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Obscure Gi Bleeding

The document discusses obscure gastrointestinal bleeding (OGIB), which accounts for approximately 5% of GI bleeding cases and is often caused by small bowel pathology. It defines OGIB and categorizes it as either overt or occult bleeding. Common causes include lesions within reach but not seen on initial endoscopy, lesions that are difficult to visualize, and lesions in the small bowel beyond standard endoscopes. The document reviews challenges in evaluating OGIB and various imaging modalities used, including capsule endoscopy which has a diagnostic yield of 60% for OGIB. Repeat endoscopy, angiography, and enteroscopy are also summarized as potential diagnostic tools.

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0% found this document useful (0 votes)
83 views76 pages

Obscure Gi Bleeding

The document discusses obscure gastrointestinal bleeding (OGIB), which accounts for approximately 5% of GI bleeding cases and is often caused by small bowel pathology. It defines OGIB and categorizes it as either overt or occult bleeding. Common causes include lesions within reach but not seen on initial endoscopy, lesions that are difficult to visualize, and lesions in the small bowel beyond standard endoscopes. The document reviews challenges in evaluating OGIB and various imaging modalities used, including capsule endoscopy which has a diagnostic yield of 60% for OGIB. Repeat endoscopy, angiography, and enteroscopy are also summarized as potential diagnostic tools.

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bittuhimi24
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OBSCURE GI BLEEDING

Dr Debakanta Mishra
Introduction

• Obscure gastrointestinal bleeding (OGIB) is defined as GI


bleeding of uncertain cause after a nondiagnostic upper
gastrointestinal endoscopy,colonoscopy and barium small
bowel follow-through.
• Accounts for approximately 5% of all GI bleeding.
• Secondary to small bowel pathology in up to 75% of cases.
• Some experts recommend that this term be replaced by the
term ‘small bowel bleeding’
•Based on the presence or absence of clinically evident bleeding
categorised into:

–Obscure overt (visible blood)

–Obscure occult (positive FOBT or IDA)


• Three possibilities in Obscure GI bleeding

• The lesion was within reach of standard endoscope and


colonoscope but not recognised as bleeding site. E.g.

– Cameron’s lesion

– Angioectasiaa

– Internal hemorrhoids
• The lesion was within reach of the endoscope and colonoscope
but was difficult to visualise or present intermittently.e.g.
– Blood clot obscured visualization of the lesion
– Varices became inappropriate in a hypovolemic patient
– Lesion hidden behind mucosal fold
– Dieulafoy’s lesion

• The lesion was in the small intestine beyond the reach of the
standard endoscope.
Challenges Related to the Evaluation of “Obscure GI Bleeding (OGIB)”

