Lymphatic Vascular Malformations Workup

Updated: Feb 17, 2021
  • Author: Meir Cohen, MD, MPS; Chief Editor: Gregory Gary Caputy, MD, PhD, FICS  more...
  • Print

Imaging Studies

See the list below:

  • The most important imaging tool is contrast-enhanced MRI. [3] This diagnostic test, which requires sedation or general anesthesia for children younger than 6 years, demonstrates the extent of the lesion and helps to differentiate between hemangiomas and venous, lymphatic, and arterial lesions. It also may help to differentiate between a vascular lesion and a nonvascular lesion, such as those found in neurofibromatosis.

    • MRI scans have 3 basic images: T1-weighted spin-echo image, T2-weighted spin-echo image, and contrast-enhanced (gadolinium) T1-weighted spin-echo image. T refers to the time necessary for the protons to discontinue spinning (see the first image below). T1 refers to a value around 600 milliseconds, and T2 refers to a value around 4000 milliseconds. The typical findings in the 3 modes are presented in the second image below.

      MRI - MRI T1, T2. MRI - MRI T1, T2.
      Diagnosis – MRI. Diagnosis – MRI.
    • Lymphatic VMs have a typical solid appearance with low intensity on T1-weighted spin-echo image, which is equal to that of venous VMs and less intense than that of hemangiomas. [3] Contrast-enhanced T1-weighted images show a very low central intensity with a typical rim enhancement of the lymphatic lesion. The first image below shows a patient with cystic hygroma. The second image below (image on left) shows a moderate intensity at T2-weighted spin-echo image and very low central intensity with typical rim enhancement of the lymphatic lesion on contrast-enhanced T1-weighted image (image on right).

      Cystic hygroma. Cystic hygroma.
      MRI of cystic hygroma. MRI of cystic hygroma.
  • Ultrasonography helps differentiate a low-flow from a high-flow lesion. Prenatal ultrasonography can detect relatively large lesions as early as the second trimester. [14]

  • CT can detect calcifications, which are present in low-flow combined venous lymphatic lesions.


Histologic Findings

Lymphatic malformations are composed of dysplastic vesicles or pouches filled with lymphatic fluid. They can be described as either microcystic, macrocystic, or combined forms. Lymphatic VMs have walls of variable thickness, composed of both striated and smooth muscle, with nodular collections of lymphocytes in the connective tissue stroma. [5]