Title: Lymphangiectasia

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| name            = Lymphangiectasia
| image           =Lymphangiectasia shown on enteroscopy before and after treatment.png
| caption         =Lymphangiectasia shown on [[enteroscopy]].
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'''Lymphangiectasia''', also known as &quot;lymphangiectasis&quot;,&lt;ref&gt;{{DorlandsDict|five/000061935|lymphangiectasia}}&lt;/ref&gt; is a pathologic dilation of lymph vessels.&lt;ref name=&quot;Zachary 2007&quot;&gt;McGavin/ Zachary (2007), Pathologic Basis of Veterinary Disease&lt;/ref&gt; 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.&lt;ref name=&quot;:0&quot;&gt;{{Cite journal |last1=Waldmann |first1=T.A. |last2=Steinfeld |first2=J.L. |last3=Dutcher |first3=T.F. |last4=Davidson |first4=J.D. |last5=Gordon |first5=R.S. |year=2008 |title=The Role of the Gastrointestinal System in &quot;Idiopathic Hypoproteinemia&quot; |journal=Gastroenterology |volume=41 |issue=3 |pages=197–207 |doi=10.1016/s0016-5085(19)35130-3 |issn=0016-5085}}&lt;/ref&gt; 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.&lt;ref name=&quot;:0&quot; /&gt; This condition, first described by Waldmann in 1961, is typically diagnosed in infancy or early childhood.&lt;ref name=&quot;:0&quot; /&gt; However, it can also manifest in adults, exhibiting a broad spectrum of clinical symptoms.&lt;ref name=&quot;:0&quot; /&gt;

==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.&lt;ref name=&quot;:1&quot;&gt;{{Cite journal |last=Freeman |first=Hugh James |date=2011 |title=Intestinal lymphangiectasia in adults |journal=World Journal of Gastrointestinal Oncology |volume=3 |issue=2 |page=19 |doi=10.4251/wjgo.v3.i2.19 |issn=1948-5204|doi-access=free |pmc=3046182 }}&lt;/ref&gt; This dilation impedes the normal flow of lymph from the intestines back to the circulatory system.&lt;ref name=&quot;:1&quot; /&gt; The overflow of lymphatic fluid into the intestines leads to the loss of lymphocytes, immunoglobulins, and proteins, causing lymphopenia, hypogammaglobulinemia, and hypoalbuminemia, respectively.&lt;ref name=&quot;:1&quot; /&gt; The loss of proteins contributes to the development of protein-losing enteropathy, a major clinical manifestation of this disease.&lt;ref name=&quot;:1&quot; /&gt;

Patients with intestinal lymphangiectasia present with a range of symptoms, significantly influenced by the extent of protein loss.&lt;ref name=&quot;:1&quot; /&gt; Chronic diarrhea and malabsorption are common symptoms.&lt;ref name=&quot;:1&quot; /&gt; The loss of protein can lead to edema, particularly in the legs and abdomen, due to decreased oncotic pressure.&lt;ref name=&quot;:1&quot; /&gt; Nutritional deficiencies may develop due to malabsorption, leading to growth retardation in children and weight loss in adults.&lt;ref&gt;{{Cite journal |last1=Vignes |first1=Stéphane |last2=Bellanger |first2=Jérôme |date=2008-02-22 |title=Primary intestinal lymphangiectasia (Waldmann's disease) |journal=Orphanet Journal of Rare Diseases |volume=3 |issue=1 |doi=10.1186/1750-1172-3-5 |issn=1750-1172|doi-access=free |pmc=2288596 }}&lt;/ref&gt; Immune abnormalities resulting from lymphocyte loss can predispose patients to recurrent infections.&lt;ref name=&quot;:1&quot; /&gt;

The clinical presentation of intestinal lymphangiectasia can range from asymptomatic to severe, implying a broad clinical spectrum.&lt;ref name=&quot;:1&quot; /&gt; Some patients may exhibit minimal or subtle clinical features, diverging from the &quot;textbook&quot; presentations often associated with severe cases in adults.&lt;ref name=&quot;:1&quot; /&gt; 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 [[lacteal]]s of the [[Intestinal villus|villi]] and distension of the [[lymphatic system|lymphatic vessel]]s.&lt;ref&gt;{{cite web | last = Steiner | first = Jörg M. | title = Protein-Losing Enteropathies in Dogs | work = Proceedings of the 28th World Congress of the World Small Animal Veterinary Association | year = 2003 | url = http://www.vin.com/proceedings/Proceedings.plx?CID=WSAVA2003&amp;PID=6588&amp;O=Generic | access-date = 2007-03-20 }}&lt;/ref&gt;  Reduced [[lymphatic system|lymph]] flow leads to a [[malabsorption]] syndrome of the small intestine, especially of [[fat]] and fat-soluble [[vitamin]]s.  Rupture of the lymphatics causes protein loss into the intestines.&lt;ref&gt;{{Cite journal|last1=Nishino|first1=Koichi|last2=Yoshimi|first2=Kaku|last3=Shibuya|first3=Tomoyoshi|last4=Hayashi|first4=Takuo|last5=Mitani|first5=Keiko|last6=Kobayashi|first6=Etsuko|last7=Ichikawa|first7=Masako|last8=Asao|first8=Tetsuhiko|last9=Suzuki|first9=Yohei|last10=Sato|first10=Tadashi|last11=Shiota|first11=Satomi|date=2017-04-15|title=Protein-losing Enteropathy Caused by Intestinal or Colonic Lymphangiectasia Complicated by Sporadic Lymphangioleiomyomatosis: A Report of Two Cases|journal=Internal Medicine|volume=56|issue=8|pages=943–948|doi=10.2169/internalmedicine.56.7769|issn=0918-2918|pmc=5465412|pmid=28420844}}&lt;/ref&gt;

