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Khalifa A, Rockey DC. Role of Endoscopy in the Diagnosis, Grading, and Treatment of Portal Hypertensive Gastropathy and Gastric Antral Vascular Ectasia. Gastrointest Endosc Clin N Am 2024; 34:263-274. [PMID: 38395483 DOI: 10.1016/j.giec.2023.09.013] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/25/2024]
Abstract
Portal hypertensive gastropathy (PHG) and gastric antral vascular ectasia (GAVE) are 2 distinct gastric vascular abnormalities that may present with acute or chronic blood loss. PHG requires the presence of portal hypertension and is typically associated with chronic liver disease, whereas there is controversy about the association of GAVE with chronic liver disease and/or portal hypertension. Distinguishing between GAVE and PHG is crucial because their treatment strategies differ. This review highlights characteristic endoscopic appearances and the clinical features of PHG and GAVE, which, in turn, aid in their appropriate management.
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Affiliation(s)
- Ali Khalifa
- Digestive Disease Research Center, Medical University of South Carolina, Charleston, South Carolina, USA
| | - Don C Rockey
- Digestive Disease Research Center, Medical University of South Carolina, Charleston, South Carolina, USA.
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Manfredi G, Crinò SF, Alicante S, Romeo S, Berté R, Gandolfi S, Spinazzola A, Fiini M, Forner P, Buscarini E. Gastrointestinal bleeding in patients with hereditary hemorrhagic telangiectasia: Long-term results of endoscopic treatment. Endosc Int Open 2023; 11:E1145-E1152. [PMID: 38108019 PMCID: PMC10723967 DOI: 10.1055/a-2190-9303] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/01/2023] [Accepted: 10/04/2023] [Indexed: 12/19/2023] Open
Abstract
Background and study aims This longitudinal prospective study evaluated the long-term outcome of endoscopic treatment of gastrointestinal bleeding in hereditary hemorrhagic telangiectases (HHT), its safety and outcome predictors. Patients and methods Consecutive patients with HHT and either anemia disproportionate to epistaxis or overt gastrointestinal bleeding received endoscopic treatment of gastrointestinal telangiectases with argon plasma coagulation (APC). Hemoglobin levels and transfusion requirements were evaluated before and after treatment. Treatment effectiveness was classified as: 1) complete: hemoglobin level during the follow-up ≥9 g/dL; 2) complete with recurrence: hemoglobin ≥9 g/dL for at least 12 months with subsequent drop to <9 g/dL; or 3) absent: no improvement of hemoglobin level. Adverse events (AEs) were classified as mild, moderate, severe or fatal. Correlations were searched between treatment outcome and demographic/genetic characteristics, number, size and site of telangiectases, and hepatic arterio-venous malformations grade. Results Forty-seven patients with HHT were enrolled. At median follow-up of 134 months (range 20-243 months), 41 of 47 patients showed treatment response (complete or with recurrence) after one (14/47) or more (27/47) endoscopic treatments. Median hemoglobin levels were 7.0 g/dL and 11.9 g/dL at baseline and at the end of follow-up, respectively. Transfusion requirement decreased from 22.8 to 7.3 red cell unit/year. A higher baseline number of telangiectases was associated with a lower chance of response ( P =0.008). Only one severe AE (0.4%, jejunal perforation) was recorded. Conclusions Endoscopic treatment of gastrointestinal teleangiectases for gastrointestinal bleeding in patients with HHT is effective in the long term and safe.
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Affiliation(s)
- Guido Manfredi
- Gastroenterology and Endoscopy Department, Center of reference VASCERN HHT, Maggiore Hospital Crema, Crema, Italy
| | | | - Saverio Alicante
- Gastroenterology and Endoscopy Department, Center of reference VASCERN HHT, Maggiore Hospital Crema, Crema, Italy
| | - Samanta Romeo
- Gastroenterology and Endoscopy Department, Center of reference VASCERN HHT, Maggiore Hospital Crema, Crema, Italy
| | - Roberto Berté
- Gastroenterology and Endoscopy Department, Center of reference VASCERN HHT, Maggiore Hospital Crema, Crema, Italy
| | | | | | - Michela Fiini
- Cardiology Department, Maggiore Hospital Crema, Crema, Italy
| | | | - Elisabetta Buscarini
- Gastroenterology and Endoscopy Department, Center of reference VASCERN HHT, Maggiore Hospital Crema, Crema, Italy
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Solela G, Adamseged M, Mohammed A. Hereditary hemorrhagic telangiectasia in a 42-year-old Ethiopian man presenting with severe anemia and high-output heart failure: A case report with literature review. Clin Case Rep 2023; 11:e7694. [PMID: 37448947 PMCID: PMC10337522 DOI: 10.1002/ccr3.7694] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/08/2023] [Revised: 06/28/2023] [Accepted: 06/30/2023] [Indexed: 07/18/2023] Open
Abstract
Key Clinical Messages Hereditary hemorrhagic telangiectasia (HHT), a rare hereditary disorder, can cause recurrent massive epistaxis and gastrointestinal bleeding leading to severe anemia. Early diagnosis of HHT is essential to provide timely interventional therapies. Abstract HHT is a rare autosomal dominant hereditary disease that results in abnormal vasculogenesis in the skin, mucous membranes, and visceral organs such as the liver, lungs, and brain. Clinical diagnosis of HHT is made using the Curacao criteria, which include recurrent spontaneous nosebleeds, mucocutaneous telangiectasias, visceral organ involvement, and first-degree family history of HHT. Here, we report a patient with HHT from Ethiopia, who presented with recurrent epistaxis and gastrointestinal bleeding, and severe anemia requiring frequent blood transfusions as well as cauterization. The presented case is a 42-year-old Black Ethiopian man with frequent hospitalization for severe anemia and high-output heart failure requiring frequent blood transfusions. His mother had bilateral epistaxis since her early adulthood. Physical examination was significant for tachycardia, pale conjunctivae, and tiny erythematous lesions over his tongue, ejection systolic murmur and peripheral edema. Laboratory investigations revealed severe anemia with iron deficiency picture. Upper gastrointestinal endoscopy showed multiple telangiectasia spots and abdominal Doppler ultrasound showed hepatic arterio-venous malformations. He received supportive management and electrocauterization of nasal, gastric, and duodenal telangiectasias. To the best of our knowledge, this is the first case of HHT to be reported from Ethiopia. High degree of suspicion and early diagnosis of HHT is essential to start preventive screening and surveillance and provide timely interventional therapies. HHT can cause recurrent massive epistaxis and gastrointestinal bleeding leading to severe anemia and high-output heart failure. In resource limited settings, selective cauterization of telangiectasia will help to control bleeding, although it does not avoid recurrent bleeding.
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Affiliation(s)
- Gashaw Solela
- Department of Internal MedicineYekatit 12 Hospital Medical CollegeAddis AbabaEthiopia
| | - Michael Adamseged
- Department of Internal MedicineYekatit 12 Hospital Medical CollegeAddis AbabaEthiopia
| | - Abdulsemed Mohammed
- Division of Gastroenterology and Hepatology, Department of Internal MedicineCollege of Health Sciences, Addis Ababa UniversityAddis AbabaEthiopia
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Zhang C, Wang Y, Zhang D, Li S. Colonoscopic and Clinical Features of Colonic Angiodysplasia: A Study in 54 Patients. Surg Laparosc Endosc Percutan Tech 2023; 33:115-120. [PMID: 36944226 PMCID: PMC10065816 DOI: 10.1097/sle.0000000000001159] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/26/2022] [Accepted: 01/31/2023] [Indexed: 03/23/2023]
Abstract
OBJECTIVES Colonic angiodysplasia is a rare disease, it is nevertheless a common cause of lower gastrointestinal (GI) bleeding in older adults. The study summarized the colonoscopic and clinical features of colonic angiodysplasia to raise awareness among endoscopists regarding this disease. MATERIALS AND METHODS We performed a retrospective study of enrolled patients diagnosed with colonic angiodysplasia between September 2013 and April 2022. Clinical and colonoscopic features of the patients with active bleeding were analyzed and compared with those of patients without bleeding. The comparisons were also conducted between the patients with active lower GI bleeding caused by colonic angiodysplasia and those by other diseases. RESULTS In total, 54 eligible patients were included in this study; 55.55% of the participants were aged over 60 years. Ten patients (3 men and 7 women) with colonic angiodysplasia suffered from active lower GI bleeding, which was mainly located in the left and total colon. The patients with type 2 diabetes mellitus, radiotherapy history, antiplatelet drug use, and multiple lesions were more likely to endure lower GI bleeding. The duration between bleeding and admission was longer in the colonic angiodysplasia group than in the other diseases group ( P = 0.043). In the colonic angiodysplasia group, bleeding relapsed in 3 patients, and the recurrence rate was higher than in the other diseases group ( P < 0.001). CONCLUSION Endoscopists should perform colonoscopy scrupulously and consider colonic angiodysplasia as a differential diagnosis in patients with lower GI bleeding, especially for older women and adults with chronic diseases, such as type 2 diabetes mellitus.
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Gairing SJ, Schleicher EM, Müller L, Labenz C, Darstein F, Grimm D, Sivanathan V, Weinmann A, Wörns MA, Kloeckner R, Pitton MB, Thieringer F, Rahman KF, Galle PR, Schattenberg JM, Foerster F. Bevacizumab in combination with octreotide rescues a patient with liver cirrhosis, GAVE syndrome and refractory hemorrhage - a case report. ZEITSCHRIFT FUR GASTROENTEROLOGIE 2023; 61:275-279. [PMID: 36379462 DOI: 10.1055/a-1959-2660] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Abstract
Gastric antral vascular ectasia (GAVE) syndrome is a rare but often challenging etiology of upper gastrointestinal bleeding (UGIB).We report on a 60-year-old patient with liver cirrhosis, GAVE syndrome and recurrent and refractory GAVE-related UGIB. During a 5-month hospital stay, the patient required a total of 82 packed red blood cells (pRBCs) and 23 gastroscopies. All endoscopic approaches, including multiple argon plasma coagulation and band ligation sessions, remained unsuccessful. Antrectomy was waived because of the high perioperative mortality risk in Child-Pugh B liver cirrhosis. TIPS insertion also failed to control the bleeding. Only continuous intravenous octreotide infusion slowed the bleeding, but this forced the patient to be hospitalized. After 144 inpatient days, administration of subcutaneous octreotide allowed the patient to be discharged. However, the patient continued to require two pRBCs every 2-3 weeks. Based on recently published data, we treated the patient with bevacizumab (anti-VEGF antibody) off-label at a dose of 7.5 mg/kg body weight every three weeks in nine single doses over six months. Since the first administration, the patient has remained transfusion-free, has not required hospitalization, and leads an active life, working full-time. He remains on octreotide, which has been reduced but not yet discontinued. Additionally, no adverse events were observed.Thus, in patients with liver cirrhosis and refractory GAVE-related hemorrhage, bevacizumab combined with subcutaneous octreotide should be considered as an effective and durable pharmacological treatment option.
