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Confusion on terminology: Primary lymphedema and lymphangioma (lymphatic malformation)
Published in Byung-Boong Lee, Peter Gloviczki, Francine Blei, Jovan N. Markovic, Vascular Malformations, 2019
Lymphangiomas/LMs result from congenital or acquired abnormalities of the lymphatic system. Congenital lymphangiomas are extra-truncular LMs that form due to blockage of the lymphatic system during fetal development.1, 14 Cystic lymphangiomas are associated with genetic disorders, including trisomies 13, 18, and 21, Noonan syndrome, Turner syndrome, and Down syndrome.18 Analogous to secondary lymphedema, acquired lymphangiomas (also known as lymphangiectasias) occur as a sequela of any interruption of previously normal lymphatic drainage such as surgery, trauma, malignancy, and radiation therapy.
Basic dermatology in children and adolescents
Published in Joseph S. Sanfilippo, Eduardo Lara-Torre, Veronica Gomez-Lobo, Sanfilippo's Textbook of Pediatric and Adolescent GynecologySecond Edition, 2019
Kalyani Marathe, Kathleen Ellison
The preferred treatment of lymphangioma is complete surgical excision, though recurrence rates are high. Carbon dioxide lasers have been used to treat superficial lymphatic malformations, and sclerotherapy has been used to successfully treat deeper lymphatic malformations. If lymphatic malformation is suspected, referral to dermatology is warranted.42
Lymphatic disorders
Published in Professor Sir Norman Williams, Professor P. Ronan O’Connell, Professor Andrew W. McCaskie, Bailey & Love's Short Practice of Surgery, 2018
Professor Sir Norman Williams, Professor P. Ronan O’Connell, Professor Andrew W. McCaskie
These are associated with megalymphatics. The diagnosis may be obvious if accompanied by lymphoedema and lymphangioma. However, some patients develop chylous ascites and/or chylothorax in isolation, in which case the diagnosis can be confirmed by aspiration and the identification of chylomicrons in the aspirate. Cytology for malignant cells should also be carried out. A CT scan may show enlarged lymph nodes and CT with guided biopsy, laparoscopy or even laparotomy and biopsy may be necessary to exclude lymphoma or other malignancy. Lymphangiography may indicate the site of a lymphatic fistula that can be surgically ligated. Even if no localised lesion is identified, it may be possible to control leakage at laparotomy or even remove a segment of affected bowel. If the problem is too diffuse to be corrected surgically, a peritoneal venous shunt may be inserted, although occlusion and infection are important complications. Medical treatment comprising the avoidance of fat in the diet and the prescription of medium-chain triglycerides (which are absorbed directly into the blood rather than via the lymphatics) may reduce swelling. Chylothorax is best treated by pleurodesis, but this may lead to death from lymph-logged lungs as the excess lymph has nowhere to drain.
Percutaneous full endoscopic treatment of cystic lymphangioma of cauda equina: a case report
Published in British Journal of Neurosurgery, 2020
Hyeun Sung Kim, Nitin Maruti Adsul, Sung Ho Choi, Jee-Soo Jang, Il-Tae Jang, Seong-Hoon Oh
Lymphangioma of the spine is extremely rare and only a few cases have been reported in the literature. There are two types of lymphangioma - congenital and acquired.1 Congenital lymphatic malformations occur most commonly within first two years of life in the head, neck or axilla. They form because the primordial lymphatic sacs fail to drain into the veins or normal lymph tissue during fetal development. Acquired lymphangioma is due to obstruction of affected lymphatic channels. It is a benign condition with slow growth has a long course and rarely disappears without treatment. Its volume increases quickly when there is bleeding inside3 which may be a cause of progressive worsening of symptoms since last one month in our case.
Giant Ovarian Lymphangioma: Case Report and Review of the Literature
Published in Fetal and Pediatric Pathology, 2018
Elisa Pani, Alessandra Martin, Annamaria Buccoliero, Marco Ghionzoli, Antonio Messineo
Lymphangiomas are benign tumors/malformations, characterized by proliferation of lymphatic vessels. Approximately 50% present at birth, and 90% are diagnosed in the early childhood (before the age of 2) [1]. Lymphangiomas may arise anywhere on the body, although the most common localizations in children are the head–neck region and the axilla. In adults they most often are superficial or intraabdominal, and have been reported in the gastrointestinal tract, mesentery, liver, pancreas, gallbladder, spleen, adrenal gland, and retroperitoneum [2]. There are reported cases in the pelvic region, infrequently in ovaries and even in fallopian tubes [3–5].
Isolated cranio-orbitofacial neurofibroma mimicking vascular malformation
Published in Orbit, 2018
Harinder S. Chahal, Brandon Kuiper, Puneet S. Braich, A. Tyrone Glover
MRI is the radiographic study of choice for evaluating lymphangioma because of its superior capability to demonstrate soft tissues and to identify blood and blood products in all stages of degradation. The presence of a fluid–fluid level on MRI, seen in cases complicated by intralesional hemorrhage, is the best clue to differentiate lymphangioma from other cystic lesions of the orbit. Fluid–fluid levels or variable signal intensity is due to blood products at different ages present in the tumor related to the variable paramagnetic properties of the iron moiety of hemoglobin as it degrades into ferritin and hemosiderin.