JAVIER PACHECO PATERNINA

FACULTAD DE MEDICINARADIOLOGIA E IMÁGENES DIAGNÓSTICAS

UNIVERSIDAD DE CARTAGENA2014

Introduction

M.Y. Ng et al. / Clinical Radiology 69 (2014) e153ee162

Cystic fibrosis (CF) is an incurable autosomal recessive

disease that affects approximately 1 in 2500 live births

among caucasians.

Mutation in the gene coding for

the CF transmembrane

conductance regulator (CFTR)

protein.

Expressed in many different

organs and acts primarily as

a chloride channel.

CFTR dysfunction

In the lung leads todehydration of theairway surface liquid

cycle of pulmonaryinfection andinflammation

Reducedmucociliaryclearance

In the majority of cases, CF leads to progressive respiratory failure and premature death.

Bronchiectasis and small airway disease

M.Y. Ng et al. / Clinical Radiology 69 (2014) e153ee162

Permanently dilated

airways and is the hallmark

of CF lung disease.

«Un ciclo de infección e inflamación recurrente conlleva al

desarrollo de bronquiectasias, que afecta típicamente a los

lóbulos superiores en la FQ.»

Recent data have shown a significant correlation between the extent of bronchiectasis and

reduced survival amongst patients with CF.

Rx:

- Tram-lining and cystic lesions containing airefluid levels.

TC:

- «signo del anillo de sello»

- Engrosamiento bronquial.

Figure 1.

Typical chest radiograph of a

patient with CF demonstrating

bilateral generalized bronchial

wall thickening, tram-lining

indicating bronchiectasis, and

the presence of a TIVAD in the

right hemi-thorax.

Figure 3.

CF patient with ABPA. Extensive bronchiectasis with large mucous plugs in the middle lobe and right

lower lobe. However, this appearance is not infrequently seen in CF patients.

Chronic endobronchial colonization

M.Y. Ng et al. / Clinical Radiology 69 (2014) e153ee162

Pseudomonas aeruginosa

Staphylococcus aureus

Complejo Burkholderia cepacia• Disminución acelerada de la función

pulmonar

• Responsable de brotes devastadores dentro

de las unidades de pacientes con FQ.

B. cepacia bacteriemia neumonía necrotizante "síndrome cepacia".

Figure 2. Cepacia syndrome: fatal

necrotizing pneumonia associated with B.

cenocepacia bacteraemia in an adult with

CF. (a) Baseline check radiograph. (b)

Radiograph taken close to admission

shows early nodular consolidation in the

left lung. (c) Nine days later, further

progression of the nodular consolidation

can be clearly seen in both lungs. These

nodular areas over the next 6 days then

become mass-like, and are seen in the

lower zone bilaterally with the mass-like

consolidation in the left lower zone

showing possible cavitation.

Unfortunately, the patient developed

progressive respiratory failure and died

despite maximal medical therapy.

Aspergillus-related lung disease

M.Y. Ng et al. / Clinical Radiology 69 (2014) e153ee162

Se aísla con frecuencia de las

vías respiratorias de los pacientes

con FQ.

El espectro de la enfermedad pulmonar:

Aspergilosis

broncopulmon

ar alérgica

(ABPA)

Aspergiloma

Aspergilosis

necrotizante

crónica

Studies have indicated that 2–10% of patients with CF have ABPA.

«Puede ser muy difícil de diagnosticar radiológicamente debido a un solapamiento considerable con el

de bronquiectasias típica y taponamiento mucoso visto en la mayoría de los pacientes con FQ»Figure 4. CF patient with semi-invasive aspergillosis. The axial images show a large cavitary lesion in

the right middle lobe with thick walls. On the sagittal images, this cavitary lesion can be seen to cross

the oblique fissure and involve the upper lobe.

NTM infection

M.Y. Ng et al. / Clinical Radiology 69 (2014) e153ee162

The radiological appearances of NTM disease have been classified into two main forms:

Classical (also known as cavitary) and non-classical (bronchiectatic).

In the classical form, cavities are typically seen in the upper lobes with adjacent

nodules.

The bronchiectatic form typically involves cylindrical bronchiectasis with centrilobular

nodule formation, which can range from small to large nodules.

23% of patients with CF• Mycobacterium avium intracellulare

• Mycobacterium abscessus

Contraindicación relativa

para el trasplante de

pulmón.

Figure 5. Chronic M. abscessus infection in a 25-year-old patient with CF. The patient presented with declining lung function and pyrexia.

This thoracic high-resolution CT image shows evidence of bronchiectasis (white arrowheads) and widespread bilateral nodules

(white arrows), which would be in keeping with the non-classical/bronchiectatic form of NTM. Long-term therapy with a

combination of oral and nebulized anti-mycobacterial drugs was commenced.

