Unusual Case of Massive Obscure Gastrointestinal Bleeding : Ectopic Varices in Jejunum Caused by Arteriovenous Malformation

Small bowel ectopic varices is a rare etiology for obscure gastrointestinal bleeding. Ectopic varices in the absence of portal hypertension can be caused by congenital or familial conditions (e.g. malformation of vessel). Bleeding caused by ectopic varices can be massive and life threatening. Single Balloon Enteroscopy (SBE) is one of diagnostic modalities for obscure gastrointestinal bleeding. We report one case of obscure overt gastrointestinal bleeding with sub-acute onset. Previous esophagogastroduodenoscopy and colonoscopy cannot found the source of bleeding. On the enteroscopy we found varices at proximal jejunum with active bleeding during procedure. We applied hemostatic powder to stop the bleeding and proceed to surgery. Surgery was performed by enteroscopy guide. The jejunum section with varices was resected and the pathology confirmed the malformation of arteriovenous. Currently there is no available guideline or randomized study for the treatment of ectopic varices. Treatment options include ligation, sclerotherapy, surgery and interventional radiology. In this patient we choose surgery because of massive gastrointestinal bleeding.


INTRODUCTION
Obscure gastrointestinal bleeding still remain a challenge in diagnosis and treatment.Diagnostic approach consists of video capsule endoscopy, enteroscopy, CT/MR enterography or angiography. 1nteroscopy is one of the modality for detecting etiology of obscure gastrointestinal bleeding.It allows therapeutic procedure simultaneously and assist the surgeon during operation.
Obscure gastrointestinal bleeding cause by ectopic varices is a rare disease.Ectopic varices can cause massive gastrointestinal bleeding with high mortality.Ectopic varices can occur in portal hypertension and in the absence of portal hypertension.In the absence of portal hypertension etiology of ectopic varices caused by congenital anomalous portosystemic anastomoses, abnormal vessel structures, arteriovenous fistulae, rare familial conditions, or related to thromboses 2 This case report demonstrate the approach for obscure overt gastrointestinal bleeding and management of ectopic varices.

CASE ILLUSTRATION
Male 34 years old came to our hospital with bloody stool for 2 weeks before admission.Patient was weak and pale on admission.Patient didn't complain any abdominal pain or vomiting.No weight loss.On physical examination, we found pale conjunctiva and no abnormality in abdomen.Previously he had done colonoscopy but there was no clear source of bleeding.
Six months before patient also complaint black stool and received blood transfusion.From the previous esophagogastroduodenoscopy and colonoscopy also did not find any source of bleeding and the bleeding resolved itself.Laboratory findings show microcytic hypochromic anemia (Hb : 8.23 gr/dL) with normal hemostasis, viral marker (HbsAg and anti HCV) and tumor markers (CEA) We decided to perform single balloon enteroscopy with antegrade approach to find the source of bleeding.From the enteroscopy we found the bleeding lesion in the jejunum (110 cm from incicivus) and the bleeding occur during enteroscopy.We apply hemostatic powder on the bleeding site for temporary hemostasis.During observation patient still have bleeding and we decided to refer patient to urgent surgery the next day.
During surgery we assist the surgeon to locate the varices using single balloon enteroscopy.The jejunum part with varices was resected and send to pathologist.The result was malformation of arteriovenosus.After surgery patient didn't complain any bleeding and the hemoglobin was stable.

DISCUSSION
The definition of obscure gastrointestinal bleeding is the absence of identified source of bleeding after normal upper endoscopy, colonoscopy, & small bowel radiographic evaluation.In updated definition this definition reserved for patient with bleeding despite video capsule endoscopy, deep enteroscopy, and CT enteroclysis/MR enteroclysis examination. 1 Obscure gastrointestinal bleeding has been classified as obscure overt gastrointestinal bleeding if there is frank bleeding with or without iron deficiency and obscure occult bleeding if only guaiac test positive with iron deficiency.This patient had bloody stool with normal upper and lower endoscopy from other hospital so it is an obscure overt gastrointestinal bleeding.The prevalence for small bowel lesion as the etiology for gastrointestinal bleeding is around 5-10%. 3 The approach for obscure gastrointestinal bleeding depends on the quantity of bleeding.Patient with subacute ongoing bleeding is best evaluated using deep enteroscopy to find source of bleeding.There are 3 types of deep enteroscopy that is: single-balloon enteroscopy (SBE) by Olympus, double-balloon enteroscopy (DBE) by Fujinon Inc. and spiral overtube enteroscopy.][6] In our institution we use SBE and DBE.This patient directly underwent evaluation with SBE with antegrade approach.We found the lesion at 110 cm from incicivus which is in jejunum.The lesion appears as bluish submucosal vein enlargement with point of recent bleeding at the center.During enteroscopy there was active bleeding so we applied hemostatic powder.
Ectopic varices define as dilated portosystemic collateral veins located in unusual sites other than gastroesophageal region. 7Ectopic varices is rare cause of gastrointestinal bleeding.Approximately 5% of all variceal bleeding caused by ectopic varices.The mortality of ectopic variceal bleeding is high (up to 40%) because of massive bleeding at initial presentation. 8The most common site for ectopic varices is peristomal (26%), followed by duodenum (17%) and jejunum/ileum (17%). 9In the absence of portal hypertension the pathogenesis of ectopic varices is caused by anomalies of portal venous outflow, such as congenital anomalies or rare familial conditions. 10n this patient there were no sign of portal hypertension and the varices caused by congenital anomaly (malformation).
Management of ectopic varices depends on location, presentation, and local expertise.There are no guidelines or randomized trial for management of ectopic varices.Most publications come from case report or case series.In this patient because of massive bleeding and the location in small bowel we choose to do surgical resection of the affected area.Other modalities for ectopic varices are ligation, sclerotherapy or radiology intervention. 2

Figure 1 .Figure 2 .
Figure 1.Single balloon enteroscopy finding of jejunal ectopic varices with point of bleeding