• High miss rate for lesions on initial upper and lower


endoscopy
• The need for invasive intra-operative enteroscopy and
exploratory laparotomy to adequately examine the small
bowel
• Limited capacity of older diagnostic modalities to adequately
examine the small bowel
• Finding a lesion in the small bowel doesn’t always mean that
is the source of the problem
Causes of “Obscure GI Bleeding”
• Upper GI Tract Colon
– Cameron’s lesion Angioectasia
– Dieulafoy’s lesion Diverticulosis
Hemorrhoids
– Gastric antral vascular ectasia
varices
Small intestine
Angioectasia Aortoenteric fistula
Dieulafoy’s lesion Diverticulosis
Meckel’s diverticulum Neoplasm
Pancreatic or billiary disease Ulcerations
Causes of False-Positive Fecal Occult
Blood Test Results
Extra-intestinal blood loss
• Epistaxis
• Gingival bleeding
• Tonsillitis/pharyngitis
• Hemoptysis
Exogenous peroxidase activity
• Red meat consumption (nonhuman hemoglobin)
• Uncooked vegetable consumption (radish,
• cauliflower, broccoli, turnip, horseradish; less
• likely: cucumber, carrot, cabbage, potato,
• pumpkin, parsley, zucchini)
Clinical Clues for Specific Causes of
Gastrointestinal Bleeding
Clinical clue Likely cause of bleeding
 Age greater than 50 Carcinoma
 Chronic renal failure Vascular ectasia/angiodysplasia
 Cutaneous hemangiomas Blue rubber bleb nevus
syndrome
 Chronic diarrhea celiac sprue
 Abdominal pain celiac sprue
 HIV Kaposi’s
sarcoma,cytomegalovirus colitis
Imaging modalities
• Small bowel follow through
• Barium enteroclysis
• CT,CT enteroclysis/enterography
• CT Angiography
• MR enteroclysis/enterograpy
• Wireless Capsule endoscopy
• Enteroscopy
• Radiolabelled scanning
SMALL BOWEL FOLLOW-THROUGH
• SBFT has long been used to study the small intestine
• SBFT
 Low yield in OGIB
 Limited ability to distend the bowel & visualize the
mucosa
• Has a role only when malignancy or crohn’s disease is
suspected
BARIUM ENTEROCLYSIS
• Placement of naso-enteric tube
• Infusion of contrast at variable rate
• Insufflation of air → air contrast barium radiograph
• More accurate then SBFT (30 – 40 % sensitivity)
• Limitations
 Difficult to tolerate
 Inability to visualize mucosal angioectasias
 Not recommended for patients with acute bleeding
CT SCAN
• Has an important role in OGIB
• Advantage
 imaging extra-luminal, intramural & mucosal lesions
 demonstrate larger lesions
• Disadvantage
 Often misses, low grade tumors, small sinus tracts &
Fistulas,which are better demonstrated on
enteroclysis
 Radiation exposure
CT ENTEROGRAPHY
• CT enterography involves the ingestion of a neutral contrast
solution, such as polyethelene glycol, which also distends the bowel
• CT enteroclysis introduces contrast into the small bowel via a
nasojejunal tube
• Glucagonis often administered to reduce bowel peristalsis and
increase distension.
• Requires bowel preparation with a low-residue diet, fluids, and
cathartic agent.
• Multiphase CT scanning, such as arterial, enteric, and delayed
phases.
• Arterial phase -vascular lesions,
• Enteric phase -neoplastic lesions.
• Active bleeding is best seenon delayed phase as luminal contrast
extravasation.
• Advantages
• short scan time,
• non-invasive nature(enteroclysis is slightly more invasive)
• ready accessibility
• Disadvantage
• high amounts of radiation
• inadequate bowel distention with oral contrast due to patient intolerance
• bowel obstruction, or gastrointestinal dysmotility
• contraindication to the use of intravenous contrast in patients with renal
insufficiency or contrast allergy
CT Angiography

• IV contrast → image acquisition in enteric phase


• Identification – hyperattenuating extravasation into bowel
lumen not seen in unenhanced CT
• Useful in overt OGIB
CT Angiography
• Advantages
 etiologic diagnosis in many cases
 Determining endoscopic approach / intervention
 Can detect bleeding upto 0.5ml/min
 Identify rarer causes (jejunal varix/aortoenteric fistula)
 Sensitivity (70%) & specificity (100%)
 Yoon et al (sensitivity-90%,specificity-99% & accuracy-95%)
• Disadvantage
 Radiation
 Low yield in intermittent bleeding lesions
MRI ENTEROGRAPHY
• Depend on adequate bowel distension with oral contrast
agents, which may be negative, positive, or biphasic.
• Demonstration of bowel function using dynamic MR imaging
to create a cine clip of bowel peristalsis.
• Primarily studied in the realm of Crohn’s disease,
• Emerging data suggest a role in the investigation of obscure gi
bleeding, particularly when small bowel neoplasm is
suspected.
• There are limited data on diagnostic yield for obscure GI
bleeding,
• One study suggested lower sensitivity than CT techniques.
BLEEDING SCAN
• Involve the tagging of autologous erythrocytes with the
metastable isotope of technetium-99m (99mtc) and then
reinjecting the cells into the patient.
• Then imaged with a gamma camera to examine whether these
tagged cells are being deposited into the GI tract via a bleed.
• Can detect both arterial and venous bleeding
• Minimally invasive
• Detect blood flow rates as low as 0.04 ml/min
• 22% false-positive rate,
• Used for the purpose of guiding surgical and/or angiographic
treatment.
• Additionally, 99mtc pertechnetate scans can be helpful for the
diagnosis of ectopic gastric mucosa in a meckel’s
diverticulum, particularly in the pediatric population
CATHETER ANGIOGRAPHY
• Technique
 Femoral or radial approach
 Catherize vessel to be screened
 Nonionic contrast
• Indications
 Positive nuclearscan, postop patients,massive overt GI
bleed not amenable to endoscopic approach etc
• Advantages :
 ability to localize site of bleed
 Therapeutic intervention(coil embolization)
 Identify nonbleeding lesions on the basis of vascular
pattern
• Disadvantages
 Invasive nature
 Catheter related complications
 Intermittent bleeding
 Complications of pseudo-aneurysm, arterial thrombosis,
dissection, and bowel infarction