The most common cause [[Offal|of]] lymphangiectasia was [[congenital]] malformation of the lymphatics.&lt;ref&gt;{{cite journal |vauthors=Kull P, Hess R, Craig L, Saunders H, Washabau R |title=Clinical, clinicopathologic, radiographic, and ultrasonographic characteristics of intestinal lymphangiectasia in dogs: 17 cases (1996-1998) |journal=J Am Vet Med Assoc |volume=219 |issue=2 |pages=197–202 |year=2001 |pmid=11469575 |doi=10.2460/javma.2001.219.197}}&lt;/ref&gt;  Secondary lymphangiectasia may be caused by [[granuloma]]s 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.&lt;ref name=Fogle&gt;{{cite journal | last = Fogle | first = Jonathan E. |author2=Bissett, Sally A.  | title = Mucosal Immunity and Chronic Idiopathic Enteropathies in Dogs | journal = Compendium on Continuing Education for the Practicing Veterinarian | volume = 29 | issue = 5 | pages = 290–302 | publisher = Veterinary Learning Systems |date=May 2007 | pmid = 17724983 }}&lt;/ref&gt;

==Diagnosis==
Diagnosis is through biopsy.  The presence of hypoproteinemia, decreased blood [[lymphocyte]]s, 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 [[:wikt:striation|striation]]s in the [[intestinal mucosa]] indicating dilated lacteals.&lt;ref&gt;{{cite journal |vauthors=Sutherland-Smith J, Penninck D, Keating J, Webster C |title=Ultrasonographic intestinal hyperechoic mucosal striations in dogs are associated with lacteal dilation |journal=Vet Radiol Ultrasound |volume=48 |issue=1 |pages=51–7 |year=2007 |pmid=17236361 |doi=10.1111/j.1740-8261.2007.00204.x}}&lt;/ref&gt; Computerized tomography (CT) can show low attenuation material within the bowel walls which corresponds to lipid-containing chylous fluid within the dilated lymphatic vessels.&lt;ref&gt;{{cite journal |last1=Awiwi |first1=Muhammad O. |last2=Naik |first2=Sagar |title=Tyrosine Kinase Inhibitor–related Intestinal Lymphangiectasia |journal=Radiology |date=7 June 2022 |volume=305 |issue=2 |article-number=220137 |doi=10.1148/radiol.220137 |pmid=35670716 |s2cid=249432951 }}&lt;/ref&gt;

==Treatment==
In the case of primary intestinal lymphangiectasia, a diet of low-fat and high-[[protein]] aliments, supplemental [[calcium]] and certain [[vitamins]] has been shown to reduce symptom effects.&lt;ref name=&quot;:2&quot;&gt;{{Cite journal |last1=Kwon |first1=Yiyoung |last2=Kim |first2=Mi Jin |date=2021 |title=The Update of Treatment for Primary Intestinal Lymphangiectasia |journal=Pediatric Gastroenterology, Hepatology &amp; Nutrition |language=en |volume=24 |issue=5 |page=413 |doi=10.5223/pghn.2021.24.5.413 |issn=2234-8646|doi-access=free |pmc=8443852 }}&lt;/ref&gt; This diet, however, is not a cure. If the diet is stopped, the symptoms will eventually reappear.&lt;ref name=&quot;:2&quot; /&gt; Medication is also used to treat this disease, including Octreotide, Sirolimus, Anti-plasmin and, at least in one case, Trametinib.&lt;ref name=&quot;:2&quot; /&gt;&lt;ref&gt;{{Cite web |last=PIL |first=Unraveling Adult-Onset |title=Unraveling Adult-Onset PIL |url=http://lymphangiectasia.com/ |access-date=2023-11-30 |website=lymphangiectasia.com |language=en}}&lt;/ref&gt;

==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]].&lt;ref name=Ettinger_1995&gt;{{cite book|author1=Ettinger, Stephen J. |author2=Feldman, Edward C. |title=Textbook of Veterinary Internal Medicine|edition=4th|publisher=W.B. Saunders Company|year=1995|isbn=978-0-7216-6795-9}}&lt;/ref&gt;

==References==
{{Reflist}}

== External links ==
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{{Lymphatic disease}}

[[Category:Dermal and subcutaneous growths]]
[[Category:Dog diseases]]
[[Category:Lymphatic vessel diseases]]