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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:

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.

What causes a lymphatic malformation?

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.

What are the symptoms of a lymphatic malformation?

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.

How is a lymphatic malformation diagnosed?

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:

Treatment of lymphatic malformation:

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.

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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
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Also see:

http://www.cincinnatichildrens.org/health/info/vascular/diagnose/lymphedema.htm

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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

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Research Abstracts, Articles and Links:

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]

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=15696250&itool=iconabstr

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Lymphatic malformations: percutaneus treatment with bleomycin. 

Oct 2011

[Article in Spanish]
Cuervo JL, Galli E, Eisele G, Johannes E, Fainboim A, Tonini S, Joaquin W, Viola B, Nazar M.

Source

Servicio de Cirugía, Hospital de Niños Dr. Ricardo Gutiérrez.

Abstract

Lymphatic malformations are developmental abnormalities of the lymphatic system, which tend to complicate during their evolution. In the last decade, therapy with sclerosing agents has gained popularity over surgery due to its effectiveness, fewer complications, and excellent cosmetic results. We present a series of 24 patients treated with percutaneous bleomycin injection. Results were excellent (volume reduction ≥ 95%, without symptoms) in 12 patients, good (volume reduction between 50% and 95%, without symptoms) in 5 patients, fair (volume reduction <50%, without symptoms) in 4 patients, and poor (no change in volume from baseline and persistence of symptoms) in 3 patients. These results were directly related to the type oflymphatic malformation. The complications found had little clinical relevance. Sclerotherapy with bleomycin is an effective and safe treatment for patients with unicystic or macrocystic lymphatic malformations. It could also be used in patients with microcystic or cavernous lymphatic malformation undergoing surgery to reduce both the malformation size and postoperative lymphorrhea, and in postoperative symptomatic patients whose malformation could not be totally resected.

http://www.ncbi.nlm.nih.gov/pubmed/22042072

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Preliminary investigation of human lymphatic malformations in vitro. Nov 2011

http://onlinelibrary.wiley.com/doi/10.1002/lary.22187/abstract

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Diagnosis and management of extensive vascular malformations of the lower limb: part II. Systemic repercusion, diagnosis, and treatment. Npv 2011

http://www.eblue.org/article/S0190-9622(11)00355-0/abstract

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Diagnosis and management of extensive vascular malformations of the lower limb: part I. Clinical diagnosis. Nov 2011

http://www.eblue.org/article/S0190-9622(11)00311-2/abstract

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Treatment guidelines of lymphatic malformations of the head and neck. Dec. 2011

http://www.oraloncology.com/article/S1368-8375(11)00776-7/abstract

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Sclerotherapy of abdominal lymphatic malformations with doxycycline. Oct 2011

http://www.jvir.org/article/S1051-0443(11)01087-6/abstract

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Lymphatic malformations: clinical course and management in 64 cases. Aug 2011

http://onlinelibrary.wiley.com/doi/10.1111/j.1440-0960.2011.00777.x/abstract

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Hobnail hemangioma reclassified as superficial lymphatic malformation: A study of 52 cases. Aug 2011

http://www.sciencedirect.com/science/article/pii/S0190962211005949

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Facial nerve anatomy, dissection and preservation in lymphatic malformation management.

Elsevier

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Doxycycline sclerotherapy as primary treatment of head and neck lymphatic malformations in children.

Elsevier

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Definitive percutaneous treatment of lymphatic malformations of the trunk and extremities

Elsevier

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Volvulus of a jejunal lymphatic vascular malformation presenting with bilious vomiting and the radiological appearances of malrotation

Elsevier

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Clinical outcomes in lymphocytopenic lymphatic malformation patients.

Mary Ann Libert 

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Complex lymphatic malformations: diagnostic and therapeutical implications

PubMed

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Additional External Links:

Vascular Lymphatic Malformations

http://www.hmc.psu.edu/childrens/healthinfo/uz/vascularmalform.htm

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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://training.schneiderchildrenshospital.org/peds_html_fixed/peds/ent/lympmalform.htm

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EXIT (Ex utero Intrapartum Treatment) in lymphatic malformations of the head and neck: Discussion of three cases and proposal of an EXIT-TTP (Team Time Procedure) list. Nov 2011

http://www.sciencedirect.com/science/article/pii/S0165587611004496

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Percutaneous sclerotherapy in neonatal and infant head and neck lymphatic malformations: a single center experience. Nov 2011

http://www.jpedsurg.org/article/S0022-3468(11)00622-1/abstract

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Vascular, Lymphatic Malformations

http://www.emedicine.com/plastic/TOPIC470.HTM

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Vascular Birthmarks Foundation

http://www.birthmark.org/lm.php

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Children Anguished with Lymphatic Malformations

Address:11413 Prestige Dr
Frisco, TX 75034
Phone:(972) 377-4326

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Lymphatic malformation

DermNet NZ

http://dermnetnz.org/vascular/lymphangioma.html

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Lymphedema People Related Pages

Lymphangiomas

http://www.lymphedemapeople.com/thesite/lymphedema_lymphangiomas.htm

Cystic Hygromas

http://www.lymphedemapeople.com/thesite/lymphedema_cystic_hygroma.htm

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Diagnostic Images:

Google

http://images.google.com/images?hl=en&q=lymphangioma&um=1&ie=UTF-8&sa=N&tab=wi

Yahoo

http://images.search.yahoo.com/search/images;_ylt=A0geu.Y0gANIpl0B_YtXNyoA?ei=UTF-8&p=lymphatic%20malformations&fr2=tab-web&fr=yfp-t-501

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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

228 includes:

228 excludes:

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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Lymphedema People / Advocates for Lymphedema

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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

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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/

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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 

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)

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

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