Information on all the types of lymphangiectasia, including intestinal, pulmonary, renal, cutaneous (skin). Sponsored by Pat O'Connor

Thursday, January 25, 2007

Intestinal Lymphangiectasia

Intestinal Lymphangiectasia


“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:

Inflammatory Bowel Disease
Histoplasmosis (a fungal infection)
Intestinal Lymphosarcoma
Intestinal Lymphangiectasia

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.

Low Lymphocyte Count

Animals with lymphangiectasia have lymphocytes rolling out their lacteals and into their intestine. The low blood lymphocyte count is so consistent with lymphangiectasia that it is difficult to make this diagnosis if this finding is not present.

Low Cholesterol

Cholesterol is part of the lymph fluid being lost.

Low Albumin Level

Low blood albumin level is the most consistent finding in lymphangiectasia, though it is possible to have lymphangiectasia in a small portion of the intestine only and still maintain a normal albumin level.

There are only four ways a patient can develop a low albumin level.

A protein-losing enteropathy
Glomerular disease (such as glomerulonephritis) in which albumin is lost through diseased kidneys
Reduced albumin production in the liver due to liver failure
Serum leakage through extreme skin damage (such as third degree burns)

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.

Original Article

Monday, January 22, 2007

A review of the surgical treatment of lymphangiectasia and vulval lymphangioma: four case reviews.

A review of the surgical treatment of lymphangiectasia and vulval lymphangioma: four case reviews.

J Plast Reconstr Aesthet Surg. 2006;59(12):1442-5. Epub 2006 Mar 9.

Makh DS,
Mortimer P,
Powell B.

Department of Plastic Surgery, Plastic Surgery Unit, St George's Hospital, London, SW17 ORE, UK.

OBJECTIVE: To evaluate whether surgical management of vulval lymphoedema and/or lymphangiectasia conveys any longstanding patient benefit.

PATIENT AND METHODS: A qualitative analysis of signs and symptoms that occurred before and after surgical treatment for vulval lymphoedema and/or lymphangiectasia was performed. This was done by analysis of patient notes and telephone conducted interview.

RESULTS: From a hospital database search, four patients were found to have had surgical treatment - three for lymphangioma and one for lymphangiectasia. Overall there was a clear improvement in the signs and symptoms associated with these conditions. In particular, all patients reported an improvement (i.e. a reduction or elimination) in the amount of oedema following surgery.

CONCLUSION: Carbon dioxide laser therapy and superficial radiotherapy have been previously described for the management of vulval lymphangioma and lymphangiectasia with limited success, whereas our data suggests surgery offers a more permanent solution. In particular, labial reduction seems to be more successful than methods such as lymphovenous anastomoses and lymphangioplasties. A single operation may provide benefit for up to ten years. This approach has the potential to allow patients to be rehabilitated to normal life and activity.


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Vulval lymphangiectasia.

Department of Dermatology, Bristol Royal Infirmary, Bristol, UK.

Three cases of lymphangiectasia of the vulva are reported. One case followed Wertheim's hysterectomy for carcinoma of the cervix; two other cases had Crohn's disease with perineal involvement. Lymphangiectasia is a secondary phenomenon resulting from obstruction of previously normal lymphatics. This is in contrast with lymphangioma which is an anatomical abnormality. A review of the literature suggests that the vulval skin may be particularly susceptible to the formation of lymphangiectasia, and our patients' experiences suggest that they are easily mis-diagnosed.

PMID: 2684843 [PubMed - indexed for MEDLINE]

See Also:

Vulvar lymphangiectasias in Crohn's disease

Tuesday, January 16, 2007

Protein-losing enteropathy (primary intestinal lymphangiectasia) after the Fontan operation: clinical analysis of nine cases.

Chang Gung Med J. 2006 Sep-Oct;29(5):505-12.

Lin WS,
Hwang MS,
Chung HT,
Chu JJ,
Lai MW,
Yang JS,
Huang SC,
Huang JL,
Su WJ.
Department of Pediatrics, Chang Gung Children's Hospital. 5, Fushing Street, Gueishan Shiang, Taoyuan, Taiwan 333, ROC.

