Lymphangiomas
Lymphangiomas and Lymphedema
Depending on the stage of your lymphedema,
there will be other changes that
occur
in the
skin of the affected limb. Not only do these
complications involve
lymphangiomas, but include changes in skin texture, areas
of discoloration, possible growths such as
skin
tags, dermatofibromas,
warts,
and even cysts.
Furthermore, lymphangiomas can be associated with other syndromes that include lymphedema, these are:
Turner
syndrome,
Hydrops
fetalis,
Down
syndrome and other
trisomy
disorders,
Noonan
syndrome.
--------------------------------
Lymphangiomas
Lymphangiomas, also known as
lymphatic
malformations, are abnormal, localized or
generalized growths consisting of newly formed, enlarged (dilated)
lymphatic
vessels. Maybe expressed in lymphedema as
blisters containing
lymph.
Lymphangiomas,
or lymphangiomata, are embryologic malformations of the
lymphatic system
consisting of
benign lymphatic
tumors,
usually present at birth. Most (70-90%)
of these vascular malformations are usually identified by the end of
the 1st
year of postpartum life.
These
lymph channel or cystic
lymph spaces lined by
endothelium are probably the
result of abnormalities in
lymphangiogenesis
(lymphatic
development).
Lymphangiomata or lymphatic endothelial “cysts” usually grow slowly and
may
gradually compress surrounding structures, but they do not undergo
malignant
changes.
Localization
Lymphangiomata
can arise almost anywhere in the skin,
subcutaneous tissue, intermuscular septa
and mucous membranes. The most common sites are the head and neck
(including the
tongue), the proximal extremities, trunk and buttocks. Rarely they can
be found
in the abdominal viscera (liver,
spleen,
intestines, heart,
pancreas).
Clinical
manifestations:
There
are four classical types of lymphangioma:
1-
Lymphangioma simplex
These
are single, well-circumscribed, usually smooth, subcutaneous tumors.
Definition:
2-
Lymphangioma circumscriptum
A
very common form of cutaneous lymphangioma consisting of multiple
clusters of
clear, pink or red vesicles. Pink or red appearance indicates the
presence of
blood mixed in with the lymph; in this case, mixed vascular
malformations are
present.
Definition: (1) A congenital nevoid lesion consisting of a circumscribed group of tense lymph vesicles. (2) A skin lesion that develops from enlarged lymphatic_vessels. Most commonly seen in young children, it may be pink or yellow and may grow to several centimeters in size.
3-
Cavernous lymphangioma
A
large soft tissue tumor composed of cavernous lymphatic spaces. This
condition
is rare.
Definition: (1) Conspicuous dilation of lymphatic vessels in a circumscribed region, frequently with the formation of lymph filled cavities. (2) A tumor formed by dilated lymphatic_vessels and filled with lymph that is often mixed with coagulated blood. The lesion may cause extensive enlargement of the affected tissue.
4- Cystic lymphangioma or cystic hygroma
These
benign lymphatic tumors are unilocular or multilocular masses composed
of a
collection of thin-walled
vesicles resembling a bunch of grapes, filled with
clear or yellowish lymph fluid.
These
cysts are usually soft, translucent and painless.
They
are most commonly located in the neck (hygroma colli), head, intraoral
(tongue,
floor of the mouth, salivary glands), mediastinum, axilla, groin, and
popliteal
fossa.
Definition: A condition characterized by a fairly circumscribed group of several cystlike dilated vessels or spaces lined with endothelium and filled with lymph.
Complications
Lymphangiomata
may swell. On rare occasion they become infected or hemorrhage.
Their extension
may lead to respiratory or digestive problems,
chylothorax or chylopericardium
(accumulation of
chyle or lymph in the thorax or pericardium, respectively).
Cervicofacial Lymphangiomas
The most common complication is incomplete resection and recurrence. A recurrence rate as high as 50% has been reported. Cranial nerve injury exceeds the rate of 20%; the facial nerve is the most common neural deficit reported.
