Lymphatic malformations

Last revised by Rohit Sharma on 28 Jan 2024

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:

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 This expression is out-of-date according to the 2018 ISSVA classification 5.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 1:6000-16,000 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

  • 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:

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

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:

  • hemangioma

  • venous malformation: demonstrate internal blood flow and central enhancement

  • other cystic neck masses

    • teratoma: demonstrates fat or calcification

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