II. Definitions

  1. Gastrointestinal Angiodysplasia or Arteriovenous Malformation
    1. Abnormal vascular structures (arteries, veins, capillaries) in the gastrointestinal tract
  2. Dieulafoy Vascular Malformation
    1. Tortuous arteriole in the submucosa of the Stomach which may result in upper gastrointestinal Hemorrhage

III. Epidemiology

  1. Relatively rare cause of Gastrointestinal Bleeding
  2. Most common in age over 60 years
    1. Chronic Kidney Disease may predispose to earlier onset

IV. Pathophysiology

  1. Abnormal vascular structures form in response to chronic low grade intermittent submucosal vein obstruction
  2. Increased Vascular Endothelial Growth Factor dependent proliferation also contrubutes

V. Types

  1. Upper Gastrointestinal Bleeding
    1. Responsible for 4 to 7% of non-variceal Upper Gastrointestinal Bleeding
  2. Lower Gastrointestinal Bleeding (most common)
    1. Cecum and ascending colon (>54% of lesions)

VII. Symptoms

  1. Recurrent painless Gastrointestinal Bleeding
  2. May present as acute Gastrointestinal Bleeding or chronic Gastrointestinal Bleeding
  3. May present as Upper Gastrointestinal Bleeding or lower Gastrintestinal bleeding

VIII. Diagnosis: Colonoscopy

  1. Abnormal ectatic, dilated or tortuous blood vessels lined by endothelium (but no smooth muscle)
  2. Vessels are small (typically <10 mm)
  3. Abnormal vessels lie within the intestinal mucosa and submucosa
  4. More than one lesion is seen in 40-60% of patients

IX. Management

  1. See Gastrointestinal Bleeding
  2. See Lower Gastrointestinal Bleeding
  3. Treatment of nonbleading lesions is not typically recommended
  4. Persistent bleeding
    1. Endoscopic argon plasma coagulation (first-line)
      1. Associated with 7-15% recurrence rate
    2. Endoscopic direct contact thermal electrocautery and possible endoclip placement
  5. Longterm bleeding prevention
    1. Lanreotide (Somatuline)
      1. Improves Hemoglobin, and decreases hospitalizations, transfusions and endoscopies
      2. Frago (2018) Scand J Gastroenterol 53(12):1496-502 [PubMed]
      3. Chetcuti (2017) Scand J Gastroenterol 52(9): 962-8 [PubMed]
    2. Hormonal management (e.g. Ethinyl Estradiol and norethisterone)
      1. Does not appear to decrease bleeding episodes or transfusions
      2. Junquera (2001) Gastroenterology 121(5): 1073-9 [PubMed]

X. Course

  1. Self-resolves in 40-45% of cases

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Related Studies

Ontology: Angiodysplasia of intestine (C0267367)

Concepts Disease or Syndrome (T047)
ICD9 569.84
SnomedCT 32868004, 235853006
English Angiodysplasia of intestine (without mention of hemorrhage), Angio intes w/o hmrhg, angiodysplasia intestine, angiodysplasia of intestine, angiodysplasia intestines, Angiodysplasia of intestinal tract (disorder), Localized arteriovenous malformations of intestinal tract, Localised arteriovenous malformations of intestinal tract, Angiodysplasia of intestine (without mention of haemorrhage), Angiodysplasia of intestinal tract, Angiodysplasia of intestine, Intestinal vascular dysplasia, Angiodysplasia of intestine (disorder), Angiodysplasia of intestinal tract, NOS, Localized arteriovenous malformations of intestinal tract, NOS, Angiodysplasia of intestine without mention of hemorrhage
Dutch angiodysplasie van darmen (zonder vermelding van bloeding), angiodysplasie van ingewanden (zonder vermelding van bloeding)
French Angiodysplasie de l'intestin sans mention d'hémorragie, Angiodysplasie de l'intestin (sans mention d'hémorragie)
German Angiodysplasie des Darms (ohne Erwaehnung einer Blutung)
Italian Angiodisplasia intestinale (senza menzione di emorragia), Angiodisplasia dell'intestino, senza menzione di emorragia
Portuguese Angiodisplasia do intestino (sem referência a hemorragia)
Spanish Angiodisplasia de intestino (sin mención de hemorragia), angiodisplasia del tracto intestinal, malformaciones arteriovenosas localizadas en el intestino, angiodisplasia del tracto intestinal (trastorno), angiodisplasia del intestino (trastorno), angiodisplasia del intestino, displasia vascular intestinal
Japanese 腸管血管異形成(出血の記載のないもの), チョウカンケッカンイケイセイシュッケツノキサイノナイモノ
Czech Angiodysplazie střeva (bez zmínky o krvácení)
Hungarian Bél angiodysplasia (vérzés említése nélkül), Bél angiodysplasiája (vérzés említése nélkül)

