Skip to main content
SpringerLink
Log in

Beyond pulmonary embolism: Alternative diagnosis and incidental findings on CT pulmonary angiography in sickle cell disease

  • Original Article
  • Published:
Emergency Radiology Aims and scope Submit manuscript

Abstract

Background

Sickle cell disease (SCD) is a genetic hematological disorder associated with severe complications, such as vaso-occlusive crises, acute chest syndrome (ACS), and an increased risk of thromboembolic events, including pulmonary embolism (PE). The diagnosis of PE in SCD patients presents challenges due to the overlapping symptoms with other pulmonary conditions. Our previous study revealed that nearly 96% of computed tomography pulmonary angiography (CTPA) scans in SCD patients were negative for PE, highlighting a gap in understanding the significance of CTPA findings when PE is absent.

Methods

In this retrospective follow-up study conducted at the Salmaniya Medical Complex in Bahrain, we examined SCD patients with HbSS genotypes who underwent CTPA from January 1, 2018, to December 31, 2021, for suspected PE, but the results were negative. The aim of this study was to identify alternative diagnoses and incidental findings from CTPA scans. Experienced radiologists reviewed the CTPA images and reports to assess potential alternative diagnoses and incidental findings, incorporating an additional analysis of chest X-rays to evaluate the diagnostic value of CTPA. Incidental findings were classified based on their location and clinical significance.

Results

Among the 230 evaluated SCD patients (average age 39.7 years; 53% male) who were CTPA negative for PE, 142 (61.7%) had identifiable alternative diagnoses, primarily pneumonia (49.1%). Notably, 88.0% of these alternative diagnoses had been previously suggested by chest radiographs. Furthermore, incidental findings were noted in 164 (71.3%) patients, with 11.0% deemed clinically significant, necessitating immediate action, and 87.8% considered potentially significant, requiring further assessment. Notable incidental findings included thoracic abnormalities such as cardiomegaly (12.2%) and an enlarged pulmonary artery (11.3%), as well as upper abdominal pathologies such as hepatomegaly (19.6%), splenomegaly (20.9%), and gallstones (10.4%).

Conclusion

This study underscores the limited additional diagnostic yield of CTPA for identifying alternative diagnoses to PE in SCD patients, with the majority of diagnoses, such as pneumonia, already suggested by chest radiographs. The frequent incidental findings, most of which necessitate further evaluation, highlight the need for a cautious and tailored approach to using CTPA in the SCD population.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7

Similar content being viewed by others

References

  1. Collaborators GBDSCD (2023) Global, regional, and national prevalence and mortality burden of sickle cell disease, 2000–2021: a systematic analysis from the global burden of Disease Study 2021. Lancet Haematol 10(8):e585–e599

    Article  Google Scholar 

  2. Ogu UO, Badamosi NU, Camacho PE, Freire AX, Adams-Graves P (2021) Management of Sickle Cell Disease complications Beyond Acute chest syndrome. J Blood Med 12:101–114

    Article  PubMed  PubMed Central  Google Scholar 

  3. Conran N, De Paula EV (2020) Thromboinflammatory mechanisms in sickle cell disease - challenging the hemostatic balance. Haematologica 105(10):2380–2390

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  4. Tivnan P, Billett HH, Freeman LM, Haramati LB (2018) Imaging for Pulmonary Embolism in Sickle Cell Disease: a 17-Year experience. J Nucl Med 59(8):1255–1259

    Article  PubMed  Google Scholar 

  5. Mekontso Dessap A, Deux JF, Abidi N, Lavenu-Bombled C, Melica G, Renaud B, Godeau B, Adnot S, Brochard L, Brun-Buisson C et al (2011) Pulmonary artery thrombosis during acute chest syndrome in sickle cell disease. Am J Respir Crit Care Med 184(9):1022–1029

    Article  PubMed  Google Scholar 

  6. Adedeji MO, Cespedes J, Allen K, Subramony C, Hughson MD (2001) Pulmonary thrombotic arteriopathy in patients with sickle cell disease. Arch Pathol Lab Med 125(11):1436–1441

