Surgical correction of severe cervical kyphosis in patients with neurofibromatosis Type 1

Report of 3 cases

View More View Less
  • 1 Departments of Orthopaedic Surgery and
  • 2 Advanced Therapy for Spine and Spinal Cord Disorders, Keio University, Tokyo, Japan
Restricted access

Purchase Now

USD  $45.00

Spine - 1 year subscription bundle (Individuals Only)

USD  $369.00

JNS + Pediatrics + Spine - 1 year subscription bundle (Individuals Only)

USD  $600.00
Print or Print + Online

Severe cervical kyphosis requiring surgical treatment is rare in patients with neurofibromatosis Type 1 (NF1). When it occurs, however, dystrophic changes in the vertebrae make surgical correction and fusion of the deformity extremely difficult.

The authors report on 3 cases of severe cervical kyphosis associated with NF1 that were successfully treated with combined anterior and posterior correction and fusion. All patients underwent halo-gravity traction for approximately 1 month prior to surgery to correct the deformity gradually. Posterior correction and fusion were performed with segmental spinal instrumentation consisting of lateral mass screws, lamina screws, pedicle screws, and polyethylene tape for sublaminar wiring. Anterior spinal fusion was performed using a fibula strut to induce solid bone fusion. All patients used a halo vest for postoperative external fixation.

Preoperative CT scans showed dystrophic cervical spine changes, and MR images demonstrated extensive neurofibromas outside the cervical spine in all 3 patients. The preoperative kyphotic angles were as follows: Case 1, 140°; Case 2, 81°; and Case 3, 72°; after halo-gravity traction, the kyphosis angles improved to 50°, 55°, and 51°, respectively; and after surgery, they were 50°, 15°, and 27°, respectively. Solid bone union was observed in all patients at the latest follow-up. All three patients experienced postoperative complications consisting of superficial infection, severe pneumonia, and partial dislocation of the distal fibula graft after removing the halo vest, in one patient each.

Although dystrophic cervical vertebral changes in these patients with NF1 complicated the correction of severe cervical kyphosis, the use of preoperative halo-gravity traction, a combination of spinal instrumentations, an anterior strut bone graft, and postoperative halo-vest fixation made it possible to correct the kyphosis, maintain the correction, and achieve solid bone fusion.

Abbreviations used in this paper:EBL = estimated blood loss; JOA = Japanese Orthopaedic Association; NF1 = neurofibromatosis Type 1; SCI = spinal cord injury.

Spine - 1 year subscription bundle (Individuals Only)

USD  $369.00

JNS + Pediatrics + Spine - 1 year subscription bundle (Individuals Only)

USD  $600.00

Contributor Notes

Address correspondence to: Morio Matsumoto, M.D., Department of Orthopedic Surgery, Keio University School of Medicine, 35 Shinanomachi, Shinjuku, Tokyo 160-8582, Japan. email: morio@a5.keio.jp.

Please include this information when citing this paper: published online January 4, 2013; DOI: 10.3171/2012.11.SPINE12417.

  • 1

    Akbarnia BA, , Gabriel KR, , Beckman E, & Chalk D: Prevalence of scoliosis in neurofibromatosis. Spine (Phila Pa 1976) 17:8 Suppl S244S248, 1992

    • Search Google Scholar
    • Export Citation
  • 2

    Asazuma T, , Yamagishi M, , Nemoto K, , Amako M, , Osada M, & Fujikawa K: Spinal fusion using a vascularized fibular bone graft for a patient with cervical kyphosis due to neurofibromatosis. J Spinal Disord 10:537540, 1997

    • Search Google Scholar
    • Export Citation
  • 3

    Bourdais L, , Hamel A, , Hamel O, , Pannier M, & Duteille F: [Use of a vascularized fibula for spinal reconstruction in neurofibromatosis.]. Ann Chir Plast Esthet 53:293297, 2008. (Fr)

    • Search Google Scholar
    • Export Citation
  • 4

    Craig JB, & Govender S: Neurofibromatosis of the cervical spine. A report of eight cases. J Bone Joint Surg Br 74:575578, 1992

  • 5

    Crawford AH, & Herrera-Soto J: Scoliosis associated with neurofibromatosis. Orthop Clin North Am 38:553562, 2007

