I often describe them to patients as like a bunch of grapes. You can see each grape, and that has therapeutic implications because if they're full of fluid the size of a grape, you can imagine sticking a needle in, sucking out the fluid, and instilling a sclerosis.
guidelineThe terms 'cystic hygroma' and 'lymphangioma' are outdated and should not be used; the correct term is 'lymphatic malformation' because it describes malformed embryologic lymphatic tissue.↗
▶Ep 1 · 4:29
clinicalMacrocystic lymphatic malformations have cysts large enough to be individually visualized (like a bunch of grapes), making them amenable to needle aspiration and sclerotherapy.↗
▶Ep 1 · 4:29
clinicalMicrocystic lymphatic malformations have cysts too small to see individually, presenting as a soft tissue mass like a porous sponge, making sclerotherapy less practical.↗
▶Ep 1 · 4:29
quoteIf we use the wrong names, we confuse ourselves, we confuse our colleagues, and we confuse our patients.↗
▶Ep 1 · 8:51
clinicalDifferential diagnosis for a 2-year-old with a new mass includes sarcomas, teratomas, and infectious lesions, not just lymphatic malformations.↗
▶Ep 1 · 8:51
clinicalFour general treatment options for lymphatic malformations are: observation/reassurance, microinterventional therapy (sclerotherapy), resective surgery, and pharmacological therapy.↗
▶Ep 1 · 8:51
opinionFor localized macrocystic lesions, sclerotherapy is preferred if one or two sessions can achieve a durable result without leaving a concerning scar; for very small lesions, minor resection may be preferred for a more durable outcome.↗
▶Ep 1 · 8:51
opinionVery large macrocystic lesions extending from axilla to pelvis may warrant upfront surgical resection rather than multiple sclerotherapy sessions, because shrinking all cysts still leaves redundant skin and septa that may require eventual resection anyway.↗
▶Ep 1 · 8:51
opinionEXIT (ex utero intrapartum treatment) procedures are not really necessary for lymphatic lesions, as they are soft and compressible and intubation is always achievable, unlike firm teratomas where EXIT plays a role.↗
▶Ep 1 · 8:51
clinicalMost lymphatic malformations are present at birth and visible at birth, though some may present later (e.g., at age 2) with sudden expansion of a previously undetected lesion.↗
▶Ep 1 · 8:51
quoteThese are not cancers. They're often not dangerous, and we talk about the risks of doing nothing or the risk of intervention.↗
▶Ep 1 · 18:29
opinionPurely macrocystic lesions are unlikely to benefit from sirolimus therapy; the drug is more useful for microcystic and complex lesions.↗
▶Ep 1 · 18:29
opinionDr. Fishman routinely operates on complex lesions (e.g., Klippel-Trénaunay syndrome) without stopping sirolimus preoperatively, finding that the tissue is softer and skin flaps more pliable, allowing more extensive resection with better closure.↗
▶Ep 1 · 22:04
clinicalBabies with congenital lymphedema of the lower extremities can have regression to the point of non-detection on physical exam, though this is uncommon.↗
▶Ep 1 · 22:04
opinionFor lesions not impinging on the airway or causing functional impairment, observation is a safe option, and the choice to intervene can be deferred.↗
▶Ep 1 · 22:04
opinionLesions impinging on the airway that could cause an emergency if infected or if they bleed internally should not be observed; early intervention is indicated.↗
▶Ep 1 · 22:04
opinionMicrocystic lesions on the face, tongue, or floor of the mouth are now treated very early in infancy, as early treatment likely yields better long-term results than delayed treatment.↗
▶Ep 1 · 22:04
clinicalMost lymphatic malformations do not spontaneously resolve, though rare cases of macrocystic lesions have deflated and remained quiescent for over a decade.↗
▶Ep 1 · 22:04
clinicalDr. Fishman observed a massive cervical and mediastinal macrocystic lymphatic malformation detected prenatally that became undetectable on physical exam within less than a year after birth, without intervention.↗
▶Ep 1 · 22:04
quoteSir, I'm really sorry that I was wrong, but I'm really glad I was wrong. I said this is a great outcome.↗
▶Ep 1 · 26:46
opinionFollow-up intervals for observed lymphatic malformations should be individualized: monthly initially for new parents, then extending to 3, 6, or 12 months as appropriate.↗
▶Ep 1 · 28:53
clinicalDr. Fishman encountered a small leg lesion thought to be a vascular malformation that was ultimately diagnosed as synovial sarcoma on frozen section during resection; the tumor was metastatic and the patient did poorly.↗
▶Ep 1 · 28:53
clinicalDr. Fishman was involved in a case of a newborn with what was thought to be multifocal hemangioma of the liver, which turned out to be metastatic neuroblastoma.↗
▶Ep 1 · 28:53
guideline'Burrows' rule' (named after interventional radiologist Pat Burrows) states that if a lesion is not classic on history, physical, and imaging, tissue biopsy is necessary to confirm diagnosis.↗
▶Ep 1 · 28:53
clinicalUltrasound with Doppler is the first-line imaging modality for suspected lymphatic malformations, as it is least invasive and does not require sedation or anesthesia.↗
▶Ep 1 · 28:53
clinicalMRI is the next step if ultrasound is inconclusive.↗
▶Ep 1 · 33:56
clinicalVenous sclerotherapy should almost always be performed under fluoroscopy to map venous drainage and prevent intravascular injection of sclerosant.↗
▶Ep 1 · 33:56
