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Proposal of a CT scoring system of the paranasal sinuses in diagnosing cystic fibrosis

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Abstract

The purpose of this study was to develop a paranasal sinus CT scoring system that could be used as a diagnostic tool to discriminate cystic fibrosis (CF) patients from control patients examined for sinonasal disease. The model should include as few and easily applicable criteria as possible, supported by statistical analyses and clinical judgement. We used data from 116 CF and 136 control patients. The CF patients were grouped according to the number of confirmed CF mutations: genetically verified (CF-2), or based on sweat testing and clinical findings alone (CF-1, CF-0). Nine paranasal sinus CT criteria, including development, pneumatisation variants and inflammatory patterns, were evaluated. The final model included three criteria: (a) frontal and (b) sphenoid sinus development, and (c) absence of three pneumatisation variants. This model discriminated CF-2 from controls with overlap of summed scores in only 8 of 206 patients. When this model was applied in the CF-1 and CF-0 groups, two populations seemed to exist. A larger group with summed scores overlapping that of the CF-2 group and a smaller group with summed scores overlapping that of the control group. We conclude that this CT scoring system may support, as well as exclude, a CF diagnosis in cases of diagnostic uncertainty.

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References

  1. The Cystic Fibrosis Genetic Analysis Consortium (CFGAC) (2000). www.genet.sickkids.on.ca/cftr

  2. Riordan JR, Rommens JM, Kerem B, Alon N, Rozmahel R, Grzelczak Z, Zielenski J, Lok S, Plavsic N, Chou JL et al. (1989) Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 245:1066–1073

    CAS  PubMed  Google Scholar 

  3. Smith JJ, Karp PH, Welsh MJ (1994) Defective fluid transport by cystic fibrosis airway epithelia. J Clin Invest 93:1307–1311

    CAS  PubMed  Google Scholar 

  4. Rosenstein BJ, Cutting GR (1998) The diagnosis of cystic fibrosis: a consensus statement. Cystic Fibrosis Foundation Consensus Panel. J Pediatr 132:589–595

    CAS  PubMed  Google Scholar 

  5. Stern RC (1997) The diagnosis of cystic fibrosis. N Engl J Med 336:487–491

    Article  CAS  PubMed  Google Scholar 

  6. Nathanson I, Conboy K, Murphy S, Afshani E, Kuhn JP (1991) Ultrafast computerized tomography of the chest in cystic fibrosis: a new scoring system. Pediatr Pulmonol 11:81–86

    CAS  PubMed  Google Scholar 

  7. Santis G, Hodson ME, Strickland B (1991) High resolution computed tomography in adult cystic fibrosis patients with mild lung disease. Clin Radiol 44:20–22

    CAS  PubMed  Google Scholar 

  8. Cartier Y, Kavanagh PV, Johkoh T, Mason AC, Muller NL (1999) Bronchiectasis: accuracy of high-resolution CT in the differentiation of specific diseases. AJR 173:47–52

    CAS  Google Scholar 

  9. Eggesbø HB, Søvik S, Dølvik S, Kolmannskog F (2002) CT characterization of inflammatory paranasal sinus disease in cystic fibrosis. Acta Radiol 43:21–28

    Article  PubMed  Google Scholar 

  10. Eggesbø HB, Søvik S, Dølvik S, Eiklid K, Kolmannskog F (2001) CT characterization of developmental variations of the paranasal sinuses in cystic fibrosis. Acta Radiol 42:482–493

    Article  PubMed  Google Scholar 

  11. Eggesbø HB, Eken T, Eiklid K, Kolmannskog F (1999) Hypoplasia of the sphenoid sinuses as a diagnostic tool in cystic fibrosis. Acta Radiol 40:479–485

    PubMed  Google Scholar 

  12. April MM, Zinreich SJ, Baroody FM, Naclerio RM (1993) Coronal CT scan abnormalities in children with chronic sinusitis. Laryngoscope 103:985–990

    CAS  PubMed  Google Scholar 

  13. Brihaye P, Clement PA, Dab I, Desprechin B (1994) Pathological changes of the lateral nasal wall in patients with cystic fibrosis (mucoviscidosis). Int J Pediatr Otorhinolaryngol 28:141–147

