24.1.1 History of Lymphatic Channel and LVA

The description of the lymph duct began with the coinage of the word “white blood” by Hippocrates in the 5th century BC. The nature of lymphatic vessels was first revealed by Gasparo Asellius (1581–1626) of the University of Pavia, Pavia, Italy. Asellius, while dissecting a dog immediately after it had been fed, observed a series of vessels filled with a white milky fluid in the peritoneum and intestines. He misunderstood this as a blood vessel leading to the liver and named it the “chilo-duct.” From the 1600s, the anatomy of lymphatic vessels using mercury began and its detailed distribution became clear. In Japan, Genpaku Sugita et al listed lymphatic vessels for the first time in 1774 in the new anatomical book “Kaitai Schinsyo” translated from Dutch to Japanese. Professor Buntaro Adachi, Kyoto University’s first anatomy professor, was passionate about the study of lymphatic vessels, and his successor Professor Shogo Funaoka performed lymphangiography in rabbits for the first time in the world and published it as a German paper in 1929. Furthermore, in 1969 Dr. Yamada of Nagoya University performed a lymphaticovenular anastomosis (LVA) in a dog and patients with lymphedema using a microscope. Efforts of the pioneers in Japan have led to the current treatment of lymphedema.

About 1988, Professor O’Brien (Melbourne) was the only person who performed lymph bypass surgery for the treatment of lymphedema. ¹ This method had already been tried by many surgeons, but found to be ineffective. In his last speech on lymphedema for us, O’Brien expressed unhappiness at the fact that there were no followers of surgical treatment of lymphedema. This has been the motivation for Dr. Koshima to continue the use of surgery for the treatment of lymphedema since 1990. ^{2 ,​ 3} Around 1980, the authors developed supermicrosurgical techniques that have made possible the anastomosis of more precise vessels of 0.3- to 0.8-mm caliber size. This technique allows the use of 12–0 nylon with a 50- to 30-μm needle in vascular anastomosis of vessels less than 0.8 mm in diameter. Even in legs with lymphedema of long duration, we found that there remain a sizable number of precise lymphatics and subdermal venules (0.5 mm). With the supermicrosurgical techniques, anastomosis of precise lymphatics and the subdermal venular system with a higher patency rate is possible. The application of these techniques to the drainage of lymphatic fluid into the subdermal precise venular system (LVA) has been achieved with improved results. Since 1990, the authors have performed LVA in 2,000 patients including primary edema of the legs (300 upper arms and 1,700 legs) under local anesthesia. As a result, complete recovery and/or remarkable improvement was obtained in mild and moderate cases with primary edema, ^{2 ,​ 3} and now new strategies combining LVA with functional lymph channel transfers are established for severe cases with primary edema.

24.3.1 Primary (idiopathic) Lymphedema

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  Congenital edemas are those already developed at birth. Sometimes, there is an example showing unilateral seminal edema including the face. There are cases in which edema of the limb is combined with neonatal thoracic fluid and ascites, often complicating protein leaky gastroenteropathy. Although it was considered a congenital edema up to now, the authors believe this is the secondary because it is caused by stenosis or obstruction of the thoracic duct. Hereditary lymphedema (Milroy disease) is congenital edema of both lower limbs and is a rare disorder with autosomal dominant inheritance without other anomalies. Recent developments in molecular biological techniques have revealed vascular endothelial growth factor receptor 3 (VEGFR3), which is responsible for establishing the causative gene and expression of vascular endothelium. This makes it possible to perform genetic diagnosis and it is said to recognize Ala → Thr mutation in exon 18 constituting the kinase domain of VEGFR3.

  
  
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  Premature edemas are those developed at adolescents without incentives or at younger than 35 years.

  
  
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  Delayed edemas: As for these idiopathic edemas, lymphatic vessels always exist when they are confirmed by surgery. The existence of congenital missing or obstruction (hypoplasia) of thoracic duct is also conceivable, but the details are unknown at this time.

24.3.2 Secondary Lymphedema

Depending on the cause of the condition, it is subdivided as follows:

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  After treatments for malignant disease: onset after lymph node dissection and radiotherapy and/or chemotherapy.

  
  
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  Those accompanying progression and recurrence of malignant tumor: so-called malignant lymphedema.

  
  
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  Trauma: damage of lymphatic vessel, or extensive scarring.

  
  
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  Infections: filariasis, etc.

Secondary edema is lymphedema of the limbs after the surgery for breast or uterine cancers, and most cases in the upper limb are secondary. In the lower limb, 87% of the cases are secondary after uterine cancer surgery, but there are male cases, such as after testicular tumor resection. In the lower extremity cases, 29% of cases are bilateral and tend to progress from one side to both sides.