Congenital Lymphatic Malformations

by Joseph Alpert, MD

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    00:00 Well let’s talk for a few moments about congenital malformations of the lymph system.

    00:04 They’re very rare. Very, very rare. And almost always seen in childhood and almost always taken care of in childhood. So adult physicians almost never see this except in a very, very rare circumstance.

    00:19 You can have obstruction of some of the primitive lymph vessels during embryonic life. And what this does is it produces an abnormal lymphatic system with almost what looks like a cyst as you can see on this child’s neck: a collection of fluid.

    00:37 It’s more commonly seen in the head and neck and the armpit. And it is almost always taken care of surgically. These are removed and they’re benign. They’re not cancerous.

    00:49 Sometimes you’ll see little small ones inside the mouth, in the oral cavity. And sometimes they’ll look a little bit like frogs’ eggs, little tiny translucent bubbles, little cysts usually less than 2 cm across. They’ve been called frogs’ eggs or rice pudding.

    01:10 They can also be seen in the neck and just below the jaw. And here you see a picture of an infant with one that’s quite substantial. And again, these are often taken care of surgically in early childhood. And they almost never are seen in adults.

    01:27 There was a time when people tried various drugs squirting alcohol or one of the chemotherapeutic agents bleomycin or doxycycline – one of the relatives of tetracycline antibiotic – into them. But usually that doesn’t work. If you have a very small one, yes it might work in that. If they become infected, of course, you’re going to use antibiotics. But usually surgical excision is the mainstay of therapy for these. And they’re not neoplastic. They are not cancer. They do not metastasize and they’re not life threatening. They’re usually cosmetic although sometimes, if they’re large enough, they can interfere with normal function.

    02:13 The peripheral lymphatic malformations outside of the head and neck require some surgical intervention or they can be dealt with with laser. If they’re very small, sometimes just a compressive stocking or an elastic bandage can control a very localized one.

    02:39 But, usually, they’re obliterated with laser or they’re resected surgically.

    02:44 The ones that are the most trouble – you saw in the picture of the infant already – are ones that are in the head and neck.

    About the Lecture

    The lecture Congenital Lymphatic Malformations by Joseph Alpert, MD is from the course Diseases of the Lymphatic System.

    Included Quiz Questions

    1. Neck
    2. Soles
    3. Palms
    4. Thorax
    5. Back
    1. Surgical removal
    2. Antibiotics
    3. Beta-blockers
    4. Angioplasty
    5. Chemotherapy

    Author of lecture Congenital Lymphatic Malformations

     Joseph Alpert, MD

    Joseph Alpert, MD

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