Lymphangiectasia: Difference between revisions
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'''Lymphangiectasia''', also known as "lymphangiectasis",<ref>{{DorlandsDict|five/000061935|lymphangiectasia}}</ref> is a pathologic dilation of lymph vessels.<ref name="Zachary 2007">McGavin/ Zachary (2007), Pathologic Basis of Veterinary Disease</ref> When it occurs in the [[intestine]]s |
'''Lymphangiectasia''', also known as "lymphangiectasis",<ref>{{DorlandsDict|five/000061935|lymphangiectasia}}</ref> is a pathologic dilation of lymph vessels.<ref name="Zachary 2007">McGavin/ Zachary (2007), Pathologic Basis of Veterinary Disease</ref> When it occurs in the [[intestine]]s it is known as intestinal lymphangiectasia, colloquially recognized as [[Waldmann disease|Waldmann's disease]] in cases where there is no secondary cause.<ref name=":0">{{Cite journal |last=Waldmann |first=T.A. |last2=Steinfeld |first2=J.L. |last3=Dutcher |first3=T.F. |last4=Davidson |first4=J.D. |last5=Gordon |first5=R.S. |date=1961-09 |title=The Role of the Gastrointestinal System in “Idiopathic Hypoproteinemia” |url=http://dx.doi.org/10.1016/s0016-5085(19)35130-3 |journal=Gastroenterology |volume=41 |issue=3 |pages=197–207 |doi=10.1016/s0016-5085(19)35130-3 |issn=0016-5085}}</ref> The primary defect lies in the inability of the lymphatic system to adequately drain lymph, resulting in its subsequent accumulation and leakage into the intestinal lumen.<ref name=":0" /> This condition, first described by Waldmann in 1961, is typically diagnosed in infancy or early childhood.<ref name=":0" /> However, it can also manifest in adults, exhibiting a broad spectrum of clinical symptoms.<ref name=":0" /> |
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==Signs and symptoms== |
==Signs and symptoms== |
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The pathophysiology of intestinal lymphangiectasia is centered around the dilation of the lymphatic vessels in the intestinal mucosa, submucosa, and sometimes the mesentery.<ref name=":1">{{Cite journal |last=Freeman |first=Hugh James |date=2011 |title=Intestinal lymphangiectasia in adults |url=http://dx.doi.org/10.4251/wjgo.v3.i2.19 |journal=World Journal of Gastrointestinal Oncology |volume=3 |issue=2 |pages=19 |doi=10.4251/wjgo.v3.i2.19 |issn=1948-5204}}</ref> This dilation impedes the normal flow of lymph from the intestines back to the circulatory system.<ref name=":1" /> The overflow of lymphatic fluid into the intestines leads to the loss of lymphocytes, immunoglobulins, and proteins, causing lymphopenia, hypogammaglobulinemia, and hypoalbuminemia, respectively.<ref name=":1" /> The loss of proteins contributes to the development of protein-losing enteropathy, a major clinical manifestation of this disease.<ref name=":1" /> |
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Chronic diarrhea is almost always seen with lymphangiectasia, but most other signs are linked to low [[blood protein]] levels ([[hypoproteinemia]]), which causes low [[oncotic pressure]]. These signs include [[ascites]], [[pleural effusion]], and [[edema]] of the limbs and trunk. Weight loss is seen with long-term disease.<ref name=Fogle/> |
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Patients with intestinal lymphangiectasia present with a range of symptoms, significantly influenced by the extent of protein loss.<ref name=":1" /> Chronic diarrhea and malabsorption are common symptoms.<ref name=":1" /> The loss of protein can lead to edema, particularly in the legs and abdomen, due to decreased oncotic pressure.<ref name=":1" /> Nutritional deficiencies may develop due to malabsorption, leading to growth retardation in children and weight loss in adults.<ref>{{Cite journal |last=Vignes |first=Stéphane |last2=Bellanger |first2=Jérôme |date=2008-02-22 |title=Primary intestinal lymphangiectasia (Waldmann's disease) |url=http://dx.doi.org/10.1186/1750-1172-3-5 |journal=Orphanet Journal of Rare Diseases |volume=3 |issue=1 |doi=10.1186/1750-1172-3-5 |issn=1750-1172}}</ref> Immune abnormalities resulting from lymphocyte loss can predispose patients to recurrent infections.<ref name=":1" /> |
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The clinical presentation of intestinal lymphangiectasia can range from asymptomatic to severe, implying a broad clinical spectrum.<ref name=":1" /> Some patients may exhibit minimal or subtle clinical features, diverging from the "textbook" presentations often associated with severe cases in adults<ref name=":1" />. This variability underscores the importance of considering PIL in differential diagnoses, even when clinical manifestations are not severe or typical. |
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==Cause== |
==Cause== |
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Revision as of 04:53, 30 November 2023
| Lymphangiectasia | |
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| Lymphangiectasia shown on enteroscopy. | |
| Specialty | Angiology  |
