Lymphatic Malformations
A lymphatic malformation is a mass in the head or neck that results from an abnormal formation of lymphatic vessels. Lymphatic vessels are small canals that lie near blood vessels and help carry tissue fluids from within the body to the lymph nodes and back to the bloodstream.
There are two main types of lymphatic malformations:
Nearly all cystic hygromas occur in the neck, although some lymphangiomas can occur in the mouth, cheek, and tissues surrounding the ear, as well as other parts of the body. Over half of lymphatic malformations are congenital (present at birth), and most are detected by age 2. Some lymphatic malformations can spread into surrounding tissues and affect the proper development of the area. Lymphatic malformations may also enlarge and become infected following an upper respiratory infection.
Lymphatic malformations are sometimes seen in children with certain chromosome abnormalities and genetic conditions, including Down syndrome and Turner syndrome.
A lymphatic malformation is a congenital (present from birth) defect that occurs during early embryonic development when the lymphatic vessels do not properly form. The vessels may become blocked and enlarged as lymphatic fluid collects in the vessels, forming a mass or a cyst.
An internal lymphatic malformation in the mouth or cheek may not be noticed until it becomes infected and enlarges. Cystic hygromas can grow very large and may affect breathing and swallowing. The following are the most common symptoms of a lymphatic malformation. However, each child may experience symptoms differently. Symptoms may include:
The symptoms of a lymphatic malformation may resemble other neck masses or medical problems. Always consult your child's physician for a diagnosis.
During pregnancy, a fetal ultrasound may detect some large lymphatic malformations. Ultrasound is a diagnostic tool used to evaluate organs and structures inside the body with high-frequency sound waves. After birth, diagnosis of a lymphatic malformation is generally determined by a physical examination. In addition to a complete medical history and physical examination, diagnostic procedures for a lymphatic malformation may include the following:
Specific treatment of lymphatic malformation will be determined by your child's physician based on:
Treatment may include:
A small percentage of lymphangiomas can regrow and are often detectable within a year after surgery. Regrowth is more likely with larger and more complicated malformations.
==========
Lymphatic
Malformations
Cincinnati Children's
Hospital and Medical Center
Lymphatic Malformations
Related Areas:
Hemangioma and Vascular Malformation Center
Pediatric Surgery
What are they? | What do they look like? | How are they diagnosed? |
What are
possible complications? | How are they managed? | Are there risks
associated
with treatment?
What are lymphatic malformations?
Lymphatic malformations are sponge-like collections of abnormal
channels and
spaces that contain clear fluid. The lymphatic system normally collects
excess
fluid from the tissues and transports it through a series of small
vessels back
into the venous system. However, with a lymphatic malformation,
transfer of this
fluid through these vessels is slowed. The excess fluid accumulates and
dilates
the vessels, resulting in a swelling of the affected area and sometimes
in more
extensive enlargement of
soft tissues and bones.
These
lesions are most commonly seen in the neck (illustration 1)
and axilla
(armpit) (illustration 2), but they can involve any area of the body.
In certain
areas the dilated vessels tend to be small (illustration 3), while in
other
areas, they tend to be large (illustration 4). As with other vascular
malformations, lesions may be superficial or deep, localized or
diffuse. They
steadily increase in size, although some enlarge more rapidly than
others.
Conditions such as infection or
trauma can result in a sudden, but temporary
enlargement.
Although the exact cause of lymphatic malformations is unknown, they
are thought
to be caused by errors in the formation and development of
lymphatics during
fetal development. The cause is not related to any known drug or
medication that
may have been taken during pregnancy or to any environmental exposure
that may
have occurred during that time.
What do lymphatic malformations look like?
There are two types of lymphatic malformations and these are often
combined.
Macrocystic malformations are large, soft, smooth clear masses under
normal or
bluish skin. Microcystic malformations present as small, raised lesions
containing clear fluid. Superficial lesions may present as tiny clear
bubbles
that sometimes become dark red due to bleeding. Both macrocystic and
microcystic
malformations may cause enlargement of any structure (illustration 5)
(e.g., the
lip, cheek, ear, tongue, limb, finger, or toe).
How are lymphatic malformations diagnosed?