Additional complications include ocular motility problems, difficulty swallowing, aspiration, lingual and hypoglossal nerve injury, spinal accessory nerve loss, brachial plexus, and phrenic nerve injury. Secondary infection, cosmetic deficits, thrombocytopenia, and secondary airway obstruction have also been recorded.(2)
Oral lymphangiomas
Dental caries are proportionately prevalent in these patients with oral lymphangiomas and are most likely a result of patient's and dentist's difficulties in maintaining adequate oral hygiene. Dysmorphogenesis of the maxillofacial skeleton is frequently observed in association with oral and cervicofacial malformations. Speech pathologies are common. Feeding difficulties are common in neonates. Alternate enteral feeding routes, including a gastrostomy, are often necessary. Repeated paroxysms of bleeding may occur secondary to trauma. Complications can result from surgical treatment. Complication rates of surgical debulking are typically 20-30%. Complications include airway obstruction, seromas and hematomas, infections, and cranial nerve palsies. Reported surgical mortality rates are 2.5-11.4%. (3)
Diagnosis
Generally, lymphangiomas are diagnosed by simple clinical appearance. However, imaging techniques also play an important role in the diagnoses, evaluation and treatment process.
The radiology studies used are ultrasonography, MRI (magnetic resonance imaging) and CT or CAT Scans. At times, artiography may be used for cervicofacial lymphaomas. Lymphoscintigraphy is not used in lymphangiomas.
Treatment
These
conditions usually require surgery,
laser therapy or
sclerotherapy.
Some lesions (especially lymphangiomata circumscripta and cavernous lymphangiomata) have high recurrence rates after excision.
Prognosis
Because lymphangiomas are benign lymphatic malformations, prognosis is
excellent, although in some cases the lymphangiomas do grow back.
===========================
Research Abstracts and Articles:
..................
Nonsurgical therapies for lymphangiomas: A systematic review.
Otolaryngol Head Neck Surg. 2008 Apr
Department of Otolaryngology-Head and Neck Surgery, Walter Reed Army Medical Center, Washington, DC.
OBJECTIVE: Systematically review the published literature regarding the efficacy of nonsurgical therapies in the treatment of head and neck (H&N) lymphatic malformations (LM) in children.
DATA SOURCE: MEDLINE.
REVIEW METHODS: MEDLINE was searched for literature relating to nonsurgical treatments for H&N LM.
RESULTS: The initial search returned 1876 articles, with 22 meeting criteria. The majority (20) were case series. All therapies were percutaneous, with OK-432 or bleomycin sclerotherapy being most common. Random-effects modeling revealed 43% (CI = 28.9%-57%) of patients undergoing OK-432 for LM achieved a complete/excellent response, 23.5% (CI = 5.8%-41.3%) achieved a good response, 16.9% (CI = 10.3%-23.4%) achieved a fair/poor response, and 15.4% (CI = 8.6%-22.2%) observed no response. In the bleomycin group, the results were: 35.2% (CI = 15.7%-54.6%) excellent, 37.1% (CI = 22%-52.3%) good, 18.4% (CI = 2.7%-34.2%) fair/poor, and 11.6% (CI = 3.5%-19.6%) no response. Seven major complications were noted out of the 289 patients in the series, including two mortalities.
CONCLUSIONS: The literature indicates that sclerotherapy for H&N LM achieves excellent/good clinical response in a majority of patients, with few complications, and anecdotally does not complicate future surgery.
PMID: 18359347
PubMed
http://www.ncbi.nlm.nih.gov/pubmed/18359347
..................
Mediastinal cavernous lymphangioma in an adult.
Gen Thorac Cardiovasc Surg. 2008 Feb
Department of Surgery, Shiga University of Medical Science, Seta-Tsukinowa, Otsu, Shiga 520-2192, Japan. teramoto@belle.shiga-med.ac.jp
Cavernous lymphangioma is a rare mediastinal benign tumor. A 43-year-old woman presented with cough and dyspnea for 1 month. Computed tomography of the chest showed a 3-cm well-circumscribed cystic mass in the posterior mediastinum. At thoracotomy, a cystic tumor in the mediastinum that was adherent to the descending aorta and esophagus was removed completely. The tumor, the cystic space of which was filled with lymph fluid, was diagnosed as cavernous lymphangioma based on pathological findings. Mediastinal lymphangiomas may insinuate into surrounding organs. As incomplete resection can result in recurrence, complete tumor removal should be performed based on accurate preoperative evaluation.
