Lymphoedeme Congenital

www.lymphoedemacenter.com

Summary

Lymphoedema is the retention of fluid due to lesions of the lymphatic system: the volume of the limb can become impressive, and the chronic infections are producing true deformities.  .

The lymphoedema can also occur during childhood and young adults.

The congenital lymphoedema do present multi different forms (hypoplasy, hyperplasy, multi-syndroma) .Genetic mutations and chromosomic abnormalities can be associated with congenital lymphedema.

The only way to have good results in the treatment is the comprehension of the damages, by good investigations and good clinical examinationIt is much more complicated to evaluate and localize the defect(s).  The true advance for the diagnosis are: the lymphoMRI showing the map of all the lymphatic system, and the lymphography with indocyanine (and soon, with ultrasonography) to visualize the superficial lymphatic system.

Résumé:

le lymphoedème congénital est dû à une rétention de fluide extra cellulaire dans le corps qui conduit à un oedème très important qui peut déformer un membre ou une partie du corps et même, conduire l'éléphantiasis.

 

 

 

Certaines pathologies sont apparues à la naissance ou souvent, à la puberté.

Elles sont liées à une erreur génétique dont la nature, avec les énormes progrès, commence à être connue.

Bientôt, il va peut-être être possible de la corriger  grâce aux nouvelles découvertes

en génétique...

Le meilleur moyen de comprendre la symptomatologie est de faire une lymphoIRM qui permettra de comprendre si la situation est due à une hypoplasie glandulaire et/ou canalaire, ou à une hyperplasie désordonnée. La fluorescence permet aussi de visualiser le passage, notamment dans les lymphangites.

Actually, microsurgical procedures can give excellent results in long terms, and must be widely spread, with a good teaching, because those techniques are difficult, and the indications must be precisely selected.

Actuellement, les techniques microchirurgicales peuvent donner des excellents résultats, voire des normalisations chez les jeunes, lorsque l'évaluation et le choix des techniques est adéquat

The reconstruction with a free microvascular transplant containing lymph nodes can be an answer . The positive effects of the introduction of endogene VGEFc factors when the edema is moderate and/or acute, but even chronic lymphedema and elephantiasis cases is to be considered as a logic way.

La reconstruction avec une autotransplantation ganglionnaire peut être une technique de reconstruction adéquate dans les hypoplasies ganglionnaires puisque elle consiste en une greffe de ganglions. Ceux-çi sont vascularisés et, contenant des hormones trophiques, ils permettent une régénération des canaux lymphatiques, et les ganglions greffés vont pomper le liquide lymphatique et se battre contre les infections

The combination with excisions of the folds and liposuctions are extremely helpful in advanced cases to reduce the deformations.

La combinaison avec les excisions des plis et les liposculptures, permettent d'améliorer les résultats dans les formes importantes.

For the cases where some lymphatic vessels are still of good quality, lympho-venous bypass (to reduce the hyper pressure in the lymphatic system), can give good results, but the cases must be well selected (lymphangiography with IGG). 

Pour les cas d'hyperplasie lymphatique, si les canaux sont de bonne qualités et encore valvules (formes non fibrotiques), les dérivations lymphoveineuses peuvent diminuer la pression dans le système lymphatique en envoyant le liquide dans les veinules.Ces techniques demandent une instrumentation ultravide et une imagerie ultraprécise pour ne pas être déçu des résultats

Some extensive liposuctions can give excellent results by removing all the fatty tissue, but complete strong compression (8 strenght) are mandatory day and night for the life.

La technique de liposuccion complète de toute la graisse sous-cutanée peut fonctionner dans les cas gras de lymphoedème, non fibroses, mais vont nécessiter à vie, une compression n°8 (2X4) nuit et jour...ce qui est très difficile à supporter surtout dans les pays chauds.

Post-operative physiotherapy is important and must be adapted to different cases

La physiothérapie post opérative (drainages, bandages) est très importante en  période postopératoire pendant quelque mois..

Examples:

Hypoplasic cases of upper and lower extremity in adult (nodes and vessels). The upper and lower limbs can be affected simustaneously. This edema did develop during infancy. Many infections did destroy progressively the lymphatic system and the illness do progress year after year.

Cas  hypoplasique des extrémités supérieures et inférieures à cause d'une hypoplasie ganglionnaire et canalaire

 

 

 

 

 

 

 7 months old child born with genital and lower limb lymphoedema. (hypoplasy of nodes)

Enfant de 7 mois né avec hypoplasie lymphatique importante

 

 

 

Hyperplasic cases

Photodynamic eye (PDE) is a new exam used to evaluate the transportation of fluorescein in the lymphatic vessels.  The laser can follow (dynamic) the superficial network after injection of indocyanine, but not deeper as1 cm.

cas d 'hyperplasie : la fluoroscopie permet de visualiser de façon dynamique , les canaux lymphatiques superficiels (le laser ne pénètre pas à plus d'un cm de profondeur, donc difficile chez les patients présentant de gros dépôts graisseux, mais l'IRML montre le réseau profond

 

 

 

 

Description of the actual techniques and indications:

1.Macrosurgical procedures:

The dermolipectomies do consist in elliptic resection of skin and fat, reducing the volume and removing the folds. Compressive bandages are needed for 4 weeks. Those techniques do improve the efficacity of the bandages and reduce the mycotic colonization of the folds. They are combined later with lymphnodes transplantations in the inguinal region if the whole leg is involved, or at the knee region, if the distal part is only concerned. Combination with liposculptures are common.

