GI Board Review GI Board Review

EsophagusEsophagus

GERDGERD

Middle aged overweight male with retrosternal Middle aged overweight male with retrosternal burning and regurgitation worse with citrus and burning and regurgitation worse with citrus and smoking. smoking.

GERDGERD

SymptomsSymptoms Retrosternal burning – post Retrosternal burning – post

prandial/recumbantprandial/recumbant RegurgitationRegurgitation Dysphagia Dysphagia Water BrashWater Brash Chronic CoughChronic Cough

Historical clues: Pregnancy, Scleroderma, Historical clues: Pregnancy, Scleroderma, Obesity, hiatal herniaObesity, hiatal hernia

Gold standard – 24 hour pH probeGold standard – 24 hour pH probe

GERDGERD

Complications Complications Barrett’sBarrett’s AdenocarcinomaAdenocarcinoma Strictures/RingsStrictures/Rings Hoarseness/AsthmaHoarseness/Asthma

Empiric treatment – Sensitivity of 80%Empiric treatment – Sensitivity of 80% When refer for endoscopy?When refer for endoscopy?

Evaluate for Barrett’s/anatomyEvaluate for Barrett’s/anatomy Don’t respond to therapyDon’t respond to therapy Alarm symptoms – dysphagia, bleeding, Alarm symptoms – dysphagia, bleeding,

weight loss, anemia, odynophagiaweight loss, anemia, odynophagia ChronicChronic

GERD - treatmentGERD - treatment On the Boards, remember to be cost On the Boards, remember to be cost

effectiveeffective Lifestyle modificationLifestyle modification (Weight loss most important and (Weight loss most important and

avoidance of foods that cause LES relaxation i.e. peppermint, chocolate, avoidance of foods that cause LES relaxation i.e. peppermint, chocolate, alcohol, fatty foods)alcohol, fatty foods)

Acid suppressionAcid suppression PPIPPI > H2 blocker(80% control symptoms) > H2 blocker(80% control symptoms) PPI better in endoscopically proven esophagitisPPI better in endoscopically proven esophagitis

PromotilityPromotility – – Reglan/Cisapride (Minimal Data)Reglan/Cisapride (Minimal Data) SurgerySurgery – – Nissen FundoplicationNissen Fundoplication

Equivalent to PPI therapy – 0.2% mortalityEquivalent to PPI therapy – 0.2% mortality 2/3 will be on acid suppression in 5 years2/3 will be on acid suppression in 5 years No evidence that prevents Barrett's or CANo evidence that prevents Barrett's or CA

Endoscopic Therapy (Endoscopic Therapy (Stretta, Endocinch, etc) Stretta, Endocinch, etc) Rarely performedRarely performed..

Barrett’s EsophagusBarrett’s Esophagus

55 year old white male with 10 55 year old white male with 10 years of pyrosis, mildly improved years of pyrosis, mildly improved over past year, on PPI daily.over past year, on PPI daily.

Barrett’s EsophagusBarrett’s Esophagus Middle aged and older, M>F (2:1)Middle aged and older, M>F (2:1) WhitesWhites and Hispanics predominantly and Hispanics predominantly About 5-10% of patients with GERD (though in About 5-10% of patients with GERD (though in

multiple studies, also present in 0%-25% of multiple studies, also present in 0%-25% of asymptomatic patients) asymptomatic patients)

Defining characteristic: Change in squamous Defining characteristic: Change in squamous esophageal epithelium to intestinal metaplasiaesophageal epithelium to intestinal metaplasia

0.5% per person per year chance of developing 0.5% per person per year chance of developing adenocarcinomaadenocarcinoma

TreatmentTreatment Control GERD Symptoms – PPI vs. surgeryControl GERD Symptoms – PPI vs. surgery No therapy definitively shown to reduce risk of progression No therapy definitively shown to reduce risk of progression

to malignancy to malignancy Surveillance endoscopySurveillance endoscopy

Esophageal CancerEsophageal Cancer

75 year old female with history of tobacco 75 year old female with history of tobacco use and alcohol use with progressive solid use and alcohol use with progressive solid food dysphagia and 15 pound weight loss food dysphagia and 15 pound weight loss over past year.over past year.

