関: vascular anomaly

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/07/16 14:03:59」(JST)

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In medicine (gastroenterology), angiodysplasia is a small vascular malformation of the gut. It is a common cause of otherwise unexplained gastrointestinal bleeding and anemia. Lesions are often multiple, and frequently involve the cecum or ascending colon, although they can occur at other places. Treatment may be with colonoscopic interventions, angiography and embolization, medication, or occasionally surgery.

Signs and symptoms

Although some cases present with black, tarry stool (melena), the blood loss can be subtle, with the anemia symptoms predominating. Fecal occult blood testing is positive when bleeding is active. If bleeding is intermittent the test may be negative at times.

Diagnosis

Diagnosis of angiodysplasia is often accomplished with endoscopy, either colonoscopy or esophagogastroduodenoscopy (EGD). Although the lesions can be notoriously hard to find, the patient usually is diagnosed by endoscopy. A new technique, pill enteroscopy, has been a major advance in diagnosis, especially in the small bowel which is difficult to reach with traditional endoscopy. With this technique a pill that contains a video camera and radio transmitter is swallowed, and pictures of the small intestine are sent to a receiver worn by the patient. Recently, multiphase CT angiography (without positive oral contrast) has been shown to play a promising role in the diagnoses of small and large bowel angiodysplasia, especially when associated with active hemorrhage

Angiodysplasiae in the small bowel can also be diagnosed and treated with double-balloon enteroscopy, a technique involving a long endoscopic camera and overtube, both fitted with balloons, that allow the bowel to be accordioned over the camera.^[1]

In cases with negative endoscopic findings and high clinical suspicion, selective angiography of the mesenteric arteries is sometimes necessary, but this allows for interventions at time of the procedure. An alternative is scintigraphy with red blood cells labeled with a radioactive marker; this shows the site of the bleeding on a gamma camera but tends to be unhelpful unless the bleeding is continuous and significant.^[2]

Pathophysiology

Histologically, it resembles telangiectasia and development is related to age and strain on the bowel wall.^[3] It is a degenerative lesion, acquired, probably resulting from chronic and intermittent contraction of the colon that is obstructing the venous drainage of the mucosa. As time goes by the veins become more and more tortuous, while the capillaries of the mucosa are gradually dilate and precapillary sphincter becomes incompetent. Thus is formed an arteriovenous malformation characterized by a small tuft of dilated vessels.^[4]^[5]

Although angiodysplasia is probably quite common, the risk of bleeding is increased in disorders of coagulation. A classic association is Heyde's syndrome (coincidence of aortic valve stenosis and bleeding from angiodysplasia).^[6] In this disorder, von Willebrand factor (vWF) is proteolysed due to high shear stress in the highly turbulent blood flow around the aortic valve. vWF is most active in vascular beds with high shear stress, including angiodysplasias, and deficiency of vWF increases the bleeding risk from such lesions.^[3]

Warkentin et al. argue that apart from aortic valve stenosis, some other conditions that feature high shear stress might also increase the risk of bleeding from angiodysplasia.^[3]

Therapy

If the anemia is severe, blood transfusion is required before any other intervention is considered. Endoscopic treatment is an initial possibility, where cautery or argon plasma coagulation (APC) treatment is applied through the endoscope. Failing this, angiography and emolization with particles is another microinvasive treatment option, which avoids the need for surgery and bowel resection. Here, the vessel supplying the angiodysplasia is selectively catheterized and embolizaed with microparticles.^[7] Resection of the affected part of the bowel may be needed if the other modalities fail. However, the lesions may be widespread, making such treatment impractical.

If the bleeding is from multiple or inaccessible sites, systemic therapy with medication may be necessary. First-line options include the antifibrinolytics tranexamic acid or aminocaproic acid. Estrogens can be used to stop bleeding from angiodysplasia. Estrogens cause mild hypercoaguability of the blood. Estrogen side effects can be dangerous and unpleasant in both sexes. Changes in voice and breast swelling is bothersome in men, but older women often report improvement of libido and perimenopausal symptoms. (The worries about hormone replacement therapy/HRT, however, apply here as well.)

In difficult cases, there have been positive reports about octreotide^[8] and thalidomide.^[9]

In severe cases or cases not responsive to either endoscopic or medical treatment, surgery may be necessary to arrest the bleeding.

