Imaging of central lymphatics in children with Both Trisomy 21 and congenital heart disease

Abstract

Objective

To describe the lymphatic imaging findings in patients with both Trisomy 21 and congenital heart disease and to depict the most common central lymphatic abnormalities in these patients.

Materials and methods

We conducted a single-center retrospective review of patients with a confirmed history of Trisomy 21 and congenital heart disease who presented for lymphatic imaging over a 7-year period. Clinical history and outcomes were extracted from the medical records. Lymphatic distribution and flow were evaluated by two pediatric radiologists from dynamic contrast-enhanced MR lymphangiography studies, which included T2-weighted and dynamic contrast-enhanced T1-weighted images, conventional lymphangiography studies were also evaluated.

Results

We identified 16 patients (12 male): 8 infants, 6 children, and 2 adults. 12 patients had cardiac surgery including Fontan (n = 5), hemi-Fontan (n = 1), Glenn (n = 1), and other cardiac surgeries (n = 5). Presenting symptoms included chylothorax, plastic bronchitis, chylous ascites, protein losing enteropathy, pericardial effusion, lymphedema, and anasarca. Two patients died (12 %) by the time of data collection. T2-weighted MR imaging demonstrated lymphatic edema in all patients. T1-weighted dynamic imaging revealed abnormal pulmonary and/or mesenteric lymphatic perfusion in 15 patients (88 %) across intranodal, intrahepatic, and intramesenteric access types. The thoracic duct was tortuous and/or dilated in most cases. Conventional lymphangiography confirmed thoracic duct obstruction.

Conclusion

This is a descriptive study of central lymphatic diseases in patients with Trisomy 21, congenital heart disease and clinical evidence of lymphatic dysfunction. Common findings in these patients include retrograde dermal lymphatic flow, pulmonary and mesenteric lymphatic perfusion as well as the presence of a dilated and tortuous duct.