    Article  CAS  PubMed  Google Scholar 

  7. Chamberlin JH, Ogbonna A, Abrol S, Maisuria D, Miller E, McGuire A, Knight H, O’Doherty J, Baruah D, Schoepf UJ et al (2024) Enhancing diagnostic precision for acute chest syndrome in sickle cell disease: insights from dual-energy CT lung perfusion mapping. Emerg Radiol 31(1):73–82

    Article  PubMed  Google Scholar 

  8. Fakunle EE, Eteng K, Shokunbi WA (2012) DD dimer levels in patients with sickle cell disease during bone pain crises and in the steady state. Pathol Lab Med Int 4:21–25

    Article  CAS  Google Scholar 

  9. Al Dandan O, Hassan A, Alnasr A, Al Gadeeb M, AbuAlola H, Alshahwan S, Al Shammari M, Alzaki A (2020) The use of clinical decision rules for pulmonary embolism in the emergency department: a retrospective study. Int J Emerg Med 13(1):23

    Article  PubMed  PubMed Central  Google Scholar 

  10. Konstantinides SV, Meyer G, Becattini C, Bueno H, Geersing GJ, Harjola VP, Huisman MV, Humbert M, Jennings CS, Jimenez D et al (2019) 2019 ESC guidelines for the diagnosis and management of acute pulmonary embolism developed in collaboration with the European Respiratory Society (ERS): the Task Force for the diagnosis and management of acute pulmonary embolism of the European Society of Cardiology (ESC). Eur Respir J 54(3):1901647

    Article  PubMed  Google Scholar 

  11. Al Dandan O, Hassan A, AbuAlola H, Alzaki A, Alwaheed A, Alalwan M, Al Shammari M, AlShamlan N, Alsaif HS (2020) Clinical and imaging profiles of pulmonary embolism: a single-institution experience. Int J Emerg Med 13(1):47

    Article  PubMed  PubMed Central  Google Scholar 

  12. Winer JC, Yee ME, Ataga KI, Lebensburger JD, Zahr RS (2022) Patients with sickle cell disease who develop end-stage kidney disease continue to experience poor survival - A 19-year United States Renal Data System study. Br J Haematol 199(5):e43–e47

    Article  PubMed  PubMed Central  Google Scholar 

  13. Jensen J, Lin T, Fishman EK, Johnson PT (2017) Pulmonary CTA in sickle cell patients: quantitative assessment of enhancement quality. Emerg Radiol 24(6):667–674

    Article  PubMed  Google Scholar 

  14. Hassan A, Taleb M, Hasan W, Shehab F, Maki R, Alhamar N (2023) Positive rate and quality assessment of CT pulmonary angiography in sickle cell disease: a case–control study. Emerg Radiol 30(2):209–216

    Article  PubMed  PubMed Central  Google Scholar 

  15. Eskandari A, Narayanasamy S, Ward C, Priya S, Aggarwal T, Elam J, Nagpal P (2022) Prevalence and significance of incidental findings on computed tomography pulmonary angiograms: a retrospective cohort study. Am J Emerg Med 54:232–237

    Article  PubMed  Google Scholar 

  16. Anjum O, Bleeker H, Ohle R (2019) Computed tomography for suspected pulmonary embolism results in a large number of non-significant incidental findings and follow-up investigations. Emerg Radiol 26(1):29–35

    Article  PubMed  Google Scholar 

  17. Ferreira EV, Gazzana MB, Sarmento MB, Guazzelli PA, Hoffmeister MC, Guerra VA, Seligman R, Knorst MM (2016) Alternative diagnoses based on CT angiography of the chest in patients with suspected pulmonary thromboembolism. J Bras Pneumol 42(1):35–41

    Article  PubMed  PubMed Central  Google Scholar 

  18. Perelas A, Dimou A, Saenz A, Rhee JH, Teerapuncharoen K, Rowden A, Eiger G (2015) Incidental findings on computed tomography angiography in patients evaluated for pulmonary embolism. Ann Am Thorac Soc 12(5):689–695

    Article  PubMed  Google Scholar 

  19. van Es J, Douma RA, Schreuder SM, Middeldorp S, Kamphuisen PW, Gerdes VEA, Beenen LFM (2013) Clinical impact of findings supporting an alternative diagnosis on CT pulmonary angiography in patients with suspected pulmonary embolism. Chest 144(6):1893–1899

