Type:
Educational Exhibit
Keywords:
Foetal imaging, Paediatric, Thorax, MR, CT, Conventional radiography, Diagnostic procedure, Foetus, Congenital, Cysts
Authors:
R. Llorens, G. Montoliu, A. Moreno , F. Menor; Valencia/ES
DOI:
10.1594/ecr2013/C-0898
Background
Congenital thoracic malformations represent a wide spectrum of developmental anomalies whose origin is not well stablished.
Although they are rare,
its clinical importance is remarkable.
We are going to focus on those malformations of cystic nature.The most common of them are those affecting the lungs:
- Congenital pulmonary airway malformation or CPAM.
- Bronchopulmonary sequestration and hybrid lesions.
- Bronchogenic cyst.
- Bronchial atresia.
There are even more rare congenital cystic thoracic anomalies that do not affect the lung but other chest structures:
- Mediastinal or pericardial teratomas.
- Mediastinal or axillary lymphangiomas.
- Chest wall tumors.
Diaphragmatic hernia is also another entity to be aware of because its prenatal appeareance might be confused with a cystic pulmonary malformation.
Nowadays,
congenital thoracic malformations are usually detected in prenatal studies.
At present, ultrasound (US) and magnetic resonance imaging (MRI) are the diagnostic tools by which non-invasively examine fetal chest development.
Although US remains as the primary screening method for thoracic anomalies and for identifying specific lung lesions,
mediastinal shift or the presence of pleural effusion,
foetal MRI can obtain further relevant information about the nature or prognosis of these lesions,like the presence of lung hypoplasia.
Therefore,
advanced prenatal imaging techniques help to parental counseling and perinatal assessment.
Postnatal imaging should always start with a radiography because it gives the most valuable information in a fast and unexpensive fashion. If a cystic component or vascular supplies are suspected within a lesion, US and Doppler examination are very useful. Once the diagnosis of a congenital thoracic mass or lesion is made by radiography and/or Doppler-US,
other studies like contrast enhancement Computed Tomograghy (CeCT) or MRI might be required to confirm prenatal diagnosis,rule out complications and decide the surgical planning.
Surgical removal is the most frequent treatment of these malformations and the time of surgery will depend on the clinical status of the patient.
For instance,
if a neonate presents with respiratory distress because of a big CPAM,
surgery must be performed in first days of life.
Nevertheless,if the neonate is asymptomatic and the diagnosis of a CPAM is made with a plain film and/or US,
another study like a CeCT would be not necessary until surgery is considered to be performed (usually around 6 months of life).