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Lymphangiectasia is a condition in which the lymphatics are dilated and is commonly associated with lymphedema..  When the lymph channels,vessels become dilated, the lymph valves cease to work correctly and the lymph fluid flows backward. Eventually, the fluid leaks into the interstitium causing swelling.

This disease is characterized by lymphatic vessel dilation, and can affect the skin, eyelids, intestines, lungs and other various organs.

The condition can be either primary (present from birth or congenital) or secondary (acquired through trauma or injury to the lymphatics).  Specific causes of secondary lymphangiectasia include radical mastectomy with or without radiation therapy; irradiation alone for various malignancies; metastatic lymph node obstruction; and various scarring processes, such as infections, keloids, scleroderma, and scrofuloderma. It has also been described in the penis and the scrotum after removal of a sacrococcygeal tumor; they may also arise on the vulva and the inner thigh after surgery for cervical cancer.

Other complications associated with lymphangiectasia are determined by the area of the body that is effected. For a complete detail of each type, please see the internal Lymphedema People links at the bottom of the page. 

Historically, intestinal lymphangiectasia (IL) was thought to be the most common type.  However, there is more and more documentation in the medical literature of the fact the lymphedema patients have secondary cutaneous (skin) lymphangiectasia as a result of the streatching and swelling involved with that condition.  Other areas of recorded lymphangiectasia include the lungs, kidney (Renal), vulva, penis,scrotum and even conjunctiva.

Also, should the lymphedema move into the abdominal and/or chest cavities, intestinal and/or pulmonary lymphangiectasia can result.



Is a condition resulting in  dilation of the lymphatic vessels that is caused by lymphatic damage, which leads to the blockage of local lymphatic drainage.

This can be either a congenital or an acquired disorder of the lymphatic system.  The most common form of lymphangiectasia is the intestinal form.  (IL) The condition may however, be in other parts of the body.  Other forms include pulmonary (lungs), renal (kidneys), skin, or 

On microscopic examination, lymphangiectases are characterized by large, dilated lymph vessels lined by a single layer of endothelial cells. They are usually found in the papillary dermis and the reticular dermis. Involvement in the deeper dermis is rare. The overlying epidermis is usually associated with acanthosis and hyperkeratosis but is less marked than in angiokeratomas. At times, acquired lymphangiectases grow above the level of adjacent skin.

Lymphangiectases lack the subcutaneous muscle-coated cisternae characteristic of lymphangioma circumscriptum. Although no specific histologic criteria exist with which to differentiate lymphangioma circumscriptum from lymphangiectasia, some believe that the absence of subcutaneous cisternae in lymphangiectases is a true histologic difference and that this finding reflects the difference in the pathogenesis of both disorders.

Risk Factors

Signs and Symptoms




“Protein-losing Enteropathy” is a fancy way of saying there is something wrong with the intestine such that protein is being lost from the body through the intestine. This is a serious problem as the body’s proteins are not easily replaced and the only way to replace them involves the absorption of protein constituents (the amino acids that make up proteins) from the intestine. If the intestine is actually leaking nutrients out instead of absorbing them in, the result is a nutritional disaster.

The main protein which one cannot afford to lose is called “Albumin.” This protein normally is produced by one’s liver and circulates in the bloodstream acting as a carrier for biochemicals that require transport but cannot actually dissolve in blood. Albumin can be considered sort of a mass transit system in the bloodstream, a bus or subway, if you will, carrying important biochemicals from one place to another.

Albumin, by being the most prevalent blood protein, also is responsible for actually keeping water in one’s bloodstream.  When water cannot be held within the vasculature, it leaks out causing fluid accumulation in tissue (i.e. edema) or in within the chest or abdomen (i.e. effusion).

Of course, in a protein-losing enteropathy, other proteins are lost, too. Antibodies, proteins of blood clotting, enzymes, etc. all leak out the intestine and are forever lost in the feces that exits the body.

The body tries hard to maintain its albumin level by extracting protein from other sources (like muscle), and having the liver make albumin from the components of these other proteins.  This may help maintain a workable amount of albumin in the bloodstream but it comes at the expense of muscle tissue and other protein.

There are four chief cases of protein-losing enteropathy:

The first three conditions are reviewed elsewhere and will not be discussed here.


Lymph is a fluid that circulates through the body similar to the way blood does; though blood is pumped actively through the body by the heart while lymph is pumped passively via the normal muscle activity of the body.  Lymph consists of cells called “lymphocytes,” which are cells of the immune system. Lymph also consists of fluid which collects from the tissues and shunts into actual vessels similar to veins.

