Scoliosis in cystic fibrosis: is it idiopathic?

Spine (Phila Pa 1976). 2004 Sep 15;29(18):1990-5. doi: 10.1097/01.brs.0000138307.07863.c5.


Study design: This is a retrospective study of all the patients registered with the Yorkshire Regional Cystic Fibrosis unit from 1982 to 1997. Of the 316 patients, there were 184 adults (age 17 years and above) and 132 children (age 0-6 years).

Objectives: This study was aimed at determining the prevalence of scoliosis in people with cystic fibrosis and describes the characteristics and progression of scoliosis in these patients and highlights predictive factors, which account for high prevalence of scoliosis in this condition.

Summary of the background data: Two previous North American studies (1978 and 1982) have indicated a high prevalence of scoliosis in patients with cystic fibrosis.

Methods: The patients were divided into 3 groups based on their chronologic age as on January 1998. Chest, abdomen, and whole spine radiographs were studied for the presence of any spinal deformity, and measurements were made using the Oxford Cobbmeter (Oxford Metrics). The extent, apex, and the side of the curves were described. The disease-specific scores, ie, Chrispin-Norman score (score for radiologic severity of lung disease) and Shwachman score (score for general condition), were noted from the patient follow-up database maintained by the Regional Cystic Fibrosis Unit. Multiple linear regression analysis was used to study the correlation between Cobb angle and the previously mentioned scores.

Results: In the 4- to 16-year age group, the prevalence of scoliosis was 15.6%, which is 20 times the prevalence in 15,793 school children with a similar age and sex distribution from the same geographic area. The majority of curves were single-thoracic, apical around T6-T8 with no side predilection. In the adult population (above 16 years), the prevalence was 9.8%, which is higher than that of the general population. These curves were thoracic, apical around T7-T8, and approximately two thirds of them were right-sided. Infantile curves are described for the first time in our study; these tend to be nonprogressive, right-sided, upper thoracic curves.

Conclusion: Our study shows a high prevalence of scoliosis in people with cystic fibrosis. These are benign short midthoracic curves, apical between T6-T8 with no side predilection.

Publication types

  • Comparative Study
  • Review

MeSH terms

  • Adolescent
  • Adult
  • Age Distribution
  • Child
  • Child, Preschool
  • Cystic Fibrosis / complications*
  • Cystic Fibrosis / epidemiology
  • Disease Progression
  • England / epidemiology
  • Female
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Prevalence
  • Radiography
  • Registries
  • Retrospective Studies
  • Scoliosis / diagnostic imaging
  • Scoliosis / epidemiology
  • Scoliosis / etiology*
  • Severity of Illness Index
  • Thoracic Vertebrae / diagnostic imaging