Lymphatic Malformation
Everything in the library about lymphatic malformation — built automatically from the recorded discussions that name it
Educational content from recorded physician discussions — not medical advice. Always talk to your child's care team about your child's situation.
Content of this collection
Case-Based Learning
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Update Course Rewind 2025: Neck Pathologies - The Diagnostic Challenge
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This video, featuring Dr. Douglas von Allmen from Cincinnati Children's Hospital, explores the differential diagnosis and management of cystic neck masses in pediatric patients, specifically ranulas and lymphatic malformations. It presents
video3:55 · Jul 2026
In-Depth Reviews
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Lymphatic Anomalies
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This podcast is an interactive discussion about lymphatic malformations between Dr. Todd Ponsky, Dr. Belinda Dickie, Dr. Steve Fishman and Dr. Denise Adams. Dr. Belinda Dickie is an Assistant Professor of Surgery at Harvard Medical School a
podcast43:16 · Sep 2018
Lymphatic Anomalies
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This podcast is an interactive discussion about lymphatic malformations between Dr. Todd Ponsky, Dr. Belinda Dickie, Dr. Steve Fishman and Dr. Denise Adams. Dr. Belinda Dickie is an Assistant Professor of Surgery at Harvard Medical School a
podcast43:16 · Dec 2020
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Every expert statement below comes from the recorded discussions, with its speaker and moment.
Update Course Rewind 2025: Neck Pathologies - The Diagnostic Challenge
A ranula is a benign cyst that forms under the tongue caused by saliva leaking from a blocked sublingual gland.
host_summaryJill Knepprath0:50 ↗
Lymphatic malformations are clusters of lymph vessels that are often present at birth.
host_summaryJill Knepprath0:58 ↗
It can be really hard to differentiate a giant ranula from a lymphatic malformation.
clinicalDoug Van Alman1:14 ↗
The lab tends to have trouble running thick fluid from aspirated neck masses.
clinicalDoug Van Alman1:24 ↗
Lymphatic malformations near glandular tissue can have leaching of saliva into them, resulting in false positive amylase testing.
clinicalDoug Van Alman1:28 ↗
Removal of the sublingual gland has been the best practice for ranula treatment.
guidelineDoug Van Alman2:10 ↗
When a ranula has a cervical component, you can aspirate that and as long as you remove the sublingual gland, it should go away.
clinicalDoug Van Alman2:16 ↗
Micromarsupialization is a new technique for treating ranula with an intraoral component, involving placement of silk sutures through the cyst for 30 days to develop a fistula that spontaneously drains into the mouth.
clinicalDoug Van Alman2:25 ↗
For lymphatic malformations, there are 3 procedural treatments: laser, sclerotherapy, and excision.
host_summaryJill Knepprath2:43 ↗
There are 3 medical therapies for lymphatic malformations that are still undergoing clinical trials.
host_summaryJill Knepprath2:49 ↗
Gene panels now guide medical therapy for lymphatic malformations, particularly PIK3CA mutations which suggest that alpelisib (a PI3K alpha inhibitor) can be useful.
clinicalDoug Van Alman2:53 ↗
For congenital developmental neck masses, you have to deal with the embryological component and etiology to prevent recurrence, not just excise the mass.
clinicalDoug Van Alman3:15 ↗
Lymphatic Anomalies
The ISSVA classification system was updated in 2014 and is based on John Mulligan's original classification from Boston Children's Hospital
guidelineDenise Adams2:12 ↗
Lymphatic anomalies are classified into lymphatic tumors and lymphatic malformations, with malformations further divided into macrocystic, microcystic, combined lesions, generalized lymphatic anomaly, Gorham-Stout disease, and central conducting anomalies
clinicalDenise Adams2:58 ↗
Using incorrect terminology like cystic hygroma and lymphangioma causes confusion among clinicians, colleagues, and patients
opinionSteve Fishman4:45 ↗
Macrocystic lymphatic malformations can be described to patients as like a bunch of grapes where each grape is visible and can be accessed with a needle for sclerotherapy
clinicalSteve Fishman5:43 ↗
Microcystic lesions are like a small porous sponge with cysts too tiny to see individually, making sclerotherapy less practical
clinicalSteve Fishman6:14 ↗
Most lymphatic malformations are present at birth and visible at birth, though some may present later with sudden expansion
clinicalSteve Fishman9:06 ↗
There are four general treatment options for lymphatic malformations: observation with reassurance, microinterventional therapy with needle or catheter, resective surgery, and pharmacological therapy
clinicalSteve Fishman10:05 ↗
For very large truncal lesions extending from axilla to pelvis, surgical resection may be preferred over multiple sclerotherapy sessions to avoid significant radiation exposure and potentially unsatisfactory outcomes from residual tissue
opinionSteve Fishman12:30 ↗
Sirolimus (rapamycin) is an mTOR inhibitor that blocks the PIK3CA pathway, which has been shown to be a somatic mutation in many lymphatic malformations
clinicalDenise Adams15:43 ↗
Boston Children's Hospital received FDA funding in 2008 to study sirolimus for complicated vascular anomalies based on the hypothesis that the PIK3CA pathway controls blood vessel growth
clinicalDenise Adams16:18 ↗
Sirolimus improved quality of life, decreased pain, and reduced infection frequency in patients with complicated lymphatic anomalies
clinicalDenise Adams16:59 ↗
Preoperative sirolimus therapy can soften lymphatic tissue and decrease lesion size, making surgical procedures less extensive
clinicalDenise Adams17:41 ↗
Operating on patients while continuing sirolimus makes tissue softer, allows easier elevation of skin flaps, and enables more extensive resection with better closure compared to operating without the medication
clinicalSteve Fishman19:31 ↗
Sirolimus is unlikely to be useful for purely macrocystic lesions, and the tissue expansion effect from large cysts can actually make surgery easier
opinionSteve Fishman20:25 ↗
Spontaneous resolution of macrocystic lymphatic malformations occurs in less than a handful of cases out of several thousand patients
epidemiologicalSteve Fishman22:33 ↗
EXIT procedures are not really necessary for lymphatic lesions because they are soft and compressible, allowing intubation, unlike firm teratomas
clinicalSteve Fishman23:27 ↗
Babies with congenital lymphedema of the lower extremities can have regression to the point of non-detection on physical exam, though this is uncommon
clinicalSteve Fishman25:11 ↗
For lesions impinging on the airway that could cause emergency if infected or bleeding occurs, observation is not a good option
clinicalSteve Fishman25:55 ↗
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