The word “lymphangiectasia” means “dilated lymph vessels.” In the intestinal tract, lymphangiectasia is usually caused by some kind of inflammation which puts back pressure on the lymph vessels leading them to dilate. Lymph flow may be blocked by the inflammatory events in the intestine or local structures.

Lacteals are special lymph vessels in the intestinal tract designed to absorb nutritional fats. When there is high pressure within the lymph vessels, the tender lacteals burst and instead of absorbing fats, the lymph inside, its cells, fats, and precious proteins are lost.  The intestine may be able to reabsorb some of these valuable substances at other sites but if the inflammatory intestinal disease that started the problem in the first place is widespread, the balance may have shifted to nutritional loss rather than gain.


Weight loss is the most consistent sign along with chronic diarrhea, vomiting, and fluid accumulation in the abdomen creating a bloated appearance.


In most cases, an obviously sick dog is brought to the veterinarian. Sometimes the above classical signs are present but sometimes there is no specific hint of this condition until blood test results are in.

These conditions can be easily ruled out one by one. It is obvious if there are third degree burns present. If there are none, a routine urinalysis will indicate if there is significant protein loss in the urine and if glomerular disease should be pursued. A liver function test such as a bile acids test will indicate whether or not there is liver failure latently present. If none of these three conditions are present, then there must be a protein-losing enteropathy.


As mentioned, there are four likely causes of protein-losing enteropathy. To distinguish them and initial the correct treatment, an intestinal biopsy is essential. This can be done surgically or via endoscopy but rational treatment is not possible without a tissue sample.


The first step in treatment is to address the underlying cause. In most cases of lymphangiectasia, the underlying cause involves inflammation and most treatment of lymphangiectasia involves suppression of inflammation.

Medications such as prednisone, and/or azathioprine are commonly used, especially if inflammatory bowel disease is present.

The second step in treatment is dietary though success has been mixed. Traditionally, rather nasty tasting Medium Chain Triglycerides have been used in lymphangiectasia treatment. Triglycerides (a fancy word for “fats”) are very long molecules. Some are longer than others. The more usual dietary fats are called “Long Chain Triglycerides” and, when absorbed into one’s body, must be repackaged into fat globules called “chylomicra” and are normally absorbed into the lymph vessels. In lymphangiectasia, we want to reduce the pressure in the lymph vessels. We want less lymph. The idea was that if the patient ate shorter fat chains, the fats could be absorbed right into bloodstream directly and bypass the lymph system altogether. Whether or not this actually happens is still a matter of controversy but the addtion of Medium Chain Triglycerides (or “MCT’s”) in conjunction with a low fat diet are common recommendations in the therapy of lymphangiectasia.

Other treatments include the use of diuretics (such as furosemide) to help increase urination and ultimately reduce fluid accumulation in the chest or abdomen. Actual tapping of the body cavity and suctioning the fluid affected may be needed periodically.

If the underlying condition is treatable then prognosis for lymphangiectasia is good.  It should be understood that lymphangiectasia is unlikely to be cured and at best can be managed.


Pulmonary lymphangiectasia 

Primary pulmonary lymphangiectasia in infancy and childhood

P.M. Barker1, C.R. Esther, Jr1, L.A. Fordham2, S.J. Maygarden3 and W.K. Funkhouser3

Depts of 1 Paediatrics (Division of Paediatric Pulmonology), 2 Radiology, and 3 Pathology, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA

Correspondence: P.M. Barker, Division of Paediatric Pulmonology, Dept of Paediatrics, 200 Mason Farm Road, CB 7220, University of North Carolina at Chapel Hill, Chapel Hill, NC, 27599, USA. Fax: 1 9199666179. E-mail:

Keywords: Growth, pathology, radiology, spirometry

Received: February 4, 2004
Accepted May 20, 2004

C.R. Esther Jr is supported by a Parker B. Francis Pulmonary Fellowship.

Primary pulmonary lymphangiectasia (PPL) is a rare disorder of unknown aetiology characterised by dilatation of the pulmonary lymphatics. PPL is widely reported to have a poor prognosis in the neonatal period and little is known about the clinical features of patients who survive the newborn period.

The current authors report the outcome in nine patients diagnosed in infancy with PPL over a 15-yr period at a single university-based hospital clinic and followed for a median of 6 yrs.