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Affiliation(s)
- Simon Johannes Gairing
- Department of Internal Medicine I, University Medical Centre of the Johannes Gutenberg University Mainz, Mainz, Germany
| | - Eva Maria Schleicher
- Department of Internal Medicine I, University Medical Centre of the Johannes Gutenberg University Mainz, Mainz, Germany
| | - Lukas Müller
- Department of Diagnostic and Interventional Radiology, University Medical Centre of the Johannes Gutenberg University Mainz, Mainz, Germany
| | - Christian Labenz
- Department of Internal Medicine I, University Medical Centre of the Johannes Gutenberg University Mainz, Mainz, Germany
| | - Felix Darstein
- Department of Internal Medicine I, University Medical Centre of the Johannes Gutenberg University Mainz, Mainz, Germany
| | - Daniel Grimm
- Department of Internal Medicine I, University Medical Centre of the Johannes Gutenberg University Mainz, Mainz, Germany
| | - Visvakanth Sivanathan
- Department of Internal Medicine I, University Medical Centre of the Johannes Gutenberg University Mainz, Mainz, Germany
| | - Arndt Weinmann
- Department of Internal Medicine I, University Medical Centre of the Johannes Gutenberg University Mainz, Mainz, Germany
| | - Marcus-Alexander Wörns
- Department of Gastroenterology, Hematology, Oncology and Endocrinology, Hospital Dortmund, Dortmund, Germany
| | - Roman Kloeckner
- Department of Diagnostic and Interventional Radiology, University Medical Centre of the Johannes Gutenberg University Mainz, Mainz, Germany
| | - Michael B Pitton
- Department of Diagnostic and Interventional Radiology, University Medical Centre of the Johannes Gutenberg University Mainz, Mainz, Germany
| | - Florian Thieringer
- Department of Internal Medicine I, University Medical Centre of the Johannes Gutenberg University Mainz, Mainz, Germany
| | - Khan Fareed Rahman
- Department of Internal Medicine I, University Medical Centre of the Johannes Gutenberg University Mainz, Mainz, Germany
| | - Peter Robert Galle
- Department of Internal Medicine I, University Medical Centre of the Johannes Gutenberg University Mainz, Mainz, Germany
| | - Jörn M Schattenberg
- Department of Internal Medicine I, University Medical Centre of the Johannes Gutenberg University Mainz, Mainz, Germany
| | - Friedrich Foerster
- Department of Internal Medicine I, University Medical Centre of the Johannes Gutenberg University Mainz, Mainz, Germany
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Kwon HJ, Lee SH, Cho JH. Influences of etiology and endoscopic appearance on the long-term outcomes of gastric antral vascular ectasia. World J Clin Cases 2022; 10:6050-6059. [PMID: 35949841 PMCID: PMC9254169 DOI: 10.12998/wjcc.v10.i18.6050] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/18/2021] [Revised: 01/31/2022] [Accepted: 04/24/2022] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Gastric antral vascular ectasia (GAVE) has diverse associations and presumed causes, which include liver cirrhosis, chronic kidney disease, and autoimmune disease. This heterogeneity of underlying disorders suggests that the pathogenesis of GAVE may be variable.
AIM To compare the clinical features and long-term outcomes of GAVE according to endoscopic patterns and etiologies.
METHODS The medical records and endoscopic images of 23 consecutive patients diagnosed with GAVE by endoscopy at Yeungnam University Hospital from January 2006 to December 2020 were retrospectively reviewed. Patients were allocated to cirrhosis (16 patients) and non-cirrhosis groups (7 patients). GAVE subtypes, as determined by endoscopy, were categorized as punctate (a diffuse, honeycomb-like appearance, 17 patients) or striped (a linear, watermelon-like appearance, 6 patients).
RESULTS All GAVE patients with cirrhosis (16/16, 100%) had a punctate pattern by endoscopy, whereas the majority of patients (6/7, 85.7%) without cirrhosis had a striped pattern (P < 0.001). Overt GAVE bleeding (10/23, 43%) was significantly more common in the non-cirrhosis group than in the cirrhosis group (6/7, 85.7% vs 4/16, 25.0%; P = 0.019), and more common in the striped group than in the punctate group (5/6, 83.3% vs 5/17, 29.4%; P = 0.052). However, mean numbers of admissions due to GAVE bleeding and argon plasma coagulation (APC) sessions to address overt bleeding were similar in the cirrhosis and non-cirrhosis groups and in the punctate and striped groups. All patients with GAVE bleeding were successfully treated by APC, and no patient died from GAVE-related blood loss during a median follow-up of 24 mo.
CONCLUSION Punctate-type GAVE is strongly associated with liver cirrhosis, and GAVE patients without cirrhosis tend to be more prone to overt bleeding. However, the presence of cirrhosis and endoscopic patterns did not influence long-term clinical courses or outcomes in cases of overt bleeding.
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Affiliation(s)
- Hyo Jin Kwon
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Yeungnam University College of Medicine, Daegu 42415, South Korea
| | - Si Hyung Lee
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Yeungnam University College of Medicine, Daegu 42415, South Korea
| | - Joon Hyun Cho
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Yeungnam University College of Medicine, Daegu 42415, South Korea
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Jargielo A, Rycyk A, Kasztelan-Szczerbinska B, Cichoz-Lach H. A Rare Case of Upper Gastrointestinal Bleeding: Osler-Weber-Rendu Syndrome. MEDICINA (KAUNAS, LITHUANIA) 2022; 58:333. [PMID: 35334510 PMCID: PMC8951266 DOI: 10.3390/medicina58030333] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 11/29/2021] [Revised: 02/14/2022] [Accepted: 02/16/2022] [Indexed: 11/30/2022]
Abstract
Osler-Weber-Rendu disease, also known as hereditary hemorrhagic telangiectasia (HHT), is a rare, autosomal dominant condition that affects approximately 1 in 5000 patients causing abnormal blood vessel formation. HHT patients have mucocutaneous telangiectasias and arteriovenous malformations in various organs. The most prominent symptom of HHT is epistaxis, which, together with gastrointestinal bleeding, may cause iron deficiency anemia. This study is a case report of a 62-year-old patient who was admitted to the Department of Gastroenterology due to acute upper gastrointestinal bleeding and a history of recurrent epistaxis and melena for 4 days, which was confirmed in digital rectal examination. Urgent upper gastrointestinal endoscopy revealed active bleeding from multiple angioectatic spots with bright-looking salmon-colored patches in the antrum and the body suggestive of HHT. The bleeding from two angioectatic spots was stopped by argon plasma coagulation, and four clips were placed to provide good hemostasis. The patient was treated with a proton pomp inhibitor infusion and iron infusion. She was discharged with no signs of GI bleeding, normalized iron levels and a diagnosis of HHT. She was referred to further genetic testing, including evaluation of first-degree relatives. She also had performed unenhanced thin-cut computed tomography (CT) with angiography to exclude the presence of pulmonary arteriovenous malformations (PAVMs). Due to the fact that the patient did not manifest any other HHT-related symptoms and that the instrumental screening discloses no silent AVMs in other organs, the "watch-and-wait strategy" was applied. Although, Osler-Weber-Rendu syndrome is widely described in the medical literature, effective treatment of gastrointestinal telangiectasias is not always available and still lacks standardization to date, which makes the management of gastroenterological involvement still a challenging issue.
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Affiliation(s)
- Anna Jargielo
- Banacha Campus, Medical University of Warsaw, Zwirki i Wigury 61 St., 02-091 Warsaw, Poland;
| | - Anna Rycyk
- Department of Gastroenterology with Endoscopy Unit, Medical University of Lublin, Jaczewskiego 8 St., 20-954 Lublin, Poland; (B.K.-S.); (H.C.-L.)
| | - Beata Kasztelan-Szczerbinska
- Department of Gastroenterology with Endoscopy Unit, Medical University of Lublin, Jaczewskiego 8 St., 20-954 Lublin, Poland; (B.K.-S.); (H.C.-L.)
| | - Halina Cichoz-Lach
- Department of Gastroenterology with Endoscopy Unit, Medical University of Lublin, Jaczewskiego 8 St., 20-954 Lublin, Poland; (B.K.-S.); (H.C.-L.)
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O'Morain NR, O'Donovan H, Conlon C, Shannon E, Manning D, Slattery E. Is Endoscopic Band Ligation a Superior Treatment Modality for Gastric Antral Vascular Ectasia Compared to Argon Plasma Coagulation? Clin Endosc 2021; 54:548-554. [PMID: 33435659 PMCID: PMC8357586 DOI: 10.5946/ce.2020.236] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/24/2020] [Accepted: 10/13/2020] [Indexed: 12/13/2022] Open
Abstract
BACKGROUND/AIMS Gastric antral vascular ectasia (GAVE) is a rare acquired vascular lesion of the gastric antrum. The most frequent presentation of GAVE is iron deficiency anemia. Endoscopic therapy is the mainstay of treatment. However, there is no consensus regarding the optimal treatment modality. METHODS A retrospective cohort study was performed on patients with GAVE, including patients receiving endoscopic therapy. Treatment was with either argon plasma coagulation (APC) or endoscopic band ligation (EBL). Basic demographic data, indication for index procedure, number of sessions, and pre- and post-hemoglobin levels were collected. The aim of the study was to compare outcomes across the two treatment modalities. RESULTS One hundred and seventeen diagnoses of GAVE were made. Sixty-two patients (53%) required endoscopic treatment for symptomatic GAVE (female, n=38, 61%; mean age of 74.4 years). Two hundred and eighteen procedures were performed during the study period. APC was performed (n=161, 74%) more frequently than EBL (n=57, 26%). Patients treated with APC at index required a median 5 subsequent therapeutic interventions (APC or EBL), while those treated with EBL at index required a further 2.9 treatments (EBL only) (p<0.05). CONCLUSION APC was the most common treatment modality employed. We demonstrate an increasing incidence of EBL. Patients treated with EBL at index treatment required fewer subsequent treatment sessions and had a greater mean rise in hemoglobin. This suggests a more effective endoscopic response with EBL.
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Affiliation(s)
| | - Helen O'Donovan
- Department of Gastroenterology, University Hospital Galway, Galway, Ireland
| | - Caroline Conlon
- Department of Gastroenterology, University Hospital Galway, Galway, Ireland
| | - Eileen Shannon
- Department of Gastroenterology, University Hospital Galway, Galway, Ireland
| | - Diarmuid Manning
- Department of Gastroenterology, University Hospital Galway, Galway, Ireland
| | - Eoin Slattery
- Department of Gastroenterology, University Hospital Galway, Galway, Ireland
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Pizzuto M, Ellul S, Shoukry M. Gastric antral vascular ectasia in children, rare presentation. BMJ Case Rep 2020; 13:13/11/e236896. [PMID: 33257371 DOI: 10.1136/bcr-2020-236896] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
A 14-year-old boy, a known case of perinatal hypoxic cerebral palsy, presented to paediatric emergency with acute melaena and blood staining around feeding gastrostomy site. Physical examination revealed pallor, but no signs of distress with an unremarkable abdominal examination. Routine blood tests revealed normochromic. Abdominal ultrasound scan and Meckel's scan were unremarkable. The patient underwent examination under anaesthesia of the perianal area and joint upper and lower gastrointestinal endoscopy. Streak-like gastritis with no signs of active bleeding lesions were noted and patchy areas of colitis involving the descending and sigmoid colon and the rectum. All clinical findings and evidence-based diagnosis matched gastric antral vascular ectasia. He was successfully managed conservatively with elemental hydrolysed feeding formula.
