Pediatric Lymphatic Malformation

  • Etiology: endothelial lined cavernous lymphatic spaces
  • Imaging: may be multicentric, transpatial extension, mediastinal extension with airway compression, macrocystic (mean diameter of cystic lesions > 1 cm)/ microcystic (mean diameter of cystic lesions < 1 cm) / mixed, in microcystic form individual cysts may not be perceptible, cysts my be heterogeneous depending on their protein or hemorrhage content, hemorrhage into cysts forms fluid-fluid levels, thickened septa contains venous component which may enhance
  • US: contents usually anechoic, may contain debris, high-lipid content, infection, blood
    arterial and venous flow in septa
  • CT: usually homogenous and cystic
  • MR: T1 variable signal intensity depending on protein content and hemorrhage, T2 high signal intensity with fluid-fluid levels from hemorrhage, septal enhancement
  • Clinical: present at birth and grow with patient, often present with acute swelling from spontaneous hemorrhage in venolymphatic malformation or infection, often in posterior cervical space, 75% on head / neck / torso, 25% in axilla, can also be in orbit, if seen prenatally consider delivery via an EX utero Intrapartum Treatment (EXIT) procedure

Radiology Cases of Lymphatic Malformation

MRI of lymphatic malformation
Transverse (above) and sagittal (below) US of the neck and coronal (above) and axial T2 MRI without contrast of the neck shows an infiltrating multicystic mass of the neck surrounding the trachea that has minimal vascularity.
MRI of lymphatic malformation of orbit
Coronal T1 MRI without contrast (left) shows a round low signal intensity left medial canthus lesion that is high signal intensity on T2 (middle) and that does not enhance with contrast (right).
MRI of lymphatic malformation of the chest wall
Axial T1 without contrast (top), T2 (middle) and T1 with contrast (bottom) MRI of the chest shows a mass composed of multiple large fluid-filled structures separated by thin septations which faintly enhance.
CXR and CT of lymphatic malformation of the thymus
CXR AP obtained intially (left) shows a normal appearing exam with a normal appearing thymus. CXR AP obtained 10 days later for continued respiratory distress (right) showed interval development of an anterior mediastinal mass. Axial+coronal CT with contrast of the chest shows a large low density anterior mediastinal mass with multiple thin septations.

Clinical Cases of Lymphatic Malformation

Clinical image of lymphatic malformation
Clinical images (above) show a large bilateral neck and facial mass which was demonstrated by transillumination (below) to be cystic.