Although all of the patients initially experienced respiratory distress, respiratory symptoms improved in most patients after infancy and were notably better by the age of 6 yrs. Many patients had poor weight gain in the first years of life, which eventually improved. Radiological scans showed progressive resolution of neonatal infiltrates, but were characterised by hyperinflation and increased interstitial markings in older children. Most patients had evidence of bronchitis and grew pathogenic organisms from quantitative bronchoalveolar lavage culture. Pulmonary function tests showed predominantly obstructive disease that did not deteriorate over time.

In conclusion, these results suggest that primary pulmonary lymphangiectasia does not have as dismal a prognosis as previously described and symptoms and clinical findings improve after the first year of life.


Congenital dilatation of the pulmonary lymphatics

SN Javett, I Webster and JL Braudo
904 Medical Centre Jeppe Street, Johannesburg, South Africa

The disease variously referred to as congenital dilatation of the pulmonary, lymphatics congenital pulmonary cystic lymphangiectasis, and congenital lymphangiomatosis of the lung is a rarity known almost exclusively to pediatric pathologists. Up to 1959, 21 cases had appeared in world literature, with only two reports in clinical journals. Congenital dilatation of the pulmonary lymphatics has been observed as a chance finding in fetuses dying in utero, stillborn infants, and neonates, without any mention being made as to its possible significance. The great majority of those born alive have suffered in the immediate neonatal period from an acute respiratory disease exhibiting dyspnea and cyanosis, death following within a few hours. As a consequence, the opportunity for clinical observation and investigation has been strictly limited. Confirmation has been possible only by autopsy. With virtually no clinical information to work on, the pathologists have centered interest almost entirely on the identification of lymphatics, the histopathology, and theories of causation without being in a position to offer correlation between histologic findings and symptoms. In the absence of any clinical build-up, too, it is not surprising that the disease has escaped the attention of most clinicians. We have had the opportunity of observing and investigating a patient with congenital dilatation of the pulmonary lymphatics, still alive, albeit not well, at the age of 8 months. It is the purpose of this paper to describe as a clinical entity a condition hitherto regarded as a pathological curiosity.


Lymphangiectasia, retroperitoneal

Lymphangioma, retroperitoneal,
a developmental malformation and not a true neoplasm, though it presents as a retroperitoneal mass. Lymphangiectasia is the dilatation of abnormal lymphatic channels, which have failed to establish normal communication with the rest of the lymphatic system. The dilated lymphatic channels conglomerate and form a unilocular or multilocular cystic mass, known as a lymphangioma.

The diagnosis of retroperitoneal lymphangioma is usually made in infancy. It occurs with equal frequency in males and females. Common clinical findings include pain, abdominal distension, fever, fatigue, weight loss and haematuria.

A retroperitoneal lymphangioma can be imaged by ultrasound, CT or MRI. It may be elongated in contour and characteristically spans more than one retroperitoneal compartment. Uncomplicated fluid is seen within the mass or locules, which are separated by thin septa. The fluid appears complicated in the presence of haemorrhage or infection. Rarely, calcification and chyle are present


Renal Lymphangiectasia

Lorenz T. Ramseyer, MD

1 From Bass Baptist Memorial Hospital, 600 S Monroe, Enid, OK 73701. Received May 28, 1999; revision requested July 20; revision received August 26; accepted August 30. Address correspondence to the author (e-mail:

Index terms: Diagnosis Please • Kidney, CT, 81.12112 • Lymphatic system, abnormalities, 994.829 • Lymphatic system, CT, 994.12912 • Urography, 81.11


A 28-year-old obese man had microscopic hematuria. Physical examination results were normal. His medical history was unremarkable, and there was no pertinent family history. Excretory urography (Fig 1) and computed tomography (CT) of the abdomen  were performed after oral and intravenous administration of contrast material.


The excretory urogram demonstrated bilateral nephromegaly with distortion of the pelvocaliceal systems (Fig 1). The CT scan of the abdomen showed fluid collections in the perinephric space bilaterally, surrounding the renal cortex. In addition, there were peripelvic fluid collections bilaterally, with distortion of the pelvocaliceal systems. There were fluid collections in the retroperitoneum that crossed the midline at the level of the renal hila, adjacent to the abdominal aorta and the inferior vena cava.