..................
Laser excision of multiple esophageal lymphangiomas: A case report and review of the literature.
Auris Nasus Larynx. 2008 Jun
Yale University School of Medicine Division of Otolaryngology, Head & Neck Surgery, New Haven, CT, USA.
Lymphangiomas of the gastrointestinal tract are rare and benign submucosal tumors. We present the first case of multiple lymphangiomas in the cervical esophagus and only the 15th case of an esophageal lymphangioma. A 63-year-old male presented to our clinic with two years of dysphagia and an esophageal mass noted on MRI. A barium swallow confirmed a polypoid mass of the upper esophagus with operative findings of two smooth pedunculated lymphangiomas occupying the entire lumen of the esophagus. Previous reports have documented successful surgical intervention through open or endoscopic techniques, whereas the favorable location of the tumors in this case allowed for the first successful excision utilizing a CO(2) laser. We demonstrate here that esophageal laser surgery can provide an accurate, complete, and minimally invasive excision technique for lymphangiomas of the upper digestive tract with minimal morbidity for the patient. A complete review of all published cases is presented, with a focus on the clinical presentation and surgical treatment of this disease
..................
A case of cardiac lymphangioma presenting as a cystic mass in the right atrium.
Yonsei Med J. 2007 Dec 31
Department of Internal Medicine, Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan, Korea, 290-3 Jeonha-dong, Dong-gu, Ulsan 682-714, Korea.
A 44-year-old woman underwent surgery for an asymptomatic primary tumor of the heart located in the right atrium. The tumor was detected incidentally during follow-up computed tomography for a resected breast cancer. The mass, lying along the lower portion of the right atrial septum, was homogenous and cystic in nature, as detected by transthoracic and transesophageal echocardiography. Complete resection was performed via a median sternotomy under cardiopulmonary bypass. The postoperative course was uneventful. However, the histological result was surprising: the mass was a cardiac lymphangioma.
Pub Med
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2628184/?tool=pubmed..................
Cystic lymphangioma of the gallbladder: report of a case.
Surg Today. 2008
Department of Surgery II, Nagoya University Graduate School of Medicine, 65 Tsuruma-cho, Showa-ku, Nagoya 466-8550, Japan.
Abdominal lymphangioma is usually diagnosed within the first 2 years of life and is extremely rare in adults. The most common location of abdominal lymphangioma is the mesentery, but there are sporadic reports of its development in the gallbladder. A 66-year-old woman was found to have a cystic lesion near the gallbladder. Preoperative studies, including endoscopic ultrasonography, computed tomography, and magnetic resonance imaging, showed a tumor with multilocular cystic structure, originating in the gallbladder fossa. The patient underwent exploratory laparotomy, and the mass was resected en bloc with the gallbladder, as there was no evidence of malignancy on intraoperative ultrasonography. Macroscopically, the tumor was a multilocular cystic mass, 6 x 3 x 2 cm in size, with a rough, sponge-like appearance. Histologically, the cystic tumor was diagnosed as a lymphangioma, originating in the gallbladder. To our knowledge, only three other cases of a cystic lymphangioma originating in the gallbladder have been reported in the medical literature of the world.
===========================
External Links:
Lymphangioma (1)
http://www.emedicine.com/DERM/topic866.htm
Cervicofacial Lymphangiomas (2)
http://www.emedicine.com/ent/topic575.htm
Oral Lymphangiomas (3)
http://emedicine.medscape.com/article/1076948-overview
Lymphangiomas
http://www.wrongdiagnosis.com/l/lymphangiomas/intro.htm
What is Lymphangiomas?
http://www.wrongdiagnosis.com/l/lymphangiomas/basics.htm
Lymphangiomas of the Head and Neck
http://www.actaitalica.it/issues/2008/1-08/grasso.pdf
Bilateral symmetrical lymphangiomas of the gingiva: case report
http://www.diagnosticpathology.org/content/1/1/9
Sclerosing treatment of lymphangiomas with OK-432
http://adc.bmj.com/cgi/content/abstract/82/4/316
Cavernous, arteriovenous, and mixed hemangioma-lymphangioma of the rectosigmoid: rare causes of rectal bleeding-case series and review of the literature.
