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Summary

The lymphoedema can occur during childhood (even at birth) and puberty . It 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. Different pathologies can be combined with the lymphoedema and the system can show different etiologies. 

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. Regarding the hypoplasic forms, the reconstruction with a free microvascular transplant containing lymph nodes can be an fantastic option .The positive effects of the introduction of endogene VGEFc factors can cure  some hypoplasic  lymphoedema in babies and very young children. The combination with excisions of the folds and mini  liposculptures  are extremely helpful, in advanced cases.

For the cases with lesions of thoracic ducts, cysts, and  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). 

Post-operative physiotherapy is important, and must be adapted to different cases and teached to the parents.

 

Introduction:

The lymphoedema is a complex pathology.. The incidence of the congenital cases is estimated  around  1,33/1000 (births) and related with consanguinity.(ref 1).

The congenital lymphoedema do present multi different forms (hypoplasy, hyperplasy, multi-syndroma) and the only way to have good results in the treatment is the comprehension of the damages, by good investigations and good clinical examination.

Genetic mutations and chromosomic abnormalities can be associated with congenital lymphedema. Cardiac malformations, renal pathology, mental retardation is possible. Sometimes, facial defects, cleft palate, double eyelashes and yellows nails, can be observed. The lymphedema-distichiasis syndrome is due to lack of intraluminal valves of the lymphatic collectors, and this malformation results in lymph reflux. Mutation in the transcription factor FOXC2 is identified at the locus q24-3 on the chromosome 16.

- Milroy’s syndrome is due to a mutation of the gene which encodes VEGFR-3 (5q35.3). This syndrome is associated with limb edema, and sometimes genital edema and upper limb edema. The hereditary familial lymphedema known as disease of Milroy, is not a gross macrostructural defect of the lymphatic vessels. The initial and collecting lymphatics are present, but there is an impairment of absorption at the level of the initial lymphatics that reflect a functional defect.

- Meige’ s syndrome affects lower limbs and appears during puberty and the genetic mutation is unknown.

 -Lymphedema may coexist with complex vascular malformations, Proteus syndrome and Klippel-Trenaunay syndrome.

- Lymphedema in Turner’s syndrome has characteristic features. In this case, lymphedema is frequent, bilateral, distal, symmetric, and with lymphangiomas.

Other diseases associated with lymphedema are hypertension, diabetes, and hypothyroidism.

 Pluri-disciplinary approach is necessary, good imaging, and the combinations of the different surgical techniques and special physiotherapy and new devices will be the key. Genetic corrections will be perhaps solutions in the future, for some cases. 

The aspects can be various  and the solutions well documented

 

 

Material and methods:: 

Overview of the study:

This study do examine 48 children with hypoplasy of the channels and/or nodes. In the lower extremities All those children did benefit from lymphnodes transfer, combined with excision of excess of tissue (folds and fat) in 20% of the cases.

This study concern 70% of boys age between 6 months and 11 years old and suffering from lymphoedema since the birth.

The follow up  is from 2 years to 9 years of for some children.

Methods to investigate :

Magnetic resonance lymphography (MRI) is based on heavily T2 weighted fast spin-echo sequences and maximum-intensity-projection reconstruction. It appears to be a useful noninvasive technique to evaluate the lymphatic system. This exam is performed without any injection, with slight sedation in very young children. Lymphatic magnetic resonance imaging is the method of choice and gives so much more informations compared to the isotopic lymphogaphies.  The lymphoMRI  can map the lymphatic system: its forms, quality, distribution, and malformations and can show the superficial and deep lymphatic system, the pathways, the nodes, the quantity of fat and water in different parts (and compare the evolution), the thickness of the skin etc.

 

 hyperplasic cases

 Thoracic channel malformation

hypoplasic case

honey cumbs 

 

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. 

 

The combination of both techniques can provide static and dynamic informations.

Surgical technique:

General anesthesiology is performed .For the lower extremity, the flap will be inserted in the inguinal region in lymphoedema of the whole leg, and in the knee region if the lymphoedema is distal.
First, the recipient vessels are isolated : circonflex superficial  iliac vessels in the inguinal area, and, in the knee region, deeply, at the intersection between the superficial and deep system : the deep saphenous vessels will be isolated, at the inner part of the quadriceps. Those vessels are tiny in babies, but in experimented hands, the microsurgical anastomosis are possible.

The donor site for the lower extremity is located in the thoracic region, regarding the lateral side of the breast region. The zone 4 of the breast is involved. Incision is done in front of the border of the dorsalis muscle. Then the thoracodorsalis vessels are isolated, an the branches going in those nodes. Above those nodes, it is possible to find the branches of the external mammary vessels. The choice of the perforans branches depends of their size. The, a fatty flap is designed around the vessels . This flap contains 3, 4 nodes. Hemostasis are well performed and the mini lymphatic vessels are clipped distally. Then the flap is elevated and the vessels are clipped and putted on a mini clamp. 

 

 

 

The flap will be inserted in the inguinal or knee region, and the vessels will be anastomosed with nylon 10X0 or 11X0, arteria on the arteria and vein on the vein.  Then the wound will be sutured in 2 layers with a drainage.

 

Results: The results section should provide complete details of the experiment that are required to support the conclusion of the study. The results should be written in the past tense when describing findings in the authors'experiments. Previously published findings should be written in the present tense. Results and discussion may be combined or in a separate section. Speculation and detailed interpretation of data should not be included in the results but should be put into the discussion section.

 

The children are evaluated every month by measurements compared to the normal side. Every 6 months, the child is reviewed and the lymphoMRI is performed after 1 year.

The bandaging is performed during 3 weeks after the surgery, continuously, then just at night for the 3 following months. Stockings are sometimes proposed but it is complicated because the fast growth of the children.

 

The chidren are divided in groups

33 children are under 6 years

15 children between 7 and 15 years old.

Used techniques are lymphnodes transfer in all those cases, combined with local resections of the distal folds in 10% of the cases.

Considering the groups, the volumetry of the limb, compared to the normal side  do reduce faster in the children under 2 years old.Complete recovery with normalisation is observed in 80% of the babies 1 year after the surgery

 

 12 months old girl.Lymphnode flap is inserted in the inguinal flap.

Result 7 months later