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.

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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: A circumscribed region or focus of several to numerous lymphatic vessels that are moderately dilated.

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 also referred to as Lymphangioma cavernosa or cavernosum

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).

Lymphangioma circumscriptum may occur.
- This condition is associated with minor bleeding, recurrent cellulitis, and lymph fluid leakage.
- Two cases of lymphangiosarcoma arising from lymphangioma circumscriptum have been reported. However, in both of the patients, the preexisting lesion was exposed to extensive x-ray therapy. Therefore, radiation therapy should be avoided in lymphangiomas.
In cystic hygroma, large cysts can cause dysphagia, respiratory problems, and serious infection if they involve the neck.(1)

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

The severity of the sequelae varies with the extent of the lesion.
Airway compromise is a possible complication.
- Oral and hypopharyngeal lesions often result in a compromised airway. Approximately 50% of children with oral lymphangiomas require tracheotomy. Close cooperation between the surgeon and an anesthesiologist skilled in fiberoptic intubation is imperative.
- The surgeon must be cognizant that these lesions may later expand and compromise the airway as a result of trauma, hemorrhage, infection, or upper respiratory tract infection. Surgeons should have a low threshold for performing a tracheostomy in patients in whom the potential for airway compromise exists.

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.

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Research Abstracts and Articles:

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Nonsurgical therapies for lymphangiomas: A systematic review.

Otolaryngol Head Neck Surg. 2008 Apr

Acevedo JL, Shah RK, Brietzke SE.

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

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Mediastinal cavernous lymphangioma in an adult.

Gen Thorac Cardiovasc Surg. 2008 Feb

Teramoto K, Suzumura Y.

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.

Springerlink

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Laser excision of multiple esophageal lymphangiomas: A case report and review of the literature.

Auris Nasus Larynx. 2008 Jun

Best SR, Coelho DH, Ahrens WA, Atez G, Sasaki CT.

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

Elsevier

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A case of cardiac lymphangioma presenting as a cystic mass in the right atrium.

Yonsei Med J. 2007 Dec 31

Kim SJ, Shin ES, Kim SW, Shin JK, Cheong JP, Kim YM, Lee SG.

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

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Cystic lymphangioma of the gallbladder: report of a case.

Surg Today. 2008

Shikano T, Takeda S, Sakai M, Sugimoto H, Kanazumi N, Nomoto S, Nakao A.

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.

Springerlink

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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.

Springerlink

Acquired lymphangioma circumscriptum of the vulva.

Dermatology Online Journal

Surgical management of primary vulvar lymphangioma circumscriptum and postradiation: case series and review of literature.

Elsevier

Ultrasound biomicroscopy of eyelid lymphangioma in a child.

PubMed

Establishment of lymphangioma model and a study on the promoting effect of murine melanoma cell B16-F1 on the lymphangiogenesis in vitro.

PubMed

Gastrointestinal tract lymphangiomas: findings at CT and endoscopic imaging with histopathologic correlation.

Springerlink

Pulmonary lymphangioma.

Elsevier

http://www.sciencedirect.com/science/article/pii/S0003497507015561

Lymphangioma scroti. Lymphangioma of the Tongue

Blackwell Synergy

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 Tongue

http://journals.lww.com/jcraniofacialsurgery/pages/articleviewer.aspx?year=2009&issue=01000&article=00066&type=abstract

Small Bowel Lymphangoima

http://www.laparoscopy.com/pictures/lap_lymp.html

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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

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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

228 includes:

angioma (benign) (cavernous) (congenital) NOS
cavernous nevus
glomus tumor
hemangioma (benign) (congenital)

228 excludes:

benign neoplasm of spleen, except hemangioma and lymphangioma (211.9)
glomus jugulare (227.6)
nevus:
- NOS (216.0-216.9)
- blue or pigmented (216.0-216.9)
- vascular (757.32)

2008 ICD-9-CM Diagnosis 228.1

Lymphangioma any site

228.1 is a specific code that can be used to specify a diagnosis
228.1 contains 11 index entries
View the ICD-9-CM Volume 1 228.* hierarchy

228.1 also known as:

Congenital lymphangioma
Lymphatic nevus

DiseasesDB	7665
MedlinePlus	000148
eMedicine	derm/866
MeSH	D008202

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Diagnostic Images:

Google

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

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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

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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

http://www.lymphedemapeople.com/wiki/doku.php?id=how_to_treat_a_lymphedema_wound

For information about Lymphedema Treatment

http://www.lymphedemapeople.com/wiki/doku.php?id=treatment

For information about Exercises for Lymphedema

http://www.lymphedemapeople.com/wiki/doku.php?id=exercises_for_lymphedema

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

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Lymphedema People - Support Groups

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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

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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

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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

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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

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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

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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

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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.

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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