Central Conducting Lymphatic Anomaly (CCLA) is a disease that affects large lymphatic vessels in the middle of the torso, resulting in leakage or blockage of normal drainage of lymph fluid. Symptoms can include fluid build-up in and around the lungs, around the heart, in the abdomen and in the feet and legs. CCLA can also affect the bones.

Some CCLAs are associated with a EPHB4 or ARAF mutation.

The lungs in dire straits

When chyle accumulating in the linings of the heart and/or lungs dangerous – also lethal – situations can occur. The accumulations are described based on location:

  • chylothorax is chyle in the chest, compressing the lungs
  • chylopericardium is chyle trapped inside the sac surrounding the heart
  • chyloascites is chyle trapped in the linings of the abdomen and abdominal organs

The presence of chyle in these places can also be part of GLA and GSD.

Who has CCLA?

CCLA can occur at any age, but the incidence is highest in children and teenagers. Signs and symptoms are typically present before the age of 20. The condition is often under-recognized in adults. It strikes males and females of all races and exhibits no inheritance pattern. The medical literature contains case reports from every continent.

After Michio Ozeki, Toshiyuki Fukoa, 2019.