HSS Journal

, Volume 3, Issue 1, pp 71–76 | Cite as

Posterior Vertebral Column Resection for VATER/VACTERL Associated Spinal Deformity: A Case Report

  • Matthew E. Cunningham
  • Gina Charles
  • Oheneba Boachie-Adje
Original Article


The VATER/VACTERL association is a syndrome notable for congenital vertebral malformations, anal atresia, cardiovascular anomalies, tracheoesophageal fistula, esophageal atresia, and renal or limb malformations. Vertebral malformations may include the entire spectrum of congenital spinal deformities, including kyphosis, as was seen in this case. A 14-year-old girl presented to our institution with severe rigid sagittal deformity in the thoracolumbar spine that had recurred following three prior spinal fusion surgeries: the first posterior only, the second anterior and posterior, and the third a posterior only proximal extension. These surgeries were performed to control progressive kyphosis from a complex failure of segmentation that resulted in a 66° kyphosis from T11 to L3 by the time she was 9 years old. Our evaluation revealed solid arthrodesis from the most recent procedures with resultant sagittal imbalance, and surgical options to restore balance included anterior and posterior revision spinal fusion with osteotomies, multiple posterior extension osteotomies with circumferential spine fusion, and posterior vertebral column resection with circumferential spine fusion. She was advised that multiple posterior extension osteotomies would likely be insufficient to restore sagittal balance in the setting of solid arthrodesis from anterior and posterior surgery, and that the posterior-only vertebral column resection would provide results equivalent to revision anterior and posterior surgery, without the morbidity of the anterior approach. She successfully underwent posterior vertebrectomy and circumferential spinal fusion with instrumentation and is doing well 2 years postoperatively. Severe rigid sagittal deformity can be effectively managed with a posterior-only surgical approach, vertebrectomy, and circumferential spinal fusion with instrumentation.