Medical history and physical examination are generally sufficient to
diagnosis
lymphatic malformations. However, since they can be confused with
venous
anomalies, magnetic resonance imaging (MRI) and computed tomography
(CT) are
sometimes performed to confirm the diagnosis and also to determine the
extent of
the lesion.
What are the possible complications of a lymphatic malformation?
Spontaneous lymphatic leakage from the skin commonly occurs. As well as
being a
nuisance, this can lead to or be associated with cellulitis (infection
of tissue
in and beneath the skin).
Recurrent
cellulitis,
which causes pain and cosmetic
disfigurement, can also occur and may lead to serious infection.
Bacteria may
readily enter via open vesicles and quickly spread through tissues
affected by
these lesions. When this occurs, aggressive antibiotic therapy is
essential.
Another major acute complication is bleeding into the lymphatic
malformation.
Additional complications may occur, depending upon the specific area of
the body
that is affected by the lesion:
A lesion involving the trachea (windpipe), tongue or chest can cause
difficulty
breathing.
A lesion in the gastrointestinal tract (stomach and intestines) can
cause
protein loss.
A lesion in the thorax (chest) can cause chylothorax,
which is a leakage of
lymphatic fluid into the chest cavity. It can also cause heart and lung
complications.
A lesion in the abdomen can cause
chylous
ascites, which is a leakage of
lymphatic fluid into the abdomen.
While certain complications cannot be prevented, they can sometimes be
diminished. Developing an infection in a lymphatic malformation of the
head/neck
region is less likely if careful attention is paid to oral hygiene.
Teeth should
be brushed regularly and thoroughly and should be cleaned by a dental
hygienist
more frequently than is usual.
How are lymphatic malformations managed?
Surgical excision (removal). Some lesions, especially those that are
localized,
are generally treated by surgical excision. As well, surgical excision
is very
often used as part of an overall treatment plan that includes one or
more of the
other treatment approaches listed below. When lesions are extensive and
surround
many vital structures, a surgical approach may be difficult or
impossible.
Sclerotherapy (direct injection of an irritating agent). For large
macrocystic
lesions,
sclerotherapy with alcohol or Picibanil (a new
investigational drug)
has been reported to cause total or near-total shrinkage in 80% of
cases
(illustrations 8-9).
Laser therapy. Depending on the complexity of the lesion, either the
Nd:YAG, KTP,
or CO2 (carbon dioxide) laser is used selectively with beneficial
results. These
lasers are generally used as a component of an overall treatment plan
that
integrates other approaches as well.
Laser therapy requires multiple treatments
that are spaced over several months. It is done under general
anesthesia.
Radio frequency ablation. This is a relatively new technique that
employs
high-energy radio frequency sound waves to selectively destroy
superficial
lesions. As with other treatment approaches, it may be used as a
component of an
overall treatment strategy involving several approaches over a period
of time.
Are there any risks associated with treatment?
Despite the benefits, each of these management approaches has drawbacks
and
limitations:
Surgical excision. This cannot be done without some scarring, and years
after
excision, lymphatic vesicles may become apparent again in or around a
scar.
Additionally, surgical excision may cause damage to structures involved
with the
lesion.
Sclerotherapy. This approach may result in deep tissue scarring. Also,
in some
cases, the lesion may not respond at all to treatment. In other cases,
it may
reappear.
Laser therapy. This may result in some tissue scarring and/or changes
in skin
pigmentation. These can sometimes be improved by additional cosmetic
procedures.
Radio frequency ablation. Minor bleeding and infection are the primary
risks of
this technique.
http://www.cincinnatichildrens.org/health/info/vascular/diagnose/lymphatic.htm
..........
Also see:
http://www.cincinnatichildrens.org/health/info/vascular/diagnose/lymphedema.htm
..........
Cincinnati Children's Hospital Medical Center
3333 Burnet Avenue, Cincinnati, Ohio 45229-3039
513-636-4200 | 1-800-344-2462 | TTY: 513-636-4900
http://www.cincinnatichildrens.org/about/corporate/leader.htm
===========================
Research Abstracts and Articles:
Current concepts in lymphatic
malformations.