Several differential diagnostic possibilities are to be considered with bilateral nephromegaly with pelvocaliceal splaying and distortion. Adult polycystic kidney disease, lymphoma, nephroblastomatosis, and other causes of multiple renal masses, such as von Hippel-Lindau disease and tuberous sclerosis, are considerations. Adult polycystic kidney disease has characteristic findings of numerous bilateral renal cysts, with or without hepatic or pancreatic cysts. The cysts typically vary in size and are scattered throughout the parenchyma. Lymphoma and other malignancies can demonstrate soft-tissue masses involving the kidneys, pelvocaliceal systems, or retroperitoneum. The fluid attenuation of the perinephric collections in the test case, as evidenced by attenuation measurements of 0–10 HU in  exclude lymphoma and other soft-tissue masses. Perinephric and retroperitoneal soft-tissue masses can be seen in retroperitoneal fibrosis but are again excluded by the fluid attenuation in the test case. Nephroblastomatosis is a cause of nephromegaly in children and is characterized by multiple subcapsular and parenchymal soft-tissue nodules composed of metanephric blastema (1).

Renal lymphangiectasia is a rare disorder. Patient symptoms described in the literature (24) include hematuria, flank pain, and abdominal pain. The condition has been found in children and in adults (2,47). The origin of this disorder is speculative. There is a familial association in some cases, which argues for a congenital cause (6). There was no known family history of renal lymphangiectasia in the test case. Others argue for an acquired cause, which suggests that the lymphatic vessels may become blocked owing to inflammation or other obstruction and so cause lymphatic ectasia (8). Others have suggested that these lesions may be a true neoplasm (9). The nomenclature of this disorder is confusing and has evolved in recent years. Other names have included "renal lymphangiomatosis" (3,6), "renal lymphangioma" (5), "peripelvic lymphangiectasia" (2), and "renal peripelvic multicystic lymphangiectasia" (8). "Renal lymphangiectasia" is the preferred name, given that the disorder is characterized by ectatic perirenal, peripelvic, and intrarenal lymphatic vessels (10,11).

Imaging findings of renal lymphangiectasia include peripelvic cysts and perirenal fluid collections. The finding of retroperitoneal fluid collections, presumably dilated lymphatic vessels, is a more variable finding but has been noted in multiple cases in the literature (24,6).

Renal lymphangiectasia has been found associated with renal venous thrombosis and hypertension (4,6,12). Ascites and large perinephric fluid collections have been found and are exacerbated by pregnancy (6). The natural history of this disorder is not completely understood. In a neonatal case, partial regression was reported (7).

The diagnosis of renal lymphangiectasia can be confirmed with needle aspiration of chylous fluid from the perinephric fluid collections (4). However, the ultrasonographic and CT findings are characteristic for this disease and allow the diagnosis to be made confidently (2,6). Treatment is not usually necessary. Complicated cases may be treated with nephrectomy, percutaneous drainage, or marsupialization (4).

In the case presented here, the diagnosis of renal lymphangiectasia was based on the characteristic CT findings. The perirenal, peripelvic, and retroperitoneal collections had attenuation measurements consistent with fluid (0–10 HU) rather than with the soft-tissue attenuation seen with lymphoma or other causes of bilateral renal soft-tissue masses . No invasive procedures to confirm diagnosis were deemed appropriate for this patient.

Our congratulations to the 26 individuals who submitted the most likely diagnosis (renal lymphangiectasia) for Diagnosis Please, Case 34. The names and locations of the individuals, as submitted, are as follows

Marc P. Banner, Philadelphia, Pa; Lawrence R. Bigongiari, MD, Hope, Ark; Marc G. de Baets, MD, Lugano, Switzerland
Kemal Demir, MD, Ataköy, Istanbul, Turkey; Giovanna Demurtas, MD, Cagliari, Italy; Luis E. Fajre, MD, Tucuman, Argentina; Sandra K. Fernbach, Chicago, Ill; Milton R. Fuentealba, MD, General Roca, Rio Negro, Argentina;
Celso Ichihara, Brazil; Kartik Jhaveri, MD, Mumbai, India; Douglas S. Katz, MD, Mineola, NY; Glenn Krinsky, MD, New York, NY;  Prof. Dr. Luis Mendez Uriburu, Tucuman, Argentina;  Sergio J. Moguillansky, MD, Cipolletti, Rio Negro, Argentina;  Adalberto Montanhini Júnior, Brazil;  Steven Perlmutter, MD, Mineola, NY;  Timothy J. Phalen, MD, Cincinnati, Ohio;  Dr. Arturo Ramos-Pablos, Cd. Obregón, Son., México;  Luiz Antonio Rossi, São Paulo, Brazil;  Anthony J. Scuderi, MD, Johnstown, Pa;  Matt Shapiro, MD, Lowell, Mass;  Paolo Siotto, MD, Cagliari, Italy;  Douglas L. Teich, MD, Brookline, Mass; Christopher Vittore, MD, Rockford, Ill; Keith Wittenberg, MD, Rochester, Minn; Joe Yut, Olathe, Kan


Part 1 of this case appeared 4 months previously and may contain larger images.