Key words

VATER VACTERL kyphosis kyphectomy spinal deformity recurrent 


  1. 1.
    Lawhon SM, MacEwen GD, Bunnell WP (1986) Orthopaedic aspects of the VATER association. J Bone Jt Surg, Am 68(3):424–429Google Scholar
  2. 2.
    Muraji T, Mahour GH (1984) Surgical problems in patients with VATER-associated anomalies. J Pediatr Surg 19(5):550–554PubMedCrossRefGoogle Scholar
  3. 3.
    Weber TR, Smith W, Grosfeld JL (1980) Surgical experience in infants with the VATER association. J Pediatr Surg 15(6):849–854PubMedCrossRefGoogle Scholar
  4. 4.
    Botto LD, Khoury MJ, Mastroiacovo P, Castilla EE, Moore CA, Skjaerven R, Mutchinick OM, Borman B, Cocchi G, Czeizel AE, Goujard J, Irgens LM, Lancaster PA, Martinez-Frias ML, Merlob P, Ruusinen A, Stoll C, Sumiyoshi Y (1997) The spectrum of congenital anomalies of the VATER association: an international study. Am J Med Genet 71(1):8–15PubMedCrossRefGoogle Scholar
  5. 5.
    Patankar JZ, Vidyadhar M, Prabhakaran K, Bo L, Lsk Loh D (2004) Urogenital sinus, rectovaginal fistula, and an anterior stenosed anus—another cloacal variant. Pediatr Surg Int 20(7):556–558PubMedCrossRefGoogle Scholar
  6. 6.
    James HE, Chesnut R, Krous H, Billman G, Jones KL, Jones M (1994) Distal spinal cord pathology in the VATER association. J Pediatr Surg 29(11):1501–1503PubMedCrossRefGoogle Scholar
  7. 7.
    Warf BC, Scott RM, Barnes PD, Hendren WH, 3rd (1993) Tethered spinal cord in patients with anorectal and urogenital malformations. Pediatr Neurosurg 19(1):25–30PubMedCrossRefGoogle Scholar
  8. 8.
    Pang D (1993) Sacral agenesis and caudal spinal cord malformations. Neurosurgery 32(5):755–778; discussion 78–79Google Scholar
  9. 9.
    Tongsong T, Wanapirak C, Piyamongkol W, Sudasana J (1999) Prenatal sonographic diagnosis of VATER association. J Clin Ultrasound 27(7):378–384PubMedCrossRefGoogle Scholar
  10. 10.
    del Rio Holgado M, Martinez JM, Gomez O, Casals G, Bargallo N, Fortuny A, Puerto B (2005) Ultrasonographic diagnosis of Jarcho–Levin syndrome at 20 weeks’ gestation in a fetus without previous family history. Fetal Diagn Ther 20(2):136–140PubMedCrossRefGoogle Scholar
  11. 11.
    Hanauer A, Young ID (2002) Coffin–Lowry syndrome: clinical and molecular features. J Med Genet 39(10):705–713PubMedCrossRefGoogle Scholar
  12. 12.
    Yassir WK, Grottkau BE, Goldberg MJ (2003) Costello syndrome: orthopaedic manifestations and functional health. J Pediatr Orthop 23(1):94–98PubMedCrossRefGoogle Scholar
  13. 13.
    Lalonde F, Jarvis J (1999) Congenital kyphosis in myelomeningocele. The effect of cordotomy on bladder function. J Bone Jt Surg Br 81(2):245–249CrossRefGoogle Scholar
  14. 14.
    Lintner SA, Lindseth RE (1994) Kyphotic deformity in patients who have a myelomeningocele. Operative treatment and long-term follow-up. J Bone Jt Surg Am 76(9):1301–1307Google Scholar
  15. 15.
    Martin J Jr, Kumar SJ, Guille JT, Ger D, Gibbs M (1994) Congenital kyphosis in myelomeningocele: results following operative and nonoperative treatment. J Pediatr Orthop 14(3):323–328PubMedGoogle Scholar
  16. 16.
    Mintz LJ, Sarwark JF, Dias LS, Schafer MF (1991) The natural history of congenital kyphosis in myelomeningocele. A review of 51 children. Spine 16(8 Suppl):S348–350PubMedGoogle Scholar
  17. 17.
    Niall DM, Dowling FE, Fogarty EE, Moore DP, Goldberg C (2004) Kyphectomy in children with myelomeningocele: a long-term outcome study. J Pediatr Orthop 24(1):37–44PubMedGoogle Scholar
  18. 18.
    Nolden MT, Sarwark JF, Vora A, Grayhack JJ (2002) A kyphectomy technique with reduced perioperative morbidity for myelomeningocele kyphosis. Spine 27(16):1807–1813PubMedCrossRefGoogle Scholar
  19. 19.
    Sarwark JF (1999) Kyphosis deformity in myelomeningocele. Orthop Clin North Am 30(3):451–455, viii–ixGoogle Scholar
  20. 20.
    Lonstein JE (1977) Post-laminectomy kyphosis. Clin Orthop Relat Res (128):93–100Google Scholar
  21. 21.
    Otsuka NY, Hey L, Hall JE (1998) Postlaminectomy and postirradiation kyphosis in children and adolescents. Clin Orthop Relat Res (354):189–194Google Scholar
  22. 22.
    Papagelopoulos PJ, Peterson HA, Ebersold MJ, Emmanuel PR, Choudhury SN, Quast LM (1997) Spinal column deformity and instability after lumbar or thoracolumbar laminectomy for intraspinal tumors in children and young adults. Spine 22(4):442–451PubMedCrossRefGoogle Scholar
  23. 23.
    Farcy JP, Schwab FJ (1997) Management of flatback and related kyphotic decompensation syndromes. Spine 22(20):2452–2457PubMedCrossRefGoogle Scholar
  24. 24.
    Mason DE, Sanders JO, MacKenzie WG, Nakata Y, Winter R (2002) Spinal deformity in chondrodysplasia punctata. Spine 27(18):1995–2002PubMedCrossRefGoogle Scholar
  25. 25.
    Basu PS, Elsebaie H, Noordeen MH (2002) Congenital spinal deformity: a comprehensive assessment at presentation. Spine 27(20):2255–2259PubMedCrossRefGoogle Scholar
  26. 26.
    Cil A, Yazici M, Alanay A, Acaroglu RE, Uzumcugil A, Surat A (2004) The course of sagittal plane abnormality in the patients with congenital scoliosis managed with convex growth arrest. Spine 29(5):547–552; discussion 52–53Google Scholar
  27. 27.
    Walhout RJ, van Rhijn LW, Pruijs JE (2002) Hemi-epiphysiodesis for unclassified congenital scoliosis: immediate results and mid-term follow-up. Eur Spine J 11(6):543–549PubMedCrossRefGoogle Scholar
  28. 28.
    Dias RC, Miller F, Dabney K, Lipton GE (1997) Revision spine surgery in children with cerebral palsy. J Spinal Disord 10(2):132–144PubMedCrossRefGoogle Scholar
  29. 29.
    Lenke LG (2004) Kyphosis of the thoracic and thoracolumbar spine in the pediatric patient: prevention and treatment of surgical complications. Instr Course Lect 53:501–510PubMedGoogle Scholar
  30. 30.
    McMaster MJ, Singh H (1999) Natural history of congenital kyphosis and kyphoscoliosis. A study of one hundred and twelve patients. J Bone Jt Surg Am 81(10):1367–1383Google Scholar
  31. 31.
    McMaster MJ, Singh H (2001) The surgical management of congenital kyphosis and kyphoscoliosis. Spine 26(19):2146–2154; discussion 55Google Scholar
  32. 32.
    Montgomery SP, Hall JE (1982) Congenital kyphosis. Spine 7(4):360–364PubMedCrossRefGoogle Scholar
  33. 33.
    Shimode M, Kojima T, Sowa K (2002) Spinal wedge osteotomy by a single posterior approach for correction of severe and rigid kyphosis or kyphoscoliosis. Spine 27(20):2260–2267PubMedCrossRefGoogle Scholar
  34. 34.
    Smith JT, Gollogly S, Dunn HK (2005) Simultaneous anterior–posterior approach through a costotransversectomy for the treatment of congenital kyphosis and acquired kyphoscoliotic deformities. J Bone Jt Surg Am 87(10):2281–2289CrossRefGoogle Scholar

Copyright information

© Hospital for Special Surgery 2006

Authors and Affiliations

  • Matthew E. Cunningham
    • 1
  • Gina Charles
    • 1
  • Oheneba Boachie-Adje
    • 1
  1. 1.Department of Orthopaedic Surgery, Scoliosis ServiceHospital for Special SurgeryNew YorkUSA

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