Lymphangiectasia, also known as "lymphangiectasis",[1] is a pathologic dilation of lymph vessels.[2] When it occurs in the intestines it is known as intestinal lymphangiectasia, colloquially recognized as Waldmann's disease in cases where there is no secondary cause.[3] The primary defect lies in the inability of the lymphatic system to adequately drain lymph, resulting in its subsequent accumulation and leakage into the intestinal lumen.[3] This condition, first described by Waldmann in 1961, is typically diagnosed in infancy or early childhood.[3] However, it can also manifest in adults, exhibiting a broad spectrum of clinical symptoms.[3]
Signs and symptoms
The pathophysiology of intestinal lymphangiectasia is centered around the dilation of the lymphatic vessels in the intestinal mucosa, submucosa, and sometimes the mesentery.[4] This dilation impedes the normal flow of lymph from the intestines back to the circulatory system.[4] The overflow of lymphatic fluid into the intestines leads to the loss of lymphocytes, immunoglobulins, and proteins, causing lymphopenia, hypogammaglobulinemia, and hypoalbuminemia, respectively.[4] The loss of proteins contributes to the development of protein-losing enteropathy, a major clinical manifestation of this disease.[4]
Patients with intestinal lymphangiectasia present with a range of symptoms, significantly influenced by the extent of protein loss.[4] Chronic diarrhea and malabsorption are common symptoms.[4] The loss of protein can lead to edema, particularly in the legs and abdomen, due to decreased oncotic pressure.[4] Nutritional deficiencies may develop due to malabsorption, leading to growth retardation in children and weight loss in adults.[5] Immune abnormalities resulting from lymphocyte loss can predispose patients to recurrent infections.[4]
The clinical presentation of intestinal lymphangiectasia can range from asymptomatic to severe, implying a broad clinical spectrum.[4] Some patients may exhibit minimal or subtle clinical features, diverging from the "textbook" presentations often associated with severe cases in adults[4]. This variability underscores the importance of considering PIL in differential diagnoses, even when clinical manifestations are not severe or typical.
Cause
Biopsy of the small intestine shows dilation of the lacteals of the villi and distension of the lymphatic vessels.[6] Reduced lymph flow leads to a malabsorption syndrome of the small intestine, especially of fat and fat-soluble vitamins. Rupture of the lymphatics causes protein loss into the intestines.[7]
The most common cause of lymphangiectasia was congenital malformation of the lymphatics.[8] Secondary lymphangiectasia may be caused by granulomas or cancer causing lymphatic obstruction, or increased central venous pressure (CVP) causing abnormal lymph drainage. Increased CVP can be caused by pericarditis or right-sided heart failure. Inflammatory bowel disease can also lead to inflammation of the lymphatics and lymphangiectasia through migration of inflammatory cells through the lymphatics.[9]
Diagnosis
Diagnosis is through biopsy. The presence of hypoproteinemia, decreased blood lymphocytes, and decreased cholesterol support the diagnosis. Hypocalcemia (low calcium) is also seen due to poor absorption of vitamin D and calcium, and secondary to low protein binding of calcium. Medical ultrasonography may show striations in the intestinal mucosa indicating dilated lacteals.[10] Computerized tomography (CT) can show low attenuation material within the bowel walls which corresponds to lipid-containing chylous fluid within the dilated lymphatic vessels.[11]
Treatment
Treatment is multifactorial. A diet very low in fat and high in high quality protein is essential.[12] By limiting a dog's fat intake, the amount of intestinal lymph that is formed is reduced which also reduces pressure within these faulty ducts. Less pressure means less lymph leakage and a reduction of symptoms. Diets for dogs with lymphangiectasia should not have more than 20% of their calories coming from fat. Treatment of humans can also involve the use of MCT (medium-chain triglycerides) oil and/or the drug octreotide. In dogs, fat soluble vitamins (A, D, E, and K) should be supplemented. Corticosteroid treatment may be required for life. Antibiotics can be used to treat bacterial overgrowth. With a very low serum albumin, transfusion with blood plasma or an infusion of hetastarch may be necessary to treat the signs until the diet can take effect.[13] Lymphangiectasia is rarely cured but can remain in remission for a long time. It can be fatal when unresponsive to treatment.[citation needed]
In animals
Dog breeds commonly affected by lymphangiectasia and/or protein-losing enteropathy include the Soft-Coated Wheaten Terrier, Norwegian Lundehund, Basenji, and Yorkshire Terrier.[14]
References
- ^ "lymphangiectasia" at Dorland's Medical Dictionary
- ^ McGavin/ Zachary (2007), Pathologic Basis of Veterinary Disease
- ^ a b c d Waldmann, T.A.; Steinfeld, J.L.; Dutcher, T.F.; Davidson, J.D.; Gordon, R.S. (1961-09). "The Role of the Gastrointestinal System in "Idiopathic Hypoproteinemia"". Gastroenterology. 41 (3): 197–207. doi:10.1016/s0016-5085(19)35130-3. ISSN 0016-5085.