 Overall sensitivity of approximately 60 %


 Sensitivity can be improved with drug provocation
 ↑es with active ongoing bleed
SECOND-LOOK ENDOSCOPY
• May yield a bleeding source even when the initial EGD is
negative
• Suspected sources of bleeding were found within the reach of a
standard EGD in
• In 2.8% and 4.7% of patients during ce,
• In 26% of patients in studies that used push enteroscopy
• In 13.1% of patients undergoing DAE
• Factors associated with an increased yield of repeat EGD
include
– Large hiatal hernias,
– Hematemesis,
– History of NSAID use.
SMALL BOWEL CAPSULE
ENDOSCOPY
• Answer to the challenge of finding a non-invasive technology
to visualize the entire small intestine
• Originally approved in 2000
• Diagnostic capability of ce with respect to OGIB is 60.5%
• Most common diagnosis made with ce is angiodysplasia
(50%), followed by ulcers (26.8%) and neoplastic lesions
(8.8%)
• Many studies have shown that ce is far superior to PE and
radiographic imaging.
• A meta-analysis of 14 studies showed that the detection yield
for ce was 63%, which is far greater than that of both PE and
radiographic studies (26% and 8%, respectively)
• The diagnostic yield of ce is higher if performed within 2
weeks (greatest yield in 48 to 72 hours) of an overt bleeding
episode
• Timing of capsule endoscopy can influence the diagnosis and
outcomes in patients with small-bowel bleeding by identifying
patients for early intervention.
• Factors identifying patients with a greater chance of positive
results on ce
– Hemoglobin <10 g/dl,
– Longer duration of bleeding (>6 months)
– Presentation with overt bleeding,
– Male sex,
– Age >60 years,
– Inpatient status
• ce findings leading to endoscopic or surgical interventions or
change in medical management have been reported in 37% to
87% of patients.
• Repeat bleeding rates after a ce study with negative results are
generally low (6%-11%)
• If the ce study fails to identify the cause of small-bowel
bleeding, a second ce study may be considered, particularly at
the time of repeat bleeding.
• In a prospective study of 76 patients with persistent small-
bowel bleeding and initial nondiagnostic ce results, a second-
look ce showed positive results in 49% of patients.
• Limitations of ce
– Inability to provide direct therapy
– Inability to localize a lesion in the small intestine,
– Erratic passage of the capsule through the small bowel resulting
in missed lesions
– Not recommended for use in patients with pacemakers.
– Risk of retaining a capsule due to strictures, nsaid use, or
previous abdominal surgeries.
– Katsinelos et al., Found in a study that only 2/101 patients
(1.98%) retained capsules due to neoplasms and required
surgical intervention to remove both the capsule and neoplasm
• RELATIVE CONTRAINDICATION OF ce
– Pediatric patients,
– Patients with dysphagia
– Patients with prior altered surgical anatomy
– With the development of a capsule endoscope delivery
device, placement of the small bowel pillcam capsule
endoscope can be safely and successfully performed with
deployment directly into the small bowel
PUSH ENTEROSCOPY
• Does not involve the use of a balloon overtube.
• Passed only to about 50–150 cm distal to the ligament of
treitz.
• Identifies potential bleeding site in 40-50% of patients out of
which 50% are within reach of standard endoscope.
• Difficult to prevent looping and associated with patient
discomfort.
• Limited to endoscopic therapeutics in patients who have
proximal small bowel lesions seen on capsule endoscopy.
• SONDE ENTEROSCOPY
• Dependent on peristaltic propagation of a flexible enteroscope
through the SB.
• No longer utilised in clinical practice due to patient discomfort
and longer duration inability to perform biopsies or deliver
therapy.
DEVIce ASSISTED ENTEROSCOPY
• Encompasses both BAE (ie, single-balloon system,double-
balloon systems) and spiral enteroscopy.
• Total enteroscopy may be achieved through a combination of
antegrade and retrograde approaches
• In multiple large studies the diagnostic yield ranged from 43%
to 81%.
• A meta-analysis of 11 studies comparing the yield of ce and
double-balloon enteroscopy (DBE), including 375 patients
with smallbowel disease, reported comparable diagnostic
yields(60% vs 57%, respectively; p z .42) for all indications.
• However, a recent meta-analysis suggested a higher yield for
DBE when it was performed after a ce with a positive result as
compared with a negative result (75% vs 27.5%; p z .02).
• Limited data are available in reference to the role of early
DBE in the management of overt small-bowel bleeding.
• A recent study reported a high diagnostic and therapeutic yield
(90%) with early (within 24 hours) DBE in 10 patients with
overt small-bowel bleeding.
• Another retrospective study showed a higher diagnostic yield
and lower incidence of recurrent bleeding with urgent DBE
(within 72 hours after the last visible GI bleeding) compared
with non-urgent DBE (diagnostic yield of 70% vs 30%; p
< .05) in 120 patients with obscure smallbowel bleeding.
• These studies suggest that early intervention with DBE may
yield better outcomes.
• The diagnostic yield of single-balloon enteroscopy ranged
from 58% to 74%
• Initial series reported a low diagnostic yield with spiral
enteroscopy (33%), but a more recent prospective study
reported a higher yield (57%-62%).
• A modeled cost-minimization analysis of the management of
small-bowel bleeding proposed BAE as the most cost-effective
initial test after standard endoscopy if the goal is treatment or
definitive diagnosis, as opposed to visualization alone.
SPIRAL ENTEROSCOPY

