Small bowel varices secondary to chronic superior mesenteric vein thrombosis in a patient with heterozygous Factor V Leiden mutation: a case report

Abstract

IntroductionBleeding ectopic small bowel varices pose a clinical dilemma for the physician, given their diagnostic obscurity and the lack of evidence-based medicine to guide therapy. They often occur in the context of portal hypertension, secondary to either liver disease or extrahepatic causes. Rarely is their presence associated with chronic superior mesenteric vein thrombosis and hereditary coagulopathies.Case presentationA 74-year-old white woman, with a heterozygous Factor V Leiden mutation and no underlying liver disease or portal hypertension, presented over the course of 13 months for recurrent episodes of melena and per rectal bleeding. An initial endoscopy showed a clean-based chronic gastric ulcer, while colonoscopies showed multiple, non-bleeding angioectasias which were treated with argon plasma coagulation. Subsequent video capsule endoscopy and double balloon enteroscopy revealed red wale marks overlying engorged submucosal veins in her distal ileum, consistent with ectopic varices. A chronic superior mesenteric vein thrombus, found via computed tomography venogram, was the cause of the ileal varices. She underwent curative surgical resection of the affected bowel, with no re-bleeding episodes 17 months post-surgery, despite needing lifelong anticoagulation for recurrent venous thromboembolisms.ConclusionsClinicians should consider ectopic varices in patients who present with obscure gastrointestinal bleeding, even in the absence of portal hypertension or liver disease. In those with a known thrombophilia, patients should be screened for splanchnic thrombosis, which may precipitate ectopic varices.

Jejunal Varices Bleeding in a Patient with Extensive Portomesenteric Thrombosis Secondary to Factor V Leiden Mutation: A Management Dilemma

“…Glue injection was deemed high risk and difficult due to the presence of multiple large jejunal varices that will need high volume injection, so decision was to go for surgical resection of the affected small bowel segment after a multidisciplinary team meeting. Good evidence that surgery carried a very low recurrence rate was previously reported in a patient with a very similar presentation to our patient [6].…”

“…Chronic portomesenteric thrombosis leads to development of venous collaterals which can bleed. This is only the second reported case of ectopic varices with mesenteric vein thrombosis attributed to heterozygous factor V Leiden mutation [6]. Factor V Leiden is one of the most common inherited thrombophilia.…”

Várices yeyunales secundarias a trombosis de la vena mesentérica superior: una causa inusual de sangrado del intestino delgado

“…Las VID en nuestro paciente, fueron secundarias a la trombosis crónica aislada de la VMS. En la literatura, se han descrito pocos casos de VID secundarias a trombosis aislada de esta vena (4)(5)(6)(7) . Las etiologías de la trombosis venosa reportadas incluyen coagulopatías como deficiencia de las proteínas C, S y antitrombina III, mutación del factor V de Leiden.…”

“…Sin embargo, existen otras etiologías menos frecuentes como el antecedente quirúrgico abdominal, patologías inflamatorias crónicas y la trombosis de la vena mesentérica superior (VMS) (1,2) . Las várices del intestino delgado (VID) secundarias a trombosis aislada de la VMS son muy infrecuentes, se han documentado menos de 25 casos en la literatura (4)(5)(6)(7) , por lo que la evidencia de las posibles intervenciones terapéuticas se reserva a lo descrito en reportes y series de casos (3) . Representan un reto diagnóstico y terapéutico debido la baja prevalencia descrita, la insuficiencia de datos clínicos que orienten a sospecharlas (en contraste con los pacientes con factores de riesgo para hipertensión portal), y la limitada evidencia de las modalidades terapéuticas.…”

A rare case of obscure gastrointestinal bleeding: small bowel varices flowing into the inferior epigastric vein

“…Recently, double balloon endoscopy and capsule endoscopies have made it possible to observe such varices in the small intestine. [5,6] Although it is not clear whether the inferior mesenteric vein has any communication with the portal system, ectopic varices in the small intestine are usually associated with adhesions between the small intestine and the abdominal wall. Furthermore, small bowel varices can develop as a consequence of previous operations or abdominal injury.…”