{{cite journal}}: Check date values in:|date=(help) - ^ a b c d e f g h i j Freeman, Hugh James (2011). "Intestinal lymphangiectasia in adults". World Journal of Gastrointestinal Oncology. 3 (2): 19. doi:10.4251/wjgo.v3.i2.19. ISSN 1948-5204.
{{cite journal}}: CS1 maint: unflagged free DOI (link) - ^ Vignes, Stéphane; Bellanger, Jérôme (2008-02-22). "Primary intestinal lymphangiectasia (Waldmann's disease)". Orphanet Journal of Rare Diseases. 3 (1). doi:10.1186/1750-1172-3-5. ISSN 1750-1172.
{{cite journal}}: CS1 maint: unflagged free DOI (link) - ^ Steiner, Jörg M. (2003). "Protein-Losing Enteropathies in Dogs". Proceedings of the 28th World Congress of the World Small Animal Veterinary Association. Retrieved 2007-03-20.
- ^ Nishino, Koichi; Yoshimi, Kaku; Shibuya, Tomoyoshi; Hayashi, Takuo; Mitani, Keiko; Kobayashi, Etsuko; Ichikawa, Masako; Asao, Tetsuhiko; Suzuki, Yohei; Sato, Tadashi; Shiota, Satomi (2017-04-15). "Protein-losing Enteropathy Caused by Intestinal or Colonic Lymphangiectasia Complicated by Sporadic Lymphangioleiomyomatosis: A Report of Two Cases". Internal Medicine. 56 (8): 943–948. doi:10.2169/internalmedicine.56.7769. ISSN 0918-2918. PMC 5465412. PMID 28420844.
- ^ Kull P, Hess R, Craig L, Saunders H, Washabau R (2001). "Clinical, clinicopathologic, radiographic, and ultrasonographic characteristics of intestinal lymphangiectasia in dogs: 17 cases (1996-1998)". J Am Vet Med Assoc. 219 (2): 197–202. doi:10.2460/javma.2001.219.197. PMID 11469575.
- ^ Fogle, Jonathan E.; Bissett, Sally A. (May 2007). "Mucosal Immunity and Chronic Idiopathic Enteropathies in Dogs". Compendium on Continuing Education for the Practicing Veterinarian. 29 (5). Veterinary Learning Systems: 290–302. PMID 17724983.
- ^ Sutherland-Smith J, Penninck D, Keating J, Webster C (2007). "Ultrasonographic intestinal hyperechoic mucosal striations in dogs are associated with lacteal dilation". Vet Radiol Ultrasound. 48 (1): 51–7. doi:10.1111/j.1740-8261.2007.00204.x. PMID 17236361.
- ^ Awiwi, Muhammad O.; Naik, Sagar (7 June 2022). "Tyrosine Kinase Inhibitor–related Intestinal Lymphangiectasia". Radiology. 305 (2): 220137. doi:10.1148/radiol.220137. PMID 35670716. S2CID 249432951.
- ^ Matz, M.E. (2006). "Dietary Management of Gastrointestinal Disease". Proceedings of the North American Veterinary Conference. Retrieved 2007-03-20.
- ^ Willard, Michael (2005). "Protein-Losing Enteropathy in Dogs and Cats". Proceedings of the 30th World Congress of the World Small Animal Veterinary Association. Retrieved 2007-03-20.
- ^ Ettinger, Stephen J.; Feldman, Edward C. (1995). Textbook of Veterinary Internal Medicine (4th ed.). W.B. Saunders Company. ISBN 978-0-7216-6795-9.