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Castillo E, Prada-Arismendy J. Bevacizumab as a treatment option in gastrointestinal bleeding associated to hereditary hemorrhagic telangiectasia. Case Report. REVISTA DE LA FACULTAD DE MEDICINA 2019. [DOI: 10.15446/revfacmed.v67n3.68417] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/09/2022] Open
Abstract
Introduction: Hereditary Hemorrhagic Telangiectasia (HHT) is an autosomal dominant genetic disease characterized by the presence of arteriovenous malformations in the nasal mucosa, the tips of fingers, and sometimes in the lungs, the gastrointestinal tract, the liver, the pancreas, the marrow and the brain. Its treatment is based on symptomatic control measures, but recently, the administration of anti-vascular endothelial growth factor (VEGF) molecules has been proposed as a treatment alternative, especially in patients with recurrent bleeding.Case presentation: The case of a 67-year-old man diagnosed with HHT and suffering from potentially life-threatening gastrointestinal GI bleeding is presented. The patient underwent several esophagogastric cauterization procedures but not positive outcomes were obtained, so he had to go to the Emergency Service of the hospital multiple times due to having low levels of hemoglobin (as low as 3.5g/dl). A bevacizumab based treatment was started by using a novel dosage regimen consisting of the administration of 6 5mg/kg bevacizumab dosages every 14 days. During the first week of treatment, hemoglobin levels increased to 14g/dl and the condition was stabilized.Conclusions: The findings reported here suggest that bevacizumab may be a therapeutic choice to be considered when treating patients with recurrent and refractory GI bleeding caused by HHT. However, a larger sample is required to determine if administering this medication is safe for these patients, as well as the appropriate dosage.
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Abstract
PURPOSE OF REVIEW Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant inherited disease characterized by telangiectasia and arteriovenous malformations (AVMs). To date, five genetic types of HHT and one combined juvenile polyposis syndrome and HHT are known. Clinical and genetic screening of patients suspected with HHT is recommended to confirm the diagnosis and to prevent complications associated with HHT. The aim of this article is to give an overview of the evidence and to formulate a recommendation for clinicians concerning screening for HHT. RECENT FINDINGS Complications of HHT such as stroke, brain abscess and intracranial hemorrhage are caused by pulmonary and cerebral AVMs (CAVMs) and can often be prevented by screening and treatment when possible. Screening and treatment of these AVMs will result in an increased life expectancy comparable with that of the general population as opposed to unscreened and untreated HHT patients. SUMMARY Screening of HHT patients and their first-degree relatives is recommended to prevent severe complications including stroke, brain abscess and intracranial hemorrhage.
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Singh K, Zubair A, Prindle A, Nadeem AJ, Khan G. Diagnostic yield of capsule endoscopy for small bowel arteriovenous malformations in patients with hereditary hemorrhagic telangiectasia: a systematic review and meta-analysis. Endosc Int Open 2019; 7:E282-E289. [PMID: 30705963 PMCID: PMC6353713 DOI: 10.1055/a-0799-9820] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/04/2018] [Accepted: 10/02/2018] [Indexed: 12/11/2022] Open
Abstract
Background and study aims Small bowel arteriovenous malformations (AVMs) pose a bleeding risk and have traditionally been diagnosed by invasive enteroscopic procedures in patients with hereditary hemorrhagic telangiectasia (HHT). Capsule endoscopy (CE) is emerging as a safe and non-invasive alternative for small intestinal evaluation, but its diagnostic yield and utility in diagnosing small bowel AVMs in HHT patients are understudied. The aim of this study was to meta-analyze the utility of CE for diagnosing AVMs in HHT patients. Methods A meta-analysis and systematic review of the literature on CE in HHT patients identified in the PubMed, EMBASE, Scopus, and Cochrane databases from inception to March 2018 were conducted. Summary effects were estimated using a random effects model. Results After applying exclusion criteria, five studies (n = 124 patients) were eligible for meta-analysis. The pooled diagnostic yield for visualization of small bowel AVMs by CE was 77.0 % (95 % CI 65.8 - 85.4 %, P < 0.001). Conclusions CE has a good diagnostic yield for small bowel AVMs in HHT. It can be regarded as a sufficient, noninvasive diagnostic modality for identifying small bowel AVMs in HHT patients.
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Affiliation(s)
- Kevin Singh
- Department of Internal Medicine, New York University School of Medicine, Woodhull Medical and Mental Health Center, Brooklyn, New York, United States
| | - Ayla Zubair
- Department of Internal Medicine, New York University School of Medicine, Woodhull Medical and Mental Health Center, Brooklyn, New York, United States
| | - Andrew Prindle
- Department of Internal Medicine, New York University School of Medicine, Woodhull Medical and Mental Health Center, Brooklyn, New York, United States
| | - Ahmed Jamal Nadeem
- Department of Hematology and Oncology, Lehigh Valley Health Network, Allentown, Pennsylvania, United States
| | - Gulam Khan
- Department of Gastroenterology and Hepatology, New York University School of Medicine, Woodhull Medical and Mental Health Center, Brooklyn, New York, United States
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Abstract
Non-variceal upper gastrointestinal bleeding (NVUGIB) is bleeding that develops in the oesophagus, stomach or proximal duodenum. Peptic ulcers, caused by Helicobacter pylori infection or use of NSAIDs and low-dose aspirin (LDA), are the most common cause. Although the incidence and mortality associated with NVUGIB have been decreasing owing to considerable advances in the prevention and management of NVUGIB over the past 20 years, it remains a common clinical problem with an annual incidence of ∼67 per 100,000 individuals in the United States in 2012. NVUGIB is a medical emergency, and mortality is in the range ∼1-5%. After resuscitation and initial assessment, early (within 24 hours) diagnostic and therapeutic endoscopy together with intragastric pH control with proton pump inhibitors (PPIs) form the basis of treatment. With a growing ageing population treated with antiplatelet and/or anticoagulant medications, the clinical management of NVUGIB is complex as the risk between gastrointestinal bleeding events and adverse cardiovascular events needs to be balanced. The best clinical approach includes identification of risk factors and prevention of bleeding; available strategies include continuous treatment with PPIs or H. pylori eradication in those at increased risk of developing NVUGIB. Treatment with PPIs and/or use of cyclooxygenase-2-selective NSAIDs should be implemented in those patients at risk of NVUGIB who need NSAIDs and/or LDA.
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Abstract
Gastric antral vascular ectasia (GAVE) is a rare but an important cause of upper gastrointestinal bleeding (UGIB) and commonly presents as occult bleeding that manifests as iron deficiency anemia (IDA). GAVE is commonly associated with chronic illnesses, most frequently liver cirrhosis and connective tissue diseases. The pathogenesis of GAVE is still obscure, and many hypotheses such as mechanical stress, hormonal factors, and autoimmune factors, have been proposed. Upper gastrointestinal endoscopy has a major role in the diagnosis and treatment of GAVE.
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Hsu WH, Wang YK, Hsieh MS, Kuo FC, Wu MC, Shih HY, Wu IC, Yu FJ, Hu HM, Su YC, Wu DC. Insights into the management of gastric antral vascular ectasia (watermelon stomach). Therap Adv Gastroenterol 2018; 11:1756283X17747471. [PMID: 29399041 PMCID: PMC5788127 DOI: 10.1177/1756283x17747471] [Citation(s) in RCA: 27] [Impact Index Per Article: 3.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/26/2017] [Accepted: 10/30/2017] [Indexed: 02/04/2023] Open
Abstract
Gastric antral vascular ectasia (GAVE) is an uncommon but important cause of chronic gastrointestinal bleeding. It is often associated with systemic diseases such as autoimmune diseases, liver cirrhosis, chronic renal insufficiency and cardiovascular disease. The etiology of GAVE has not been fully explored and remains controversial. Diagnosis is mainly based on endoscopic presentation with flat or raised erythematous stripes radiating from the pylorus to the antrum and resembles a watermelon. Clinical presentation may range from iron-deficiency anemia secondary to occult blood loss, melena to hematemesis. In past decades, many therapeutic modalities including medical, endoscopic and surgical intervention have been introduced for GAVE treatment with variable efficacy. Herein, we review the efficacy and safety of these treatment options for GAVE.
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Affiliation(s)
- Wen-Hung Hsu
- Division of Gastroenterology, Department of Internal Medicine, Kaohsiung Medical University Hospital, Kaohsiung, TaiwanDepartment of Medicine, Faculty of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan
| | - Yao-Kuang Wang
- Division of Gastroenterology, Department of Internal Medicine, Kaohsiung Medical University Hospital, Kaohsiung, TaiwanDepartment of Internal Medicine, Kaohsiung Municipal Hsiao-Kang Hospital, Kaohsiung, Taiwan
| | - Meng-Shu Hsieh
- Division of Gastroenterology, Department of Internal Medicine, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan
| | - Fu-Chen Kuo
- Shool of Medicine, College of Medicine, E-Da Hospital, I-Shou University, Kaohsiung, Taiwan
| | - Meng-Chieh Wu
- Division of Gastroenterology, Department of Internal Medicine, Kaohsiung Medical University Hospital, Kaohsiung, TaiwanDepartment of Internal Medicine, Kaohsiung Municipal Ta-Tung Hospital, Kaohsiung, Taiwan
| | - Hsiang-Yao Shih
- Division of Gastroenterology, Department of Internal Medicine, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan
| | - I-Chen Wu
- Division of Gastroenterology, Department of Internal Medicine, Kaohsiung Medical University Hospital, Kaohsiung, TaiwanDepartment of Medicine, Faculty of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan
| | - Fang-Jung Yu
- Division of Gastroenterology, Department of Internal Medicine, Kaohsiung Medical University Hospital, Kaohsiung, TaiwanDepartment of Medicine, Faculty of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan
| | - Huang-Ming Hu
- Division of Gastroenterology, Department of Internal Medicine, Kaohsiung Medical University Hospital, Kaohsiung, TaiwanDepartment of Medicine, Faculty of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan
| | - Yu-Chung Su
- Division of Gastroenterology, Department of Internal Medicine, Kaohsiung Medical University Hospital, Kaohsiung, TaiwanDepartment of Medicine, Faculty of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan
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Amann A, Steiner N, Gunsilius E. Bevacizumab: an option for refractory epistaxis in hereditary haemorrhagic telangiectasia. Wien Klin Wochenschr 2015; 127:631-4. [PMID: 25986996 DOI: 10.1007/s00508-015-0789-4] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/15/2014] [Accepted: 04/09/2015] [Indexed: 12/17/2022]
Abstract
BACKGROUND Recurrent epistaxis in hereditary haemorrhagic telangiectasia (HHT) patients significantly decreases their quality of life. Treatment in therapy refractory patients is limited although various options have been tested so far. CASE REPORT Herein, one patient is described that was treated for HHT for over 20 years with only intermediate benefits. As epistaxis duration and frequency increased continuously, bevacizumab 5 mg/kg was administered every 2 weeks. During the time of treatment (six doses) and up to 3 month afterwards clinical symptoms, blood pressure, cardiac output, pulmonary arterial hypertension (PAH), bleeding duration and frequency were assessed as criteria for treatment benefit. RESULTS Duration and frequency of epistaxis decreased immediately after the first application resulting in reduced need of blood transfusions. After completion of six cycles, a further decrease in frequency and duration of bleeding was noted. Cardiac output and PAH decreased or remained stable, respectively, during time and after treatment. No increase in blood pressure could be found but a significant increase in heart rate was experienced after completion of all six applications. Unfortunately, the patient died due to a cerebral abscess. CONCLUSION Bevacizumab led to an improvement of HHT related epistaxis, refractory to other treatments.
