BRIEF COMMUNICATION

Recurrent gastrointestinal bleeding in a ITlan with Osler-Weber-Rendu syndrome and intestinal varices

ROHERT N. CLARK. MD, FRCPC, WILLIAM G. PATERSON. MD, FRCPC. LAURJNmON R. DA COSTA. MD, FRCP (LOND). FACP, FRCPC, IVAN T. BECK. MD. PHO, FRCPC, FACP

larly over the past year, during which time ABSTRACT: A patient with Osler-Weber-Rendu disease and idiopathic intestinal he had been transfused with over 20 l'arices who presented with recurrent gastrointestinal hemorrhage and iron deficien­ cy anemia is reported. The unique coexistence of these two rare disorders is dis­ units of packed red blood cells. Esopha­ w~scd and the literature reviewed. Can J Gastroenterol 1988;2(2):65-66 gogastroduodenoscopy, small bowel en­ ema and air con trast bariu m enema had Key Words: Bleed mi, Gascro,nreswwl, lncestinal va1·ices, Telangieccasws failed to demonstrate any abnormality. In particular no varices were visualized ERl'DITARY I 11-MORRHAG JCTl:LAN· and on many occasions subsequentl y, he in the esophagus, stomach or duodenum Hgiecras ia is a rare but well docu­ had developed profound iron deficien­ during upper endoscopy. At colonoscopy mented cause of recurrent intestinal cy anemia, often to the range of 60 to 70 large colonic varice~ ( Figure I) were ob­ bleeding i::vcn more rare is the devel­ g/L, and not compensated for by chronic served d iffusely throughout the left and opment of colonic vnrices in the absence oral iron therapy. He had developed right colon and also in the terminal ile­ oi portal hypcnension. This paper re­ unstable angina on several occasions dur­ um (Figure 2). In addition, there were l ports a unique ca~e of recu rrent lower ing periods of worsening anemia and, to 2 mm diameter tclangiectatic vascu­ gastrointestinal hemorrhage where both in 1986, underwent coronary arter y lar lesions scattered in the region of the of these conditions were present. bypass graft surgery for occlusive cor­ splenic fl exure and descending colon onary disease. (Figure 3 ). Several of these lesions were CASE PRESENTATION At no time over the past 10 years had coagulated with a bicap electrode, A 44-year-old male inmate of a Feder­ the patient been seen and assessed while (ACM!, BC-60B BICAP), despite uncer­ al penitentiary was recently investigat­ actively bleeding, but scools had been tainty that these were che cause of the ed in this unit for recurrent episodes of positive for occult blood on several oc­ gastrointestinal hemorrhage. gamointcstinal bleeding. casions. The remainder of the histor y, During the same admission a superi­ The patient was first recognized as hav­ physical examination and laboratory in­ or mesenteric artery angiogram, with de­ ing Osler-Webcr-Rendu disease in 1977 vestigations were normal. In particular, layed venous phase, fai led to identify any when the typical tclangiectasias were first the re was no cl inical or biochemical evi­ abnormality. Ultrasound of the abdo­ noted on his lips. For the previous seven dence of underl ying liver disease or men showed the portal to be pa­ years he had experienced repeated bouts portal . T here was no sug­ te n t and the spleen to be of normal size. pf red blood per rectum, attributed up gestion o f coagulopathy at any time. The liver parenchyma was unremark­ until that time to . A hem­ T he re had been no previous abdomi­ able. however, a 3 to 4 cm d iameter nrrhoidectomy did not influence the al­ nal surge ry. hyperchoic lesion was detected in the mo~l monthly epi~odcs of bleeding. The patient had been extensively in­ right lobe. A technetium 99 sulphur col­ At the time of his initial assessment, vc~tigated on several occasions, particu- loid liver-spleen scan also demonstrat­ ed a 3 cm filling defect in the right lobe Gc1srro111rc,rinal D1.1ctrsc., Research UnH <1nd 1he De/Jartmcnr of Medicme, Q11een's University, of the liver. Otherwise, the hepatic up­ 1'111.1(11011. Ontario take of technetium 99 was homogeneous Corre.1/mndcnce ,rnJ reprnm Dr William G Pmcrson. Di1•is1on of Gasrroenrerology. and there was no bone marrow u ptake Drpur111w11 of Mcdrcme. Hord Drett Ho1/1ital . /66 Bruck Srrcct, Kmgsron, Onwrio K?L .5G2. Tde/1hm1c ( 6/ l 1 ~49 ~o/ ~ or splenomegaly to suggest portal hyper­ R,xcrt'cd /or /11d1/1carro11 Dewnher 7. /98i. Acce/1rccl Fclmwry 12, 1988 tension. Subsequently a pooled techne-