• It involves use of an overtube which has grooves, and by


rotating the overtube, it pleats the small bowel.
• The endoscopy can thus be advanced and one can achieve
deep intubation of the small bowel.
• The advantage of the system is the rapid deep intubation, thus
reducing the procedure time.
• The main disadvantages are the requirement of two operators
– one to rotate the overtube and the other to advance the tube.
• Also, in a recent prospective cross-over trial, it was noted that
the depth of small bowel intubation with spiral enteroscopy
was shorter than DBE.
INTRAOPERATIVE ENTEROSCOPY

• Considered as a last resort


• Endoscopic evaluation can be performed orally, rectally,
and/or through enterotomies at the time of surgery.
• Diagnostic yields between 58% and 88%.
• Common adverse events include serosal tears, avulsion of
mesenteric vessels, and prolonged ileus.
• Reserved for rare cases in which other modalities have failed
to identify a lesion
Surgery for OGIB

Indicated for patients who continue to bleed and have had


negative endoscopic and radiologic workups, and are
operative candidates
 Surgery is recommended in patients:
• Patients require blood transfusions despite optimal iron
replacement
• Have associated symptoms such as weight loss or
abdominal pain
• Have a history of tumors that could metastasize to the
small bowel (i.e., lung, melanoma, breast, kidney)
 Overall yield: 70-93%, however, rebleeding may occur in up
to 50% of patients
Gastro 118:201-221, 200
PHARMACOLOGIC PROVOCATION
• Endoscopy combined with the administration of antiplatelet and/or
anticoagulant agents to stimulate bleeding in order to define a
source.
• A retrospective review of 824 dae procedures was completed to
identify a total of 38 instances in which provocation was attempted
in 27 patients.
• The diagnostic yield of provocative testing per procedure was 53%
in the provocation-experienced group, 27% in the provocation-naïve
group, and 71% in the full protocol group.
• Provocative testing was revealing in 15 of 27 patients; angioectasias
and dieulafoy lesions were the most common pathologies
• This novel technique should be considered only after standard
management has failed to define a bleeding source, and bleeding
continues to recur.
• In the 2015 ACG guideline, the term obscure GI bleeding was
replaced by the term small bowel bleeding,
• The term obscure reserved for patients without a source identified
on ce, deep enteroscopy, or enterography examination.
• The reason for this change in terminology was related to several
advancements:
• The literature on repeat endoscopic examinations of the upper
and/or lower tracts demonstrated findings in 30% to 40% of patients,
• Utilization of ce or deep enteroscopy detected lesions in the small
bowel in50%to60%of patients, and
• In patients with normalce or enteroscopy examinations, disease is
found on CTE or MRE in 40% to 50% of patients.
TREATMENT
Pharmacological therapy
Hormonal therapy (estrogens and progesterone)
• Estrogens and progesterone therapy has been widely used in
OGIB, with contradictory results, although some reports
have observed a significant reduction in transfusion
requirements
– hereditary hemorrhagic telangiectasia,
– vonWillebrand disease,
– chronic kidney failure and gastric antral vascular ectasia
(GAVE)