  • 6

    Etame AB, , Wang AC, , Than KD, , La Marca F, & Park P: Outcomes after surgery for cervical spine deformity: review of the literature. Neurosurg Focus 28:3 E14, 2010

    • Search Google Scholar
    • Export Citation
  • 7

    Funasaki H, , Winter RB, , Lonstein JB, & Denis F: Pathophysiology of spinal deformities in neurofibromatosis. An analysis of seventy-one patients who had curves associated with dystrophic changes. J Bone Joint Surg Am 76:692700, 1994

    • Search Google Scholar
    • Export Citation
  • 8

    Gardner A, , Millner P, , Liddington M, & Towns G: Translaminar screw fixation of a kyphosis of the cervical and thoracic spine in neurofibromatosis. J Bone Joint Surg Br 91:12521255, 2009

    • Search Google Scholar
    • Export Citation
  • 9

    Goffin J, & Grob D: Spondyloptosis of the cervical spine in neurofibromatosis. A case report. Spine (Phila Pa 1976) 24:587590, 1999

  • 10

    Gould EP: The bone changes occurring in von Recklinghausen's disease. Q J Med 11:221228, 1918

  • 11

    Horsley M, , Taylor TK, & Sorby WA: Traction-induced rupture of an extracranial vertebral artery aneurysm associated with neurofibromatosis. A case report. Spine (Phila Pa 1976) 22:225227, 1997

    • Search Google Scholar
    • Export Citation
  • 12

    Hsu LC, , Lee PC, & Leong JC: Dystrophic spinal deformities in neurofibromatosis. Treatment by anterior and posterior fusion. J Bone Joint Surg Br 66:495499, 1984

    • Search Google Scholar
    • Export Citation
  • 13

    Inoue M, , Sairyo K, , Sakai T, & Yasui N: Significance of surgical treatment for severe dystrophic changes in the cervical spine associated with neurofibromatosis type I: a case report. J Pediatr Orthop B 19:270275, 2010

    • Search Google Scholar
    • Export Citation
  • 14

    Inoue S, , Kataoka H, , Tajima T, , Tajima N, , Nakano N, & Hasue M, : Assessment of treatment for low back pain. J Jpn Orthop Assoc 60:391394, 1986

    • Search Google Scholar
    • Export Citation
  • 15

    Johnston TL, , Karaikovic EE, , Lautenschlager EP, & Marcu D: Cervical pedicle screws vs. lateral mass screws: uniplanar fatigue analysis and residual pullout strengths. Spine J 6:667672, 2006

    • Search Google Scholar
    • Export Citation
  • 16

    Jones EL, , Heller JG, , Silcox DH, & Hutton WC: Cervical pedicle screws versus lateral mass screws. Anatomic feasibility and biomechanical comparison. Spine (Phila Pa 1976) 22:977982, 1997

    • Search Google Scholar
    • Export Citation
  • 17

    Kothe R, , Rüther W, , Schneider E, & Linke B: Biomechanical analysis of transpedicular screw fixation in the subaxial cervical spine. Spine (Phila Pa 1976) 29:18691875, 2004

    • Search Google Scholar
    • Export Citation
  • 18

    Lammert M, , Kappler M, , Mautner VF, , Lammert K, , Störkel S, & Friedman JM, : Decreased bone mineral density in patients with neurofibromatosis 1. Osteoporos Int 16:11611166, 2005

    • Search Google Scholar
    • Export Citation
  • 19

    Laohacharoensombat W, , Wajanavisit W, & Woratanarat P: Cervical neurofibromatosis with quadriparesis: management by fibular strut graft. Indian J Orthop 44:9597, 2010

    • Search Google Scholar
    • Export Citation
  • 20

    Ma J, , Wu Z, , Yang X, & Xiao J: Surgical treatment of severe cervical dystrophic kyphosis due to neurofibromatosis Type 1: a review of 8 cases. Clinical article. J Neurosurg Spine 14:9398, 2011

    • Search Google Scholar
    • Export Citation
  • 21

    Mummaneni PV, , Dhall SS, , Rodts GE, & Haid RW: Circumferential fusion for cervical kyphotic deformity. Clinical article. J Neurosurg Spine 9:515521, 2008

    • Search Google Scholar
    • Export Citation
  • 22

    Nijland EA, , van den Berg MP, , Wuisman PI, , van Royen BJ, , Winters HA, & van Ouwerkerk WJ: Correction of a dystrophic cervicothoracic spine deformity in Recklinghausen's disease. Clin Orthop Relat Res 349:149155, 1998