Pneumothorax

M.Y. Ng et al. / Clinical Radiology 69 (2014) e153ee162

Pneumothorax has an incidence of 0.64% per year among patients with CF.

Risk factors for pneumothorax in CF include: P. aeruginosa or B. cepacia complex

infection, ABPA, previous massive haemoptysis, and a forced expiratory volume in 1 s

(FEV1) of <30%–predicted.

Pneumothoraces in CF are a poor prognostic indicator with nearly 50% of patients

dying within 2 years.

Figure 6. Left-sided pneumothorax in end-stage CF. This patient had end-stage CF lung disease and was on the

transplant list. Unfortunately, the patient died of progressive respiratory failure within 2 weeks of pneumothorax

despite drainage, pleurodesis, intravenous antibiotics, and supportive interventions

Atelectasis, lobar collapse, and idiopathic collapsed lung

M.Y. Ng et al. / Clinical Radiology 69 (2014) e153ee162

Atelectasis and lobar collapse are well-recognized complications of CF and occur in

4–11% of patients.

The upper lobes are most frequently affected and mucus plugging is thought to

have a central role in the pathophysiology of this process.

Lung transplantation is an option in severely asymmetrical lung disease but is

considered high risk.

Figure 8.

Right upper lobe collapse in a 18-year-old man

with CF who was chronically infected with

Ralstonia mannitolilytica and methicillin-resistant

S. aureus. (a) The baseline appearances for this

patient with the subsequent development of right

upper-zone opacification (b). (c) Evidence of

progression to complete collapse of the right

upper lobe as evidenced by volume loss with

a vertically orientated horizontal fissure, right

hilum is pulled up, and the trachea is

displaced to the right. At this point, the patient

received intensive in-patient therapy from a

specialist CF team. Treatment included

intravenous antibiotics, inhaled mucolytics, chest

physiotherapy, and intermittent positive-pressure

breathing with resolution of the lobar collapse (d).

A new cavitary lesion was noted in the right upper

zone, which cleared with further antibiotic therapy.

La Figura 9.

Idiopática pulmón colapsado. (a) Radiografía de tórax de un paciente con FQ mayores de 18 años. (b) En una radiografía posterior 2 años

más tarde, nueva consolidación se muestra con ligera pérdida de volumen en el pulmón derecho. (c) 1 año más tarde, la radiografía de

tórax muestra la consolidación más florida y el colapso casi total del pulmón derecho. (d) CT imagen coronal que muestra el pulmón

derecho colapsado con broncogramas y bronquiectasia sin evidencia de obstrucción en las vías respiratorias visibles. (e-f).

Pulmonary hypertension and cor pulmonale

M.Y. Ng et al. / Clinical Radiology 69 (2014) e153ee162

Pulmonary hypertension is defined as a mean pulmonary arterial pressure ≥25 mmHg and in CF

patients may result from chronic hypoxia, which is characteristic of advanced CF lung disease.

Definitive diagnosis of pulmonary hypertension requires right heart catheterization, but the

diagnosis may be strongly suggested by echocardiography or a number of imaging methods.

The chest radiograph in pulmonary hypertension may show:

Enlargement of the pulmonary trunk

Elevation of the cardiac apex suggesting an increase in the size of the right heart

Enlargement of the right interlobar artery (>15 mm for females and 16 mm for males), or

pruning of the peripheral arteries.

At thoracic CT, the signs most likely to indicate pulmonary hypertension are

enlargement of the pulmonary artery by >31.5 mm or a mean pulmonary artery

diameter/ascending aorta diameter (mPAD/AAD) ratio >1

Figure 10.

Pulmonary hypertension in an adult with CF subsequently confirmed with right heart catheterization. (a)

Axial CT section showing a dilated right ventricle, which is much larger than the left ventricular

cavity. (b) A dilated pulmonary trunk measuring 35 mm (red line), while the ascending thoracic aortic

diameter measures 24 mm, which indicates a mPAD/AAD ratio >1.

Complications of totally implantable venous access devices(TIVAD)

M.Y. Ng et al. / Clinical Radiology 69 (2014) e153ee162

Venous stenosis due to a TIVAD. (a) A SVC venogram demonstrating mid-SVC occlusion. (b) A wire is

passed through the occlusion and the tip of the TIVAD has been snared into the right brachiocephalic vein

in order that the stent can be deployed. (c) The stent is deployed across the SVC with its superior edge

below the confluence of the brachiocephalic veins. There is a significant waist to the SVC stent, which is

subsequently dilated with a 15 mm balloon. (d) The stent with no significant waist and the tip of the TIVAD

is now back in the SVC having been snared and re-sited to its original position. A repeat venogram shows

successful opening of the occlusion.

Role of CT and chest radiography

M.Y. Ng et al. / Clinical Radiology 69 (2014) e153ee162

M.Y. Ng et al. / Clinical Radiology 69 (2014) e153ee162