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The lymphatic system is the least understood part of the vascular system. Lymphatic malformations such as chylous disorders, cystic hygromas, and lymphocysts are rare; acquired disorders such as lymphoceles and chylous effusions are also uncommon. However, local interruption and obstruction of the lymphatic vessels occur frequently from either congenital or acquired causes (see Ch. 167 , Lymphedema: Evaluation and Decision Making). In developed countries, most acquired lymphatic obstructions are iatrogenic, caused by medical procedures. In developing countries, the most frequent cause of lymphatic obstruction resulting in chronic lymphedema is filariasis.
The development of lymphedema can be described as an imbalance between the lymphatic load (the amount of lymph that has to be cleared from a body part within a given time) and the lymphatic transport capacity (the amount of lymph that can be transported out of a body part within a given time), which is dependent on the number and function of lymphatic vessels and nodes (see Ch. 10 , Lymphatic Pathophysiology). Reduced lymphatic flow due to obstruction leads to secondary tissue changes, a process that is not yet fully understood. Lymphatic outflow disorders are manifested mainly with advanced secondary changes and chronic lymphedema, a condition that can be difficult to treat unless the underlying problem of reduced lymphatic outflow is resolved. Historically, secondary tissue changes leading to excess fibrous and adipose tissue were treated solely by excisional procedures, without correcting the underlying cause. However, modern surgical concepts have been developed to attempt to correct the underlying pathophysiologic mechanism to the extent possible.
Because conservative therapy consisting of limb elevation, compression garments, complex decongestive therapy, and compression pump therapy should be the first step (see Ch. 168 , Lymphedema: Nonoperative Treatment), the question arises whether and at what time surgery is indicated. If the only purpose of surgery is resection, it is wise to reserve it as a last option. If, however, surgical reconstruction is possible, this procedure should be considered and offered to the patient early in the course of lymphedema.
The most radical excisional approach is the classic operation first described by Charles in 1912. It involves complete and circumferential resection of the skin, subcutaneous tissue, and deep fascia, followed by split-skin grafting. However, this procedure is associated with significant complications, and follow-up studies revealed hyperkeratosis, papillomatosis, and ulcerations in the grafted areas. , Modifications of this technique, using the resected skin for grafting and performing the surgery in two stages, reduced the surgical trauma and the rate of complications.
A less invasive way to reduce the amount of subcutaneous tissue is liposuction. It was first described by Illouz as a method for treating lymphedema. More recently, Brorson and Svensson demonstrated lasting volume reduction if elastic compression garments are worn after the surgical procedure; this can result in an extremity that is even slimmer than the healthy limb.
The first attempts to divert lymph from the subcutaneous to the muscular compartment through partial or complete resection of the fascia were described by Lanz and Kondoleon. Redirection of lymph from the superficial to the deep compartment is also a component of the Thompson method. Thompson resected the fascia along with parts of the subcutaneous tissue, created a flap in two stages, and de-epithelialized the rim of the flap to allow the outflow of lymph. Subsequently, he buried the flap near the deep vessels to facilitate the creation of spontaneous lymphatic anastomoses.
The use of veins for the reconstruction of an interrupted lymphatic system was investigated by Holle and Mandl experimentally and performed in two patients clinically. , Campisi and colleagues reported on a larger series using this technique.
Currently, the most common way to drain lymph from edematous tissue is the construction of connections between the lymphatic ( Fig. 169.1 ) and venous systems in the periphery. The first reports on lymphonodular and lymphovenous anastomoses were provided by Laine and Howard, Nielubowicz and Olszewski, Rivero and coworkers, and Allen and Taylor. Degni designed a special needle to facilitate the insertion of lymphatic vessels into veins. , Further improvements were described by O’Brien and colleagues using microsurgical techniques. , In some patients, excisional methods were combined with lymphovenous shunting. A large cohort of patients successfully treated with microsurgical lymphovenous anastomosis was reported by Campisi and associates.
However, experimental studies revealed problems with thrombotic occlusion at the site of anastomosis, with a patency of 20% after 5 months of follow-up. Specific preparations that ensured an undisturbed connection to the venous valve led to an improved patency rate of 44% after 6 months. , Gloviczki and colleagues reported on results of experimental microsurgical end-to-end anastomoses between normal femoral lymph vessels and a tributary of the femoral vein in dogs and noted a 50% patency rate up to 8 months after the operation.
