Vascular malformation of the gastrointestinal tract

Vascular lesions of the Vascular lesions of the Gastrointestinal TractGastrointestinal Tract

By By Omar Mohammad Saleh Omar Mohammad Saleh

Assistant lecturer of tropical medicine and Assistant lecturer of tropical medicine and gastroenterology , Hadramout University, Yemen.gastroenterology , Hadramout University, Yemen.

Main points to be discussedMain points to be discussed:: Nomenclature and PathogenesisNomenclature and PathogenesisClassificationClassification Clinical significanceClinical significanceClinical presentationClinical presentationDiagnosisDiagnosis TreatmentTreatment

Nomenclature and Pathogenesis:Nomenclature and Pathogenesis:

* Normal blood vessels are derived from two * Normal blood vessels are derived from two

processes : vasculogenesis and angiogenesis.processes : vasculogenesis and angiogenesis.

* * Vascular ectasia is a broad term that Vascular ectasia is a broad term that

includes angiodysplasia , gastric antral includes angiodysplasia , gastric antral

vascular ectasia (GAVE), and telangectasia vascular ectasia (GAVE), and telangectasia

associated with multi-system disease such associated with multi-system disease such

as hereditary hemorrhagic telangectasia as hereditary hemorrhagic telangectasia

(HHT)(HHT)..

•**Venous malformations (VMs) are still called Venous malformations (VMs) are still called cavernous cavernous

hemangiomahemangioma, , venous hemangiomavenous hemangioma, and , and cavernouscavernous angiomaangioma in many medical text books in many medical text books..

* *Intestinal Phlebectasia are venous varicosities Intestinal Phlebectasia are venous varicosities consisting of a markedly dilated and tortuous vein with consisting of a markedly dilated and tortuous vein with a normal vascular wall and scant connective tissuea normal vascular wall and scant connective tissue . .

*The abnormal vascular structures in HHT (also known as Osler–Weber–Rendu syndrome) result from mutations in genes (most commonly endoglin or ACVRL1) whose protein products influence TGF-ß superfamily signalling in vascular endothelial Cells.

*HHT was first described as a familial disease characterized by severe recurrent nasal and gastrointestinal bleeding with associated anemia, and visible dilated blood vessels (telangectasia) on the lips and finger tips.

HHTHHT

Dieulafoy’s diseaseDieulafoy’s disease::

?? Secondary to a vascular aneurysm in the Secondary to a vascular aneurysm in the gastric wall associated with arteriosclerosisgastric wall associated with arteriosclerosis..

? ?Congenital or acquired vascular Congenital or acquired vascular malformationmalformation..

Now generally accepted that the bleeding is Now generally accepted that the bleeding is caused by an abnormally large artery caused by an abnormally large artery running in the submucosarunning in the submucosa..

Gastric antral vascular ectasiaGastric antral vascular ectasia * *The aetiology is unknownThe aetiology is unknown..

* *Hypothetically , caused by repeated antral Hypothetically , caused by repeated antral mucosal trauma due to pyloric prolapse mucosal trauma due to pyloric prolapse and disturbed motilityand disturbed motility..

* *Capillary type, dilated, tortuous, mucosal Capillary type, dilated, tortuous, mucosal capillaries , often occluded by thrombi as capillaries , often occluded by thrombi as well as submucosal veins , are present in well as submucosal veins , are present in the antrumthe antrum..

AngiodysplasiaAngiodysplasiaThe pathogenesis is not clearThe pathogenesis is not clear..

?? These lesions can be regarded as degenerative These lesions can be regarded as degenerative lesions of aging or as neurohormonal lesions of aging or as neurohormonal abnormality causing increased flow and abnormality causing increased flow and

relaxation of smooth musclesrelaxation of smooth muscles . .

Histologically , they present only in the superficial Histologically , they present only in the superficial layers of the bowel wall (mucosa and layers of the bowel wall (mucosa and submucosa). Vessels are dilated , tortuous , and submucosa). Vessels are dilated , tortuous , and very thin walled , with no inflammation or very thin walled , with no inflammation or fibrosisfibrosis..

Ectatic mucosal blood vessels characteristic of angiodysplasia, filed by barium-gelatine. Note the cluster of associated submucosal arterioles and the lack of surface ulceration, inflammation or fibrosis.

ClassificationClassification

Clinical significanceClinical significance **Vascular anomalies of the GIT are an Vascular anomalies of the GIT are an

important source of both acute and important source of both acute and chronic blood losschronic blood loss..

**They present difficult management They present difficult management problems as they are often multiple, problems as they are often multiple, involving more than one part of the involving more than one part of the GIT, and may give rise to bleeds of GIT, and may give rise to bleeds of increasing frequency and severity over increasing frequency and severity over a period of many yearsa period of many years..

