Tracheal narrowing in children and adults with mucopolysaccharidosis type IVA: evaluation with computed tomography angiography
| Autor: | Colleen Ditro, Shunji Tomatsu, Christian Pizarro, Lauren W. Averill, Heidi H. Kecskemethy, William G. Mackenzie, Mary C. Theroux, Michael B. Bober |
|---|---|
| Rok vydání: | 2021 |
| Předmět: |
congenital
hereditary and neonatal diseases and abnormalities Morquio syndrome medicine.diagnostic_test business.industry Thyroid Ultrasound Anatomy respiratory system medicine.disease Mucopolysaccharidosis Type IVA 030218 nuclear medicine & medical imaging 03 medical and health sciences 0302 clinical medicine medicine.anatomical_structure medicine.artery Pediatrics Perinatology and Child Health cardiovascular system Brachiocephalic artery medicine Radiology Nuclear Medicine and imaging Respiratory system business 030217 neurology & neurosurgery Computed tomography angiography Neuroradiology |
| Zdroj: | Pediatric Radiology. 51:1202-1213 |
| ISSN: | 1432-1998 0301-0449 |
| DOI: | 10.1007/s00247-020-04946-0 |
| Popis: | Mucopolysaccharidosis type IVA (MPS IVA) is characterized by progressive skeletal dysplasia and respiratory issues with difficult airway management during anesthesia. To characterize tracheal abnormalities in children and adults with MPS IVA including interplay of the trachea, vasculature, bones and thyroid at the thoracic inlet. Computed tomography (CT) angiograms of the chest were analyzed for trachea shape, narrowing and deviation at the thoracic inlet, course of vasculature, bone alignment and thyroid location. The tracheal cross-sectional area was measured at the cervical, thoracic inlet and intrathoracic levels. Thirty-seven patients (mean age: 18.1 years) were included. The mean tracheal cross-sectional area narrowing at the thoracic inlet was 63.9% (range: −2.1–96%), with a trend for increased tracheal narrowing in older children. The trachea was commonly deviated rightward posterior (22/37, 59%). T- or W-shaped tracheas had two times greater tracheal narrowing than D- or U-shaped tracheas (P |
| Databáze: | OpenAIRE |
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