Lee BB, Kim YW, Seo JM, Hwang JH, Do YS, Kim DI, Byun HS, Lee
SK, Huh SH,
Hyun WS.
Department of Surgery, Sungkyunkwan University School of Medicine and
Samsung
Medical Center, Seoul, Korea. bblee38@comcast.net
A lymphatic malformation (LM) is the most common form of congenital
vascular
malformation (CVM). The new Hamburg classification of CVM distinguishes
the
truncular (T) form from the extratruncular (ET) form of LMs. Both are
consequences of a developmental arrest at the different stages of
lymphangiogenesis as a result of defective genes. The
purpose of this review was
to evaluate the current management results of both forms of LMs. A
retrospective
review of the clinical data of 315 patients with a diagnosis of LMs
treated
between September 1994 and December 2001 was performed.
Lymphoscintigraphy
was
the most frequent diagnostic test. The patients with the ET form were
treated
with sclerotherapy with OK-432 and/or ethanol. Combinations of CDP (complex
decongestive physiotherapy) and/or compressotherapy were used
to treat all the
T-form patients. In addition, surgery, either reconstructive or
ablative, was
offered to patients with the T form who failed to respond to the proper
CDP. A
multidisciplinary team performed the management of LM, and the results
were
evaluated every 6 months. Among 797 patients with CVM, 315 were
confirmed to
have LMs, either as the T form (226) or the ET form (89). Another 66
LMs were
diagnosed with hemolymphatic malformations (HLM). Most of the ET forms
(89/315)
were the cystic type (70/89), while the T forms included aplasia and/or
an
obstruction (204/226). The ET form was most frequent in the head, neck,
and
thorax (69/89). The T form was located most frequently to the
extremities
(202/226), mostly to the lower limb (180/202). Two hundred and
twenty-six T
forms belonged to the various clinical stages: stages I-32, II-104,
III-48,
IV-18, and an unclear stage-24. The ET form was treated with
sclerotherapy using
OK-432 (108/120) and absolute ethanol (12/120). Among the 11 patients
with the
multiple ET form, 7 patients underwent perioperative sclerotherapy with
OK-432
and a subsequent surgical excision. The clinical response of the T form
at the
extremity to CDP was excellent to good in a majority of clinical stages
I to II
(121/136) but decreased to a good to fair degree in stages III to IV
(31/66).
The additional surgical therapy, either reconstructive (10/19) or
ablative
(9/19), provided limited success in improving CDP efficacy, owing
mainly to poor
compliance. The long-term outcome of the initial success through
self-motivated
home-maintenance care during the follow-up period of up to 48 months
was totally
dependent on patient compliance. OK-432 sclerotherapy to 51 ET forms
has shown
excellent results on 88.9% of the cystic type (40/45) and 50% (3/6) of
the
cavernous type (minimum follow-up for 24 months). Seventeen ET forms in
7
patients were treated with a preoperative OK-432 sclerotherapy and a
subsequent
surgical excision, which provided good to excellent results in 14 for a
minimum
of 24 months. Primary lymphedema,
which is the T form of LMs, can be managed
safely by a combination of CDP with compressotherapy. Patients with
good
compliance can benefit from additional surgical therapy, either
reconstructive
or ablative. The ET form, particularly the cystic type, can be treated
with
various scleroagents that are preferably less toxic as the primary
therapy. A
surgical excision with or without perioperative sclerotherapy provides
good
results for patients with the localized cavernous type of the ET form.
A
multidisciplinary team approach is essential for the proper care of LM.
PMID: 15696250 [PubMed - in process]
..........................
Facial nerve anatomy, dissection and preservation in lymphatic malformation management.
..........................
Doxycycline sclerotherapy as primary treatment of head and neck lymphatic malformations in children.
..........................
Definitive percutaneous treatment of lymphatic malformations of the trunk and extremities
..........................
Volvulus of a jejunal lymphatic vascular malformation presenting with bilious vomiting and the radiological appearances of malrotation
..........................
Clinical outcomes in lymphocytopenic lymphatic malformation patients.
..........................
