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Lymphatic malformations are benign lesions of vascular origin that show lymphatic differentiation. Specifically, they are vascular malformations and not vascular tumors as per the 2018 ISSVA classification of vascular anomalies 5.
This article focuses on the general features of lymphatic malformations. For a specific discussion in other locations, please refer to:
- head and neck: cystic hygroma (cystic or nuchal lymphangioma)
- hepatic lymphangioma
- splenic lymphangioma
- pancreatic lymphangioma
- renal lymphangioma
- retroperitoneal lymphatic malformation
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These malformations were formerly called lymphangiomas. This expression is out-of-date according to the 2018 ISSVA classification 5.
They can present at any age but most often occur in the pediatric population (~90% in those less than 2 years old 3). The worldwide incidence of lymphangiomas is 1:6000-16000 live births. Males and females are equally affected.
Generally, the presentation may be with symptoms related to local mass effect and/or hemorrhage.
For example, a lymphatic malformation within the orbit may present with progressive proptosis with acute deterioration in symptoms, the mass effect resulting in compressive optic neuropathy, diplopia/ocular muscle weakness, and orbital bruising.
The clinical examination may reveal soft, non-tender masses on palpation with a doughy consistency.
Typically comprised of thin-walled cystic masses and can be classified according to the size of the cystic lesions 6:
macrocystic lymphatic malformation
- previously known as cystic hygroma / cystic lymphangioma, although a term still commonly used when large in the cervical region
- mean diameter of cystic lesions >1 cm
microcystic lymphatic malformation
- previously known as cavernous lymphangioma
- mean diameter of cystic lesions <1 cm
- mixed type lymphatic malformation: both macroscopic and microscopic features
Their wall consists of connective tissue, smooth muscle, fat, blood vessels, nerve, and/or lymphatic tissue.
They can occur at almost any location:
- marked predilection for the head and neck: 95% in the neck and axillary regions
- mesentery, retroperitoneum, abdominal viscera, lung, and mediastinum: ~5%
- Noonan syndrome
- Turner syndrome
- trisomies 13, 18 and 21
Lymphatic malformations may involve more than one compartment. For instance, in the head and neck region, larger lesions tend to occupy more than one deep space, sandwiching between normal structures.
- multilocular cystic masses
- internal septa of varying thickness
- cystic contents: usually anechoic; hyperechoic if contain debris, high lipid concentration, infection or hemorrhage
- wide variations exist: solid areas, or mostly solid with cystic foci
- color Doppler: +/- arterial or venous flow in the septa
Most lymphatic malformations appear homogeneous and cystic on CT, but some appear inhomogeneous because of the presence of proteinaceous, fluid, blood, or fat components within the lesion. It is rare for CT to demonstrate intrinsic septations. There is only minimal or no displacement/compression of adjacent structures.
Fluid-fluid levels may be seen if complicated by hemorrhage. Signal characteristics include:
- T1: can be variable especially dependent on protein content
- T2: usually high signal
Treatment and prognosis
Surgical excision or interventional sclerotherapy (with interferon, OK-432, or bleomycin) is often necessary 3. Other possible treatment methods include steroid therapy, laser treatment, aspiration, radiofrequency ablation, or cautery.
Differentials will depend on which compartment or viscera is considered. Possible imaging differential considerations include:
- venous malformation: demonstrate internal blood flow and central enhancement
- other cystic neck masses
- teratoma: demonstrates fat or calcification
- 1. Levy AD, Cantisani V, Miettinen M. Abdominal lymphangiomas: imaging features with pathologic correlation. AJR Am J Roentgenol. 2004;182 (6): 1485-91. AJR Am J Roentgenol (full text) - Pubmed citation
- 2. Shaffer K, Rosado-de-christenson ML, Patz EF et-al. Thoracic lymphangioma in adults: CT and MR imaging features. AJR Am J Roentgenol. 1994;162 (2): 283-9. AJR Am J Roentgenol (abstract) - Pubmed citation
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- 4. Bansal AG, Oudsema R, Masseaux JA et-al. US of Pediatric Superficial Masses of the Head and Neck. (2018) Radiographics : a review publication of the Radiological Society of North America, Inc. 38 (4): 1239-1263. doi:10.1148/rg.2018170165 - Pubmed
- 5. ISSVA classification for vascular anomalies © (Approved at the 20th ISSVA Workshop, Melbourne, April 2014, last revision May 2018) https://www.issva.org/UserFiles/file/ISSVA-Classification-2018.pdf
- 6. Paul D. Losty, Alan W. Flake, Risto J. Rintala et-al. Rickham's Neonatal Surgery. (2018) ISBN: 9781447147213