Isolated type III tracheal agenesis without associated congenital anomalies is very rare. Therefore, infants with this type of isolated defects are potential candidates for surgical correction. We present three cases of tracheal agenesis (Floyd's type III) with no other major abnormalities and reviewed the literature. We also discussed the previously attempted surgical procedures with the hope that in future, with advances in surgical reconstruction, there may be a better solution for this lethal anomaly.
We describe prenatal sonographic findings in a 34-week fetus with Jeune syndrome or asphyxiating thoracic dystrophy (ATD). The long bones measured were less than third percentile; the thoracic circumference (TC) measured 216 mm (< 2.5th percentile); the abdominal circumference (AC) measured 303.5 mm (50th-75th percentiles) and the rib cage perimeter (RCP) measured was 98 mm. The TC/AC was 0.70 (normal, 0.85) and the RCP/TC was 0.45 (normal, 0.68). Following birth diagnosis of Jeune syndrome was made based on radiographic analysis, which was subsequently confirmed by clinical and postmortem examination. This case highlights the utility of both TC/AC and RCP/TC in diagnosis of ATD and other skeletal dysplasias associated with a small thorax.
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