BACKGROUND: Protein-losing enteropathy (PLE) is a serious complication of a Fontan operation and has a very high mortality rate. The purpose of this study was to investigate the incidence, clinical manifestations, diagnostic approaches, laboratory findings, therapeutic modalities and outcome of patients with PLE at our institution.

METHODS: The diagnosis of PLE was based on clinical manifestations and laboratory studies. We reviewed medical records of patients who received a Fontan operation at our hospital form July 1985 to October 2005.

RESULTS: A total 101 patients underwent various modifications of the Fontan procedure during this period. Nine of the 75 patients (12%) who survived 30 days after surgery developed PLE, including 4 boys and 5 girls. The median time interval between the Fontan operation and onset of PLE was 3.7 years (range 1.2 to 9.7 years). Laboratory examination showed low serum albumin levels and increased fecal alpha-1-antitrypsin excretion. Lymphangiectasia was found on intestinal biopsy. Six patients had cardiac catheterization after development of PLE which demonstrated an elevated mean right atrium pressure (22.5 +/- 6.4 mmHg, range 16 to 33 mmHg) and mean pulmonary artery pressure (22.3 +/- 6.4 mmHg, range 16 to 33 mmHg).

Treatment included diet modification, albumin infusion, diuretics, inotropes, corticosteroids, heparin, and surgery. Four patients received medical treatment only. Two of these patients died due to sepsis and heart failure and 2 survived with partial relief of PLE. The remaining five received surgery for PLE after medical treatment failure. Three of them died after the operation and the two survivors were free of PLE, but one died of ventricular tachycardia 8 years later. The overall mortality rate was 67% (6/9).

CONCLUSIONS: The current treatment for PLE is associated with a very high mortality rate. Further investigation is needed to determine the exact mechanism of the disease and to develop new therapeutic approaches.

PMID: 17214396 [PubMed - in process]

Thursday, January 11, 2007

Congenital pulmonary lymphangiectasis sequence: a rare, heterogeneous, and lethal etiology for prenatal pleural effusion.

Congenital pulmonary lymphangiectasis sequence: a rare, heterogeneous, and lethal etiology for prenatal pleural effusion.


Wilson RD,
Pawel B,
Bebbington M,
Johnson MP,
Lim FY,
Stamilio D,
Silber A,
Zakii E,
Flake AW.
The Center for Fetal Diagnosis and Treatment, Department of Surgery at The Children's Hospital of Philadelphia/University of Pennsylvania School of Medicine, Philadelphia, PA 19104-4399, USA.

OBJECTIVE: Case report and literature review for congenital pulmonary lymphangiectasis (CPL)

CASE REPORT: Male fetus with bilateral pleural effusion, thoracoamniotic shunt, preterm delivery, and prolonged neonatal course with neonatal death at 3 months. Autopsy-identified CPL.

DISCUSSION: Review of pathology, clinical course, and genetics of CPL. CONCLUSION: This postnatal diagnosis of CPL/Hennekam syndrome must be considered with prenatal counseling regarding a fetus with bilateral pleural effusions. This pathological entity is autosomal recessive and has a significant risk of lethality.

Wiley Interscience

* * * * * *
Related Abstract

* * * * * *

Pleural effusions in the neonate.


Rocha G,
Fernandes P,
Rocha P,
Quintas C,
Martins T,
Proenca E.

Division of Neonatology, Department of Pediatrics, Hospital de Sao Joao, University Hospital, Porto, Portugal.

INTRODUCTION: Pleural effusions are rare in the neonate and may be associated to several clinical conditions. Only a few series of pleural effusions in the fetus and newborn are described in the literature. AIM: This study was undertaken to determine more accurately the causes and prognostic significance of pleural effusions in a population of high-risk neonates.

MATERIALS AND METHODS: A retrospective chart review of 62 neonates admitted to the neonatal intensive care unit of six medical centers in the north of Portugal, between 1997 and 2004, that presented the diagnosis of pleural effusion.

