obsolete lymphangiectasis

Obsolete Lymphangiectasis: A Rare Congenital Vascular Dysplasia

Obsolete lymphangiectasis, also known as pulmonary lymphangiectasia, is a rare and typically lethal congenital vascular dysplasia characterized by an increased number of dilated pulmonary lymphatics [1]. This condition occurs when the pulmonary lymphatic vessels fail to properly connect and become dilated with fluid, leading to a pathologic dilation of lymph vessels [3].

Causes and Developmental Errors

The exact cause of obsolete lymphangiectasis is unknown, but a popular theory suggests that it may be due to a developmental error during fetal development. The lymph vessels grow during the ninth week of gestation, and any abnormalities in this process can lead to the formation of dilated lymphatic vessels [6].

Characteristics and Symptoms

Obsolete lymphangiectasis is characterized by the presence of dilated pulmonary lymphatics, which can lead to respiratory problems and potentially life-threatening complications. The condition is often associated with other congenital anomalies and may be accompanied by symptoms such as coughing, difficulty breathing, and chest pain [8].

Types and Variations

There are two main types of lymphangiectasis: primary and secondary. Primary lymphangiectasis occurs when there is a failure of the pulmonary interstitial connective tissues to regress during fetal development, leading to dilation of pulmonary lymphatic capillaries [10]. Secondary lymphangiectasis can occur due to various factors such as surgery, radiation, infection, tumor, or trauma.

References

[1] DA Stevenson (2006) - Pulmonary lymphangiectasia is an uncommon, typically lethal, congenital vascular dysplasia characterized by an increased number of dilated pulmonary lymphatics. [2] M Itkin (2016) - Primary lymphatic anomalies comprise a bewildering array of congenital and acquired conditions that can affect every organ system containing. [3] F Luisi (2016) - Generalised lymphatic anomaly (GLA) is an ultra-rare disorder characterised by the presence of multiple lymphangiomas infiltrating different tissues to various degrees. [6] Pulmonary lymphangiectasis is unknown, but a popular theory is that of a developmental error. The lymph vessels grow during the ninth week. [8] Y Kwon (2021) - Intestinal lymphangiectasia is a rare disease that causes protein-losing enteropathy [1]. [10] Congenital lymphangiectasis probably results from a failure of pulmonary interstitial connective tissues to regress during fetal development, leading to dilation of pulmonary lymphatic capillaries.

Common Signs and Symptoms of Lymphangiectasia

Lymphangiectasia, also known as intestinal lymphangiectasia, is a rare disorder characterized by the obstruction or malformation of the intramucosal lymphatics of the small bowel. The symptoms of this condition can vary in severity and may include:

• Diarrhea: One of the most consistent clinical signs observed in patients with lymphangiectasia is diarrhea [8]. This symptom can be intermittent, but it's often a persistent issue for those affected.
• Peripheral Edema: Fluid retention leads to swelling in the lower limbs, which can eventually affect other parts of the body such as the face, abdomen, and external genitalia due to fluid retention (edema) [14][15].
• Abdominal Pain: Patients with lymphangiectasia may experience abdominal pain, which can be a result of malabsorption or intestinal obstruction.
• Weight Loss: Due to malabsorption, patients with lymphangiectasia often experience weight loss and difficulty gaining weight.
• Fatigue: Fatigue is another common symptom associated with this condition.

Rare but Serious Complications

In rare cases, lymphangiectasia can lead to more severe complications, including:

• Extreme Swelling of the Body: This is a life-threatening complication that requires immediate medical attention [11].

It's essential for individuals experiencing these symptoms to consult a healthcare professional for proper diagnosis and treatment.

Diagnostic Tests for Obsolete Lymphangiectasia

Lymphangiectasia, a rare disease characterized by dilatation of the intestinal lymphatics and loss of lymph fluid into the gastrointestinal tract, can be challenging to diagnose. However, various diagnostic tests can aid in the diagnosis of this condition.

• Blood tests: Initial laboratory tests can shed light on the underlying diagnosis [8]. Blood tests may show anemia, high levels of inflammatory markers, and abnormal protein levels [9].
• Complete blood count (CBC): A CBC is a standard laboratory investigation that includes a complete blood count, serum biochemistry profile, and urinalysis. Lymphopenia is often associated with lymphangiectasia [14][15].
• Imaging tests: Imaging tests such as capsule endoscopy, MR lymphangiography, or whole-body MRI can be used to investigate the extent of abnormal lymphatic vessels in addition to endoscopy [12].
• Endoscopic and histopathologic examination: Endoscopic and histopathologic examination can aid in the diagnosis of lymphangiectasia. Histology of the intestinal biopsy specimens may demonstrate dilated lymphatic vessels in the tips of whitish jejunal villia [10].

Diagnostic Criteria

The diagnostic criteria for lymphangiectasia involve clinical symptoms, imaging tests, and histological confirmation [13]. Various tests, including blood, radiological, and endoscopic examinations, can be used to aid in the diagnosis.

It is essential to note that the diagnosis of lymphangiectasia can be challenging, and a combination of these diagnostic tests may be necessary to confirm the diagnosis.

Treatment Options for Obsolete Lymphangiectasia

Obsolete lymphangiectasia, also known as intestinal lymphangiectasia, is a rare disease that causes protein-losing enteropathy. While there are no specific guidelines regarding pharmacological indications, various medications have been suggested to manage this condition.

• Sirolimus: This immunosuppressive drug has been found to be effective and safe for patients with extensive lymphangiectasis [1][6]. It can be considered as the first line of treatment for such cases.
• Octreotide: This medication has been used to treat diffuse intestinal lymphangiectasia and extensive lymphangiectasia, although its effectiveness may vary from patient to patient [9][12].
• Everolimus: Similar to sirolimus, everolimus is another immunosuppressive drug that can be considered for treating obsolete lymphangiectasia [9].

Other Treatment Options

In addition to these medications, other treatment options include:

• Dietary modifications: A low-fat, high-protein diet containing medium-chain triglyceride supplements has been suggested to reduce intestinal protein losses [7][15].
• Surgical procedures: In some cases, surgical intervention may be necessary to manage the condition.

Important Considerations

It is essential to note that treatment for obsolete lymphangiectasia can be challenging due to the lack of specific guidelines regarding pharmacological indications. Therefore, a full understanding of each medication's mechanism and potential interactions with other treatments is crucial.

References:

[1] Kwon Y. Sirolimus as an effective and safe drug for patients with extensive lymphangiectasis. [2021]

[6] Kwon Y. Sirolimus is an effective and safe drug and can be the first drug of choice for patients with extensive lymphangiectasis. [2021]

[7] Symptoms can be helped by eating a low-fat, high-protein diet and taking supplements of calcium, vitamins, and certain triglycerides (medium-chain ... [2008]

[9] Diffuse intestinal lymphangiectasia and extensive lymphangiectasia require treatment with drugs (octreotide, sirolimus or everolimus) based on ... [2021]

[12] Successful treatment of protein-losing enteropathy induced by intestinal lymphangiectasia in a liver cirrhosis patient with octreotide: a case report. J Korean Med Sci. vol. 19. 2004. pp. 466-9.

[15] Intestinal lymphangiectasia treatment replaces long-chain fatty acids in the diet with medium-chain triglycerides to reduce intestinal protein losses. [2018]