Esophageal CancerEsophageal Cancer Essentially equal prevalence in Essentially equal prevalence in

United States of esophageal SCC United States of esophageal SCC and adenocarcinomaand adenocarcinoma

Squamous CellSquamous Cell – proximal esophagus – proximal esophagus SSmoking moking TTylosis ylosis AAchalasia chalasia PPlummer-Vinsonlummer-Vinson LLye ye EEthanol thanol SSprue/Sclerodermaprue/Scleroderma

Adenocarcinoma Adenocarcinoma – Distal esophagus– Distal esophagus GERD/GERD/Barrett’sBarrett’s, Obesity, Tobacco, Obesity, Tobacco

Esophageal CancerEsophageal Cancer

SymptomsSymptoms Progressive Solid Food DysphagiaProgressive Solid Food Dysphagia 75% also weight loss/anorexia75% also weight loss/anorexia

Endoscopy with Biopsy – DiagnosticEndoscopy with Biopsy – Diagnostic StagingStaging

1. CT chest/abd/pelvis vs. PET for Mets1. CT chest/abd/pelvis vs. PET for Mets 2. EUS for T and N staging2. EUS for T and N staging

DysphagiaDysphagia

Dysphagia

Solids Solids/liquids

Intermittent Progressive Intermittent Progressive

Ring or Stricture Esophageal CA DES Achalasia / Scleroderma

DysphagiaDysphagia

History and PhysicalHistory and Physical Solid vs. liquidSolid vs. liquid Intermittent vs. progressiveIntermittent vs. progressive Oropharyngeal vs. EsophagealOropharyngeal vs. Esophageal

Barium swallow (13mm pill)Barium swallow (13mm pill) EGD (with possible dilation)EGD (with possible dilation) Esophageal manometryEsophageal manometry

Peptic StricturePeptic Stricture

Progressive solid food dysphagiaProgressive solid food dysphagia History of GERDHistory of GERD No weight lossNo weight loss

Patients have normal appetitePatients have normal appetite Majority (60-70%) are peptic in Majority (60-70%) are peptic in

originorigin Result of chronic esophageal Result of chronic esophageal

inflammationinflammation

Esophageal RingEsophageal Ring

38 year old female with frequent heartburn, controlled on PPI, with intermittent solid food dysphagia.

AchalasiaAchalasia

35 year old male with progressive solid and liquid food dysphagia and fatigue, with regurgitation of undigested food.

AchalasiaAchalasia “failure to relax”“failure to relax” 1/100,0001/100,000 M:F 1:1M:F 1:1 Age 25-60 (If older>60, think Age 25-60 (If older>60, think

pseudoachalasia pseudoachalasia especially gastric cancerespecially gastric cancer)) Increased risk for squamous cell cancerIncreased risk for squamous cell cancer Hallmarks:Hallmarks:

AperistalsisAperistalsis Failure of LES to relaxFailure of LES to relax Dysphagia to solid and liquidDysphagia to solid and liquid Postural changes to help swallowingPostural changes to help swallowing Regurgitation of undigested foodRegurgitation of undigested food

Autoimmune vs. Viral Autoimmune vs. Viral Chagas Disease (Chagas Disease (Trypanosoma cruzei)Trypanosoma cruzei)

Achalasia - diagnosisAchalasia - diagnosis

Barium SwallowBarium Swallow – Dilated esophagus – Dilated esophagus with column of barium and “Birds Beak” with column of barium and “Birds Beak” taper. Test of choice if suspectedtaper. Test of choice if suspected

EndoscopyEndoscopy – rule out pseudoachalasia – rule out pseudoachalasia ManometryManometry (Used to confirm diagnosis) (Used to confirm diagnosis)

(1)Loss of peristalsis, (1)Loss of peristalsis, (2)failure of LES to relax, (2)failure of LES to relax, (3)possibly LES high pressure(3)possibly LES high pressure

Chest X-rayChest X-ray – wide mediastinum and air – wide mediastinum and air fluid levelfluid level

Eosinophilic EsophagitisEosinophilic Esophagitis

20 year old male with history of asthma and eczema with recurrent food impactions.

