Ileo-caecal arterio-venous malformation associated with extrahepatic portal hypertension: a case report

Tatekawa, Yukihiro; Muraji, Toshihiro; Tsugawa, Chikara

doi:10.1007/s00383-005-1524-4

Cited by 7 publications

(5 citation statements)

References 13 publications

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“…Several reports have documented abnormal anatomy of the portal venous system in individuals with Down syndrome [2]. In another case report, a boy with Down syndrome was diagnosed with a combination of multiple vascular abnormalities, including extrahepatic portal hypertension, ileocecal AVMs, caval/iliac venous malformation, and rectal bleeding [10]. Only one published case of an adult with Down syndrome and pelvic AVM complicated by hematuria has been reported, resulting in death after embolization and cystectomy [2].…”

Section: Discussionmentioning

confidence: 99%

Treatment of Iliac Arteriovenous Malformation Associated with Trisomy 21: A Case Report

Yang,

Zhao,

Liang

et al. 2024

Preprint

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Both arteriovenous malformation (AVM) and trisomy 21 are rare diseases. Studies have shown that individuals with trisomy 21 may have potential vascular malformations, with the main site of onset being the portal system. This case involved an adult patient with trisomy 21 who presented with pelvic iliac arteriovenous malformations. The patient underwent interventional treatment, and the prognosis was favorable. It is important to enhance the screening for vascular malformations in patients with trisomy 21 syndrome. At the same time, interventional treatment for AVMs is effective and increasingly important for managing AVMs.

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Section: Discussionmentioning

confidence: 99%

Treatment of Iliac Arteriovenous Malformation Associated with Trisomy 21: A Case Report

Yang,

Zhao,

Liang

et al. 2024

Preprint

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“…1 There is a case report which describes a boy with trisomy 21 and a novel combination of multiple vascular anomalies: extrahepatic portal hypertension, an arteriovenous malformation at the ileocecal junction, and caval/iliac vein anomalies. 37 Among vascular abnormalities of hepatic portal system in trisomy 21 patients, those involving umbilical vein, splenic vein, hepatic vein, and mesenteric superior vein are reported rarely. 38 However, our case had trisomy 21 along with a rare vascular malformation APF.…”

Section: Discussionmentioning

confidence: 99%

Transarterial and Transhepatic Endovascular Intervention to Alleviate Portal Hypertension Secondary to Arterioportal Fistula in a Trisomy 21 Infant

Yazıcı

Çil

Bayrakçı

et al. 2017

J Pediatr Intensive Care

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Individuals with trisomy 21 have an 80% risk reduction of vascular anomalies compared with general population. However, an association of trisomy 21 and portal vascular and arteriovenous anomalies has been defined in the literature. The primary hemodynamic abnormality in portal hypertension is increased resistance to portal blood flow. In various case reports in adults and pediatric age group patients, transarterial coil embolization of hepatoportal fistula was described. One of the authors of this article has previously reported successful treatment of congenital arterioportal fistula (APF) with percutaneous transhepatic liquid embolization in a patient who previously had transarterial coil embolization. To date, eight patients with trisomy 21 (Down syndrome) and congenital portosystemic shunts were reported of which four were treated with embolization. Here, we describe a 3-month-old infant with trisomy 21 and intrahepatic APF associated with extrahepatic portal hypertension and massive ascites. In the current report, a rare case of a patient with a diagnosis of trisomy 21 is discussed who was attempted to be treated with transarterial coil embolization and percutaneous transhepatic liquid embolization of the congenital APF in a single session.

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“…Moreover, AVMs of the superior mesenteric artery and its branches to the ileum are exceedingly rare, with only sporadic case reports found in the review of the literature, and involving adults (4–6). In the pediatric literature we could equally find only a few case reports of AVM involving ileocecal segment (7), cecal segment (8), and right colon (9). In some cases, despite being severe, the site of gastrointestinal bleeding may be difficult to diagnose, but contemporary capsule endoscopy and double‐balloon enteroscopy have made the investigation of the small bowel easier and less invasive (2).…”

Section: Discussionmentioning

confidence: 99%

Diagnostic and Therapeutic Tricks in a Rare Case of Pediatric Ileal Congenital Arteriovenous Malformation

Silveri

Falappa²,

Casciani³

et al. 2010

J. pediatr. gastroenterol. nutr.

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C ongenital arteriovenous malformations (AVMs) are not usually considered initially in the diagnostic workup of a pediatric melena (1). After excluding conditions requiring nonoperative management, conventional endoscopic evaluations and radionuclide scans are, in most patients, able to define the site of bleeding and address the type of operative treatment. Furthermore, capsule endoscopy or double-balloon enteroscopy can provide additional information in doubtful cases, even of pediatric age (2). Nevertheless, there are some cases in which the diagnosis remains difficult, setting up an ''obscure melena'' requiring additional investigations. AVMs are rare in children, usually small, single, predominantly located in the rectum and descending colon, and relatively uncommon in the small bowel (3). An exceedingly rare case of congenital AVM arising from the ileal branch of the ileocolic artery in a baby girl is described as well as the original method of angiographic wire placement, as an intraoperative locating mark, to help surgical resection planning.

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Ileo-caecal arterio-venous malformation associated with extrahepatic portal hypertension: a case report

Cited by 7 publications

References 13 publications

Treatment of Iliac Arteriovenous Malformation Associated with Trisomy 21: A Case Report

Treatment of Iliac Arteriovenous Malformation Associated with Trisomy 21: A Case Report

Transarterial and Transhepatic Endovascular Intervention to Alleviate Portal Hypertension Secondary to Arterioportal Fistula in a Trisomy 21 Infant

Diagnostic and Therapeutic Tricks in a Rare Case of Pediatric Ileal Congenital Arteriovenous Malformation

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