    • Search Google Scholar
    • Export Citation
  • 23

    Parisini P, , Di Silvestre M, , Greggi T, , Paderni S, , Cervellati S, & Savini R: Surgical correction of dystrophic spinal curves in neurofibromatosis. A review of 56 patients. Spine (Phila Pa 1976) 24:22472253, 1999

    • Search Google Scholar
    • Export Citation
  • 24

    Rinella A, , Lenke L, , Whitaker C, , Kim Y, , Park SS, & Peelle M, : Perioperative halo-gravity traction in the treatment of severe scoliosis and kyphosis. Spine (Phila Pa 1976) 30:475482, 2005. (Erratum in Spine (Phila Pal 1976) 30:994)

    • Search Google Scholar
    • Export Citation
  • 25

    Singh K, , Samartzis D, & An HS: Neurofibromatosis type I with severe dystrophic kyphoscoliosis and its operative management via a simultaneous anterior-posterior approach: a case report and review of the literature. Spine J 5:461466, 2005

    • Search Google Scholar
    • Export Citation
  • 26

    Sirois JL III, & Drennan JC: Dystrophic spinal deformity in neurofibromatosis. J Pediatr Orthop 10:522526, 1990

  • 27

    Tanaka J, , Kuramochi A, , Nishi N, , Yuasa M, & Heshiki A: Preoperative transarterial embolization enhances the surgical management of diffuse plexiform neurofibroma: a case report. Cardiovasc Intervent Radiol 28:686688, 2005

    • Search Google Scholar
    • Export Citation
  • 28

    Tsirikos AI, , Ramachandran M, , Lee J, & Saifuddin A: Assessment of vertebral scalloping in neurofibromatosis type 1 with plain radiography and MRI. Clin Radiol 59:10091017, 2004

    • Search Google Scholar
    • Export Citation
  • 29

    Tsirikos AI, , Saifuddin A, & Noordeen MH: Spinal deformity in neurofibromatosis type-1: diagnosis and treatment. Eur Spine J 14:427439, 2005

    • Search Google Scholar
    • Export Citation
  • 30

    Wang Z, & Liu Y: Research update and recent developments in the management of scoliosis in neurofibromatosis type 1. Orthopedics 33:335341, 2010

    • Search Google Scholar
    • Export Citation
  • 31

    Wilde PH, , Upadhyay SS, & Leong JC: Deterioration of operative correction in dystrophic spinal neurofibromatosis. Spine (Phila Pa 1976) 19:12641270, 1994

    • Search Google Scholar
    • Export Citation
  • 32

    Winter RB, , Lonstein JE, & Anderson M: Neurofibromatosis hyperkyphosis: a review of 33 patients with kyphosis of 80 degrees or greater. J Spinal Disord 1:3949, 1988

    • Search Google Scholar
    • Export Citation
  • 33

    Winter RB, , Moe JH, , Bradford DS, , Lonstein JE, , Pedras CV, & Weber AH: Spine deformity in neurofibromatosis. A review of one hundred and two patients. J Bone Joint Surg Am 61:677694, 1979

    • Search Google Scholar
    • Export Citation
  • 34

    Wu F, , Zhang L, , Liu Z, , Sun Y, , Li F, & Wang S: Cervical neurofibromatosis with tetraplegia: management by cervical suspensory traction a case report. Spine (Phila Pa 1976) 37:E858E862, 2012

    • Search Google Scholar
    • Export Citation
  • 35

    Yonezawa I, , Arai Y, , Tsuji T, , Takahashi M, & Kurosawa H: Anterior fusion and posterior correction of severe cervical kyphosis using pedicle screw fixation in a patient with neurofibromatosis: a case report. J Spinal Disord Tech 16:493496, 2003

    • Search Google Scholar
    • Export Citation
  • 36

    Yong-Hing K, , Kalamchi A, & MacEwen GD: Cervical spine abnormalities in neurofibromatosis. J Bone Joint Surg Am 61:695699, 1979

Metrics

All Time Past Year Past 30 Days
Abstract Views 630 198 37
Full Text Views 213 40 6
PDF Downloads 238 28 2
EPUB Downloads 0 0 0