Sánchez-Capilla AD World J Gastro&Pathophysiology . 2014 Aug:271-83.


Somatostatin analogs
• Octreotide reduces splancnic arterial flow by inhibiting
angiogenesis and endothelial related growth factors.
• Octreotide can also inhibit angiogenesis by inhibiting
endothelial cell proliferation

Non-selective beta-blockers
• They reduce splancnic flow, pulse and cardiac output. They
are usually used in portal hypertension related OGIB

Thalidomide
• It also displays antiangiogenic activity, which may be useful
for the treatment of gi bleeding.
• Have shown some benefit in eliminating the need for blood
transfusions and iron supplementation in patients with chronic
blood loss from angiectasias
• Other drugs :
– Antifibrinolytics like tranexemic acid
– Danazol
– Desmopressin and
– Recombinant factor VII for massive GI bleed.
Endoscopic therapy

• Argon plasma coagulation


–APC has been used for a variety of bleeding lesions,
including angiodysplasia,
–in these lesions submucosal saline injection prior to
treatment with APC may protect against deep wall injury.

• Electrocoagulation
– Bipolar or heater probe coagulation is effective for
treatment of angiodysplasia in the colon or upper
gastrointestinal tract
– Risk of perforation increases in small bowel beyond
duodenum
• Mechanical hemostasis
– Endoscopic clips have the advantage of avoiding tissue
injury, which may be particularly desirable in patients
taking anticoagulants and/or antiplatelet agents, or in
patients with coagulation defects.

– Band ligation for Dieulafoy lesion


ANGIOGRAPHY

• Agents used for embolization include biodegradable gelatin


sponge, polyvinyl alcohol particles, liquid agents, and
metallic coils
• Microcoils , preferred agent for embolizing bleeding
vessels and can be deployed by means of a micro catheter
to the site of bleeding.
• Complications include
– hematomas, arterial thrombosis, dissection, embolism,
pseudo aneurysm formation, and bowel infarction.
Infusion of vasoactive drugs (vasopressin)
– Vasopressin causes generalized vasoconstriction
– used with caution in patients with coronary artery
disease, congestive cardiomyopathy, severe
hypertension, or severe peripheral vascular disease.
– Other side effects arrhythmia and hyponatremia due
fluid retention
• Recent advance
– Hemostatic powder spray for GI bleeding
– The powder is applied as a non-contact spray by
using a pressurized carbon dioxide canister for
propulsion via a through-the-scope delivery catheter

Wong Kee Song LM, Banerjee S, Barth BA, et al. Emerging technologies for endoscopic hemostasis. Gastrointest Endosc
2012;75:933-7.
ASGE GUIDELINE
• For patients with signs or symptoms consistent with recurrent
upper or lower GI sources of bleeding, EGD and colonoscopy,
should be repeated respectively, before small-bowel
evaluation.
• ce should be done as the initial test for patients with overt or
occult small-bowel bleeding. Positive ce results should be
followed with push enteroscopy if within reach or DAE.
• DAE or push enteroscopy should be done if ce is unavailable
or nondiagnostic in patients with overt smallbowel bleeding.
• In select circumstances (eg, high levelof suspicion of small-
bowel angiectasias or in patients with surgically altered
anatomy) DAE may be considered as the initial small-bowel
diagnostic procedure in patients with small-bowel bleeding
• After an appropriate negative evaluation, clinically stable patients
without recurrent bleeding may be treated with iron therapy and
clinically followed if iron deficiency is present.
• Multiphase CTE or MRE in patients with obscure bleeding and
suspected small-bowel neoplasms.
• Following appropriate hemodynamic resuscitation, angiography
for selective embolization in patients who present with
hemodynamically unstable suspected small-bowel bleeding.
• CTA or RBC scan should be done for localization of the bleeding
site and to guide timing of angiography in hemodynamically
stable patients with suspected active small-bowel bleeding.
THANK YOU
ANGIODYSPLASIA
• Angioectasias or angiodysplasias, are aberrant blood vessels
found throughout the GI tract that develop with advancing
age( > 60).
• More frequently found in the colon
– cecum - 37%;
– Ascending colon - 17%
– Transverse colon - 7%
– Descending colon - 7%
– Sigmoid colon - 18%
– Rectum - 14%
PATHOGENESIS

