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Ectopic varices in portal hypertension D. Sharma, S. P. Misra1 Department of Surgery, NSCB Government Medical College, Jabalpur, Madhya Pradesh, India; 1Department of Gastroenterology, MLN Medical College, Allahabad, Uttar Pradesh, India For correspondence: D Sharma, Department of Surgery, NSCB Government Medical College, P-10, Medical College Campus, Jabalpur, Madhya Pradesh – 482003, India. E-mail: [email protected] ABSTRACT Varices most commonly occur and bleed in the gastro-esophageal region but ectopic varices can arise at extra-gastro-esophageal locations in the gastrointestinal tract like duodenum, jejunum, ileum, colon, rectum, biliary tree and at the site of a surgical ostomy. These varices pose diagnostic as well as therapeutic challenges during endoscopic procedures. Ectopic varices can also occur outside the gastrointestinal tract, giving rise to diagnostic difficulties on imaging and unusual hemorrhage. Although ectopic varices bleed less commonly than gastro-esophageal varices, they are difficult to diagnose and problematic to treat. The absence of stigmata of recent esophageal or gastric variceal bleeding and certain clues in the patient’s history and clinical presentation should raise the clinician’s suspicion of an extra-gastro-esophageal site of variceal bleeding. Patients with extrahepatic causes of portal hypertension, Review Article Review Article Review Article Review Article Review Article cirrhotic patients with a prior history of gastrointestinal surgery and patients who present with profound bleeding but without hematemesis should in particular be evaluated further if an obvious site of gastro esophageal variceal bleeding is not observed at UGI endoscopy. Accurate preoperative diagnosis is often difficult in patients bleeding from ectopic varices from extra-gastro-esophageal sites, rebleeding is certain even in those patients who respond to medical treatment, and the optimal surgical decision making protocol has not yet been evolved due to the low prevalence of these ectopic varices. Nevertheless, an appreciation and awareness of these unusual causes of gastro-intestinal bleeding, allied with prompt and appropriate diagnosis can lead to successful endoscopic, radiological or surgical management of ectopic variceal bleeding. Key words: Cirrhosis, ectopic varices, gastrointestinal bleeding, portal hypertension How to cite this article: Sharma D, Misra SP. Ectopic varices in portal hypertension. Indian J Surg 2005;67:246-52. Portal hypertension and the development of DUODENAL VARICES varices in areas of portosystemic venous anas tomoses can lead to dramatic and life-threat- Duodenal varices occur in about 0.4% in all patients ening hemorrhage. Varices most commonly with portal hypertension and account for one third of occur and bleed in the gastro-esophageal re- bleeding episodes from ectopic varices. [1,2] Early de gion, but ectopic varices can arise at extra-gas- tection is important, as duodenal varices are a poten tro-esophageal locations in the gastrointesti- tial source of massive hemorrhage. At upper gastroin nal tract like the duodenum, jejunum, ileum, testinal endoscopy, an uninitiated observer may mis colon, rectum, and biliary tree. They can also interpret bleeding from duodenal varices as that from occur at the site of a surgical ostomy. Although duodenal ulcer. These should be considered in all pa ectopic varices bleed less commonly than es- tients with duodenal tumoral lesions and suspected ophageal varices, they can be far more diffi- portal hypertension. In this context, duodenal biopsy cult to diagnose and treat. can be dangerous and should be avoided. A diminu tion in the volume of the duodenal varices with in- Paper Received: August, 2003. Paper Accepted: October, 2005. spiratory movements may help in the differential di- Source of Support: Nil. agnosis during endoscopy.[3] FreeIndian full J Surgtext available| October from 2005 http://www.indianjsurg.com | Volume 67 | Issue 5 241 Sharma D, et al. The duodenal bulb is the most common site of duode munication) in adhesions. Possible physiological ori nal varices, the second portion of the duodenum ap gins of this entity were studied in Edward’s demon pears to be the next most common site but duodenal stration of network of fine communication between varices in the other portions are rare.[4] Hashizume the parietal surface of the viscera and the posterior et al. studied these varices angiographically and his abdominal wall, arising in the embryo due to the jux topathologically; and found that the duodenal varix taposition of the developing systemic and visceral ve consisted of a single vessel with afferent and efferent nous plexus.