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Affiliation(s)
- Arno Amann
- Department of Internal Medicine V (Haematology and Oncology), Medical University Innsbruck, Anichstraße 35, 6020, Innsbruck, Austria,
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Garg N, Khunger M, Gupta A, Kumar N. Optimal management of hereditary hemorrhagic telangiectasia. J Blood Med 2014; 5:191-206. [PMID: 25342923 PMCID: PMC4206399 DOI: 10.2147/jbm.s45295] [Citation(s) in RCA: 36] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/23/2022] Open
Abstract
Hereditary hemorrhagic telangiectasia (HHT), also known by the eponym Osler-Weber-Rendu syndrome, is a group of related disorders inherited in an autosomal dominant fashion and characterized by the development of arteriovenous malformations (AVM) in the skin, mucous membranes, and/or internal organs such as brain, lungs, and liver. Its prevalence is currently estimated at one in 5,000 to 8,000. Most cases are due to mutations in the endoglin (HHT1) or ACVRLK1 (HHT2) genes. Telangiectasias in nasal and gastrointestinal mucosa generally present with recurrent/chronic bleeding and iron deficiency anemia. Larger AVMs occur in lungs (~40%-60% of affected individuals), liver (~40%-70%), brain (~10%), and spine (~1%). Due to the devastating and potentially fatal complications of some of these lesions (for example, strokes and brain abscesses with pulmonary AVMs), presymptomatic screening and treatment are of utmost importance. However, due to the rarity of this condition, many providers lack an appreciation for the whole gamut of its manifestations and complications, age-dependent penetrance, and marked intrafamilial variation. As a result, HHT remains frequently underdiagnosed and many families do not receive the appropriate screening and treatments. This article provides an overview of the clinical features of HHT, discusses the clinical and genetic diagnostic strategies, and presents an up-to-date review of literature and detailed considerations regarding screening for visceral AVMs, preventive modalities, and treatment options.
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Affiliation(s)
- Neetika Garg
- Department of Medicine, Beth Israel Deaconess Medical Center, Boston, MA, USA
| | - Monica Khunger
- Department of Medicine, All India Institute of Medical Sciences, New Delhi, India
| | - Arjun Gupta
- Department of Medicine, UT Southwestern Medical Center, Dallas, TX, USA
| | - Nilay Kumar
- Department of Medicine, Cambridge Health Alliance/Harvard Medical School, Cambridge, MA, USA
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Swanson E, Mahgoub A, MacDonald R, Shaukat A. Medical and endoscopic therapies for angiodysplasia and gastric antral vascular ectasia: a systematic review. Clin Gastroenterol Hepatol 2014; 12:571-82. [PMID: 24013107 DOI: 10.1016/j.cgh.2013.08.038] [Citation(s) in RCA: 65] [Impact Index Per Article: 5.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/08/2013] [Revised: 07/24/2013] [Accepted: 08/19/2013] [Indexed: 02/07/2023]
Abstract
BACKGROUND & AIMS Few studies have compared the efficacy and complications of endoscopic or medical therapies for bleeding angiodysplasias or gastric antral vascular ectasias (GAVE). We conducted a systematic review to evaluate therapies. METHODS We performed a PubMed search for studies (written in English from January 1, 1980, through January 1, 2013) of medical or endoscopic treatment of bleeding angiodysplasias and GAVE. Measured outcomes included levels of hemoglobin, transfusion requirements, rebleeding rates, complications, treatment failures, and overall mortality. RESULTS We analyzed data from 63 studies that met inclusion criteria; 50 evaluated endoscopic treatment (1790 patients), 13 evaluated medical treatment (392 patients), and 12 were comparative studies. In patients with angiodysplasias, the combination of estrogen and progesterone did not significantly reduce bleeding episodes, compared with placebo (0.7/y vs 0.9/y, respectively), and increased mortality, compared with conservative therapy (33% vs 21%). A higher percentage of patients receiving octreotide were free of rebleeding at 1 and 2 years vs placebo (77% vs 55% and 68% vs 36%, respectively; P = .03). Thalidomide reduced the number of bleeding episodes (-8.96/y), compared with iron therapy (-1.38/y, P < .01), but neither treatment reduced mortality. More patients with GAVE treated by endoscopic band ligation were free from rebleeding (92%) than those treated with argon plasma coagulation (32%, P = .01). CONCLUSIONS In a systematic review, we found a low quality of evidence to support treatment of angiodysplasias with thalidomide or the combination of estrogen and progesterone and insufficient evidence to support treatment with octreotide. There is also insufficient evidence for endoscopic therapy of angiodysplasia or GAVE. Well-designed randomized controlled trials are needed to study the efficacy and complications of medical and endoscopic treatments for patients with angiodysplasias or GAVE.
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Affiliation(s)
- Eric Swanson
- Department of Medicine, Veterans Affairs Medical Center and University of Minnesota, Minneapolis, Minnesota
| | - Amar Mahgoub
- Department of Medicine, Veterans Affairs Medical Center and University of Minnesota, Minneapolis, Minnesota; Section of Gastroenterology, Veterans Affairs Medical Center and University of Minnesota, Minneapolis, Minnesota
| | - Roderick MacDonald
- Minnesota Evidence based Practice Center, Center for Chronic Disease and Outcome Research, Minneapolis, Minnesota
| | - Aasma Shaukat
- Department of Medicine, Veterans Affairs Medical Center and University of Minnesota, Minneapolis, Minnesota; Section of Gastroenterology, Veterans Affairs Medical Center and University of Minnesota, Minneapolis, Minnesota.
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Yoshida H, Takahashi H, Akoshima H, Chida N, Noguchi K, Tanabe N, Mano Y, Ukai K, Tadokoro K. Successful treatment of refractory gastric antral vascular ectasia using transcatheter arterial embolization. Clin J Gastroenterol 2013; 6:231-6. [DOI: 10.1007/s12328-013-0385-y] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/22/2013] [Accepted: 04/30/2013] [Indexed: 12/11/2022]
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Imai Y, Mizuno Y, Yoshino K, Watanabe K, Sugawara K, Motoya D, Oka M, Mochida S. Long-term efficacy of endoscopic coagulation for different types of gastric vascular ectasia. World J Gastroenterol 2013; 19:2799-2805. [PMID: 23687417 PMCID: PMC3653154 DOI: 10.3748/wjg.v19.i18.2799] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/24/2012] [Revised: 02/01/2013] [Accepted: 03/07/2013] [Indexed: 02/06/2023] Open
Abstract
AIM: To examine the long-term therapeutic efficacies of endoscopic cauterization for gastric vascular ectasia, according to the type of lesion.
METHODS: Thirty-eight patients with hemorrhagic gastric vascular ectasia (VE) were treated by endoscopic cauterization: 13 by heater probe coagulationand 25 by argon plasma coagulation. Depending on the number of lesions, 14 and 24 patients were classified into localized VE (≤ 10; LVE) and extensive VE (> 10; EVE), respectively. The patients were followed-up by repeated endoscopic examinations after the therapy, and the incidences of VE recurrence and re-bleeding from the lesions were evaluated.
RESULTS: Although the VE lesions disappeared initially in all the patients after the therapy, the recurrence of VE developed in 25 patients (66%) over a mid-term observation period of 32 mo, and re-bleeding occurred in 15 patients (39%). The recurrence of VE was found in all patients with EVE, with re-bleeding occurring in 14 patients (58%). In contrast, only 1 patient (7%) with LVE showed recurrence of the lesions and complicating hemorrhage. Both the cumulative recurrence-free rates and cumulative re-bleeding-free rates were significantly lower in the EVE group than in the LVE group (P < 0.001 and P < 0.001, respectively). Moreover, the cumulative re-bleeding-free rate in the EVE group was 47.6% at 1 year and 25.4% at 2 years in patients with chronic renal failure, which were significantly lower than the rates in the patients without chronic renal failure (83.3% and 74.1%, respectively) (P < 0.05).
CONCLUSION: The recurrence of VE and re-bleeding from the lesions was more frequent in the patients with EVE, especially in those with complicating renal failure.
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Fuccio L, Mussetto A, Laterza L, Eusebi LH, Bazzoli F. Diagnosis and management of gastric antral vascular ectasia. World J Gastrointest Endosc 2013; 5:6-13. [PMID: 23330048 PMCID: PMC3547119 DOI: 10.4253/wjge.v5.i1.6] [Citation(s) in RCA: 74] [Impact Index Per Article: 6.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/05/2012] [Revised: 09/26/2012] [Accepted: 12/01/2012] [Indexed: 02/05/2023] Open
Abstract
Gastric antral vascular ectasia (GAVE) is an uncommon but often severe cause of upper gastrointestinal (GI) bleeding, responsible of about 4% of non-variceal upper GI haemorrhage. The diagnosis is mainly based on endoscopic pattern and, for uncertain cases, on histology. GAVE is characterized by a pathognomonic endoscopic pattern, mainly represented by red spots either organized in stripes radially departing from pylorus, defined as watermelon stomach, or arranged in a diffused-way, the so called honeycomb stomach. The histological pattern, although not pathognomonic, is characterized by four alterations: vascular ectasia of mucosal capillaries, focal thrombosis, spindle cell proliferation and fibrohyalinosis, which consist of homogeneous substance around the ectatic capillaries of the lamina propria. The main differential diagnosis is with Portal Hypertensive Gastropathy, that can frequently co-exists, since about 30% of patients with GAVE co-present a liver cirrhosis. Autoimmune disorders, mainly represented by Reynaud’s phenomenon and sclerodactyly, are co-present in about 60% of patients with GAVE; other autoimmune and connective tissue disorders are occasionally reported such as Sjogren’s syndrome, systemic lupus erythematosus, primary biliary cirrhosis and systemic sclerosis. In the remaining cases, GAVE syndrome has been described in patients with chronic renal failure, bone marrow transplantation and cardiac diseases. The pathogenesis of GAVE is still obscure and many hypotheses have been proposed such as mechanical stress, humoural and autoimmune factors and hemodynamic alterations. In the last two decades, many therapeutic options have been proposed including surgical, endoscopic and medical choices. Medical therapy has not clearly shown satisfactory results and surgery should only be considered for refractory severe cases, since this approach has significant mortality and morbidity risks, especially in the setting of portal hypertension and liver cirrhosis. Endoscopic therapy, particularly treatment with Argon Plasma Coagulation, has shown to be as effective and also safer than surgery, and should be considered the first-line treatment for patients with GAVE-related bleeding.