Some attribute angiodysplasia due to a mild chronic venous


obstruction. This hypothesis is concordant with the
observation of a higher number of these lesions in the right
colon, where the wall tension is higher

•mucosal chronic ischemia, which could appear in episodes of


bowel obstruction
•local ischemia
•patients with heart, vascular or lung disease
•younger patients, could be congenital or associated to
hereditary syndromes;
On endoscopy, angioectasias appear as 2- to 10-
mm red lesions, with arborizing ectatic blood
vessels that emanate from a central vessel

.Application of pressure on an angioectasia with


an endoscopic probe may cause the lesion to
blanch.
• Usually, lesions are multiple in a given
segment of intestine.
• ~20% of patients have angioectasias in at
least 2 sections of the GIT.
• Associated with
– chronic kidney disease( 47%)
– cirrhosis,
– rheumatoid disorders,
– and cardio vascular disease
Cardiovascular disease, such as mild- moderate AS, aortic sclerosis,
hypertrophic cardiomyopathy, and
peripheral vascular disease may contribute to bleeding angioectasia
( high shear stress disrupt vWF )

• Acquired von Willebrand’s disease associated with selective loss


of the largest multimeric forms of vWF (explain why bleeding
occurs in pts with angioectasia)

• Angiodysplasia size (> 1 cm) and number (> 10) is related with a
higher mortality (20% vs 4% and 25% vs 0%) respectively.

• HEYDE SYNDROME: Aortic stenosis has been associated with GI


bleeding from angioectasias
• Treatment :
– Endoscopic treatment of angioectasia can be
performed with various modalities , including injection
with epinephrin ,thermal probe coagulation ,argon
coagulation & EVL
– Hormonal therapy with estrogen is suggested to have a
benefit in controlling bleeding from angioectasia with
CKD
– Thalidomide ( angiogenesis inhibitor) may be effective
in selected pts with vascular malformations
• A study revealed that thalidomide treated pts had a
significant decrease in the number of bleeding episodes,
transfusions & hospitalizations
Meckel’s Diverticulum
• A Meckel’s diverticulum is a congenital blind intestinal
pouch that results from incomplete obliteration of the
vitelline duct during gestation.
• RULE OF TWO
– 2% population,
– 2 feet of the ileocecal valve,
– 2 inches long,
– 2% complicate,
– 2 ectopic tissue (gastric and pancreatic)
– 2 years of age : usual presentation
– > 2:1 M:F ratio
Most common complications are bleeding,
obstruction, and diverticulitis

• The diagnostic test for a Meckel’s diverticulum


is a 99mTc pertechnetate scan (Meckel’s scan),
because technetium pertechnetate has an
affinity for gastric mucosa

Specificity is 90% in those with ectopic gastric


mucosa
Fibro
us
cord

Umbili
Umbilic
cal
al cyst
sinus
NSAID ULceR
• NSAID enteropathy : increased intestinal
permeability, inflammation, and subtle bleeding

• single or multiple and range from tiny punched-


out ulcers to confluent areas of deep ulcer with
stricture formation
• PATHOGENESIS
disturbance in the microcirculation of the villi –
loss of enterocytes
Pathogenesis
Small bowel tumors
• Leiomyoma
• Polyps
• Neoplasms
Dieulafoy’s lesion
• Etiology is unknown
• Lesions are normally found in the proximal stomach, in the
lesser curvature, near the GE junction.