Complex lymphatic malformations: diagnostic and therapeutical implications
===========================
External Links:
Vascular
Lymphatic Malformations
http://www.hmc.psu.edu/childrens/healthinfo/uz/vascularmalform.htm
..........................
Lymphatic Malformations
What is a lymphatic malformation?
A lymphatic malformation is a mass in the head or neck that results
from an
abnormal formation of lymphatic vessels. Lymphatic vessels are small
canals that
lie near blood vessels and help carry tissue fluids from within the
body to the
lymph nodes and back to the bloodstream.
There are two main types of lymphatic malformations:
lymphangioma - a group of lymphatic vessels that form a mass or lump. A
cavernous lymphangioma contains greatly enlarged lymphatic vessels.
cystic hygroma - a large cyst or pocket of lymphatic fluid that results
from
blocked lymphatic vessels. A cystic hygroma may contain multiple cysts
connected
to each other by the lymphatic vessels....
http://www.schneiderchildrenshospital.org/peds_html_fixed/peds/ent/lympmalform.htm
..........................
Vascular, Lymphatic Malformations
http://www.emedicine.com/plastic/TOPIC470.HTM
..........................
Vascular Birthmarks Foundation
http://www.birthmark.org/lm.php
..........................
Children Anguished with Lymphatic Malformations
..........................
Lymphatic malformation
DermNet NZ
http://dermnetnz.org/vascular/lymphangioma.html
===========================
Lymphedema People Related Pages
Lymphangiomas
http://www.lymphedemapeople.com/thesite/lymphedema_lymphangiomas.htm
Cystic Hygromas
http://www.lymphedemapeople.com/thesite/lymphedema_cystic_hygroma.htm
===========================
Diagnostic Images:
http://images.google.com/images?hl=en&q=lymphangioma&um=1&ie=UTF-8&sa=N&tab=wi
Yahoo
===========================
Diagnostic Codes and External Resources:
| D18.140: |
Cystic hygroma |
| D18.130: |
Lymphangioma circumscriptum |
| D18.130: |
Lymphangioma circumscriptum cutis |
| D18.140: |
Lymphangioma, cavernous |
| D18.110: |
Lymphangioma, simple |
ICD-9 Codes
Hemangioma and lymphangioma any site
228.0 is a non-specific code that cannot be used to specify a diagnosis
| DiseasesDB | 7665 |
|---|---|
| MedlinePlus | 000148 |
| eMedicine | derm/866 |
| MeSH | D008202 |
===========================
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Advocates for Lymphedema
Dedicated to be an advocacy group for lymphedema patients. Working towards education, legal reform, changing insurance practices, promoting research, reaching for a cure.
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| Subscribe: | AdvocatesforLymphedema-subscribe@yahoogroups.com |
Pat O'Connor
Lymphedema People / Advocates for Lymphedema
===========================
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Lymphedema Glossary
http://www.lymphedemapeople.com/wiki/doku.php?id=glossary:listing
===========================
Lymphedema People - Support Groups
-----------------------------------------------
Children
with Lymphedema
The time has come for families, parents, caregivers to have a support
group of
their own. Support group for parents, families and caregivers of
chilren with
lymphedema. Sharing information on coping, diagnosis, treatment and
prognosis.
Sponsored by Lymphedema People.
http://health.groups.yahoo.com/group/childrenwithlymphedema/
Subscribe: childrenwithlymphedema-subscribe@yahoogroups.com
......................
Lipedema
Lipodema Lipoedema
No matter how you spell it, this is another very little understood and
totally
frustrating conditions out there. This will be a support group for
those
suffering with lipedema/lipodema. A place for information, sharing
experiences,
exploring treatment options and coping.
Come join, be a part of the family!
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Subscribe: lipedema_lipodema_lipoedema-subscribe@yahoogroups.com
......................
MEN WITH LYMPHEDEMA
If you are a man with
lymphedema; a man with a loved one with lymphedema who you are trying
to help
and understand come join us and discover what it is to be the master
instead of
the sufferer of lymphedema.
http://health.groups.yahoo.com/group/menwithlymphedema/
Subscribe: menwithlymphedema-subscribe@yahoogroups.com
......................