Eosinophilic EsophagitisEosinophilic Esophagitis

Atopic history and food impactionsAtopic history and food impactions Ringed esophagus / linear furrowsRinged esophagus / linear furrows >15 eosinophils per high power field>15 eosinophils per high power field Some (minority) have peripheral Some (minority) have peripheral

eosinophiliaeosinophilia Oral fluticasone 220 mcg/puff 2 puffs bid for Oral fluticasone 220 mcg/puff 2 puffs bid for

6-8 weeks. Recurrence frequent.6-8 weeks. Recurrence frequent. Other possible treatments:Other possible treatments:

PPI, singulair (large doses up to 100 mg), PPI, singulair (large doses up to 100 mg), elimination diets (children predominantly) and elimination diets (children predominantly) and dilationdilation

SclerodermaScleroderma

Historical Key: 70 year old woman with Historical Key: 70 year old woman with sclerodactyly telangiectasias, Raynaud’s sclerodactyly telangiectasias, Raynaud’s with GERD, resistant to PPIwith GERD, resistant to PPI 75% have esophageal involvement75% have esophageal involvement Severe GERD, frequently resistant to PPISevere GERD, frequently resistant to PPI Incompetent LES and lack of peristalsisIncompetent LES and lack of peristalsis Control GERD with PPIControl GERD with PPI

StomachStomach

Upper GI bleedUpper GI bleed

PresentationPresentation

Hematemesis Hematemesis (Not to be confused with (Not to be confused with hemoptysis)hemoptysis)

Melena (Black, Tar-Like – not solid)Melena (Black, Tar-Like – not solid) Nausea/vomiting common with PUDNausea/vomiting common with PUD Orthostasis (Bedside orthostatics)Orthostasis (Bedside orthostatics) Abdominal painAbdominal pain Hematochezia Hematochezia (10% of maroon stool from (10% of maroon stool from

upper source – On test will be unstable)upper source – On test will be unstable)

Upper GI BleedUpper GI Bleed Peptic Ulcer Disease – 55%Peptic Ulcer Disease – 55% Esophageal Varices – 14%Esophageal Varices – 14% AVM’s / GAVE – 6%AVM’s / GAVE – 6% Mallory-Weiss tear – 5%Mallory-Weiss tear – 5% Dieulafoy’s – 1%Dieulafoy’s – 1% Cameron’s lesionCameron’s lesion TumorsTumors Esophagitis (Most common cause of UGIB Esophagitis (Most common cause of UGIB

in hospitalized patients, likely due to NGT in hospitalized patients, likely due to NGT and reflux in supine position)and reflux in supine position)

Peptic Ulcer DiseasePeptic Ulcer Disease

65 year old female with arthritis, taking ibuprofen, with melena and lightheadedness.

Risk Factors for NSAID-Risk Factors for NSAID-induced Ulcersinduced Ulcers

Definite:Definite: Prior PUDPrior PUD Advanced ageAdvanced age Concomitant use of Concomitant use of

glucocorticoidsglucocorticoids Concomitant use of Concomitant use of

anticoagulantsanticoagulants High doses or High doses or

combinations of NSAIDs, combinations of NSAIDs, including low-dose aspirinincluding low-dose aspirin

Comorbid illness (RA, Comorbid illness (RA, CAD, etc)CAD, etc)

Ethanol useEthanol use

Possible:Possible: H. pyloriH. pylori infection infection smokingsmoking

Helicobacter pyloriHelicobacter pylori

Most peptic ulcers caused by Most peptic ulcers caused by Helicobacter Helicobacter pyloripylori 60-80% of GU’s and 90% DU’s60-80% of GU’s and 90% DU’s

2 clinical presentations of 2 clinical presentations of H. pyloriH. pylori Antrum predominantAntrum predominant

Increased acid production, Duodenal Ulcers, no cancerIncreased acid production, Duodenal Ulcers, no cancer Body predominantBody predominant

Decreased acid production, Gastric Ulcers, Gastric Decreased acid production, Gastric Ulcers, Gastric Cancer (<1% of those infected, Cag A strain)Cancer (<1% of those infected, Cag A strain)

Eradication of Hp dramatically decreases Eradication of Hp dramatically decreases PUD and its complicationsPUD and its complications

Tests for H. pyloriTests for H. pylori Serologic AntibodySerologic Antibody (90% sens / 90% spec)(90% sens / 90% spec)

Remains positive for several yearsRemains positive for several years Do not use for evaluation for eradicationDo not use for evaluation for eradication

Endoscopy with Histology Endoscopy with Histology (95% sens / 98% spec)(95% sens / 98% spec) Endoscopy w/Rapid Urease Test (CLO) Endoscopy w/Rapid Urease Test (CLO) (90% (90%

/98%)/98%) Urease Breath TestUrease Breath Test (C13 / C14) (C13 / C14) (95% / 95%)(95% / 95%)

Best test for eradicationBest test for eradication Stool Antigen Stool Antigen (92% / 90%)(92% / 90%) All tests (except serology) less reliable if on All tests (except serology) less reliable if on

PPI in last 2 weeks, or antibiotics or bismuth PPI in last 2 weeks, or antibiotics or bismuth in past 4 weeks.in past 4 weeks.