RESULTS: 33M/29F newborns; preterms 47 (76%); GA 33 (25-40) wk; BW 1830 (660-4270) g; C-section 39 (63%). Pleural effusions were congenital in 20 (32%) newborns and acquired in 42 (68%). Congenital pleural effusions occurred as fetal hydrops in 11 (18%) patients and as chylothorax in 9 (15%). In four cases of hydrops, the cause was a congenital chylothorax. Congenital chylothorax (n=13) was the most common (65%) congenital pleural effusion in this study. The incidence of congenital chylothorax was 1:8.600 deliveries and male:female ratio was 2:1. Mortality occurred in five newborns due to pulmonary hypoplasia. Traumatic (iatrogenic) were the most frequent (n=31) acquired pleural effusions. These included 8 (13%) cases of hemothorax and 8 (13%) cases of total parenteral nutrition leakage. Pleural effusions after intra-thoracic surgery were mainly (79%) chylothoraces. There were 11 (26%) non-iatrogenic acquired pleural effusions. No mortality was associated with acquired pleural effusions.

CONCLUSIONS: Congenital pleural effusions usually occur as hydrops or congenital chylothorax. Traumatic (iatrogenic) are the most frequent acquired pleural effusions in a tertiary NICU. Pleural effusions after intra-thoracic surgery are mainly chylothoraces. Non-iatrogenic acquired pleural effusions are associated to several clinical conditions, and mortality is usually associated to the underlying condition.

Chylothorax, hydrops, newborn, pleural effusion

Meta Press

Friday, January 05, 2007

Benign supraclavicular tumorous lymphangiectasia--a new disease?

Benign supraclavicular tumorous lymphangiectasia--a new disease?

Preyer S,
Kaiserling E,
Heinle H,
Foldi E,
Zenner HP,
Foldi M.
Department of Otorhinolaryngology, University of Tubingen, Germany.

We describe an isolated recurrent non-inflammatory tumorous swelling of the supraclavicular fossa in four premenopausal women. Ultrasonography, magnetic resonance imaging and computer tomography of the neck each suggested an inhomogeneous mass consistent with "lymphangioma."

In each patient the clinical course and histopathologic findings suggested that the swellings were due to chronic localized lymph stasis with subsequent lymphangiectasia, possibly initiated by intermittent obstruction of the juncture of the thoracic or right lymph duct with the internal jugular vein. Enlargement may have been hormonally triggered by estrogens as each woman was taking oral contraceptive pills at the onset of the disease.

To characterize this unique entity, we have termed the disorder benign supraclavicular tumorous lymphangiectasia.

PMID: 7475260 [PubMed - indexed for MEDLINE]

Monday, January 01, 2007

Videocapsule endoscopy is useful for the diagnosis of intestinal lymphangiectasia.

Videocapsule endoscopy is useful for the diagnosis of intestinal lymphangiectasia.

Dig Liver Dis. 2006 Sep

Chamouard P,
Nehme-Schuster H,
Simler JM,
Finck G,
Baumann R,
Pasquali JL.

Service d'Hepato-Gastroenterologie et d'Assistance Nutritive, Hopital de Hautepierre, 67098 Strasbourg Cedex, France.

Keywords: Intestinal lymphangiectasia; Lymphatic; Protein-losing enteropathy; Push enteroscopy; Videocapsule endoscopy

We study two authentic cases of protein-losing enteropathy, the diagnosis of which was facilitated using Given M2A videocapsule endoscopy. The first case corresponded to a primary intestinal lymphangiectasia confirmed by jejunum biopsies and the second one to a protein-losing enteropathy with lymphatic abnormalities secondary to a chronic constrictive pericarditis.

In the first case, the mucosa of jejunum presented with a diffuse oedematous aspect, whitish villi, white curved lines probably related to submucosal dilated lymphatics and lacteal juice.

In the second case, capsule endoscopy showed oedematous aspect of jejunum mucosa associated with white curved lines similar to those observed in the first case.

Videocapsule endoscopy is useful in cases of protein-losing enteropathy to identify presence of intestinal lymphangiectasia and to specify their localisation after ruling out other disorders liable to induce protein-losing gastrointestinal syndrome.

Science Direct