A Model for Mechanics of Primary Lymphatic Valves

Journal of Biomechanical Engineering -- June 2003 -- Volume 125, Issue 3, pp. 407-414

Ernesto Mendoza and Geert W. Schmid-Schönbein

Department of Bioengineering, The Whitaker Institute for Biomedical Engineering, University of California San Diego, La Jolla, CA 92093-0412

(Received Sep. 2002; revised Dec. 2002)

Keywords: physiological models, cellular transport, biological tissues,biological fluid dynamics

Recent experimental evidence indicates that lymphatics have two valve systems, a set of primary valves in the wall of the endothelial cells of initial lymphatics and a secondary valve system in the lumen of the lymphatics. While the intralymphatic secondary valves are well described, no analysis of the primary valves is available. We propose a model for primary lymphatics valves at the junctions between lymphatic endothelial cells. The model consists of two overlapping endothelial extensions at a cell junction in the initial lymphatics. One cell extension is firmly attached to the adjacent connective tissue while the other cell extension is not attached to the interstitial collagen. It is free to bend into the lumen of the lymphatic when the lymphatic pressure falls below the adjacent interstitial fluid pressure. Thereby the cell junction opens a gap permitting entry of interstitial fluid into the lymphatic lumen. When the lymphatic fluid pressure rises above the adjacent interstitial fluid pressure, the endothelial extensions contact each other and the junction is closed preventing fluid reflow into the interstitial space. The model illustrates the mechanics of valve action and provides the first time a rational analysis of the mechanisms underlying fluid collection in the initial lymphatics and lymph transport in the microcirculation.

Journal of Biomechanical Engineering 

Lymphangiectasia of the vulva, treatment with CO 2 laser. 

Jan. 2012

Indian Journal of Dermatology, Venereology and Leprology

Recurrent lymphangiectasia of the left supraclavicular fossa: an unusual cause of paroxystic swelling. 

Nov 2011

Archives of Dermatology

Ocular coherence tomography in lymphangiectasia. 

Oct 2011


Recurrent breast cellulitis associated with lymphangiectasia after tumorectomy for breast cancer 

Jun-Jul 2011



Other resources, organizations


All About Lymphangiectasia Yahoo Support Group

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.



External Links:


Support for Children with Lymphangiectasia


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Little Leaks

Hypoproteinemia in intestinal lymphangiectasia; Contribution of albumin "trapping" in the lymphedematous extremity

T Herskovic, SJ Winawer, R Goldsmith, R Klein and N Zamcheck
Department of Medicine, Yale University School of Medicine, New Haven, Connecticut


Intestinal Lymphangiectasia


Congenital Pulmonary Lymphangiectasia

New England Journal of Medicine


Primary intestinal and thoracic lymphangiectasia: a response to antiplasmin therapy



Acquired cutaneous lymphangiectasia with mesothelial cells reflux in a patient with cirrhotic ascites

Lippincott, Williams & Wilkins


Primary intestinal lymphangiectasia (Waldmann's disease).



Limb lymphedema as a first manifestation of primary intestinal lymphangiectasia (Waldmann's disease)

Keywords: Primary intestinal lymphangiectasia , Waldmann's disease , Lymphedema


Videocapsule endoscopy as a useful tool to diagnose primary intestinal lymphangiectasia



A primary intestinal lymphangiectasia patient diagnosed by capsule endoscopy and confirmed at surgery: a case report

World Journal of Gastroenterology


Chronic renal insufficiency in a boy with cystic renal lymphangiectasia: morphological findings and long-term follow-up



Immunohistochemical studies in a hydroptic fetus with pulmonary lymphangiectasia and trisomy 21.



Osteomalacia in a patient with primary intestinal lymphangiectasis (Waldmann's disease).



Renal Lymphangiectasia

British Journal of Radiology


Internal Lymphedema People Links

 Intestinal Lymphangiectasia

Pulmonary Lymphangiectasia 


Hennekam Syndrome 

Hennekam Lymphangiectasia Syndrome

Hennekam Syndrome

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Lymphangiectasia Blog Spot


Lymphangiectasia Classification

ICD-9 457.1

MeSH D008200


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