Direct reconstruction of the lymphatic system became a possibility only after the development of microsurgery. Before that time, it was commonly thought to be impossible to anastomose lymphatic vessels because of their extremely small diameters. Hence, Danese approximated lymphatic vessels as close to each other as possible and waited for spontaneous regeneration. He was able to demonstrate transport of contrast medium (through the lymphatics) with this technique. In a patient with lymphedema of the arm, he mobilized two lymphatic channels proximally and distally, approximated them in the axilla, and achieved a reduction in edema. Subsequent approaches included interpositioning veins between lymphatic vessels, implanting microsurgical lymph node grafts, , and implanting free flaps with lymphatic vessels. ,
The question of optimal reconstruction material has been the subject of two experimental studies. In a series of 14 rats, 100% of the autologous lymphatic grafts were patent (observations made between days 7 and 119 postoperatively), whereas allogeneic lymphatic grafts were patent only until day 21 after transplantation. When lymphatics were replaced by small veins ( n = 10), a patency of 70% was observed. Expanded polytetrafluoroethylene implants ( n = 10) used as lymphovascular conduits were already thrombosed by day 7. In a comparison of lymphatic and venous interpositional autografts in 71 dogs, all 26 lymphatic autografts remained patent up to the end of the observation period at 24 weeks. Of 30 venous interpositional autografts, only 4 were patent after 1 week. None of the lympholymphatic anastomoses with silicone tubing showed patency at any time.
Acland and Smith were the first to attempt to anastomose lymphatic vessels. The first successful therapeutic lympholymphatic graft was performed in 1980 by Baumeister in a patient with unilateral lymphedema of the lower extremity. This followed extensive animal experiments on thoracic duct transplants in rats and the treatment of experimental lymphedema in dogs using lymphatic autografts.
Direct contrast lymphography, using oily contrast medium and invasive administration through dissected lymphatic vessels, was introduced by Kinmonth and greatly advanced our knowledge of the lymphatic system. However, owing to the invasiveness of the procedure (and injury to the lymphatic vessels and lymph nodes), it was found to worsen lymphedema and is rarely used today. Indirect contrast lymphography, using a water-soluble contrast medium injected subepidermally, cannot visualize lymphatic vessels as successfully as direct lymphography and gained only limited use.
Attempts to visualize lymphatic vessels with magnetic resonance imaging (MRI) and subdermally administered contrast medium have been promising. This technique may be useful in the future for preprocedure planning and postoperative assessment of the patency of lymphatic reconstructions. For the detection of vascular lymphatic malformations, MRI is extremely valuable both with and without contrast medium.
MR lymphangiography and lymphoscintigraphy show a clear concordance. Inguinal lymph nodes were better visualized using lymphoscintigraphy, whereas lymph vessels and their abnormalities were better depicted by MR lymphangiography.
The most important test to evaluate chronic lymphedema and to plan surgical treatment is lymphoscintigraphy. It can be repeated and used for treatment planning and follow-up. It not only evaluates function but also visualizes routes of lymphatic transport. The lymphatic transport index summarizes the findings derived from lymphoscintigraphic studies and allows a semiquantitative evaluation of lymphatic flow without the need for standardized physical movements by the patient. The transport index ranges from 0 for an optimal lymphatic outflow to 45 for no visible transport; normal values are less than 10. It also provides a good basis for follow-up studies and can show lymphatic transport along the route of lymphatic grafts. , In measuring lymph transport at regions of interest, it is critical to standardize the dose of radiopharmaceutical and the physical activity of the patient during the procedure.
Another diagnostic tool that can be used to identify lymphatic channels is the subepidermal injection of a vital dye (patent blue dye in Europe; isosulfan blue [Lymphazurin] dye in the United States). Normally, lymphatic transport is visualized in the superficial lymphatic collecting system. In pathologic situations, dermal backflow leads to the pooling of contrast medium within the skin, resulting in a cloudlike appearance. Because allergic reactions have been reported, staining of lymphatic vessels with patent blue or isosulfan blue dye is generally performed during surgery under general anesthesia.
For lymphatic grafting, it is critical to choose and carefully evaluate the proper harvest site for lymphatic vessels to avoid the development of edema secondary to the procedure. Thus, the donor lower extremity must be evaluated by lymphoscintigraphy before harvesting. During the harvest, the narrowing lymphatic system at the medial aspect of the knee and the groin must be left untouched, and all stained lymphatic vessels other than those used as grafts should be left in place. A study including 80 patients with arm edema showed that when this method was used, the harvest site and the untouched leg were not different in size.
No pathologic values were detected after the harvest in a study of 19 consecutive patients, where the donor limb was examined pre- and postoperatively by lymphoscintigraphy. The scintigraphic follow-up was performed 48.6 months following harvest and transplantation. In all patients, the postoperative transport index was close to the preoperative baseline value and within the normal range.
Because this type of surgery is performed in the subcutaneous tissue, the surgical risk is generally low, and the procedure is well tolerated. Peripheral lymphovenous shunting is often performed under local anesthesia and is unproblematic as long as the patient tolerates local anesthetics. Because the application of patent blue or isosulfan blue dye can lead to allergic reactions, it should be used only under general anesthesia. Excisional methods typically involve more surgical trauma and the possibility of greater blood loss. Therefore, it is sometimes advisable to perform large excisional operations in two stages. For surgical intervention within the abdomen and thorax, the usual preoperative risk assessment must be done (see Ch. 34 , Preoperative Evaluation and Management).
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