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Affiliation(s)
- Lorenzo Fuccio
- Lorenzo Fuccio, Liboria Laterza, Leonardo Henry Eusebi, Franco Bazzoli, Department of Clinical Medicine, S.Orsola-Malpighi University Hospital, 40138 Bologna, Italy
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Bollinger E, Raines D, Saitta P. Distribution of bleeding gastrointestinal angioectasias in a Western population. World J Gastroenterol 2012; 18:6235-6239. [PMID: 23180943 PMCID: PMC3501771 DOI: 10.3748/wjg.v18.i43.6235] [Citation(s) in RCA: 58] [Impact Index Per Article: 4.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
AIM: To define which segments of the gastrointestinal tract are most likely to yield angioectasias for ablative therapy.
METHODS: A retrospective chart review was performed for patients treated in the Louisiana State University Health Sciences Center Gastroenterology clinics between the dates of July 1, 2007 and October 1, 2010. The selection of cases for review was initiated by use of our electronic medical record to identify all patients with a diagnosis of angioectasia, angiodysplasia, or arteriovenous malformation. Of these cases, chart reviews identified patients who had a complete evaluation of their gastrointestinal tract as defined by at least one upper endoscopy, colonoscopy and small bowel capsule endoscopy within the past three years. Patients without evidence of overt gastrointestinal bleeding or iron deficiency anemia associated with intestinal angioectasias were classified as asymptomatic and excluded from this analysis. Thirty-five patients with confirmed, bleeding intestinal angioectasias who had undergone complete endoscopic evaluation of the gastrointestinal tract were included in the final analysis.
RESULTS: A total of 127 cases were reviewed. Sixty-six were excluded during subsequent screening due to lack of complete small bowel evaluation and/or lack of documentation of overt bleeding or iron deficiency anemia. The 61 remaining cases were carefully examined with independent review of endoscopic images as well as complete capsule endoscopy videos. This analysis excluded 26 additional cases due to insufficient records/images for review, incomplete capsule examination, poor capsule visualization or lack of confirmation of typical angioectasias by the principal investigator on independent review. Thirty-five cases met criteria for final analysis. All study patients were age 50 years or older and 13 patients (37.1%) had chronic kidney disease stage 3 or higher. Twenty of 35 patients were taking aspirin (81 mg or 325 mg), clopidogrel, and/or warfarin, with 8/20 on combination therapy. The number and location of angioectasis was documented for each case. Lesions were then classified into the following segments of the gastrointestinal tract: esophagus, stomach, duodenum, jejunum, ileum, right colon and left colon. The location of lesions within the small bowel observed by capsule endoscopy was generally defined by percentage of total small bowel transit time with times of 0%-9%, 10%-39%, and 40%-100% corresponding to the duodenum, jejunum and ileum, respectively. Independent review of complete capsule studies allowed for deviation from this guideline if capsule passage was delayed in one or more segments. In addition, the location and number of angioectasias observed in the small bowel was further modified or confirmed by subsequent device-assisted enteroscopy (DAE) performed in the 83% of cases. In our study population, angioectasias were most commonly found in the jejunum (80%) followed by the duodenum (51%), stomach (22.8%), and right colon (11.4%). Only two patients were found to have angioectasias in the ileum (5.7%). Twenty-one patients (60%) had angioectasias in more than one location.
CONCLUSION: Patients being considered for endoscopic ablation of symptomatic angioectasias should undergo push enteroscopy or anterograde DAE and re-inspection of the right colon.
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Chiu YC, Lu LS, Wu KL, Tam W, Hu ML, Tai WC, Chiu KW, Chuah SK. Comparison of argon plasma coagulation in management of upper gastrointestinal angiodysplasia and gastric antral vascular ectasia hemorrhage. BMC Gastroenterol 2012; 12:67. [PMID: 22681987 PMCID: PMC3439282 DOI: 10.1186/1471-230x-12-67] [Citation(s) in RCA: 33] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/17/2011] [Accepted: 06/09/2012] [Indexed: 01/10/2023] Open
Abstract
BACKGROUND Vascular ectasias, including gastric antral vascular ectasia (GAVE) and angiodysplasia, are increasingly recognized as important sources of gastrointestinal bleeding. This study investigated and compared the efficacies and outcomes of treatment of upper gastrointestinal (UGI) angiodysplasia and GAVE hemorrhage by endoscopic argon plasma coagulation (APC). METHODS From January 2006 to December 2009, 46 patients diagnosed with upper GI bleeding caused by angiodysplasia or GAVE at a tertiary hospital were recruited into this study. They included 26 males and 20 females with an average age of 65.6 years (range, 45-90 years). All patients underwent APC for hemostasis during an endoscopic procedure. Parameters such as underlying co-morbidities, number of endoscopic treatment sessions, recurrent bleeding, and clinical outcomes during follow-up were analyzed. RESULTS The 46 patients with UGI vascular ectasia hemorrhage included 27 patients with angiodysplasia and 19 with GAVE. The patients with angiodysplasia were older than those with GAVE (71.6 ± 10.2 years versus 61.8 ± 11.9 years, P = 0.005). More GAVE patients than angiodysplasia patients had co-existing liver cirrhosis (63.2% versus 25.9%, P = 0.012). The patients with GAVE had a higher rate of recurrent bleeding (78.9% versus 7.4%, P < 0.001) and required more treatment sessions to achieve complete hemostasis (2.4 ± 1.4 versus 1.1 ± 0.1, P < 0.001) than those with angiodysplasia. Univariate analysis demonstrated that age greater than 60 years (odds ratio (OR) = 8.929, P = 0.003), GAVE (OR = 0.021, P < 0.001), and previous radiation therapy (OR = 11.667, P = 0.032) were associated with higher rates of recurrent bleeding. Further multivariate analysis revealed that GAVE was the only independent risk factor for recurrent bleeding after APC treatment (OR = 0.027, P < 0.001). CONCLUSION Endoscopic hemostasis with APC is a safe treatment modality for both angiodysplasia and vascular ectasia bleeding. The efficacy of APC treatment is greater for angiodysplasia than for vascular ectasia bleeding. GAVE patients have a higher recurrent bleeding rate and may require multiple treatment sessions for sustained hemostasis.
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Affiliation(s)
- Yi-Chun Chiu
- Division of Hepato-Gastroenterology, Department of Internal Medicine, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, 123, Ta-Pei Road, Niao-Sung District, Kaohsiung City, 833, Taiwan
| | - Lung-Sheng Lu
- Division of Hepato-Gastroenterology, Department of Internal Medicine, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, 123, Ta-Pei Road, Niao-Sung District, Kaohsiung City, 833, Taiwan
| | - Keng-Liang Wu
- Division of Hepato-Gastroenterology, Department of Internal Medicine, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, 123, Ta-Pei Road, Niao-Sung District, Kaohsiung City, 833, Taiwan
| | - William Tam
- Department of Gastroenterology and Hepatology, Royal Adelaide Hospital, Adelaide, South Australia
| | - Ming-Luen Hu
- Division of Hepato-Gastroenterology, Department of Internal Medicine, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, 123, Ta-Pei Road, Niao-Sung District, Kaohsiung City, 833, Taiwan
| | - Wei-Chen Tai
- Division of Hepato-Gastroenterology, Department of Internal Medicine, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, 123, Ta-Pei Road, Niao-Sung District, Kaohsiung City, 833, Taiwan
| | - King-Wah Chiu
- Division of Hepato-Gastroenterology, Department of Internal Medicine, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, 123, Ta-Pei Road, Niao-Sung District, Kaohsiung City, 833, Taiwan
| | - Seng-Kee Chuah
- Division of Hepato-Gastroenterology, Department of Internal Medicine, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, 123, Ta-Pei Road, Niao-Sung District, Kaohsiung City, 833, Taiwan
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Brown C, Subramanian V, Wilcox CM, Peter S. Somatostatin analogues in the treatment of recurrent bleeding from gastrointestinal vascular malformations: an overview and systematic review of prospective observational studies. Dig Dis Sci 2010; 55:2129-34. [PMID: 20393879 DOI: 10.1007/s10620-010-1193-6] [Citation(s) in RCA: 87] [Impact Index Per Article: 5.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/22/2009] [Accepted: 03/07/2010] [Indexed: 12/11/2022]
Abstract
BACKGROUND Vascular malformation of the gastrointestinal tract is an uncommon cause for gastrointestinal bleeding. Traditionally, gastroenterologists prefer to use endoscopic modalities like argon plasma coagulation and electrocoagulation to treat accessible lesions. The role of somatostatin analogues (octreotide) in preventing recurrent bleeding in these patients is unclear. The use of pharmacological treatments would be useful especially in refractory bleeding, inaccessible lesions and in patients who are at high risk for invasive interventions. AIMS To systematically review pooled clinical response rates from prospective studies using somatostatin analogues for prevention of recurrent bleeding from gastrointestinal angiodysplasia and quantify the effects that therapy has on the use of blood transfusions. METHODS We searched several electronic databases including Pubmed for full journal articles published after 1966 reporting on the use of somatostatin analogues in the treatment of gastrointestinal angiodyplasia. We hand searched the reference lists of all retrieved articles. Prospective studies involving ten or more patients were included in the analysis. We calculated the pooled proportion of patients who had a clinical response to therapy in the selected studies and the weighted mean difference in transfusion requirements before and after therapy. Heterogeneity between the studies was assessed using the I2 statistic. RESULTS A total of three studies involving 62 patients met the inclusion criteria. The proportional meta-analysis showed a clinical response to treatment of 0.76 (95% CI 0.64-0.85). The weighted mean difference in transfusion requirements before starting therapy (control group) and after treatment initiation (treatment group) was -2.2 (95% CI -3.9 to -0.5). No significant heterogeneity was seen between the studies. CONCLUSIONS A significant number of patients with bleeding gastrointestinal angiodysplasia respond to treatment with octreotide by reducing the need for blood products. As all the included studies had small sample sizes, multicenter randomized trials are needed to confirm these findings. However, it seems reasonable to administer octreotide especially in patients with refractory bleeding, inaccessible lesions and in patients at high risk for other interventions.
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Affiliation(s)
- Colin Brown
- Division of Gastroenterology and Hepatology, University of Alabama, Birmingham, AL, USA
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Dupuis-Girod S, Bailly S, Plauchu H. Hereditary hemorrhagic telangiectasia: from molecular biology to patient care. J Thromb Haemost 2010; 8:1447-56. [PMID: 20345718 DOI: 10.1111/j.1538-7836.2010.03860.x] [Citation(s) in RCA: 102] [Impact Index Per Article: 6.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/01/2023]
Abstract
SUMMARY Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disorder characterized by severe and recurrent nosebleeds, mucocutaneous telangiectases, and, in some cases, life-threatening visceral arteriovenous malformations of various types, including pulmonary, hepatic, cerebral, and spinal. Gastrointestinal telangiectases are frequent and may cause severe bleeding. HHT type 1 results from mutations in ENG on chromosome 9 (coding for endoglin), and HHT type 2 results from mutations in ACVRL1 on chromosome 12 (coding for activin receptor-like kinase 1). Mutations of either of these two genes account for most clinical cases. In addition, mutations in MADH4 (encoding SMAD4), which cause a juvenile polyposis/HHT overlap syndrome, have been described, and recently, an HHT3 locus on chromosome 5 (5q31.3-5q32) has been reported. The mutated genes in HHT encode proteins that modulate transforming growth factor-beta superfamily signaling in vascular endothelial cells. Management of patients has changed considerably in the last 20 years, in terms of both treatment and the prevention of complications. The goal of this review was to describe the underlying molecular and cellular physiopathology, explore clinical and genetic diagnostic strategies for HHT, and present clinical management recommendations in order to treat symptomatic disease and to screen for vascular malformations.