             

Resections of the folds and plasties

2.Microsurgical procedures:

1.Lymphovenous anastomosis consist in derivation of lymphatic vessels in veins: the hyper-pressure in the lymphatic system can be reduced by lympho-venous bypass. The same concept applies if blockage or absence of the thoracic duct is diagnosed. Peripheral lymphatic vessels lympho-venous shunt operations performed using microsurgical techniques are more effective in the early stage of lymphedema .The progress in quality of  the material ,the magnification microscopes , and the laser imaging  improve a lot the permeability of such vessels (0,2/0,5mm of diameter.(ref 5) Long terms results are showing local effects , that means that many anastomosis are to be done  distally, and perhaps redone later, regarding the results.

 

2.Free lymphnodes transfers:

The princeps of this technique is the transfer of a group of nodes on their own vessels, to the place where the nodes/and vessels are not existing. It is a fascio(cutaneous) flap based on arterial and vein, transferred and revascularized by microsurgical anastomosis like a finger transplantation. So, the transfer of the lymph nodes, and the resulting endogenous lymphatic growth factor expression may thereby enhance the re-growth of lymphatic network. It is a critical forward step in this technique because it allows the growth of news vessels into the area ,and do stimulate the immune function. In each node, there is a full exchange between blood and lymph (plexus like multiple lympho-venous exchanges). This is true reconstructive technique, but the dissection of the donor sites must be follow strict rules.

   

                        

 

What is it ?

 

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 dysplasic

 

DIAGNOSIS

 

The clinical aspect and the history are importantt but 

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

 Magnetic Resonance Lymphography (MRL), with T3 weighted imaging, allows visualization of the lymphatic system anatomy with greater sensitivity than

Fluoroscopy do provide information on the superficial lymphatic network

lymphoscintigraphy, without the need of any injection.

Mostly in the children,,you always have to check the body to understand the pathology.. It is like an iceberg…..

 

Ex 1./Here under , swollen hands are due first to aplasia of the thoracic duct, and the second one , due to hypoplasic lymphatic system.

 

 

 

 

 

 

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

 

Autologeous lymphnodes transplantation 

 

 Indications

 

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

the transplanted lymphnodes and the fat around

 They do contain growthing factors witch  do promote the formation of new  lymphatic vessels.

They also help fighting again infections. Because they do contain  T lymphocyte`

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

The transplanted nodes will pomp the liquid and the  pathways will growth and drain the liquid..

Where is the flap inserted?

 

In the case where the whole leg is swollen, the flap will be inserted in the inguinal region.Sometimes 2 flaps are mandatory.

 

 

here the flap will be inserted in the inguinal reg

In distal lymphoedema,the flaps will be inserted near the knee, 

 

 

RESULTS

 

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

 

The children  can be cured when they are operated early

 

 

 

 

 

this young boy  came only when he was 22y old. This is his picture when he was child…many years of suffering from chronic infections and disability.

Results after 2 implantations of nodes :in the inguinal region, then after 6 months, in the knee region

 

 

 

 

 

Even in very important lymphoedema, we have good results, combining with local resection of the bad skin.

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

 

 

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

 

 

 

Lympho MRI showing the transplant at the knee

 

 

 

 

 

combination with local resection and lymphnodes transplantation can cure definitively the children.DO NOT WAIT !!!!!

 

 

 

 

 

 

 

 

 

UPPER EXTREMITY CONGENITAL LYMPHODEMA

 

 

For the hands, lymphoedema since the birth,having receiving a lymphnode transfer in the axillar region. Result after 3 years

 

 

 

even very important lymphoedema appeared after one trauma on the hand., probably having a congenital background, can be cured by nodes transplantation at the elbow region.A local excision of the bad skin in the dorsum of the hand has been prior  realized

 

 

 

 

 

 

 

 

 

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 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.

 

 

 

 

mini flap based on a small artery and vein , coming from the breast region, just under the mammilla( zone1)

 

 

 

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.

 

 

incision in the cervical region

 

 

 

little flap containing nodes on an arteria and a vein

 

 

 

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.

 

Other tecniques :

 

 

 

Lymphovenous Anastomosis indications

 

 

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 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 indications :

Enormous lymphoedema =elephantiasis

 

 

 

 

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 recurrence

 

 

 

Local liposculptures

 

Fat deposists can be due to the hyperplasy of the mesodermis  in  utero  in children, .And also, all the fat that we are eating is  absorbed directly in the bowels in the lymphatic system.If it is  insufficient, the fat will not circulate correctly and will make deposits.The only way is to do some local liposculptures, with direct vision of the lymphvessels by visualisation of the vessels by laser, during the surgery (SPY)

 

 

 

 

 

 

local liposcultures wher no visible lymphatic ducts

 

 

 

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Genital Lymphedema

 

 

Lymphedema can involve the scrotum, penis, labia and produce leaks and a lot of functional and psychological problems.

Lymphnodes transplantation combined sometimes to local excisions, can achieve good results

 

 

 

 

 

 

 

 

 

 

 

 

OTHERS

 

Some  general deseases cans appear at the birth and are related to genetic malformations. General investigations must be performed, diat , medical treatments and surgeries must be adapted .

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