References

^ Neumann H, Mönkemüller K, Malfertheiner P (2007). "Obscure overt GI bleeding secondary to angiodysplasias at the hepaticojejunostomy diagnosed and successfully treated with double-balloon enteroscopy". Gastrointest Endosc 67 (3): 563–5. doi:10.1016/j.gie.2007.06.051. PMID 17981272.
^ Mitchell SH, Schaefer DC, Dubagunta S (2004). "A new view of occult and obscure gastrointestinal bleeding". Am Fam Physician 69 (4): 875–81. PMID 14989574.
^ ^a ^b ^c Warkentin TE, Moore JC, Anand SS, Lonn EM, Morgan DG (2003). "Gastrointestinal bleeding, angiodysplasia, cardiovascular disease, and acquired von Willebrand syndrome". Transfusion medicine reviews 17 (4): 272–86. doi:10.1016/S0887-7963(03)00037-3. PMID 14571395.
^ Jovino, R.; Pontecorvo, C.; Pesce, G.; Jovino, P. (Feb 1989). "[Angiodysplasia of the colon].". Minerva Chir 44 (4): 633–5. PMID 2654735.
^ Festa, V.; Garrone, C.; Simone, P.; Morino, M.; Toppino, M.; Miglietta, C.; Casassa Vigna, M. (May 1989). "[Angiodysplasia of the colon].". Minerva Chir 44 (9): 1389–93. PMID 2668797.
^ Obscure gastrointestinal bleeding and calcific aortic stenosis (Heyde's syndrome)
^ Template:Rossetti A, Buchs NC, Breguet R, Bucher P, Terraz S, Morel P. Transarterial embolization in acute colonic bleeding: review of 11 years of experience and long-term results. Int J Colorectal Dis. 2013 Jun;28(6):777-82.
^ Junquera F, Saperas E, Videla S, Feu F, Vilaseca J, Armengol JR, Bordas JM, Piqué JM, Malagelada JR (2007). "Long-term efficacy of octreotide in the prevention of recurrent bleeding from gastrointestinal angiodysplasia". Am. J. Gastroenterol. 102 (2): 254–60. doi:10.1111/j.1572-0241.2007.01053.x. PMID 17311647.
^ Shurafa M, Kamboj G (2003). "Thalidomide for the treatment of bleeding angiodysplasias". Am. J. Gastroenterol. 98 (1): 221–2. doi:10.1111/j.1572-0241.2003.07201.x. PMID 12526972.

UpToDate Contents

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1. 消化管の血管異形成 angiodysplasia of the gastrointestinal tract
2. 成人における下部消化管出血の病因 etiology of lower gastrointestinal bleeding in adults
3. 毛細血管奇形（ポートワイン母斑）：臨床的特徴、診断、および関連する症候群 capillary malformations port wine stains clinical features diagnosis and associated syndromes
4. 消化管出血のマネージメントにおけるアルゴンプラズマ凝固法 argon plasma coagulation in the management of gastrointestinal hemorrhage
5. 透析患者における消化管疾患 gastrointestinal disease in dialysis patients

English Journal

Review article: gastrointestinal angiodysplasia - pathogenesis, diagnosis and management.

Sami SS, Al-Araji SA, Ragunath K.SourceNottingham Digestive Diseases Centre & NIHR Biomedical research Unit, Queens Medical Centre, Nottingham, UK.
Alimentary pharmacology & therapeutics.Aliment Pharmacol Ther.2013 Oct 20. doi: 10.1111/apt.12527. [Epub ahead of print]
BACKGROUND: Angiodysplasia (AD) of the gastrointestinal (GI) tract is an important condition that can cause significant morbidity and -rarely - mortality.AIMS: To provide an up-to-date comprehensive summary of the literature evaluating this disease entity with a particular focus on pathogenesis as w
PMID 24138285

Diagnostic and Therapeutic Utility of Double Balloon Enteroscopy in Children.

Urs A, Martinelli M, Rao P, Thomson M.Source*Centre for Paediatric Gastroenterology and International Academy of Pediatric Endoscopy Training, Sheffield Children's NHS Foundation Trust, UK †Department of Paediatrics, University of Naples, "Federico II", Naples, Italy.
Journal of pediatric gastroenterology and nutrition.J Pediatr Gastroenterol Nutr.2013 Oct 11. [Epub ahead of print]
OBJECTIVES:: Diagnostic and therapeutic benefits of double-balloon enteroscopy (DBE) have been documented in adults with little pediatric data available. We evaluated the diagnostic and therapeutic utility of DBE in children.METHODS:: A Prospective assessment of 113 DBE procedures in 58 consecutive
PMID 24126830

Lymphangiopathy in neurofibromatosis 1 manifesting with chylothorax, pericardial effusion, and leg edema.

Finsterer J, Stollberger C, Stubenberger E, Tschakoschian S.SourceKrankenanstalt Rudolfstiftung, Vienna, Austria.
International journal of general medicine.Int J Gen Med.2013 Sep 9;6:743-6. doi: 10.2147/IJGM.S45825.
BACKGROUND: This case report documents the affliction of the lymph vessels as a phenotypic feature of neurofibromatosis-1 (NF-1).METHODOLOGY: Routine transthoracic echocardiography, computed tomography scan of the thorax, magnetic resonance angiography of the renal arteries, and conventional digital
PMID 24043952

Japanese Journal

大量出血をきたし緊急手術を施行した大腸 angiodysplasia の1例

上杉尚正,竹重元寛,竹本圭宏,原田栄二郎,榎忠彦,濱野公一
山口医学 60(4), 113-116, 2011-08-31
NAID 10029463572

症例報告大量出血をきたし緊急手術を施行した大腸angiodysplasiaの1例

上杉尚正,竹重元寛,竹本圭宏 [他]
山口医学 60(4), 113-116, 2011-08
… 繰り返す血便に対する経過観察中に,出血性ショックとなり緊急手術を施行した大腸angiodysplasiaの1例を経験した.症例は62歳,男性.約10年間の繰り返す血便を認めていた.腹部X線,CT検査上,小腸内に鏡面像を認めた.下部消化管内視鏡検査で,大腸内に出血源は認めなかった.出血シンチグラフィを撮影したが陽性所見は得られなかった.経過観察中,大量出血に伴う意識消失発作を認め緊急手術を施行した.術中全小腸内視鏡検査を …
NAID 40018977885