These lesions can occur sporadically These lesions can occur sporadically or in associations with other or in associations with other disorders: cirrhosis, renal failure, disorders: cirrhosis, renal failure, radiation injury, various collagen radiation injury, various collagen diseases and HHT (Osler-Weber-diseases and HHT (Osler-Weber-Rendu disease)Rendu disease)..

Clinically, Dieulafoy’s lesions manifest as massive gastrointestinal bleeding

with no preceding symptoms, If the lesion is not treated, patients

continue to bleed intermittently.

The successful diagnosis and The successful diagnosis and treatment of GI bleeding requires treatment of GI bleeding requires the involvement and expertise of a the involvement and expertise of a variety of specialists , the use of a variety of specialists , the use of a variety of sophisticated imaging variety of sophisticated imaging techniques and selection of a proper techniques and selection of a proper treatment modality from a variety of treatment modality from a variety of options. (Multidisciplinary approach)options. (Multidisciplinary approach)..

Clinical presentationClinical presentation AngiodysplasiaAngiodysplasia::

•Represent 3-6% of LGIB and 2-5% of Represent 3-6% of LGIB and 2-5% of UGIB, either acute or chronic (IDA)UGIB, either acute or chronic (IDA)Found usually in the elderlyFound usually in the elderlyLocalized mainly in the stomach and Localized mainly in the stomach and right colonright colon

Associated with CRF, CREST, Turner Associated with CRF, CREST, Turner syndrome, portal hypertension and syndrome, portal hypertension and Heyde syndromeHeyde syndrome..

DiagnosisDiagnosis::

* *Endoscopy plays a major role in the Endoscopy plays a major role in the diagnosisdiagnosis..

• * *They appear as mucosal or submucosal , They appear as mucosal or submucosal , 2-8mm wide, intensely red, dense, 2-8mm wide, intensely red, dense, network of vessels with abrupt and network of vessels with abrupt and irregular borderirregular border..

Bleeding angiodysplasia lesion on wall of large intestine

Dieulafoy’s diseaseDieulafoy’s disease::• * *Causes about 1.5% of UGIB and about 0.3% Causes about 1.5% of UGIB and about 0.3%

of LGIBof LGIB..

• * *The characteristic clinical presentation is The characteristic clinical presentation is sudden onset of painless , massive , recurrent , sudden onset of painless , massive , recurrent , intermittent hematemesis at times associated intermittent hematemesis at times associated with melena, hematochezia and hypotensionwith melena, hematochezia and hypotension..

* *Located in the upper stomach about 5-7 cm Located in the upper stomach about 5-7 cm below the GEJ in the majority of cases, but can below the GEJ in the majority of cases, but can occur in other GI partsoccur in other GI parts..

On endoscopy: Blood spurting or oozing On endoscopy: Blood spurting or oozing from a pin point mucosal defect or, in the from a pin point mucosal defect or, in the absence of bleeding , a clot without an absence of bleeding , a clot without an associated surrounding ulcerassociated surrounding ulcer..

Rectal Dieulafoy’s lesion, old age female patient presented Rectal Dieulafoy’s lesion, old age female patient presented with massive hematocheziawith massive hematochezia..

GAVE ‘Watermelon stomachGAVE ‘Watermelon stomach’’**Occurs five times more often in women than in Occurs five times more often in women than in

menmen..• * *Clinical presentation includes IDA due to Clinical presentation includes IDA due to

chronic bleedingchronic bleeding..• * *Associated with liver cirrhosis, bone-marrow Associated with liver cirrhosis, bone-marrow

transplantation, scleroderma and transplantation, scleroderma and hypochlorhydriahypochlorhydria..

* *Endoscopically, multiple parallel Endoscopically, multiple parallel longitudinal streaks converge onto the longitudinal streaks converge onto the pyloric orifice, this view resembles the pyloric orifice, this view resembles the surface of a watermelonsurface of a watermelon . .

HaemangiomasHaemangiomas• * *GIT haemangiomas are commonly associated GIT haemangiomas are commonly associated

with cutaneous haemangioma such with cutaneous haemangioma such as blueas blue rubber bleb nevus syndrome or Klippel-rubber bleb nevus syndrome or Klippel-Trenaunay syndromeTrenaunay syndrome (port-wine (port-wine haemangiomas, soft tissue hypertrophy, haemangiomas, soft tissue hypertrophy, varicose veins and large cavernous varicose veins and large cavernous haemangiomas in large or small intestine)haemangiomas in large or small intestine)..

• * *Bleeding is usually episodic and starts in Bleeding is usually episodic and starts in childhoodchildhood..

