1. K O B E U N I V E R S I T Y O R T H O P E D I C S A K O B E U N I V E R S I T Y O R T H O P E D I C S A Nippon Steel Hirohata Hospital Nippon Steel Hirohata Hospital 第 41 回人工関節学会 (25-26. Feb. 2011, Tokyo) How to Manage Dystrophic Spinal Deformities in Type 1 Neurofibromatosis Patients? How to Manage Dystrophic Spinal Deformities in Type 1 Neurofibromatosis Patients? Takuto Kurakawa, Teppei Suzuki, Koki Uno National Hospital Organization Kobe Medical Center, JAPAN JAPAN

2. K O B E U N I V E R S I T Y O R T H O P E D I C S A K O B E U N I V E R S I T Y O R T H O P E D I C S A Nippon Steel Hirohata Hospital Nippon Steel Hirohata Hospital 第 41 回人工関節学会 (25-26. Feb. 2011, Tokyo) Financial Disclosure Presenting author Relationships Disclosed Takuto Kurakawa No Relationship Co-authors Teppei Suzuki No Relationship Koki Uno No Relationship

3. K O B E U N I V E R S I T Y O R T H O P E D I C S A K O B E U N I V E R S I T Y O R T H O P E D I C S A Nippon Steel Hirohata Hospital Nippon Steel Hirohata Hospital 第 41 回人工関節学会 (25-26. Feb. 2011, Tokyo)ObjectiveObjective The aim of this study was to evaluate surgical outcome of spinal deformities in NF-1 patients with dystrophic changes. It is still a challenge to control severe dystrophic deformities in neurofibromatosis type 1 (NF-1) due to progressive destruction of the spinal column. Several authors have recommended combined anterior and posterior spinal fusion for dystrophic deformities with more than 40º of scoliosis or more than 50º of kyphosis 1,2. However, when or how we should perform surgical intervention to those patients is not clarified. Several authors have recommended combined anterior and posterior spinal fusion for dystrophic deformities with more than 40º of scoliosis or more than 50º of kyphosis 1,2. However, when or how we should perform surgical intervention to those patients is not clarified.

4. K O B E U N I V E R S I T Y O R T H O P E D I C S A K O B E U N I V E R S I T Y O R T H O P E D I C S A Nippon Steel Hirohata Hospital Nippon Steel Hirohata Hospital 第 41 回人工関節学会 (25-26. Feb. 2011, Tokyo) PatientsPatients No. of cases 21 Gender (F:M) 12 : 9 Curve types 13 scoliosis & 8 kyphoscoliosis Age at the initial surgery 10.6 (range, 2 - 22) years Follow-up period 9.0 (range, 1 - 24) years All patients surgically treated by a single surgeon in the past 24 years. * Three patients (14%) died during follow-up were included (malignant nerve sheath tumor in 2 patients and unknown sudden death in 1). * Three patients (14%) died during follow-up were included (malignant nerve sheath tumor in 2 patients and unknown sudden death in 1). **

5. K O B E U N I V E R S I T Y O R T H O P E D I C S A K O B E U N I V E R S I T Y O R T H O P E D I C S A Nippon Steel Hirohata Hospital Nippon Steel Hirohata Hospital 第 41 回人工関節学会 (25-26. Feb. 2011, Tokyo) Demographics of Patient Groups Of the 13 patients in Group A, 5 patients had growing rod surgery. Group A Group B Surgical Approach Posterior only Combined anterior and posterior No. of cases 138 Gender (F:M) 7 : 6 3 : 5 Age at the initial surgery 11.1 ±5.4 yrs. 10.6 ±3.0 yrs. Major curve magnitude 67.8 ±17.8 ° 67.4 ±19.1 ° * There was no significant difference between two groups by Mann-Whitney U test. Patients were divided into two groups according to the surgical approaches.

6. K O B E U N I V E R S I T Y O R T H O P E D I C S A K O B E U N I V E R S I T Y O R T H O P E D I C S A Nippon Steel Hirohata Hospital Nippon Steel Hirohata Hospital 第 41 回人工関節学会 (25-26. Feb. 2011, Tokyo) Changes in Major Curves At the latest follow-up, magnitude of major curve was progressed larger in Group A than in Group B. At the latest follow-up, magnitude of major curve was progressed larger in Group A than in Group B. Group A Group B P value Surgical Approach Posterior only Combined anterior and posterior Pre-op. magnitude (°) 67.8 ±17.8 67.4 ±19.1 0.86 Post-op. magnitude (°) 46.8 ±10.7 36.9 ±24.9 0.66 correction (%) 29.0 ±14.8 49.7 ±29.7 0.17 Latest f/u. magnitude (°) 52.6 ±14.9 41.9 ±28.5 0.69 correction loss (°) 12.8 ±10.9 * 5.0 ±11.1 0.22 * Excluded 5 cases of growing rod surgery. Significance was assessed by Mann-Whitney U test.