    CAS  PubMed  Google Scholar 

  14. Gentile VG,Isaacson G (1996) Patterns of sinusitis in cystic fibrosis. Laryngoscope 106:1005–1009

    CAS  PubMed  Google Scholar 

  15. Kim HJ, Friedman EM, Sulek M, Duncan NO, McCluggage C (1997) Paranasal sinus development in chronic sinusitis, cystic fibrosis, and normal comparison population: a computerized tomography correlation study. Am J Rhinol 11:275–281

    CAS  PubMed  Google Scholar 

  16. Nishioka GJ, Cook PR, McKinsey JP, Rodriguez FJ (1996) Paranasal sinus computed tomography scan findings in patients with cystic fibrosis. Otolaryngol Head Neck Surg 114:394–399

    CAS  PubMed  Google Scholar 

  17. Stern RC, Boat TF, Orenstein DM, Wood RE, Matthews LW, Doershuk CF (1978) Treatment and prognosis of lobar and segmental atelectasis in cystic fibrosis. Am Rev Respir Dis 118:821–826

    CAS  PubMed  Google Scholar 

  18. Ledesma-Medina J, Osman MZ, Girdany BR (1980) Abnormal paranasal sinuses in patients with cystic fibrosis of the pancreas. Radiological findings. Pediatr Radiol 9:61–64

    CAS  PubMed  Google Scholar 

  19. Schwartz M, Anvret M, Claustres M, Eiken HG, Eiklid K, Schaedel C, Stolpe L, Tranebjaerg L (1994) 394delTT: a Nordic cystic fibrosis mutation. Hum Genet 93:157–161

    CAS  PubMed  Google Scholar 

  20. Boman H (1996) Cystic Fibrosis Mutation Database Newsletter 69

  21. Dork T, Mekus F, Schmidt K, Bosshammer J, Fislage R, Heuer T, Dziadek V, Neumann T, Kalin N, Wulbrand U (1994) Detection of more than 50 different CFTR mutations in a large group of German cystic fibrosis patients. Hum Genet 94:533–542

    PubMed  Google Scholar 

  22. LeGrys VA (1996) Sweat testing for the diagnosis of cystic fibrosis: practical considerations. J Pediatr 129:892–897

    CAS  PubMed  Google Scholar 

  23. Libersa C, Laude M, Libersa J-C (1981) The pneumatization of the accessory cavities of the nasal fossae during growth. Anat Clin 2:265–273

    Google Scholar 

  24. Weiglein A, Anderhuber W, Wolf G (1992) Radiologic anatomy of the paranasal sinuses in the child. Surg Radiol Anat 14:335–339

    CAS  PubMed  Google Scholar 

  25. Bierny JP,Dryden R (1977) Orbital enlargement secondary to paranasal sinus hypoplasia. AJR 128:850–852

    CAS  Google Scholar 

  26. Bassiouny A, Newlands WJ, Ali H, Zaki Y (1982) Maxillary sinus hypoplasia and superior orbital fissure asymmetry. Laryngoscope 92:441–448

    CAS  PubMed  Google Scholar 

  27. Meyers RM, Valvassori G (1998) Interpretation of anatomic variations of computed tomography scans of the sinuses: a surgeon's perspective. Laryngoscope 108:422–425

    CAS  PubMed  Google Scholar 

  28. Fujioka M, Young LW (1978) The sphenoidal sinuses: radiographic patterns of normal development and abnormal findings in infants and children. Radiology 129:133

    CAS  PubMed  Google Scholar 

  29. Sonkens JW, Harnsberger HR, Blanch GM, Babbel RW, Hunt S (1991) The impact of screening sinus CT on the planning of functional endoscopic sinus surgery. Otolaryngol Head Neck Surg 105:802–813

    CAS  PubMed  Google Scholar 

  30. Collett D (1994) Strategy for model selection. In: Modelling survival data in medical research. Chapman and Hall, London, p 78

  31. Andersen DH (1938) Cystic fibrosis of the pancreas and its relation to celiac disease: a clinical and pathologic study. Am J Dis Child 56:344–399