• It is usually a submucosal, dilated, aberrant vessel that


erodes the overlaying mucosa without a previous ulcer.

• Caused by the lack of ramification of the submucosal artery


which makes its diameter ten times the normal diameter of
a mucosa capillary.

• Cardiovascular diseases , HTN, CKD, DM or alcohol abuse.


• Aberrant vessel cannot be seen unless it bleeds actively.
Dieulafoy s lesion
Hereditary hemorrhagic telangiectasia
(HHT)
• Osler-Weber-Rendu disease,
• Rare AD inherited disease
• occurs in ~ 1 in 5000 to 8000 people.
• Telangiectasias on the lips, oral mucosa, and fingertips.
• Clinical diagnosis of HHT
3 of the following 4 criteria:
1) Family history( 1st degree relative with HHT)
2) Recurrent nosebleeds
3) Mucocutaneous telangiectasia and
4) AVM in the brain, lung, liver and GI tract.
• Epistaxis : MC presenting symptom.
• The detection of > 5 telangiectasias had a
sensitivity of 75%, PPV of 86% for diagnosis of HHT.
• In GIT - MC site is small intestine ( duodenum &
proximal jejunum )
• Mutations occur in at least four genes
– ENG [encodes endoglin],
– ALK-1 [encodes activin receptor-like kinase 1],
– MADH4 and HHT3
• HHT can be lifethreatening because of embolic
strokes or brain abscesses
related to the pulmonary and cerebral AVMs.
Blue Rubber Bleb Nevus Syndrome
• Rare syndrome , only 200 cases reported till 2012.
• Venous malformations in the skin, soft tissues, and GI tract.
• Usually presents as iron def. anemia or occult GI bleeding.
• Bluish nodules measure from 0.5 to 1.5 cm and tend to refill
after compression
• Endoscopy :lesions appear as large protuberant polypoid
venous blebs
• Can occur anywhere in the GI tract, but especially in the
small bowel and colon
• Treatment - endoscopic band ligation or surgical resection

Zahedi MJ, Darvish Moghadam S, Seyed Mirzaei SM, Dehghani M, Shafiei Pour S, Rasti A.Middle East J Dig Dis. 2013 Oct;5(4):235-9.
Blue Rubber Bleb Nevus
Syndrome

Zahedi MJ, Darvish Moghadam S, Seyed Mirzaei SM, Dehghani M, Shafiei Pour S, Rasti A.Middle East J Dig Dis. 2013
Oct;5(4):235-9.
Cameron’s lesions
Linear gastric ulcers or erosions on the mucosal folds
at the diaphragmatic impression in patients with a large
hiatal hernia
• Mechanical trauma, ischemia, and acid mucosal injury
may play a role in the pathogenesis of Cameron
lesions.
• Prevalence of Cameron lesions seems to be dependent
on the size of the hernia sac, increased prevalence with
larger hernia sac.
• Found in 5.2% of patients with hiatal hernias.
• In about two thirds of the cases, multiple Cameron
lesions are noted rather than a solitary erosion or ulcer.
• Presentation :
– asymptomatic ( M C)
– acute GI bleed ( 1/3rd ),
– Chronic GI bleed with iron def. anemia.

• Concomitant acid peptic disease like reflux


esophagitis is seen in majority.

• Treatment : high dose PPI, Endoscopic therapy for


bleeders.
Hemosuccus Pancreaticus
• Definition: Bleeding from the pancreatic duct; rare cause of UGIbleeding.
• Causes: chronic pancreatitis, pancreatic pseudocysts, and pancreatic tumors.
• Pathogenesis:
– Pseudocyst or tumor erodes into a vessel, forming a direct communication between the pancreatic
duct and a blood vessel.
– May be seen after therapeutic endoscopy of the pancreas or pancreatic duct,
including pancreatic stone removal, pancreatic duct sphincterotomy,
pseudocyst drainage, or pancreatic duct stenting.
• Diagnosis: confirmed by abdominal CT scan, ERCP, angiography or intraoperative
exploration.
• CT scan is performed first (least invasive).
• Treatment:
– Mesenteric arteriography with coil embolization can control acute bleeding.
– If bleeding persists or is massive: pancreaticoduodenectomy or pseudocyst
resection and ligation of the bleeding vessel

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