Dr Corinne Becker – Lymphoedema Center

Congenital lymphedema

Lymphedema is a pathologic condition that results from a disturbance of the lymphatic system, with localized fluid retention and tissue swelling. Swelling can affect a single limb, multiple limbs, genitalia or the face.

Primary lymphedema is a congenital disorder, due to a malformation of lymph vessels and/or nodes (hypotrophic or hypertrophic).

Congenital lymphedema can appear at birth or during the first years of the life. In most of the cases it develops during puberty, perhaps due to the hypotrophy of the lymphatic system which is too small for the growing body, or dysplasia.

DIAGNOSIS

The clinical aspect and the history are important.

Complementary Investigations

Nowadays, radiological evaluation of lymphedema patients can be done through lympho MRI .

PDE (Photodynamic eye) provides information on the superficial lymphatic network.

Magnetic Resonance Lymphography (MRL), with T3 weighted imaging, allows visualization of the lymphatic system anatomy with greater sensitivity than lymphoscintigraphy, without the need of any injection.

Lymphangioscintigraphy is dynamic but provides poor results.

Lymphangioscintigraphy is dynamic but provides poor results.

Lymphatic MRI, also called Magnetic Resonance Lymphography or MRL, is the most accurate diagnostic tool for lymphedema.

Lymphatic MRI provides a better visualisation of the lymphatic system anatomy

Physiotherapic Treatment

Physiotherapy (manual drainages, pressotherapy, compression, bandages) is the usual treatment for chronic lymphedema. It is not a curative therapy, but helps to control the evolution of the disease.

Surgical Treatment

ALNT Indication

Patients with HYPOPLASIA of the lymphatic system, pain and chronic infections  are the preferred candidates for the procedure.

The flap is inserted in the area where the nodes and the lymphatic vessels are insufficient.

The healthy nodes contain a growth hormone (VGEF 3) witch induces neoformation of lymphatic ducts. They also help fighting again infections.

The lympho MRI shows where to insert the flap

The lympho MRI shows where to insert the flap

Where is the flap inserted?

In Fig.1, the flap will be inserted in the inguinal region

In Fig.2, the flaps will be inserted near the knee, where the vessels are stoppped

Outcomes

In patients with distal lymphedema

  • all patients showed a reduction of the circumference of the treated limb with normalization in 46%
  • 88% of the patients had no more infections in the follow-up period.

In patients with generalized limb lymphedema (as in Fig.1 and 2)

  • although improvements in limb perimetry were present in 98% of the patients, only 20% of them achieved complete normalization
Results 2 years after 2 lymh nodes transplant on a boy who suffered from congenital lymphedema for 19 years.


Results 2 years after 2 lymh nodes transplant on a boy who suffered from congenital lymphedema for 19 years.

These photos show a baby boy with congenital lymphedema. He underwent ALNT surgery at 19yrs and received 2 lymph nodes transfers. The right-most photo shows the result 2 years after surgery, without physiotherapy and very occasional stockings.

Results 6 months after LNT on a lady who suffered from elephantiasis with chronic infections for 20 years

Results 6 months after LNT on a lady who suffered from elephantiasis with chronic infections for 20 years

This is a 35 years old woman who had elephantiasis for 20 years, and the results 6 months after lymph node transfer

Lympho MRI showing the transplant at the kneeLympho MRI showing the transplant at the knee

Detailed Operative Technique

Lymph nodes transfers

  • In patients presenting with lymphedema on the entire limb, the flap should be transplanted to the proximal insertion of the limb (inguinal region).
  • In socket-pattern lymphedemas, the flap can be placed at the level of the knee.

MRL can help establishing the place where to put the flap.

Large lymphedemas might require 2 different flaps (inguinal and knee region).

The surgery starts at recipient site.
In the inguinal region, the incision is performed in the inguinal crease. Deeply, the circumflex iliac vessels are individualized and prepared for the micro anastomosis. A little pocket is created to receive the transplant, at the depth of the deep lymphatic system.
At the knee region, an incision of approximately 5cm is performed just above the knee (internal). The deep saphenous vessels will be the recipient pedicle.

The lymph node flap is usually taken at the thoracic region. An incision of 5 cm is performed in the low axillary region, laterally to the nipple. The fat tissue located deeply on the lateral thoracic wall and anterior to these vessels contains functional lymph nodes. It can be dissected as a lymph node flap based on small  thoracic branches. The vessels are prepared with microvascular clamps for identification on the recipient site.

Branches of the thoracodorsal or ext. mammary vessels nourishing 3-5 nodes of the breast.

Branches of the thoracodorsal or ext. mammary vessels nourishing 3-5 nodes of the breast.
Look for the nodes around those branches.

The cervical flap is based on the transverse cervical artery. Incision is performed on the internal part of the clavicle, over the sternocleidomastoidus, which is reflected.
The flap is raised as a free-style flap, in the same manner as the thoracic region.

Preparing the cervical flap

The incision is performed on the internal part of the clavicle.

The flap is transfered with microsurgical techniques, arteria on arteria, vein on vein, under microscope magnification and 10×0 nylon sutures. Skin is closed with multi-layer absorbable sutures.cervical-flap-3

LNT in Children

The indication of lymph nodes transfer in very hypoplasic cases in children diagnosed by lymphoMRI is excellent at 1 year old, allowing to observe the continuing enlargement of the limb of the kid.

The results are excellent, even better and faster than for the adults and the growth can be normal or near normal.

ALNT gives very good resulsts on congenital lymphema in children. Operate children as soon as possible.

Operate children as soon as possible

Lymphovenous Anastomosis

Hypertrophic forms of primary lymphedema diagnosed thru MRL and malformations of the thoracic duct are treated by several lymphovenous anastomoses in the inguinal region.

Hypoplasia of the thoracic duct. 2 years post-op with 4x2 compression

Hypoplasia of the thoracic duct.
2 years post-op with 4×2 compression

In the hyperplasic cases, the high pressure on the lymphatic system can be deviated by lymphovenous bypass. This is the case for patients diagnosed with blockage or absence of the thoracic duct.

Excisions

Combination of resection and ALNT

Excisions are sometimes necessary to remove the folds, where mycosis are sources of many infections and make the bandages very difficult.

The addition of lymph nodes transfer is very useful to prevent recidive.