Ontology: Angiodysplasia of colon (C0267370)

Concepts Disease or Syndrome (T047)
ICD10 K55.2
SnomedCT 90858003, 197244008
English AV - Angiodysplasia of colon, Angiodysplasia of large int, colonic angiodysplasia (diagnosis), colonic angiodysplasia, colon angiodysplasia, angiodysplasia colon, Large intestine angiodysplasia, Angiodysplasia, Vascular ectasia of colon, AV - Angiodysplasia malformation of colon, Angiodysplasia of large intestine, Telangiectasia of colon, Angiodysplasia of colon (disorder), Vascular ectasia of colon (disorder), Angiodysplasia of colon, angiodysplasia
German Angiodysplasie des Kolons, Angiodysplasie des Dickdarms
Korean 결장의 혈관형성이상
Czech Angiodysplazie tlustého střeva
Dutch angiodysplasie van dikke darm, Angiodysplasie van colon
French Angiodysplasie du gros intestin
Hungarian Vastagbél angiodysplasia
Italian Angiodisplasia dell'intestino crasso
Japanese 大腸血管異形成, ダイチョウケッカンイケイセイ
Portuguese Angiodisplasia do intestino grosso
Spanish Angiodisplasia del intestino grueso, angiodisplasia del colon (trastorno), angiodisplasia del colon, ectasia vascular del colon (trastorno), ectasia vascular del colon

Ontology: Dieulafoy's vascular malformation (C0341217)

Concepts Disease or Syndrome (T047)
SnomedCT 109558001
Spanish Malformación vascular de Dieulafoy, enfermedad de Dieulafoy, malformación vascular de Dieulafoy (trastorno), malformación vascular de Dieulafoy
French Malformation vasculaire de Dieulafoy
Dutch Dieulafoy-vaatmisvorming, Dieulafoy; ulcus, ulcus; Dieulafoy
Portuguese Malformação vascular de Dieulafoy
German Dieulafoy-Gefaessmissbildung
Italian Malformazione vascolare di Dieulafoy
Japanese デュラフォア血管奇形, デュラフォアケッカンキケイ
English Dieulafoy lesion of stomach, Dieulafoy lesion of stomach (diagnosis), Dieulafoy vascular malformation, dieulafoy lesions, dieulafoy disease, dieulafoy lesion, dieulafoy diseases, dieulafoy's ulcer, Calibre persistent artery, Caliber persistent artery, Dieulafoy disease, Dieulafoy's vascular malformation (disorder), Dieulafoy's vascular malformation, ulcer; Dieulafoy, Dieulafoy; ulcer
Czech Dieulafoyova vaskulární malformace
Hungarian Dieulafoy vascularis malformatio

Ontology: Gastrointestinal arteriovenous malformation (C0744321)