Acquired lymphangioma circumscriptum of the vulva.
Surgical management of primary vulvar lymphangioma circumscriptum and postradiation: case series and review of literature.
Ultrasound biomicroscopy of eyelid lymphangioma in a child.
Establishment of lymphangioma model and a study on the promoting effect of murine melanoma cell B16-F1 on the lymphangiogenesis in vitro.
Gastrointestinal tract lymphangiomas: findings at CT and endoscopic imaging with histopathologic correlation.
Pulmonary lymphangioma.
Elsevier
http://www.sciencedirect.com/science/article/pii/S0003497507015561
Lymphangioma scroti. Lymphangioma of the Tongue
Lymphangioma of the Tongue, A Rare Entity Giant Lymphagioma of the Tongue
http://www.nacd.in/ijda/volume-03-issue-03/150-lymphangioma-of-tongue-a-rare-entity
Giant Lymphagioma of the TongueSmall Bowel Lymphangoima
http://www.laparoscopy.com/pictures/lap_lymp.html
===========================
Related Lymphedema People Links:
Cystic Hygroma
http://www.lymphedemapeople.com/thesite/lymphedema_cystic_hygroma.htm
Lymphatic Malformations
http://www.lymphedemapeople.com/thesite/lymphedema_lymphatic_malformations.htm
===========================
Diagnostic Codes and External Resources:
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 |
===========================
Diagnostic Images:
http://images.google.com/images?hl=en&q=lymphangiomas&um=1&ie=UTF-8&sa=N&tab=wi
Yahoo
http://images.search.yahoo.com/search/images?p=lymphangiomas&fr=yfp-t-501&toggle=1&cop=mss&ei=UTF-8
===========================
Join us as we work for lymphedema patients everywehere:
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.
http://health.groups.yahoo.com/group/AdvocatesforLymphedema/
Subscribe: | AdvocatesforLymphedema-subscribe@yahoogroups.com |
Pat O'Connor
Lymphedema People / Advocates for Lymphedema
===========================
For information about Lymphedema
http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema\
For Information about Lymphedema Complications
http://www.lymphedemapeople.com/wiki/doku.php?id=complications_of_lymphedema
For Lymphedema Personal Stories
http://www.lymphedemapeople.com/phpBB2/viewforum.php?f=3
For information about How to Treat a Lymphedema Wound
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For information about Lymphedema Treatment
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For information about Exercises for Lymphedema
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For information on Infections Associated with Lymphedema
http://www.lymphedemapeople.com/wiki/doku.php?id=infections_associated_with_lymphedema
For information on Lymphedema in Children
http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_in_children
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!
http://health.groups.yahoo.com/group/lipedema_lipodema_lipoedema/?yguid=209645515
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
http://www.lymphedemapeople.com/wiki/doku.php?id=arm_lymphedema
Leg
Lymphedema
http://www.lymphedemapeople.com/wiki/doku.php?id=leg_lymphedema
Acute
Lymphedema
http://www.lymphedemapeople.com/wiki/doku.php?id=acute_lymphedema
The
Lymphedema Diet
http://www.lymphedemapeople.com/wiki/doku.php?id=the_lymphedema_diet
Exercises
for Lymphedema
http://www.lymphedemapeople.com/wiki/doku.php?id=exercises_for_lymphedema
Diuretics
are not for
Lymphedema
http://www.lymphedemapeople.com/wiki/doku.php?id=diuretics_are_not_for_lymphedema
Lymphedema
People Online
Support Groups
http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_people_online_support_groups
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
http://www.lymphedemapeople.com
Page Updated: Jan. 8, 2012