Vol 2 N<1 2. lune 19H8 65 CLt\Rf-. e1 al

F igu re I ) E11Jmco/>1c 1•1eu· o/mnccs m rhc as­ F ig ure 2) EnJo,copic 1•1cu· 0/mnce.1 in rhc wr­ Figu re 3) E11Jmco/)1c 1•icU' o/ 1clan)!1ectact1c /~­ ,;~11Jm.~ col,m mmal ileum mm 0/ 1/ic />roxi11wl dcscend111g co/011

tium red blotid cel l scan was perfo rmed in the small bowel, as was demonstrated hemangi0ma with associated venous pro­ which revealed a 'delayed hot spot' in in the present patient. Falch uk and col­ li(eration produced the colonoscopically the right lobe suggesting that the liver leagues ( 6 J reported gastrointestinal visuali zed varices in the rectosigmoiJ. lesion was a hemangioma. bleeding from ilea! varices, which were T he presen t patient presented with two believed to ,irise as a result of localized rare intestinal conditions, and the prob­ DISCUSSION mcscnteric hypertension due to ad he­ ability of these co-existi ng due to chance The associmion between Osler-Wcber­ sions. Except for such unusual circum­ alone is quite small. Thus it is tempting Rend u hereditary and co­ stances, the small bowel is generall y to speculate that the two arc interrelat­ lonic variccs has never been reported. spared extensive portosyscemic collater­ ed. It is possible that arreriovenous shunt­ Colonic variccs arc relatively rare bcmg als, wh ich are common in the cardiac­ ing a~sociated with in te~tinal tclangiec­ fo u nd in only 0.07°1, of 2912 consecu­ azygous system. abdominal wall and tasias resu lts in increased flow and ,cc· tive auwpsics reported by Feldman ct al rectum (7). A lthough ch is was nor the ondary dilatation of intestinal vei n,, how­ ( I ). In the majority of cases, colonic var­ case in the present patient, small bowel ever, the relatively ~mall number of icc~ arc.,1ttriburnblc to portal hyperten­ varices can be demonstrated by entero­ colonic tclangicccasias seen in the patient sion (2), usually in the setting of alcoholic clysis (8). Active bleeding from intesti­ would argue against this hypothesis. Nev­ liver disease. Less common causes of co­ nal varices can be d iagnosed by scinti­ ertheless, it is possible thar more exten­ lonic variccs include large bowel venous graphy, using labelled red blood cells, if sive relangiectactic lc~ions were rrescnt anomalies (3) and selective mcscnte ric the examination is performed during an deep to the mucosa and out of view of vein obstruction ( 4 ). Rarely, chronic heart episode of active hemorrhage (9). the endoscope. failure or constrictive pericarditis may be The only report which links colonic Gi\Tn the uncerraintie~ regarding the responsible fo r impaired colonic venous variccs and benign vascu lar disease of exact si te and source ofhlecding, the au­ return and result in colon ic variccs (2). the bowel is ch ar of Lieberman and oth­ thors intend to manage thb patien t with The management of recurrent colon ic ers (3 ). These auch0rs describe a patient hlood rra nsfusion as necessary, and u n­ variccal blceding from portal hyperten­ with severe lower gastrointestinal hlced­ dcnake colonic or small bowel rcsccrion sion is either resection of the affected ing, rectosigmoid varices and Gtvcrn0us only for an cxsanguinating bleed or, if portion of bowel, or portosystemic de­ hemangiomas involving the skin, l0wcr they .ire able to localize a foca l bleeding compressive shunt ( 5). extremities and distal colon. The authors source. by angiography or red blood cell Va rices arc even less likely robe found suggest th:u the underlying cavernous bleeding ,can.

REFERENCES intestinal hemorrh,1ge associated with 7 Silk OBA, William, R Portal 1 Feldman M, Smith VM. Warner CG chronil mc,cmcric venous occlusion hypcrtcnsi(>n In· Wright R. ed. Liver and Variccs of the colon JAMA Gasrmcntcrology 19H5;H8: 1964-7 Biliary Disease. Path\1phy,iology, 1%2. 179.729-3(.). 5 P1Ckcns CA. Tcdc,co FJ Cokmic varicc, Diagnosis. Mnnagcrncn1. Tc,ronto: WB 2. bak EM. Finlay JM. Colonic variccs 3 unusual cause of rccral bleeding Am J Saunders Co. 1979 case reports and a review of che li terature Gasrroentcrol 1980;7 'l 73-4 8. Agaral D. Schol: Fl Sm.ill bowel varices Am J Ga,1rocnterol 1980;7.3: l 31-6 6. Fakhuk KR. Aicllu MR. Trey C. Costello demonstrated hy cnternclysis. Radiology 'l L1chcrman DA. Knppachnc WW. Melnyk P Rl'currcnt gastrointestinal bleeding 1981 , 140 350. CS. Colonic variccs due to in testinal from ilea! varices as,ociatcd with 9 . Royal HD, Papan1cohwu N, Bcttmann M. cavcrnou, hcmangiomas. Dig Dis Sci imra·abdn1111nal adhesions: Case report McNeil BJ. Scimigraph1c identification ot 1983,28:852-8 ,,nd review of the literature. Am J bleeding duodenal vnrices. Am J 4. Soper NJ, Rikkcrs LF, Miller FJ. Gastro- Gastrocntcrol 1982; 77 :8 59-60. Ga,1rnenrcrol 1980,74.17 l-5

66 M EDIATORSof

The Scientific Journal of Research and Practice Diabetes Research Disease Markers World Journal Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation http://www.hindawi.com Volume 2014 Hindawi Publishing Corporation Hindawi Publishing Corporation http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014

Journal of International Journal of Immunology Research Endocrinology Hindawi Publishing Corporation Hindawi Publishing Corporation http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014

Submit your manuscripts at http://www.hindawi.com

BioMed PPAR Research Research International Hindawi Publishing Corporation Hindawi Publishing Corporation http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014

Journal of Obesity

Evidence-Based Journal of Stem Cells Complementary and Journal of Ophthalmology International Alternative Medicine Oncology Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014

Parkinson’s Disease

Computational and Mathematical Methods Behavioural AIDS Oxidative Medicine and in Medicine Neurology Research and Treatment Cellular Longevity Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014