Concepts Anatomical Abnormality (T190)
Italian Malformazione arterovenosa gastrointestinale
Japanese 胃腸管動静脈奇形, イチョウカンドウジョウミャクキケイ
Czech Gastrointestinální arteriovenózní malformace
Hungarian Gastrointestinalis arteriovenosus malformatio
English Gastrointestinal arteriovenous malformation, Gastrointestinal Arteriovenous Malformation
Portuguese Malformação arteriovenosa gastrointestinal
Spanish Malformación arteriovenosa gastrointestinal
Dutch maagdarmstelsel arterioveneuze misvorming
French Malformation artérioveineuse gastro-intestinale
German arteriovenoese Missbildung im Intestinaltrakt

Ontology: Gastrointestinal vascular malformation (C0851971)

Concepts Disease or Syndrome (T047)
Italian Malformazioni vascolari gastrointestinali, Malformazione vascolare gastrointestinale
Japanese 消化管血管奇形, ショウカカンケッカンキケイ, 胃腸血管奇形, イチョウケッカンキケイ
Czech Gastrointestinální cévní malformace, Vaskulární malformace v gastrointestinálním traktu
English Gastrointestinal vascular malformations, Gastrointestinal vascular malformation
Spanish Malformación vascular gastrointestinal, Malformaciones vasculares gastrointestinales
Portuguese Malformação gastrointestinal vascular, Malformações vasculares gastrointestinais
French Malformation vasculaire gastro-intestinale, Malformations vasculaires gastro-intestinales
Dutch gastro-intestinale vaatmisvorming, maagdarmstelselvaatmisvormingen
German Fehlbildung der gastrointestinalen Gefaesse, Gastrointestinale Gefaessmissbildungen
Hungarian Gastrointestinalis vascularis malformatiók, Gastrointestinalis vascularis malformatio

Ontology: Gastrointestinal angiodysplasia (C0854242)

Concepts Disease or Syndrome (T047)
Italian Angiodisplasia gastrointestinale
Japanese 胃腸管血管異形成, イチョウカンケッカンイケイセイ
Czech Gastrointestinální angiodysplazie
English Gastrointestinal angiodysplasia
Hungarian Gastrointestinalis angiodysplasia
Portuguese Angiodisplasia gastrointestinal
Spanish Angiodisplasia gastrointestinal
Dutch maagdarmstelsel angiodysplasie
French Angiodysplasie gastro-intestinale
German gastrointestinale Angiodysplasie

Ontology: Gastrointestinal angiodysplasia haemorrhagic (C0854243)

Concepts Disease or Syndrome (T047)
Italian Angiodisplasia gastrointestinale emorragica
Dutch angiodysplasie van maagdarmstelsel hemorragisch, maagdarmstelsel hemorragische angiodysplasie
French Angiodysplasie gastrointestinale hémorragique, Angiodysplasie gastro-intestinale hémorragique
German gastrointestinale Angiodysplasie, blutend, gastrointestinale Angiodysplasie haemorrhagisch
Portuguese Angiodisplasia gastrintestinal hemorrágica, Angiodisplasia gastrointestinal hemorrágica
Japanese 出血性胃腸管血管異形成, シュッケツセイイチョウカンケッカンイケイセイ
English Gastrointestinal angiodysplasia haemorrhagic, Gastrointestinal angiodysplasia hemorrhagic
Czech Gastrointestinální hemoragická angiodysplazie
Hungarian Gastrointestinalis angiodysplasia vérzéses, Gastrointestinalis angiodysplasia, haemorrhagiás
Spanish Angiodisplasia gastrointestinal hemorrágica

Ontology: Gastrointestinal telangiectasia (C1619711)

Concepts Disease or Syndrome (T047)
Dutch gastro-intestinale teleangiëctasie
French Télangiectasie gastrointestinale
German gastrointestinale Teleangiektasie
Italian Telangiectasia gastrointestinale
Portuguese Telangiectasia gastrointestinal
Spanish Telangiectasia gastrointestinal
Japanese 胃腸毛細血管拡張症, イチョウモウサイケッカンカクチョウショウ
Czech Gastrointestinální telangiektáze
Hungarian Gastrointestinalis telangiectasia
English Gastrointestinal telangiectasia