    Google Scholar 

  32. Gibson LE, Cooke RE (1959) A test for concentration of electrolytes in sweat in cystis fibrosis of the pancreas utilizing pilocarpine iontophoresis. Pediatrics 23:545–549

    CAS  Google Scholar 

  33. LeGrys VA (2001) Assessment of sweat-testing practices for the diagnosis of cystic fibrosis. Arch Pathol Lab Med 125:1420–1424

    CAS  PubMed  Google Scholar 

  34. Rosenstein BJ, Langbaum TS, Gordes E, Brusilow SW (1978) Cystic fibrosis. Problems encountered with sweat testing. J Am Med Assoc 240:1987–1988

    CAS  Google Scholar 

  35. Green A, Dodds P, Pennock C (1985) A study of sweat sodium and chloride: criteria for the diagnosis of cystic fibrosis. Ann Clin Biochem 22:171–174

    PubMed  Google Scholar 

  36. Columbo JL, Gibbs GE (1979) Cystic fibrosis and erroneous sweat tests. Nebr Med J 64:164–165

    CAS  PubMed  Google Scholar 

  37. Kerem B, Rommens JM, Buchanan JA, Markiewicz D, Cox TK, Chakravarti A, Buchwald M, Tsui LC (1989) Identification of the cystic fibrosis gene: genetic analysis. Science 245:1073–1080

    CAS  PubMed  Google Scholar 

  38. Raman V, Clary R, Siegrist KL, Zehnbauer B, Chatila TA (2002) Increased prevalence of mutations in the cystic fibrosis transmembrane conductance regulator in children with chronic rhinosinusitis. Pediatrics 109: E13

    PubMed  Google Scholar 

  39. Wang X, Moylan B, Leopold DA, Kim J, Rubenstein RC, Togias A, Proud D, Zeitlin PL, Cutting GR (2000) Mutation in the gene responsible for cystic fibrosis and predisposition to chronic rhinosinusitis in the general population. J Am Med Assoc 284:1814–1819

    CAS  Google Scholar 

  40. Hytonen M, Patjas M, Vento SI, Kauppi P, Malmberg H, Ylikoski J, Kere J (2001) Cystic fibrosis gene mutations ∆F508 and 394delTT in patients with chronic sinusitis in Finland. Acta Laryngol 121:945–947

    Article  CAS  Google Scholar 

  41. Irving RM, McMahon R, Clark R, Jones NS (1997) Cystic fibrosis transmembrane conductance regulator gene mutations in severe nasal polyposis. Clin Otolaryngol 22:519–521

    CAS  PubMed  Google Scholar 

  42. Brihaye P, Jorissen M, Clement PA (1997) Chronic rhinosinusitis in cystic fibrosis (mucoviscidosis). Acta Otorhinolaryngol Belg 51:323–337

    CAS  PubMed  Google Scholar 

  43. Kerem E, Kerem B (1995) The relationship between genotype and phenotype in cystic fibrosis. Curr Opin Pulm Med 1:450–456

    CAS  PubMed  Google Scholar 

  44. Dipple KM, McCabe ER (2000) Modifier genes convert "simple" Mendelian disorders to complex traits. Mol Genet Metab 71:43–50

    Article  CAS  PubMed  Google Scholar 

  45. Rosenstein BJ, Langbaum TS (1987) Misdiagnosis of cystic fibrosis. Need for continuing follow-up and reevaluation. Clin Pediatr 26:78–82

    Google Scholar 

  46. Smalley CA, Addy DP, Anderson CM (1978) Does that child really have cystic fibrosis? Lancet 2:415–417

    CAS  PubMed  Google Scholar 

  47. David TJ, Phillips BM (1982) Overdiagnosis of cystic fibrosis. Lancet 2:1204–1206

    CAS  PubMed  Google Scholar 

  48. Shaw NJ, Littlewood JM (1987) Misdiagnosis of cystic fibrosis. Arch Dis Child 62:1271–1273

    CAS  PubMed  Google Scholar 

  49. Barbero GJ (1995) The undoing of a diagnosis. The effect of a misdiagnosis of a disease. Arch Pediatr Adolesc Med 149:1341–1344