[18] Formation of collaterals, de novo, is vessels, forming a portosystemic shunt in the retro unlikely if the anatomy is undisturbed. In some cases peritoneum. The varix traversed the duodenum and no cause can be found. Histological examination dem was present in the submucosal layer of the posterior onstrates a massive varicose vein and several dilated wall; while the afferent vessel was the superior or in veins in the submucosa.[19] ferior pancreaticoduodenal vein originating in the por tal vein trunk or superior mesenteric vein, and the ef Although rare, bleeding from small bowel varices is ferent vein drained into the inferior vena cava.[2] They associated with a high mortality as accurate preopera have also been reported at the site of previous duode tive diagnosis is often difficult. Detection of these varic nal operations and the resultant adhesions and after es has been a challenging task and several invasive endoscopic sclerotherapy.[5,6] Duodenal varices are diagnostic techniques such as enteroclysis, Tc-99m more common in patients having extrahepatic portal RBC studies, venous phase of mesenteric arteriogra vein obstruction and in those with thrombosed porto phy, enteroscopy, color flow Doppler ultrasound and systemic shunts.[7,8] magnetic resonance angiography have been used for this purpose.[16,20-23] Intraoperative Sonde enteroscopy Apart from endoscopy, hypotonic duodenography, ul is safe and effective, providing complete visualization trasonography, computed tomography, venous phase of the small-bowel mucosa without enterotomy while of superior mesenteric angiography, and percutaneous avoiding the trauma that can be caused by push en transhepatic portography have been used to diagnose doscopy. It is the diagnostic assessment of choice.[24] duodenal varices.[4] Medical therapy, including vasopressin infusion via the superior mesenteric artery, is often useful in con Medical therapies, including vasopressin and octre trolling acute variceal bleeding.[25] Percutaneous tran otide may have limited success in controlling active shepatic embolization and transjugular intrahepatic duodenal variceal bleeding.[9] Endoscopic sclerothera portosystemic shunt are the therapeutic alterna py or endoscopic variceal ligations are the main treat tives.[26,27] Surgical treatment consists of lysis of adhe ment modalities.[10,11] Embolization and transjugular sions and bowel resection combined with portosys intrahepatic portosystemic shunt are the therapeutic temic shunt, under the presumption that the portal alternatives, if endoscopic sclerotherapy or variceal pressure in these patients has been partially decom ligation fails to control the bleeding.[12,13] pressed through these spontaneous shunts and may increase significantly after their surgical division.[28] When conservative measures cannot control the hem Patients with excellent hepatic reserve survive and orrhage, emergency laparotomy may be indicated. have no further gastrointestinal bleeding.[29] Duodenal varix suture ligation or resection results in a high rate of rebleeding.[14] End-to-side portacaval COLONIC VARICES shunt may be effective.[9] An arteriovenous fistula re quires resection of the paramural varix and surgical Colonic variceal bleeding is a rarity and is most com occlusion.[14] In view of the difficulty during the duo monly due to portal hypertension, with local mesenter denal mobilization and the precarious condition of ic vein obstruction constituting a rare cause. The true patient, it is not surprising that the operative mortali prevalence of colonic varices is not known, but Feld ty is high. man et al. found an incidence of 0.07% in autopsy material.[30] Esophageal varices were present in approx JEJUNAL AND ILEAL VARICES imately half of the group with colonic varices.[31] Bleed ing has been reported to occur in 2.5% of patients at A triad of portal hypertension (generally due to liver tending sclerotherapy sessions for esophageal varic cirrhosis), history of abdominal surgery, and hemato es.[32] In patients with portal hypertension the coro chezia without hematemesis characterizes small intes nary azygous system was the primary portosystemic tinal varices.[16] Bleeding from varices may present with channel in at least half of the cases, but in a quarter of vesical varices and gross hematuria if an intestinal seg cases it was the inferior mesenteric-internal iliac sys ment is used for an augmentation cystoplasty.[17] tem.[31] Possible etiologies of this condition may be es ophageal transection and devascularization and exten A history of abdominal surgery appears to predispose sive thrombosis of the portal vein resulting in obliter the development of ectopic varices (portosystemic com ation of the coronary-azygous anastomotic system. In 242 Indian J Surg | October 2005