    Article  PubMed  Google Scholar 

  20. Chandra S, Sarkar PK, Chandra D, Ginsberg NE, Cohen RI (2013) Finding an alternative diagnosis does not justify increased use of CT-pulmonary angiography. BMC Pulm Med 13(1):9

    Article  PubMed  PubMed Central  Google Scholar 

  21. Ballas SK, Lieff S, Benjamin LJ, Dampier CD, Heeney MM, Hoppe C, Johnson CS, Rogers ZR, Smith-Whitley K, Wang WC et al (2010) Definitions of the phenotypic manifestations of sickle cell disease. Am J Hematol 85(1):6–13

    Article  PubMed  PubMed Central  Google Scholar 

  22. Seck M, Ba O, Faye BF, Touré SA, Guèye YB, Dieng N, Sall A, Gadji M, Touré AO, Diop S (2021) Homozygous sickle cell disease related mortality in Senegal (2011–2020). eJHaem 2(4):711–715

    Article  PubMed  PubMed Central  Google Scholar 

  23. Evans CS, Arthur R, Kane M, Omofoye F, Chung AE, Moreton E, Moore C (2022) Incidental radiology findings on computed Tomography studies in Emergency Department patients: a systematic review and Meta-analysis. Ann Emerg Med 80(3):243–256

    Article  PubMed  Google Scholar 

  24. Davenport MS (2023) Incidental findings and low-value care. Am J Roentgenol 221(1):117–123

    Article  Google Scholar 

  25. Waterbrook AL, Manning MA, Dalen JE (2018) The significance of incidental findings on computed tomography of the chest. J Emerg Med 55(4):503–506

    Article  PubMed  Google Scholar 

  26. Mohamed SOO, Ibrahim OAO, Mohammad DAA, Ali AHM (2021) Correlates of gallbladder stones among patients with sickle cell disease: a meta-analysis. JGH Open 5(9):997–1003

    Article  PubMed  PubMed Central  Google Scholar 

  27. Praharaj DL, Anand AC (2021) Sickle Hepatopathy. J Clin Exp Hepatol 11(1):82–96

    Article  CAS  PubMed  Google Scholar 

  28. Simonson JL, Pandya D, Kang J, Talwar A, Zaidi GZ (2021) Enlarged pulmonary artery on computed tomography and respiratory failure in sickle cell disease acute chest syndrome. Pulm Circ 11(2):20458940211018345

    Article  PubMed  PubMed Central  Google Scholar 

  29. Klings ES, Machado RF, Barst RJ, Morris CR, Mubarak KK, Gordeuk VR, Kato GJ, Ataga KI, Gibbs JS, Castro O et al (2014) An official American Thoracic Society clinical practice guideline: diagnosis, risk stratification, and management of pulmonary hypertension of sickle cell disease. Am J Respir Crit Care Med 189(6):727–740

    Article  PubMed  PubMed Central  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Radiology Department, Governmental Hospitals, Salmaniya Medical Complex, Manama, Bahrain

    Ali Hassan, Reem Maki & Jalila Adnan

  2. Radiology Department, Eastern Health Cluster, Dammam, Saudi Arabia

    Mahdi Aljawad, Ali Alzayer, Ali Habeeb, Aqeel Alzaher, Adnan Alawami & Fatimah Alaithan

Authors
  1. Ali Hassan
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Reem Maki
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Mahdi Aljawad
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Ali Alzayer
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Ali Habeeb
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Aqeel Alzaher
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Adnan Alawami
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Fatimah Alaithan
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Jalila Adnan
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Ali Hassan.

Ethics declarations

Conflict of interest

Nothing to Disclose.

Additional information

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Hassan, A., Maki, R., Aljawad, M. et al. Beyond pulmonary embolism: Alternative diagnosis and incidental findings on CT pulmonary angiography in sickle cell disease. Emerg Radiol (2024). https://doi.org/10.1007/s10140-024-02229-x

Download citation

  • Received:

  • Accepted:

  • Published:

  • DOI: https://doi.org/10.1007/s10140-024-02229-x

Keywords

Search

Navigation