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Affiliation(s)
- S Dupuis-Girod
- Service de Génétique et Centre de Référence National Maladies Rares, maladie de Rendu-Osler, Hospices Civils de Lyon, Hôpital de l'Hôtel Dieu, Lyon, France.
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Grève E, Moussata D, Gaudin JL, Lapalus MG, Giraud S, Dupuis-Girod S, Calender A, Plauchu H, Saurin JC. High diagnostic and clinical impact of small-bowel capsule endoscopy in patients with hereditary hemorrhagic telangiectasia with overt digestive bleeding and/or severe anemia. Gastrointest Endosc 2010; 71:760-7. [PMID: 20170910 DOI: 10.1016/j.gie.2009.11.004] [Citation(s) in RCA: 22] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/17/2008] [Accepted: 11/02/2009] [Indexed: 02/08/2023]
Abstract
BACKGROUND Patients with hereditary hemorrhagic telangiectasia (HHT) often present with recurrent anemia because of epistaxis or GI bleeding in relation to telangiectases mostly located in the stomach or small bowel. Capsule endoscopy is considered a major diagnostic tool for small-bowel diseases, but the impact of capsule endoscopy imaging on patient management in HHT is poorly understood. OBJECTIVE To clarify the contribution of capsule endoscopy in selected patients with HHT. DESIGN Prospective, descriptive study. SETTING Multicenter, two university hospital tertiary-care centers, from January 2003 to June 2007. PATIENTS This study involved 30 patients with HHT and severe anemia (hemoglobin <9 g/dL; normal: 11-15 g/dL) and minimal epistaxis or moderate anemia but overt GI bleeding. INTERVENTION Capsule endoscopy investigation. MAIN OUTCOME MEASUREMENTS Clinical characteristics and capsule endoscopy results and their clinical consequences. RESULTS Capsule endoscopy detected gastric and small-bowel telangiectases in 14 (46.7%) and 26 (86.7%) cases, respectively. Active bleeding was present in 36.7% of cases. Diffuse telangiectases were detected in 42.3% without correlation with age, sex, or type of HHT mutation. Further investigations were carried out as a consequence of the capsule endoscopy results in 67% of cases. Treatment, consisting mostly of endoscopic argon plasma coagulation, was scheduled in 46.7% of patients. LIMITATIONS Our population was essentially composed of patients with the ALK1 mutation. CONCLUSION This study shows that there is a high diagnostic yield for capsule endoscopy in selected patients with HHT. Capsule endoscopy makes possible precise mapping of lesions and has a considerable impact on the management of these selected patients by using a predefined algorithm: a limited number of accessible lesions is suitable for endoscopic treatment, whereas innumerable diffuse lesions require a medical approach. We suggest that capsule endoscopy could be a first-line, noninvasive, digestive tract examination in selected patients with HHT.
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Affiliation(s)
- Emilie Grève
- Service de gastroentérologie et d'hépatologie, Centre Hospitalier Universitaire de Saint-Etienne, Hôpital Nord, Saint-Etienne, France
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Abstract
BACKGROUND AND AIMS Recurrent bleeding from gastrointestinal (GI) angiodysplasia remains a therapeutic challenge. Identification of factors predicting poor outcome of haemorrhage from angiodysplasia would help us to select the patients who may likely benefit from further therapy. Thus, we analysed risk factors for recurrence of acute GI haemorrhage from angiodysplasia. PATIENTS AND METHODS 62 patients admitted consecutively with acute GI bleeding from angiodysplasia, between June 2002 and June 2006, were included. Bivariate, multivariate and survival analysis were performed to identify risk factors for recurrence of bleeding after hospital discharge. RESULTS Recurrence of acute haemorrhage after hospital discharge occurred in 17 of 57 (30%) patients (38 men; mean age: 74+/-6 years), after a mean follow-up (33+/-40 months). On Cox analysis, earlier history of bleeding with a high bleeding rate, over anticoagulation and the presence of multiple lesions were predictive factors of recurrence in a multivariate analysis. In contrast, endoscopic argon plasma coagulation (APC) therapy was not associated with lower rates of recurrent bleeding. CONCLUSION In patients with acute GI haemorrhage from angiodysplasia, earlier bleeding with a high bleeding rate, over anticoagulation and multiple angiodisplasic lesions predict an increased risk of recurrent bleeding. Although there is a trend towards better management with endoscopic APC therapy for the prevention of recurrence of bleeding, endoscopic APC therapy is not predictive of a lower rate of recurrence.
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Laparoscopic Surgical Treatment for Refractory Gastric Antral Vascular Ectasia. Surg Laparosc Endosc Percutan Tech 2009; 19:e189-93. [DOI: 10.1097/sle.0b013e3181bb5a19] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
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Forbes A, Marie I. Gastrointestinal complications: the most frequent internal complications of systemic sclerosis. Rheumatology (Oxford) 2009; 48 Suppl 3:iii36-9. [PMID: 19487222 DOI: 10.1093/rheumatology/ken485] [Citation(s) in RCA: 73] [Impact Index Per Article: 4.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/11/2022] Open
Abstract
Manifestations of SSc in the gastrointestinal (GI) tract are common, occurring in 50-90% of patients. They typically result from the fibrosis that characterizes this disease. Manifestations of SSc can affect many sites within the GI tract, and patients may experience substantial dysfunction in the processes of motility, digestion, absorption and excretion. Oesophageal dysfunction is the most common GI manifestation, but patients may also experience dysfunction of the stomach, small intestine, colon and rectum, each of which can be responsible for severe and distressing symptoms. At present, few specific therapeutic options are available for the treatment of these patients, but relief of symptoms is often possible with appropriate knowledge and support. It is therefore particularly important to identify, monitor and manage these patients carefully, with a view to minimizing further degeneration and maximizing quality of life.
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Affiliation(s)
- A Forbes
- Department of Gastroenterology and Nutrition, University College Hospital, London, UK.
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30
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Adler DG, Chand B, Conway JD, Diehl DL, Kantsevoy SV, Kwon RS, Mamula P, Shah RJ, Wong Kee Song LM, Tierney WM. Mucosal ablation devices. Gastrointest Endosc 2008; 68:1031-42. [PMID: 19028211 DOI: 10.1016/j.gie.2008.06.018] [Citation(s) in RCA: 30] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/17/2008] [Accepted: 06/17/2008] [Indexed: 02/08/2023]
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31
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Cerra Pohl A, Alfred Werner J, Josef Folz B. Exploración multiorgánica de los pacientes con telangiectasia hemorrágica hereditaria (enfermedad de Osler). ACTA OTORRINOLARINGOLOGICA ESPANOLA 2008. [DOI: 10.1016/s0001-6519(08)75119-7] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/22/2022]
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32
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Lecleire S, Ben-Soussan E, Antonietti M, Goria O, Riachi G, Lerebours E, Ducrotté P. Bleeding gastric vascular ectasia treated by argon plasma coagulation: a comparison between patients with and without cirrhosis. Gastrointest Endosc 2008; 67:219-25. [PMID: 18226684 DOI: 10.1016/j.gie.2007.10.016] [Citation(s) in RCA: 53] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/04/2007] [Accepted: 10/02/2007] [Indexed: 02/07/2023]
Abstract
BACKGROUND Gastric vascular ectasia (GVE) is an uncommon etiology of GI bleeding. GVE can affect not only patients with cirrhosis but also patients with a variety of chronic diseases. OBJECTIVE The aim of the study was to compare clinical and endoscopic patient characteristics and responses to treatment by argon plasma coagulation (APC) of bleeding GVE between patients with cirrhosis and noncirrhotic patients. DESIGN Retrospective study of consecutive patients. PATIENTS Between January 2001 and December 2005, 30 patients were treated by APC for bleeding GVE. INTERVENTIONS Clinical and endoscopic features and APC treatment success were compared between patients with cirrhosis (group 1) and noncirrhotic patients (group 2). MAIN OUTCOME MEASUREMENTS Endoscopic treatment efficacy was assessed on the recurrence of symptoms after APC. RESULTS Seventeen patients were cirrhotic and 13 had no cirrhosis. Cirrhotic patients presented more frequently with overt bleeding (65% vs 15%) and noncirrhotic patients with occult bleeding with iron deficiency anemia (35% vs 85%, P= .01). Endoscopy in noncirrhotic patients revealed more frequently a "watermelon" appearance (23.5% vs 76.9%, P= .008). Endoscopic treatment by APC was successful in 83.3% of patients (88.2% vs 76.9%, not significant). Patients from group 2 required significantly more APC sessions to achieve a complete treatment (2.18 vs 3.77, P= .04). CONCLUSIONS APC treatment of bleeding GVE was efficient and safe in cirrhotic and noncirrhotic patients in more than 80% of cases. Noncirrhotic patients required significantly more APC sessions to achieve a complete treatment. An endoscopic watermelon appearance and the use of antiplatelet drugs were associated with failure of APC.
