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Page 2 of 8 Grünherz et al. Plast Aesthet Res 2023;10:20 https://dx.doi.org/10.20517/2347-9264.2023.24

Thoracic duct injuries typically occur in the setting of head and neck surgery such as neck dissection,
pharyngectomy, esophagectomy or thyroidectomy or after heart surgery. After lateral neck dissection, the
incidence is 4.5-8.3%, with the risk increasing with the presence of metastases at the angulus venosus as well
[3-8]
as the extent of surgery . Depending on the anatomic location involved, patients suffer from leakage of
chyle in the area of the surgical wound, recurrent thoracic (chylothorax) or abdominal chylous effusions
(chylous ascites), which favor infections and further complications due to permanent protein and fluid loss
leading to a mortality of up to 50% [9-10] .

Lesions of the central lymphatic system or lymphatic dysfunction due to high venous pressure can also
result from Fontan surgery in patients with congenital heart disease with single-ventricle physiology. In
these patients, lymphatic dysfunction can lead to hepatic lymphatic congestion, protein-losing enteropathy
and plastic bronchitis due to the formation of rubbery and caulk-like plugs in the airways, causing severe
respiratory issues. Protein-losing enteropathy is caused by permanent enteric protein loss, resulting in
[11]
hypoalbuminemia, lymphopenia, hypogammaglobulinemia, and loss of clotting factors .

Congenital malformations of the lymphatic system include central conducting lymphatic anomalies (CCLA)
and lymphocele.

Due to the rarity of central lymphatic lesions, diagnosis and treatment should be reserved for specialized
lymphatic centers. In addition to a detailed history, diagnosis is based on special imaging, preferably
magnetic resonance lymphangiography (MRL), which allows accurate visualization of the central lymphatic
[12]
lesion and is essential for precise treatment planning . In the case of lymphatic lesions in childhood, a
[1]
broad genetic workup should also be performed .
TREATMENT ALGORITHM
We previously established a treatment algorithm based on a series of cases with central lymphatic lesions,
which is shown in [Figure 1]. Initial treatment of central lymphatic lesions should be based on conservative
therapy including a diet of medium-chain triglycerides (MCT) or total parenteral nutrition (TPN).
Although success rates vary widely, this therapy can lead to spontaneous resolution of the chyle leak [13,14] . In
addition, we propose to combine dietary measures with an Octreotide therapy that is supposed to reduce
the production of chyle. Especially in patients with central conducting lymphatic anomaly (CCLA), other
substances such as propanolol, sirolimus, sildenafil or trametinib are increasingly used [15-18] .

When conservative treatment fails, lymphangiography-guided interventions remain the treatment of choice
for central lymphatic lesions and allow for different treatment modalities depending on the entity of the
lesion. Lymphangiography alone has been shown to succeed in different sites of lymphatic leakage due to its
known Lipiodol-induced selective blockage of pathological lymph ducts.

Lymphangiography-guided embolization of the affected central lymphatics by endovascular coils or liquid
embolic agents has been shown to be effective in postoperative chylous fistula, chylothorax, and chylous
ascites with cure rates of 80-90% and overall complication rate of 7% [19,22] . However, it has to be considered
that occlusion of the thoracic duct carries the risk of protein-losing enteropathy, lower extremity
lymphedema, or worsening of lymphatic reflux with a fistula at another site due to the resulting down-
stream congestion . The same accounts for the surgical ligation of the thoracic duct, which has also been
[23]
performed to treat patients with chyle leaks in the past [8,24] . In recent years, micro- and super-microsurgical
[25]
reconstruction, defined as microsurgery in less than 0.8 mm vessels , has opened new frontiers to
successfully treat rare central lymphatic lesions by thoracic duct-vein anastomoses.