A huge lymphovenous malformation in the retroperitoneum

Kawamoto, Aya; Inoue, Mikihiro; Uchida, Keiichi; Okugawa, Yoshinaga; Koike, Yuki; Ohtake, Kohhei; Kusunoki, Masato

doi:10.1016/j.jpedsurg.2007.11.002

Cited by 6 publications

(4 citation statements)

References 17 publications

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“…LVMs are collections of abnormal lymphatic and venous vessels, with normal endothelium, that result from an error in embryonic development. 45,46 These structures may be associated with syndromic conditions such as Klippel-Trenaunay syndrome (KTS), which is caused by a mutation in the PIK3CA gene 47,48 ; other studies have shown that PIK3CA encodes for p110α which is required for lymphangiogenesis, providing a mechanism by which these malformations may occur in Klippel-Trenaunay syndrome (KTS). 49 Patients with KTS exhibit malformations across organ systems including, liver, heart, and skeletal muscle, with each providing evidence of the ubiquity of the lymphatic system.…”

Section: Lymphovenous Malformationsmentioning

confidence: 99%

“…Alternatively, lymphovenous malformations may be isolated without syndromic association. 45,46 These malformations are usually present at birth and can range from being asymptomatic to having significant manifestations. 46,50,51 The sequelae of these shunts can be quite serious with cases of pharyngeal abscess secondary to lymphovenous malformations reported in the literature.…”

Section: Lymphovenous Malformationsmentioning

confidence: 99%

“…Lymphovenous malformations (LVMs) represent another example of pathologic lymphovenous anastomoses. LVMs are collections of abnormal lymphatic and venous vessels, with normal endothelium, that result from an error in embryonic development 45,46 …”

Section: Pathologic Lymphovenous Shuntsmentioning

confidence: 99%

“…Patients with KTS exhibit malformations across organ systems including, liver, heart, and skeletal muscle, with each providing evidence of the ubiquity of the lymphatic system. Alternatively, lymphovenous malformations may be isolated without syndromic association 45,46 …”

Section: Pathologic Lymphovenous Shuntsmentioning

confidence: 99%

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Lymphovenous shunts: from development to clinical applications

2021

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The lymphatic system is a vast network of vessels that functions to return excess fluid from the interstitial space to the blood stream. 1 Excess interstitial fluid is drained by lymphatic capillaries, which are composed of a single layer of lymphatic endothelial cells that overlap with each other. The overlapping cells are thought to act as primary valves allowing flow of interstitial fluid into lymphatic vessels but limiting outflow. [2][3][4] Lymph then flows from these capillaries into collecting lymphatics 5 which have a layer of lymphatic muscle cells (LMCs) that work to transport lymph through rhythmic contraction. 6,7 This intrinsic force from LMCs occurs in conjunction with extrinsic compressive force from surrounding tissue to propel lymph through the lymphatic system. 6,8 A secondary system of intraluminal valves within the lymphatic collecting ducts themselves prevents backflow of lymphatic fluid toward the capillaries. 6,8,9 Lymph then travels through lymph nodes into post-nodal lymphatic ducts which converge into either the thoracic duct or the right lymphatic duct; these are classically described as emptying into the junction of the subclavian veins and internal jugular veins on the left and right side, respectively. 9,10 These connections are the body's two major anatomic lymphovenous shunts, a term which refers to the anastomoses, be it natural or surgical, between the lymphatic system and the venous system. 11 | ANATOMIC ALLY NORMAL LYMPHOVENOUS S HUNTS | Thoracic duct and right lymphatic ductThe natural anastomosis between the thoracic duct and the subclavian vein represents a well-known anatomic lymphovenous shunt. 12 The thoracic duct (TD) most commonly originates from the cisterna

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