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Page 4 of 8 Grünherz et al. Plast Aesthet Res 2023;10:20 https://dx.doi.org/10.20517/2347-9264.2023.24

in the fluid and by conventional lymphangiography on postoperative day five. Conservative treatment based
on a low-fat medium-chain triglyceride diet and an additional treatment with Octreotide was continued for
five weeks but remained unsuccessful. A missing cisterna chyli, which would have allowed percutaneous
access to the thoracic duct, led to the decision to perform microsurgical reconstruction of the thoracic duct.
During surgical revision, parenteral administration of high-fat cream at the beginning of the surgery
allowed rapid identification of the persistent chyle leak. Subsequently, an anastomosis between the external
jugular vein and the thoracic duct was performed. The postoperative course was uneventful. Currently, the
[24]
follow-up has reached five years, with no recurrence of chyle leakage .
CONGENITAL CENTRAL LYMPHATIC LESIONS
Surgical treatment of congenital central lymphatic lesions, such as central conducting lymphatic anomalies
(CCLA), is much more complex because it usually affects the whole paraxial lymphatic system, which
[9]
results in multiple areas of chyle leakage and may include insufficient lymphatic tonus and peristalsis . The
thoracic duct may be completely absent, resulting in lymphatic collateralization in the subcutaneous or
muscular planes or leakage into the body cavities, such as pleura, pericardium or peritoneum. In addition, it
may result in significant congestion of the lymphatic system of the liver and/or the gut entailing liver
lymphorrhea and protein-losing enteropathy. Lower limb lymphedema and anasarca may additionally be
present. Often insufficient collateralization of the interrupted central lymphatic flow is present necessitating
reconstructive procedures of the central lymphatic system.

Only a few reports on central lymphatic surgery exist for these rare entities [32,33] . In a report about 14
patients, complete remission of symptoms was seen in five patients, and 50% of patients did not have any
improvement. Of note, the majority of these patients showed a patent anastomosis with drainage of contrast
agent in the venous system, confirmed by postoperative fluoroscopic contrast lymphangiography which
emphasizes the complexity of the disease with an insufficient and discoordinated lymphatic peristalsis
[33]
despite patent microsurgical anastomoses . Kovach et al. performed thoracic duct-vein anastomosis in the
neck in four patients with central lymphatic abnormalities of different etiology, achieving complete
remission in three patients and partial resolution in one patient .
[34]

Thoracic duct-vein anastomoses can be performed on different levels depending on the localization of the
lesion. In CCLA, often a lymphatic malformation can be found or severe congestion, e.g., in atresia of the
thoracic duct junction at the left lympho-venous angle, may result in a dilated and tortuous thoracic duct.
The best accessible region is the neck where usually low-pressure veins, e.g. the external jugular vein or
paravertebral veins, are available. This is why most case series describe anastomoses in this region.

However, often in CCLA, thoracic duct abnormalities and interruptions occur at lower levels within the
thorax or abdomen. This is why lympho-venous anastomoses of the thoracic duct should also be considered
in these regions. We performed thoracic duct-vein anastomosis in a 52-year-old woman and a 34-year-old
male with late onset of CCLA. Both patients were otherwise healthy but suffered from permanent
chylothorax and chylous ascites in the setting of CCLA, with daily secretions of up to 3000 ml and recurrent
infections due to immunodeficiency [Figures 2 and 3]. In both patients, stenosis of the thoracic duct with
consecutive chylolymphatic reflux into the abdomen and thorax could be identified by MRL. While
conservative treatment based on an initial MCT diet followed by total parenteral nutrition
failed,lymphangiography-guided embolization of refluxive lymph vessels or even a thoracic duct
embolization would have carried the risk of worsening chylolymphatic reflux and congestion.