7. K O B E U N I V E R S I T Y O R T H O P E D I C S A K O B E U N I V E R S I T Y O R T H O P E D I C S A Nippon Steel Hirohata Hospital Nippon Steel Hirohata Hospital 第 41 回人工関節学会 (25-26. Feb. 2011, Tokyo) ComplicationsComplications Complications in Group A (posterior only) No. of cases DetailsDuration from the initial surgery Revision surgery 1 (8%) Instrumentation failure 8 years Replacement of posterior instrumentation and extension of fusion area down to pelvis 1 (8%) Implant infection 1 year Instrumentation removal 3* (23%) Bone destruction at the apex 2 to 8 years* Final fixation surgery (graduation from growing rods) * The patients who had growing rods during lengthening period.

8. K O B E U N I V E R S I T Y O R T H O P E D I C S A K O B E U N I V E R S I T Y O R T H O P E D I C S A Nippon Steel Hirohata Hospital Nippon Steel Hirohata Hospital 第 41 回人工関節学会 (25-26. Feb. 2011, Tokyo) ComplicationsComplications Complications in Group B (combined anterior and posterior) No. of cases DetailsDuration from the initial surgery Revision surgery 3 (38%) Instrumentation failure 1 to 3 years Replacement of posterior fixation 1 (13%) Implant infection 1 year Removal of instrumentation and re-fixation due to neurological deterioration 1 (13%) Progressive bone destruction 11 year Additional anterior fusion

9. K O B E U N I V E R S I T Y O R T H O P E D I C S A K O B E U N I V E R S I T Y O R T H O P E D I C S A Nippon Steel Hirohata Hospital Nippon Steel Hirohata Hospital 第 41 回人工関節学会 (25-26. Feb. 2011, Tokyo) Case Presentation The Cobb angles of the scoliosis were 72º preoperatively and 64º at the latest follow-up. The Cobb angles of the scoliosis were 72º preoperatively and 64º at the latest follow-up. 13 -year-old female patient with dystrophic thoracic scoliosis underwent combined anterior and posterior spinal fusion using an autologous fibular graft (T4–10) and posterior instrumentation (T2–L3). 13 -year-old female patient with dystrophic thoracic scoliosis underwent combined anterior and posterior spinal fusion using an autologous fibular graft (T4–10) and posterior instrumentation (T2–L3). 3D-CT scan at 10 years after surgery demonstrates that the anterior strut grafts have been integrated into the vertebral column that can tolerate postoperative bone erosion.

10. K O B E U N I V E R S I T Y O R T H O P E D I C S A K O B E U N I V E R S I T Y O R T H O P E D I C S A Nippon Steel Hirohata Hospital Nippon Steel Hirohata Hospital 第 41 回人工関節学会 (25-26. Feb. 2011, Tokyo) SummarySummary ☑ The results of this study suggested that dystrophic curves in NF-1 patients tend to progress after posterior spinal fusion including growing rod cases. ☑ Early surgical intervention and combined antero-posterior fusion might be necessary before focal dystrophic changes develop in NF-1 patients.

11. K O B E U N I V E R S I T Y O R T H O P E D I C S A K O B E U N I V E R S I T Y O R T H O P E D I C S A Nippon Steel Hirohata Hospital Nippon Steel Hirohata Hospital 第 41 回人工関節学会 (25-26. Feb. 2011, Tokyo) ReferencesReferences [1] Ogilvie JW. Neurofibromatosis. In: Bradford DS, ed. Moe’s Textbook of Scoliosis and Other Spinal Deformities. 3rd ed. Textbook of Scoliosis and Other Spinal Deformities. 3rd ed. Philadelphia, PA: W.B. Saunders Co.; 1995: 338 – 47. Philadelphia, PA: W.B. Saunders Co.; 1995: 338 – 47. [1] Ogilvie JW. Neurofibromatosis. In: Bradford DS, ed. Moe’s Textbook of Scoliosis and Other Spinal Deformities. 3rd ed. Textbook of Scoliosis and Other Spinal Deformities. 3rd ed. Philadelphia, PA: W.B. Saunders Co.; 1995: 338 – 47. Philadelphia, PA: W.B. Saunders Co.; 1995: 338 – 47. [2] Iwai C, Taneichi H, Inami S, et al. Clinical outcomes of combined anterior and posterior spinal fusion for dystrophic thoracolumbar anterior and posterior spinal fusion for dystrophic thoracolumbar spinal deformities of neurofibromatosis-1: fate of nonvascularized spinal deformities of neurofibromatosis-1: fate of nonvascularized anterior fibular strut grafts. Spine 2013; 38: 44 – 50. anterior fibular strut grafts. Spine 2013; 38: 44 – 50. [2] Iwai C, Taneichi H, Inami S, et al. Clinical outcomes of combined anterior and posterior spinal fusion for dystrophic thoracolumbar anterior and posterior spinal fusion for dystrophic thoracolumbar spinal deformities of neurofibromatosis-1: fate of nonvascularized spinal deformities of neurofibromatosis-1: fate of nonvascularized anterior fibular strut grafts. Spine 2013; 38: 44 – 50. anterior fibular strut grafts. Spine 2013; 38: 44 – 50.