All
About Lymphangiectasia
Support group for parents, patients, children who suffer from all forms
of
lymphangiectasia. This condition is caused by dilation of the
lymphatics. It can
affect the intestinal tract, lungs and other critical body areas.
http://health.groups.yahoo.com/group/allaboutlymphangiectasia/
Subscribe: allaboutlymphangiectasia-subscribe@yahoogroups.com
......................
Lymphatic
Disorders Support Group @ Yahoo Groups
While we have a number of support groups for lymphedema... there is
nothing out
there for other lymphatic disorders. Because we have one of the most
comprehensive information sites on all lymphatic disorders, I thought
perhaps,
it is time that one be offered.
DISCRIPTION
Information and support for rare and unusual disorders affecting the
lymph
system. Includes lymphangiomas, lymphatic malformations,
telangiectasia,
hennekam's syndrome, distichiasis, Figueroa
syndrome, ptosis syndrome, plus many more. Extensive database of
information
available through sister site Lymphedema People.
http://health.groups.yahoo.com/group/lymphaticdisorders/
Subscribe: lymphaticdisorders-subscribe@yahoogroups.com
......................
All
About Lymphedema
For our Google fans, we have just created this online support group in
Google
Groups:
Homepage: http://groups-beta.google.com/group/All-About-Lymphedema
Group email: All-About-Lymphedema@googlegroups.com
......................
Lymphedema Friends
http://groups.aol.com/lymphedemafriend
If you an AOL fan and looking for a
support group in AOL
Groups, come and join us there.
===========================
Lymphedema People New Wiki Pages
Have
you seen our new
“Wiki” pages yet? Listed
below
are just a sample of the more than 140 pages now listed in our Wiki
section. We
are also working on hundred more.
Come
and take a stroll!
Lymphedema
Glossary
http://www.lymphedemapeople.com/wiki/doku.php?id=glossary:listing
Lymphedema
http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema
Arm
Lymphedema
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Leg
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The
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Exercises
for Lymphedema
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Diuretics
are not for
Lymphedema
http://www.lymphedemapeople.com/wiki/doku.php?id=diuretics_are_not_for_lymphedema
Lymphedema
People Online
Support Groups
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Lipedema
http://www.lymphedemapeople.com/wiki/doku.php?id=lipedema
Treatment
http://www.lymphedemapeople.com/wiki/doku.php?id=treatment
Lymphedema
and Pain
Management
http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_and_pain_management
Manual
Lymphatic Drainage (MLD) and Complex Decongestive Therapy (CDT)
Infections
Associated with
Lymphedema
http://www.lymphedemapeople.com/wiki/doku.php?id=infections_associated_with_lymphedema
How
to Treat a Lymphedema
Wound
http://www.lymphedemapeople.com/wiki/doku.php?id=how_to_treat_a_lymphedema_wound
Fungal
Infections Associated
with Lymphedema
http://www.lymphedemapeople.com/wiki/doku.php?id=fungal_infections_associated_with_lymphedema
Lymphedema
in Children
http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_in_children
Lymphoscintigraphy
http://www.lymphedemapeople.com/wiki/doku.php?id=lymphoscintigraphy
Magnetic
Resonance Imaging
http://www.lymphedemapeople.com/wiki/doku.php?id=magnetic_resonance_imaging
Extraperitoneal
para-aortic lymph node dissection (EPLND)
Axillary
node biopsy
http://www.lymphedemapeople.com/wiki/doku.php?id=axillary_node_biopsy
Sentinel
Node Biopsy
http://www.lymphedemapeople.com/wiki/doku.php?id=sentinel_node_biopsy
Small
Needle Biopsy - Fine Needle Aspiration
http://www.lymphedemapeople.com/wiki/doku.php?id=small_needle_biopsy
Magnetic
Resonance Imaging
http://www.lymphedemapeople.com/wiki/doku.php?id=magnetic_resonance_imaging
Lymphedema
Gene FOXC2
http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_gene_foxc2
Lymphedema Gene VEGFC
http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_gene_vegfc
Lymphedema Gene SOX18
http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_gene_sox18
Lymphedema
and
Pregnancy
http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_and_pregnancy
Home page: Lymphedema People
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Page Updated: April 14, 2008