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2019

ANUÁRIO DO HOSPITAL
DONA ESTEFÂNIA

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Cystic fibrosis – The role of imaging in a multi-organ disease

João Almeida 1, Rita Prata 2, Cecília Leal 3, Luísa Figueiredo 4

1 - Interno de Radiologia, Centro Hospitalar Lisboa Central, Lisboa, Portugal
2 - Interna de Radiologia, Hospital Central do Funchal, Funchal, Madeira, Portugal / Centro Hospitalar Lisboa Central (CHLC), Lisboa, Portugal
3 - Assistente de Radiologia do Hospital Santa Marta (CHLC), Lisboa, Portugal
4 - Chefe de Serviço de Radiologia do Centro Hospitalar Lisboa Central (CHLC), Lisboa, Portugal

- 2018 AIRP (American Institute for Radiologic Pathology) Course
- 18 - 20 Outubro. Porto – Portugal. Póster Educacional

Abstract:
Objectives: Cystic fibrosis is an autosomal recessive genetic disorder leading to impairment of exocrine function affecting many organs, most often with pulmonary, -hepatic, pancreatic and paranasal sinus involvement. It is associated with progressive disability and major impact on quality of life. Our purpose is to review the multi-organ imaging findings in cystic fibrosis based on conventional imaging, computed tomography and magnetic resonance, with clinical, diagnostic and therapeutic relevance.
Methods: We retrospectively reviewed 21 patients diagnosed with cystic fibrosis between January 2010 and August 2018 in our center. Patient sample was obtained through systematic search of the radiology department database from our institution.
Results: Pulmonary manifestations are the main findings, including thick-walled bronchiectasis with progression from cylindrical to cystic forms, mucus plugging, air trapping and recurrent pulmonary infections. Extrapulmonary manifestations may also be found, such as sinonasal disease (rhinosinusitis, nasal polyps), pancreatic (fatty replacement, cysts), hepatic (steatosis, cirrhosis) and gastrointestinal disease (meconium ileus, intussusception).
Conclusion: Early diagnosis and adequate therapy can improve the overall expectancy and quality of life of affected patients. Radiologists have an important role in the diagnosis and monitoring of cystic fibrosis and its complications.