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Kyphosis

Kyphosis - other
Objectives
  1. List 5 other causes for kyphosis other than congenital, postural, or Scherermann's kyphosis
  2. Discuss the clinical significance of kyphosis occurring in the thoracolumbar or lumbar spine

Discussion point
  1. How can one determine if a kyphotic deformity is amenable to bracing?

Discussion
A number of pediatric orthopaedic conditions can be accompanied by kyphotic deformity. Fortunately, two of the historically more frequent causes, post irradiation kyphosis and post laminectomy kyphosis are occurring less often now, thanks to advances in radiation therapy and the technique of laminoplasty, which replaces the posterior elements removed for access to the spinal canal.

Kyphosis can be manifest as part of the clinical picture of a number of generalized conditions. Children with high-level myelodysplasia generally develop lumbar kyphosis due to the absence of posterior structures. Two of the more malevolent mucopolysaccaridoses, Hunter and Hurler syndromes, may present with kyphosis in infancy. Thoracolumbar kyphosis also commonly affects infants with achondroplasia. Fortunately, most resolve with walking. Other unusual causes include Gaucher's disease, juvenile osteoporosis, and pseudoachondroplasia. Kyphosis in conditions accompanied by ligamentous laxity such as Ehlers Danlos syndrome and Marfan syndrome commonly affect the thoracolumbar or lumbar spine. Lumbar or thoracolumbar kyphosis is difficult to treat as junctional kyphosis above or below the instrumented portion of the spine often occurs unless excellent sagittal plane alignment is achieved. Kyphosis accompanying neurofibromatosis often is accompanied by severe rotatory deformity and can be very difficult to treat. Cervical kyphosis can be a part of diastrophic dysplasia or Larsen's syndrome. In parts of the world, where tuberculosis is prevalent, screening of children for kyphosis can aid earlier diagnosis. As children with cystic fibrosis live longer into adult life, kyphosis accompanying this condition is more often being reported.

For conditions such as juvenile osteoporosis or Maroteaux-Lamy syndrome, in which the kyphosis is flexible and radiographically corrects when the patient is placed supine over a bolster, bracing can be effective.

References
  1. Kocher MS, Hall JE. Surgical management of spinal involvement in children and adolescents with Gaucher's disease. Journal of Pediatric Orthopedics 2000; 20( 3): 383-8.
  2. Zeller RD, Dubousset J. Progressive rotational dislocation in kyphoscoliotic deformities: presentation and treatment. Spine 2000; 25( 9): 1092-7.
  3. Matsuyama Y, Winter RB, Lonstein JE. The spine in diastrophic dysplasia. The surgical arthrodesis of thoracic and lumbar deformities in 21 patients. Spine 1999; 24( 22): 2325-31.
  4. Sarwark JF. Kyphosis deformity in myelomeningocele. Orthopedic Clinics of North America 1999; 30( 3): 451-5, viii-ix.
  5. Remes V, Marttinen E, Poussa M, Kaitila I, Peltonen J. Cervical kyphosis in diastrophic dysplasia. Spine 1999; 24( 19): 1990-5.
  6. Belmont PJ, Jr., Polly DW, Jr. Early diagnosis of Hurler's syndrome with the aid of the identification of the characteristic gibbus deformity. Military Medicine 1998; 163( 10): 711-4.
  7. Otsuka NY, Hey L, Hall JE. Postlaminectomy and postirradiation kyphosis in children and adolescents. Clinical Orthopaedics & Related Research 1998( 354): 189-94.
  8. Levin TL, Berdon WE, Lachman RS, Anyane-Yeboa K, Ruzal-Shapiro C, Roye DP, Jr. Lumbar gibbus in storage diseases and bone dysplasias. Pediatric Radiology 1997; 27( 4): 289-94.
  9. Johnston CE, 2nd, Birch JG, Daniels JL. Cervical kyphosis in patients who have Larsen syndrome [see comments]. Journal of Bone & Joint Surgery -American Volume 1996; 78( 4): 538-45.
  10. Mooney JF, 3rd, Emans JB. Progressive kyphosis and neurologic compromise complicating spondylothoracic dysplasia in infancy (Jarcho-Levin syndrome). Spine 1995; 20( 17): 1938-42.
  11. Ogle JW, Wilson FC, McConnachie CC. Angular kyphosis as an indicator of the prevalence of Pott's disease in Transkei. South African Medical Journal 1994; 84( 9): 614-8.
  12. McMaster MJ. Spinal deformity in Ehlers-Danlos syndrome. Five patients treated by spinal fusion. Journal of Bone & Joint Surgery -British Volume 1994; 76( 5): 773-7.
  13. Henderson RC, Specter BB. Kyphosis and fractures in children and young adults with cystic fibrosis. Journal of Pediatrics 1994; 125( 2): 208-12.
  14. Osebold WR, King HA. Kyphoscoliosis in Williams syndrome. Spine 1994; 19( 3): 367-71.
  15. Bell DF, Walker JL, O'Connor G, Tibshirani R. Spinal deformity after multiple-level cervical laminectomy in children. Spine 1994; 19( 4): 406-11.
  16. 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. Journal of Bone & Joint Surgery -American Volume 1994; 76( 5): 692-700.
  17. Makipernaa A, Heikkila JT, Merikanto J, Marttinen E, Siimes MA. Spinal deformity induced by radiotherapy for solid tumours in childhood: a long-term follow up study. European Journal of Pediatrics 1993; 152( 3): 197-200.
  18. Conrad EUd, Olszewski AD, Berger M, Powell E, Bruckner J. Pediatric spine tumors with spinal cord compromise. Journal of Pediatric Orthopedics 1992; 12( 4): 454-60.

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