clinicalA small amount of ethanol injected systemically during venous sclerotherapy can cause sudden fatal pulmonary hypertension.↗
▶Ep 1 · 33:56
clinicalLarge venous anomalies (e.g., in Klippel-Trénaunay syndrome) with direct macroscopic outflow to systemic veins can result in pulmonary embolism from clot induced by sclerotherapy; the outflow must be obliterated first using intravenous laser, titanium coils, glue, or guide wires.↗
▶Ep 1 · 38:23
clinicalCentral conducting lymphatic anomalies can present with subcutaneous chyle, eroding bones, chylous ascites, chylothorax, and chyle dripping from the urethra, scrotum, vagina, or even from under toenails.↗
▶Ep 1 · 38:23
opinionCombined overgrowth syndromes like Klippel-Trénaunay and CLOVES syndrome involve multi-system and multi-tissue involvement and do not have simple treatment answers; these patients benefit from expert consultation.↗
Lymphatic Anomalies
▶Ep 3 · 4:45
opinionUsing incorrect terminology like cystic hygroma and lymphangioma causes confusion among clinicians, colleagues, and patients↗
▶Ep 3 · 5:01
quoteIf we use the wrong names, we confuse ourselves, we confuse our colleagues, and we confuse our patients.↗
▶Ep 3 · 5:43
clinicalMacrocystic 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↗
▶Ep 3 · 5:54
quoteI often describe them to patients as like a bunch of grapes. You can see each grape, and that has therapeutic implications because if they're full of fluid the size of a grape, you can imagine sticking a needle in, sucking out the fluid, and instilling a sclerosis.↗
▶Ep 3 · 6:14
clinicalMicrocystic lesions are like a small porous sponge with cysts too tiny to see individually, making sclerotherapy less practical↗
▶Ep 3 · 9:06
clinicalMost lymphatic malformations are present at birth and visible at birth, though some may present later with sudden expansion↗
▶Ep 3 · 10:05
clinicalThere are four general treatment options for lymphatic malformations: observation with reassurance, microinterventional therapy with needle or catheter, resective surgery, and pharmacological therapy↗
▶Ep 3 · 10:12
quoteThese are not cancers. They're often not dangerous, and we talk about the risks of doing nothing or the risk of intervention.↗
▶Ep 3 · 12:30
opinionFor 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↗
▶Ep 3 · 18:32
quoteI have become a passionate advocate of enter inhibition to determine whether or not interventional therapies, whether they be with a needle or a knife, are necessary.↗
▶Ep 3 · 19:31
clinicalOperating 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↗
▶Ep 3 · 20:25
opinionSirolimus is unlikely to be useful for purely macrocystic lesions, and the tissue expansion effect from large cysts can actually make surgery easier↗
▶Ep 3 · 22:33
epidemiologicalSpontaneous resolution of macrocystic lymphatic malformations occurs in less than a handful of cases out of several thousand patients↗
▶Ep 3 · 23:27
clinicalEXIT procedures are not really necessary for lymphatic lesions because they are soft and compressible, allowing intubation, unlike firm teratomas↗
▶Ep 3 · 24:46
quoteSir, I'm really sorry that I was wrong, but I'm really glad I was wrong. I said this is a great outcome.↗
▶Ep 3 · 25:11
clinicalBabies with congenital lymphedema of the lower extremities can have regression to the point of non-detection on physical exam, though this is uncommon↗
▶Ep 3 · 25:55
clinicalFor lesions impinging on the airway that could cause emergency if infected or bleeding occurs, observation is not a good option↗
▶Ep 3 · 26:09
opinionEarly treatment in infancy is now preferred for microcystic lesions on the face, tongue, and floor of mouth, with medical therapy rather than surgery↗
▶Ep 3 · 29:28
guidelineBurroughs rule states that if diagnosis is not classic on history, physical, and imaging, tissue biopsy is necessary↗
▶Ep 3 · 34:25
clinicalA small amount of ethanol systemically can cause sudden fatal pulmonary hypertension↗
▶Ep 3 · 34:25
quoteA small amount of ethanol systemically can cause sudden fatal pulmonary hypertension.↗
▶Ep 3 · 34:45
clinicalLarge venous anomalies with direct macroscopic outflow to systemic veins can result in pulmonary embolism from clot induced by sclerotherapy↗
▶Ep 3 · 34:59
clinicalVenous mapping by MR, ultrasound, or venography is essential before sclerotherapy, and direct venous outflow must be obliterated first using laser, coils, or glue to prevent fatal pulmonary embolism↗
▶Ep 3 · 35:45
quoteIt's fatal. And like I always say, don't ask me how I know.↗
▶Ep 3 · 37:12
epidemiologicalMost sclerotherapy worldwide is likely performed by surgeons rather than interventional radiologists because many countries lack image-guided fluoroscopy suites↗
▶Ep 3 · 39:38
clinicalPatients with central conducting lymphatic anomalies can present with subcutaneous chyle, eroding bones, chylous ascites, chylothorax, and chyle dripping from urethra, scrotum, vagina, or even from under toenails↗
Summaries Steve gave as host
· 2 summaries
Recaps of other experts' statements, not Steve's own clinical position.
Summaries Steve gave as host · Lymphatic Malformation2 summaries
host summarySteve Fishman summarizes what Dr. Denise Adams said: If it's not classic on history, physical, and imaging, you need to get tissue.↗
Lymphatic Anomalies
▶Ep 3 · 29:28
host summarySteve Fishman summarizes what Dr. Denise Adams said: Pat Burroughs used to say, you know, if it's not classic history, physical, and imaging, you need to get tissue.↗