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Affiliation(s)
- Stéphane Lecleire
- Department of Hepato-Gastroenterology and Nutrition, Endoscopy Unit, ADEN-EA3234/IFRMP23 Research Group, Rouen University Hospital Charles-Nicolle, Rouen, France
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Screening for Systemic Manifestations of Vascular Malformations in Patients With Hereditary Haemorrhagic Telangiectasia (Osler Disease). ACTA OTORRINOLARINGOLOGICA ESPANOLA 2008. [DOI: 10.1016/s2173-5735(08)70273-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022]
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YAMADA M, ICHIKAWA M, TAKAHARA O, TSUCHIDA T, ISHIHARA A, YAMADA M, NISHIMURA D, HOSHINO H, KATADA N, KATO K. Gastroduodenal Vascular Ectasia in Patients with Liver Cirrhosis. Dig Endosc 2007. [DOI: 10.1111/j.1443-1661.1999.tb00010.x] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/23/2023]
Affiliation(s)
| | | | - Osamu TAKAHARA
- *Department of Internal Medicine, Anjo Kosei Hospital, Aichi, Japan
| | - Takeshi TSUCHIDA
- *Department of Internal Medicine, Anjo Kosei Hospital, Aichi, Japan
| | - Akira ISHIHARA
- *Department of Internal Medicine, Anjo Kosei Hospital, Aichi, Japan
| | - Masahiro YAMADA
- *Department of Internal Medicine, Anjo Kosei Hospital, Aichi, Japan
| | | | - Hiroshi HOSHINO
- *Department of Internal Medicine, Anjo Kosei Hospital, Aichi, Japan
| | - Naoyuki KATADA
- *Department of Internal Medicine, Anjo Kosei Hospital, Aichi, Japan
| | - Katsumoto KATO
- **Department of Internal Medicine, Kamo Hospital, Aichi, Japan
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35
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SHIRAI M, JARAMILLO E, BEFRITS R, JONAS E, LJUNG T, SLEZAK P. The Endoscopic Detection and Treatment of Vascular Ectasia in the Gastrointestinal Tract —A One Year Experience at Karolinska Hospital—. Dig Endosc 2007. [DOI: 10.1111/j.1443-1661.1998.tb00560.x] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/23/2023]
Affiliation(s)
- Masato SHIRAI
- Endoscopy Unit, Department of Gastroenterology and Hepatoloygy, Division of Internal Medicine, Karolinska Hospital, Stockholm, Sweden
| | - Edgar JARAMILLO
- Endoscopy Unit, Department of Gastroenterology and Hepatoloygy, Division of Internal Medicine, Karolinska Hospital, Stockholm, Sweden
| | - Ragnar BEFRITS
- Endoscopy Unit, Department of Gastroenterology and Hepatoloygy, Division of Internal Medicine, Karolinska Hospital, Stockholm, Sweden
| | - Eduard JONAS
- Endoscopy Unit, Department of Gastroenterology and Hepatoloygy, Division of Internal Medicine, Karolinska Hospital, Stockholm, Sweden
| | - Tryggve LJUNG
- Endoscopy Unit, Department of Gastroenterology and Hepatoloygy, Division of Internal Medicine, Karolinska Hospital, Stockholm, Sweden
| | - Premysl SLEZAK
- Endoscopy Unit, Department of Gastroenterology and Hepatoloygy, Division of Internal Medicine, Karolinska Hospital, Stockholm, Sweden
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36
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IKEDA M, INOKI K, HAYASHI N, YAMAMOTO K, MASUZAWA M, TAKEDA M. Successful Endoscopic Treatment of Diffuse Antral Vascular Ectasia and Early Gastric Carcinoma in an Aged Patient. Dig Endosc 2007. [DOI: 10.1111/j.1443-1661.1995.tb00131.x] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/23/2023]
Affiliation(s)
- Masahiro IKEDA
- *Department of Gastroenterology, Osaka National Hospital, Osaka, Japan
| | - Ken INOKI
- *Department of Gastroenterology, Osaka National Hospital, Osaka, Japan
| | - Nobuhiko HAYASHI
- *Department of Gastroenterology, Osaka National Hospital, Osaka, Japan
| | - Keiji YAMAMOTO
- *Department of Gastroenterology, Osaka National Hospital, Osaka, Japan
| | - Manabu MASUZAWA
- *Department of Gastroenterology, Osaka National Hospital, Osaka, Japan
| | - Masashi TAKEDA
- **Department of Clinical Laboratory, Osaka National Hospital, Osaka, Japan
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37
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Farooq FT, Wong RCK, Yang P, Post AB. Gastric outlet obstruction as a complication of argon plasma coagulation for watermelon stomach. Gastrointest Endosc 2007; 65:1090-2. [PMID: 17451706 DOI: 10.1016/j.gie.2006.10.006] [Citation(s) in RCA: 24] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/28/2006] [Accepted: 10/05/2006] [Indexed: 02/08/2023]
Affiliation(s)
- Farees T Farooq
- Division of Gastroenterology, Department of Medicine, University Hospitals Case Medical Center, Cleveland, Ohio 44106-5066, USA
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38
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van Tuyl SAC, Letteboer TGW, Rogge-Wolf C, Kuipers EJ, Snijder RJ, Westermann CJJ, Stolk MFJ. Assessment of intestinal vascular malformations in patients with hereditary hemorrhagic teleangiectasia and anemia. Eur J Gastroenterol Hepatol 2007; 19:153-8. [PMID: 17273001 DOI: 10.1097/01.meg.0000252633.88419.04] [Citation(s) in RCA: 17] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/22/2022]
Abstract
INTRODUCTION Hereditary hemorrhagic teleangiectasia (HHT) is an autosomal dominant disorder with mucocutaneous teleangiectasia and visceral arteriovenous malformations. Mutations of endoglin and Activin A receptor like kinase-1 have different phenotypes, HHT1 and HHT2, respectively. The gastrointestinal tract is frequently affected, but limited information is available on the relationship with genotype. AIM To determine whether different genotypes have different phenotypes with respect to intestinal teleangiectasia. METHODS HHT patients, referred for anemia, underwent videocapsule endoscopy. Chart review was performed for information on genotype and HHT manifestations. RESULTS Twenty-five patients were analyzed (men/women 13/9, mean age 49+/-15 years.), 14 HHT1, eight HHT2 and three without known mutation. Epistaxis occurred in 96% of patients. Gastroduodenoscopy revealed teleangiectasia in 7/12 (58%) HHT1 and 3/8 (38%) HHT2 patients. Videocapsule endoscopy found teleangiectasia in all HHT1 and 5/8 (63%) HHT2 patients. In 9/14 HHT1 patients, teleangiectasia were large. Teleangiectasia in the colon was restricted to 6/11 (55%) HHT1 patients. Hepatic arteriovenous malformations were present in 1/7 HHT1 and 5/6 HHT2 patients. CONCLUSION Large teleangiectasia in small intestine and colon appear to occur predominantly in HHT1. Hepatic arteriovenous malformations are mainly found in HHT2. In HHT patients with unexplained anemia, videocapsule endoscopy should be considered to determine the size and extent of teleangiectasia and exclude other abnormalities.
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39
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Shibukawa G, Irisawa A, Sakamoto N, Takagi T, Wakatsuki T, Imamura H, Takahashi Y, Sato A, Sato M, Hikichi T, Obara K, Ohira H. Gastric antral vascular ectasia (GAVE) associated with systemic sclerosis: relapse after endoscopic treatment by argon plasma coagulation. Intern Med 2007; 46:279-83. [PMID: 17379994 DOI: 10.2169/internalmedicine.46.6203] [Citation(s) in RCA: 28] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022] Open
Abstract
In this report, we present a case of gastric antral vascular ectasia (GAVE) associated with systemic sclerosis and interstitial pneumonitis. This case showed resistance to endoscopic treatment using argon plasma coagulation (APC). After initial recognition of GAVE as the origin of persistent anemia, three sessions of APC were performed and dilated vessels on the antrum were eliminated completely. Five months after primary treatment, follow-up endoscopy revealed deformity of the gastric antrum caused by ulcer scars induced by APC, with no vascular ectasia. Ten months later, the patient showed anemia and recurrence of GAVE on endoscopy. Ablation using APC was performed again, thereby eradicating recurrent GAVE completely. At a two months' follow-up, however, recurrent GAVE was indicated. In spite of GAVE eradication by APC, a third recurrence of GAVE was observed after 32 months. During the follow-up period, systemic sclerosis and interstitial pneumonitis were controlled clinically by administration of methyl prednisolone with no aggravation.
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Affiliation(s)
- Goro Shibukawa
- Department of Internal Medicine 2, Fukushima Medical University School of Medicine, Fukushima
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40
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Polese L, Angriman I, Pagano D, Tenderini ML, Polese F, Frego M, D'Amico DF, Norberto L. Laser therapy and surgical treatment in transfusion-dependent patients with upper-gastrointestinal vascular ectasia. Lasers Med Sci 2006; 21:140-6. [PMID: 16897110 DOI: 10.1007/s10103-006-0389-7] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/06/2006] [Revised: 05/09/2006] [Accepted: 06/09/2006] [Indexed: 12/11/2022]
Abstract
We report the results of endoscopic laser therapy on 59 patients with upper gastrointestinal vascular ectasia. After 300 sessions, complications were two nonbleeding and three bleeding ulcers, treated successfully with endoscopic therapy. After treatment, blood transfusions were abolished in 61% and reduced in 22% of the patients, whereas 17% of the patients were nonresponders and 5% died for bleeding. Treatment outcome correlated with endoscopic healing, number of yearly transfusions, and lesions' localization. Patients, who did not respond to laser therapy by the sixth session, showed no improvement afterwards. Four patients with persistence of bleeding despite laser therapy underwent surgery and in three of them, a long-term control of bleeding was obtained. In conclusion, laser therapy was safe and effective; nonetheless, surgical treatment should be considered, after adequate staging, for those patients receiving more than 10 blood units per year or who have undergone more than six laser sessions without improvement.
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Affiliation(s)
- Lino Polese
- Department of Surgical and Gastroenterological Sciences, First Surgical Clinic, University of Padova, Padova, Italy
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41
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Szilagyi A, Ghali MP. Pharmacological therapy of vascular malformations of the gastrointestinal tract. CANADIAN JOURNAL OF GASTROENTEROLOGY = JOURNAL CANADIEN DE GASTROENTEROLOGIE 2006; 20:171-8. [PMID: 16550261 PMCID: PMC2582970 DOI: 10.1155/2006/859435] [Citation(s) in RCA: 65] [Impact Index Per Article: 3.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/22/2022]
Abstract
Vascular malformation (AVM) in the gastrointestinal tract is an uncommon, but not rare, cause of bleeding and iron deficiency anemia, especially in an aging population. While endoscopic coagulative therapy is the method of choice for controlling bleeding, a substantial number of cases require additional therapy. Adjunctive or even primary phamacotherapy may be indicated in recurrent bleeding. However, there is little evidence-based proof of efficacy for any agent. The bulk of support is derived from anecdotal reports or case series. The present review compares the outcome of AVM after no intervention, coagulative therapy or focus on pharmacological agents. Most of the literature encompasses two common AVMs, angiodysplasia and hereditary hemorrhagic telangiectasia. Similarly, the bulk of information evaluates two therapies, hormones (estrogen and progesterone) and the somatostatin analogue octreotide. Of these, the former is the only therapy evaluated in randomized trials, and the results are conflicting without clear guidelines. The latter therapy has been reported only as case reports and case series without prospective trials. In addition, other anecdotally used medications are discussed.
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Affiliation(s)
- Andrew Szilagyi
- Division of Gastroenterology, Department of Medicine, Sir Mortimer B Davis Jewish General Hospital, McGill University School of Medicine, Montreal, Quebec.
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42
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Selinger RRE, McDonald GB, Hockenbery DM, Steinbach G, Kimmey MB. Efficacy of neodymium:YAG laser therapy for gastric antral vascular ectasia (GAVE) following hematopoietic cell transplant. Bone Marrow Transplant 2005; 37:191-7. [PMID: 16284614 DOI: 10.1038/sj.bmt.1705212] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/09/2022]
Abstract
We determined the incidence of severe bleeding from gastric antral vascular ectasia (GAVE) after myeloablative hematopoietic cell transplant and the outcomes after treatment with endoscopic neodymium:YAG laser photocoagulation. From 1992 to 2005, the incidence of severe bleeding from GAVE was 6/4491 (0.13%). All patients had received oral busulfan and four had sinusoidal obstruction syndrome. Gastrointestinal bleeding began a median of 53 days after transplant (range 15-2952). After GAVE was diagnosed by endoscopic and histologic findings, a median of three (range 2-7) sessions of laser therapy were required to control the bleeding with a median of 2737 J (range 1117-6160 J) per session. A median of 16 units (range 4-44) had been transfused prior to laser therapy and a median of four additional units (range 0-113) were transfused until bleeding was controlled. All patients were followed for at least 70 days after the last laser therapy session, with no further episodes of bleeding. Complications were mild and included abdominal pain and asymptomatic ulceration; however, one patient required gastrectomy due to gastric necrosis following transarterial embolizations. In summary, severe bleeding from GAVE is rare following hematopoietic cell transplant. Treatment with endoscopic therapy using the Nd:YAG laser is safe and effective.