    Google Scholar 

  50. Schatz CJ,Becker TS (1984) Normal CT anatomy of the paranasal sinuses. Radiol Clin North Am 22:107–118

    CAS  PubMed  Google Scholar 

  51. Anderhuber W, Weiglein A, Wolf G (1992) Nasal cavities and paranasal sinuses in newborns and children. Acta Anat (Basel) 144:120–126

    Google Scholar 

  52. Davis WE, Templer J, Parsons DS (1996) Anatomy of the paranasal sinuses. Otolaryngol Clin North Am 29:57–74

    CAS  PubMed  Google Scholar 

  53. Aoki S, Dillon WP, Barkovich AJ, Norman D (1989) Marrow conversion before pneumatization of the sphenoid sinus: assessment with MR imaging. Radiology 172:373–375

    CAS  PubMed  Google Scholar 

  54. Spaeth J, Krugelstein U, Schlondorff G (1997) The paranasal sinuses in CT imaging: development from birth to age 25. Int J Pediatr Otorhinolaryngol 39:25–40

    CAS  PubMed  Google Scholar 

  55. Duvoisin B, Landry M, Chapuis L, Krayenbuhl M, Schnyder P (1991) Low-dose CT and inflammatory disease of the paranasal sinuses. Neuroradiology 33:403–406

    Google Scholar 

  56. Marmolya G, Wiesen EJ, Yagan R, Haria CD, Shah AC (1991) Paranasal sinuses: low-dose CT. Radiology 181:689–691

    CAS  PubMed  Google Scholar 

  57. Hein E, Rogalla P, Klingebiel R, Hamm B (2002) Low-dose CT of the paranasal sinuses with eye lens protection: effect on image quality and radiation dose. Eur Radiol 12:1693–1696

    PubMed  Google Scholar 

  58. Czechowski J, Janeczek J, Kelly G, Johansen J (2001) Radiation dose to the lens in sequential and spiral CT of the facial bones and sinuses. Eur Radiol 11:711–713

    CAS  PubMed  Google Scholar 

  59. Hagtvedt T, Aaløkken J, Nøtthellen J, Kolbenstvedt A (2002) A new low-dose CT examination compared with standard-dose CT in the diagnosis of acute sinusitis. Eur Radiol (in press). DOI 10.1007/s00330-002-1652-3

  60. Eggesbø HB, Ringertz S, Haanaes OC, Dølvik S, Erichsen A, Stiris M, Kolmannskog F (1999) CT and MR imaging of the paranasal sinuses in cystic fibrosis. Correlation with microbiological and histopathological results. Acta Radiol 40:154–162

    PubMed  Google Scholar 

  61. Eggesbø HB, Stiris M, Dølvik S, Søvik S, Kolmannskog F (2001) Complementary role of MR imaging of ethmomaxillary sinus disease depicted at CT in cystic fibrosis. Acta Radiol 42:144–150

    PubMed  Google Scholar 

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Acknowledgements

We thank statistician L.M. Diep for invaluable help with the statistical analyses.

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Authors and Affiliations

  1. Department of Radiology, Aker University Hospital, 0514, Oslo, Norway

    H. B. Eggesbø

  2. Department of Physiology, Department Group of Basic Medical Sciences, University of Oslo, 0317 , Oslo, Norway

    S. Søvik

  3. Department of Otorhinolaryngology, Ullevål University Hospital, 0407 , Oslo, Norway

    S. Dølvik

  4. Department of Medical Genetics, Ullevål University Hospital, 0407 , Oslo, Norway

    K. Eiklid

  5. Sentrum Røntgeninstitutt, 0155 , Oslo, Norway

    F. Kolmannskog

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  1. H. B. Eggesbø
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Correspondence to H. B. Eggesbø.

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Eggesbø, H.B., Søvik, S., Dølvik, S. et al. Proposal of a CT scoring system of the paranasal sinuses in diagnosing cystic fibrosis. Eur Radiol 13, 1451–1460 (2003). https://doi.org/10.1007/s00330-003-1825-8

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