• * *Endoscopically ;sessile polypoid lesions , Endoscopically ;sessile polypoid lesions , bluish in colour are seenbluish in colour are seen . .

**biopsy should be avoidedbiopsy should be avoided..

TreatmentTreatment The treatment will include the followingThe treatment will include the following::

• * *General measuresGeneral measures**Medical treatmentMedical treatment

* *Endoscopic therapyEndoscopic therapy * *Interventional radiology ; angiography Interventional radiology ; angiography

and embolizationand embolization * *SurgerySurgery

TreatmentTreatmentAngiodysplasiaAngiodysplasia::

11--Medical treatmentMedical treatment::Supportive treatmentSupportive treatment

??Hormonal therapy with oestrogen and Hormonal therapy with oestrogen and progestagen (case report, studies with short progestagen (case report, studies with short follow-up)follow-up)..

??somatostatin and its analogue , octreotide, have somatostatin and its analogue , octreotide, have been reported to reduce blood loss from been reported to reduce blood loss from intestinal angiodysplasia (case report)intestinal angiodysplasia (case report)..

??VEGF inhibitors , Thalidomide is a potent VEGF inhibitors , Thalidomide is a potent inhibitor of angiogenesisinhibitor of angiogenesis..

Efficacy of Thalidomide for Refractory Gastrointestinal Bleeding From Vascular Malformation, GASTROENTEROLOGY 2011;141:1629–1637 .

Endoscopic therapyEndoscopic therapy::

•Argon plasma coagulation ; currently is Argon plasma coagulation ; currently is the preferred method which provide the preferred method which provide sufficient electrocaogualtion of the lesionsufficient electrocaogualtion of the lesion..

* *Other methods such as Other methods such as thermocoagulation, electrocoagulation , thermocoagulation, electrocoagulation , or photocoagulationor photocoagulation..

Angiodysplasia of the stomach treated Angiodysplasia of the stomach treated by APCby APC

Dieulafoy’s lesionDieulafoy’s lesion:: * *Bleeding can be controlled byBleeding can be controlled by::

11--endoscopic clip placementendoscopic clip placement::Hemostasis achieved in 94% in a Hemostasis achieved in 94% in a prospective study of 34 patientsprospective study of 34 patients . .

22--Band ligationBand ligation::Retrospective study showed similar Retrospective study showed similar hemostasis charcteristics with injection hemostasis charcteristics with injection therapy (as epinephrine, ethanolamine therapy (as epinephrine, ethanolamine oleate, cyanoacrylae glue, hypertonic oleate, cyanoacrylae glue, hypertonic glucose or ehanol)with or without thermal glucose or ehanol)with or without thermal

therapytherapy..

* *Studies showed that mechanical methods Studies showed that mechanical methods (clipping, banding) are significantly better (clipping, banding) are significantly better than injection methods alone ; recurrence than injection methods alone ; recurrence of bleeding was 8.3% and 33%of bleeding was 8.3% and 33%. . SurgerySurgery::

When endoscopic therapy failed to achieve When endoscopic therapy failed to achieve hemostasishemostasis..

hemostasis was carry out with absolute alcohol hemostasis was carry out with absolute alcohol

injectioninjection  .  .

APCAPC

GAVEGAVE::**Endoscopic therapy is performed Endoscopic therapy is performed

preferably with argon plasma preferably with argon plasma coagulationcoagulation..

•**The paint-brush techniqueThe paint-brush technique –with –with application of energy only on withdrawal application of energy only on withdrawal of the catheter is usedof the catheter is used . .

* *Therapy has to be repeated every few Therapy has to be repeated every few moths due to recurrence of new vascular moths due to recurrence of new vascular abnormalitiesabnormalities . .

Portal gastropathyPortal gastropathy::Asymptomatic: no therapyAsymptomatic: no therapyChronic bleedingChronic bleeding::

--general measures; iron replacement, blood general measures; iron replacement, blood transfusion when necessarytransfusion when necessary..

--specific measures; betablockersspecific measures; betablockersAcute bleedingAcute bleeding::

--general measures; adequate resuscitationgeneral measures; adequate resuscitation--specific mesures; vasoactive medicationspecific mesures; vasoactive medication..

HaemangiomasHaemangiomas::• * *Polypectomy or band ligation have been Polypectomy or band ligation have been

performed successfully without performed successfully without complications in many described complications in many described cases ;however , this method seems to be cases ;however , this method seems to be risky and should be done with good risky and should be done with good justificationjustification..

* *Surgery is the main method , which on Surgery is the main method , which on the other hand may be difficult to the other hand may be difficult to perform due to multiplicity of lesionsperform due to multiplicity of lesions..