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Affiliation(s)
- R R E Selinger
- Division of Gastroenterology, The University of Washington Medical Center, Seattle, WA 98195, USA
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43
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Pérez del Molino A, Zarrabeitia R, Fernández A. Telangiectasia hemorrágica hereditaria. Med Clin (Barc) 2005; 124:583-7. [PMID: 15860174 DOI: 10.1157/13074142] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/21/2022]
Abstract
Hereditary Hemorrhagic Telangiectasia (HHT) or Rendu-Osler-Weber disease is a genetic disorder with a dominant autosomic transmission. Its prevalence is estimated in one in 5-8,000 individuals. Two different mutations have been described involving endoglin and ALK-1 genes, resulting in HHT type 1 and 2 respectively. It is characterized by the occurrence of spontaneous and recurrent episodes of epistaxis, telangiectasias and the presence of visceral arteriovenous malformations (mainly affecting lungs, liver, brain and digestive tract) which are responsible for the clinical manifestations and constitute a basic point in the diagnostic criteria of Curaçao. The aim of this article is to review the pathogenesis, clinical aspects, screening procedures to disclose the visceral involvement and the therapeutic options of this rare disease.
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44
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Affiliation(s)
- Shou-jiang Tang
- Center for Therapeutic Endoscopy and Endoscopic Oncology, Division of Respiratory Medicine, Department of Medicine, St. Michael's Hospital, Toronto, Ontario, Canada M5B 1W8
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45
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Abstract
BACKGROUND Upper-GI vascular ectasias, including angiodysplasia and gastric antral vascular ectasia may present with either acute or chronic bleeding. Endoscopic thermal modalities have been used to control acute bleeding and reduce transfusion requirements. METHODS Endoscopic experience was reviewed for a 6-year period during which 32 patients requiring blood transfusions for upper-GI angiodysplasia or gastric antral vascular ectasia were evaluated. Patients seen during the first 5 years were treated with either Nd:YAG laser photocoagulation or multipolar electrocoagulation. During the most recent 12 months, all patients were treated by argon plasma coagulation. Response to therapy was assessed by change in mean Hb and transfusion requirements. RESULTS Overall, 16 patients were treated by laser photoablation alone; 9, argon plasma coagulation with or without laser; and 7, multipolar electrocoagulation with or without laser. Mean follow-up for all patients was 19 months. After therapy, mean Hb concentration rose from 76 to 114 g/L for patients with gastric antral vascular ectasia and from 85 to 118 g/L for those with angiodysplasia. Endoscopic therapy abolished or reduced transfusion requirements in 93% of patients with gastric antral vascular ectasia and 76% with angiodysplasia. Patients with gastric antral vascular ectasia required a mean of 6 treatment sessions, while those with angiodysplasia required one to two sessions. CONCLUSIONS Endoscopic thermal ablation effectively controls acute bleeding and reduces transfusion requirements in most patients with upper-GI vascular ectasias. Patients with gastric antral vascular ectasia require significantly more treatment sessions to achieve this effect.
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Affiliation(s)
- Darren A Pavey
- Department of Gastroenterology, St George Hospital, Sydney, Australia
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46
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Mathou NG, Lovat LB, Thorpe SM, Bown SG. Nd:YAG laser induces long-term remission in transfusion-dependent patients with watermelon stomach. Lasers Med Sci 2004; 18:213-8. [PMID: 15042426 DOI: 10.1007/s10103-003-0284-4] [Citation(s) in RCA: 25] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/25/2003] [Accepted: 11/21/2003] [Indexed: 01/16/2023]
Abstract
Watermelon stomach (gastric antral vascular ectasia) is a rare cause of gastric bleeding which can render patients transfusion-dependent. Laser therapy can be used to stop bleeding but the long-term success of this approach is not well described. We present a retrospective analysis of 24 consecutive transfusion-dependent patients who were treated in a national referral centre with Nd:YAG laser over an 18 year period. Laser therapy stopped all bleeding in 20 patients (83%) after a median of two sessions. Median follow up was 55 months (range 9-127). Patients remained transfusion free for a median of 16 months and a second course of treatment succeeded in all those who re-bled. One gastric perforation occurred early in the series and two patients developed pyloric stenosis which was successfully treated with balloon pyloric dilatation. Oestrogens were not used in these patients. Our experience shows that long-term remission from blood transfusion is seen in most patients treated with Nd:YAG laser. If bleeding recurs, further laser treatment is usually successful.
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Affiliation(s)
- N G Mathou
- National Medical Laser Centre, Department of Surgery, Royal Free and University College Medical School, UCL, 67-73 Riding House Street, W1W 7EJ, London, UK
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47
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Sebastian S, O'Morain CA, Buckley MJM. Review article: current therapeutic options for gastric antral vascular ectasia. Aliment Pharmacol Ther 2003; 18:157-165. [PMID: 12869075 DOI: 10.1046/j.1365-2036.2003.01617.x] [Citation(s) in RCA: 97] [Impact Index Per Article: 4.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/15/2022]
Abstract
Gastric antral vascular ectasia, or 'watermelon stomach', is a rare but important cause of gastrointestinal blood loss and anaemia, which has characteristic endoscopic and histological features. The pathogenesis of this condition remains unclear; however, many associated disorders have been documented. Various medical, surgical and endoscopic therapeutic modalities have been attempted with variable success. Leading contenders for the therapeutic modality of choice include hormonal therapy, endoscopic Nd:YAG laser and argon plasma coagulation. Randomized controlled trials to identify the ideal treatment method are lacking at present.
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Affiliation(s)
- S Sebastian
- Department of Gastroenterology, Adelaide and Meath Hospital, Trinity College, Dublin, Ireland
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Novitsky YW, Kercher KW, Czerniach DR, Litwin DEM. Watermelon stomach: pathophysiology, diagnosis, and management. J Gastrointest Surg 2003; 7:652-61. [PMID: 12850679 DOI: 10.1016/s1091-255x(02)00435-3] [Citation(s) in RCA: 73] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/31/2023]
Abstract
Watermelon Stomach (WS) has been increasingly recognized as an important cause of occult gastrointestinal blood loss. Clinically, patients develop significant iron deficiency anemia and are frequently transfusion dependent. The histologic hallmark of WS is superficial fibromuscular hyperplasia of gastric antral mucosa with capillary ectasia and microvascular thrombosis in the lamina propria. Endoscopic findings of the longitudinal antral folds containing visible columns of tortuous red ectatic vessels (watermelon stripes) are pathognomonic for WS. Trauma to the mucosal epithelium overlying engorged vessels by gastric acid or intraluminal food results in bleeding. Treatment options for WS include endoscopic, pharmacologic, and surgical approaches. Endoscopic therapy, including contact and non-contact thermal ablations of the angiodysplastic lesions, is the mainstay of conservative therapy. However, many patients fail endoscopic therapy and develop recurrent acute and chronic GI bleeding episodes. Surgical resection may be the only reliable method for achieving a cure and eliminating transfusion dependency. Traditionally, surgery was used only as a last resort after patients failed prolonged medical and/or endoscopic therapy. However, based on the experience garnered from the literature we recommend a more aggressive surgical approach in patients who fail a short trial of endoluminal therapy.
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Affiliation(s)
- Yuri W Novitsky
- Department of Surgery, University of Massachusetts Medical School, Worcester, MA 01655, USA.
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Begbie ME, Wallace GMF, Shovlin CL. Hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu syndrome): a view from the 21st century. Postgrad Med J 2003; 79:18-24. [PMID: 12566546 PMCID: PMC1742589 DOI: 10.1136/pmj.79.927.18] [Citation(s) in RCA: 193] [Impact Index Per Article: 8.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/24/2023]
Abstract
Hereditary haemorrhagic telangiectasia (HHT) affects one in 5-8000, and no longer can be viewed as solely causing anaemia (due to nasal and gastrointestinal bleeding) and characteristic mucocutaneous telangiectasia. Arteriovenous malformations commonly occur, and in the pulmonary and cerebral circulations demand knowledge of risks and benefits of asymptomatic screening and treatment. HHT is inherited as an autosomal dominant trait and there is no age cut off when apparently unaffected offspring of an individual with HHT can be told they are unaffected. This review focuses on the evolving evidence base for HHT management, issues regarding pregnancy and prothrombotic treatments, and discusses the molecular and cellular changes that underlie this disease.
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Affiliation(s)
- M E Begbie
- Respiratory Medicine, National Heart and Lung Institute, Imperial College Faculty of Medicine, Hammersmith Hospital, London, UK
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Longacre AV, Gross CP, Gallitelli M, Henderson KJ, White RI, Proctor DD. Diagnosis and management of gastrointestinal bleeding in patients with hereditary hemorrhagic telangiectasia. Am J Gastroenterol 2003; 98:59-65. [PMID: 12526937 DOI: 10.1111/j.1572-0241.2003.07185.x] [Citation(s) in RCA: 67] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
OBJECTIVE Our aim was to report our experience with treating GI bleeding in patients with hereditary hemorrhagic telangiectasia (HHT). METHODS Consecutive patients with GI bleeding referred to the Yale University Vascular Malformation Center underwent clinical evaluation and endoscopy. Hb and blood transfusion requirements for 1 yr before and after evaluation were documented. Patients with a mean Hb <or= 8 mg/dl or blood transfusion requirements >or= 12 units packed red blood cells (PRBC)/yr were defined as patients with significant bleeding. Drug therapies, including ethinyl estradiol/norethindrone, danazol, and aminocaproic acid, were prescribed on an individual patient basis. RESULTS The study included 43 HHT patients with a mean age of 57 yr. Endoscopy revealed telangiectases in the esophagus (1/41), stomach (33/41), duodenum (33/41), jejunum (5/9), and colon (10/32). Patients with > 20 telangiectases visualized on esophagogastroduodenoscopy had a significantly lower mean Hb of 7.9, compared with 9.4 (p = 0.007), and a trend toward higher blood transfusion requirements. Non-HHT-related causes of GI bleeding were diagnosed in four patients. During a mean follow up of 18.9 months, the group of 40 patients with HHT-related bleeding had improvements in their mean Hb and blood transfusion requirements. CONCLUSIONS Some HHT patients with GI bleeding improve on drug therapies, but others fail. Transfusion-dependent GI bleeding is difficult to manage, and optimal management may include both medical and endoscopic treatments.
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Affiliation(s)
- Anna V Longacre
- Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut 06520, USA
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