1 elongation and growth were recorded. Results Five patients were identified. There was one patient with type I OI and two patients each with type III and type IV. Four patients had GR constructs and one a VEPTR construct. The initial scoliosis deformity averaged 80° (70°–103°), and the subsequent corrections averaged 32% for initial correction, 48% at last follow-up, and 54% for the two patients that had a final fusion. The T1–T12 and T1–S1 growth averaged 31 mm and 44 mm respectively, and yearly growth averaged 4 mm and 6 mm, respectively. Growth was notably much less in those with more severe disease. There were 13 complications in 4 patients. Nine of the 10 surgical complications were anchor failures which were corrected in 7 planned and 2 un-planned procedures. Significant migration occurred in one patient with severe OI type III. Conclusion The results varied in this heterogeneous population. In general, satisfactory deformity corrections were obtained and maintained, modest growth was obtained, and complications were similar to those reported in other series of growthfriendly surgery. Limited growth and significant anchor migration are to be anticipated in this population. Level of evidence IV. Keywords Osteogenesis imperfecta scoliosis · T1–S1 spinal growth · Growth-friendly spinal surgery · Instrumentation anchor migration · Growing spinal rods
Introduction
* Lawrence I. Karlin [email protected] 1
Department of Orthopaedic Surgery, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA
2
Department of Orthopedic Surgery, Texas Scottish Rite Hospital, Dallas, TX, USA
3
Shriners Hospital for Children, Philadelphia, PA, USA
4
Spine Unit, Department of Orthopedic Surgery, Hospital Universitario La PA, Madrid, Spain
5
Chidren’s Spine Foundation, Valley Forge, PA, USA
Osteogenesis imperfecta (OI) is a disease with a wide spectrum of clinical findings that share the characteristic of bone fragility. In its mildest form, the affected individuals are of normal height and have no orthopaedic deformities; the most severe form is fatal in the perinatal period. The repo
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