Rx of Rx of H. pyloriH. pylori

Standard : Amoxicillin, Standard : Amoxicillin, Clarithromycin, PPI bid x 14 daysClarithromycin, PPI bid x 14 days

75-80% eradication rate75-80% eradication rate Major antibiotic resistance to Major antibiotic resistance to

clarithromycin and metronidazole.clarithromycin and metronidazole. If allergic to PCN, substitute If allergic to PCN, substitute

metronidazole for amoxicillinmetronidazole for amoxicillin

Peptic Ulcer Disease – Peptic Ulcer Disease – Rebleed Risk (within 72 Rebleed Risk (within 72

hours)hours)BaselineBaseline PPI PPI/EGD PPI PPI/EGD

TxTx

Clean BaseClean Base 3-5%3-5% -------- ---- ----

Pigmented SpotPigmented Spot 7-10%7-10% -------- ---- ----

Adherent ClotAdherent Clot 22-30%22-30% 0%0% 6.7% 6.7%

Visible VesselVisible Vessel 43-52%43-52% 12%12% 6.7% 6.7%

Active BleedingActive Bleeding 55-90%55-90% 73%73% 6.7%6.7%

Zollinger-EllisonZollinger-Ellison

Gastrin producing neuroendocrine tumorGastrin producing neuroendocrine tumor 1% of PUD (Never seen clinically but all 1% of PUD (Never seen clinically but all

over board exams)over board exams) 90% will have PUD (frequently solitary 90% will have PUD (frequently solitary

duodenal ulcer but may be multiple and in duodenal ulcer but may be multiple and in unusual places i.e. jejunum) unusual places i.e. jejunum)

Frequently with abdominal pain and Frequently with abdominal pain and chronic secretory diarrheachronic secretory diarrhea

70% Duodenum/30% Pancreas70% Duodenum/30% Pancreas 1/3 metastatic at diagnosis1/3 metastatic at diagnosis

Zollinger - EllisonZollinger - Ellison Fasting Gastrin >1000 diagnostic if acidic pH Fasting Gastrin >1000 diagnostic if acidic pH

in stomach (separate low acid states in stomach (separate low acid states (atrophic gastritis and pernicious anemia) (atrophic gastritis and pernicious anemia) from ZE)from ZE) 150-1000 abnormal but can be secondary to meds 150-1000 abnormal but can be secondary to meds

(PPI) or H pylori causing atrophic gastritis(PPI) or H pylori causing atrophic gastritis Secretin Stimulation Test (secretin infusion Secretin Stimulation Test (secretin infusion

promotes gastrin release by gastrinoma cells promotes gastrin release by gastrinoma cells but not gastric G cells)but not gastric G cells) Positive test - Increased Gastrin by at least 120-Positive test - Increased Gastrin by at least 120-

200 pg/ml within 20 minutes after secretin infusion200 pg/ml within 20 minutes after secretin infusion Localize with octreoscan/EUS of pancreasLocalize with octreoscan/EUS of pancreas

MEN1MEN1 Pancreatic islet cell tumors seen with Pancreatic islet cell tumors seen with

MEN1 about 80% of time (parathyroid, MEN1 about 80% of time (parathyroid, pituitary, pancreas)pituitary, pancreas) 50-60% with MEN1 have gastrinoma, but…50-60% with MEN1 have gastrinoma, but… About 20% with gastrinoma have MEN1About 20% with gastrinoma have MEN1

Most common pancreatic islet cell Most common pancreatic islet cell tumor associated with MEN1 is a tumor associated with MEN1 is a “nonfunctioning” islet cell tumor (i.e. “nonfunctioning” islet cell tumor (i.e. releases hormone that does not cause releases hormone that does not cause symptoms like PPP)symptoms like PPP)

Gastric CancerGastric Cancer

Diffuse (Infiltrating cells, i.e. linitis Diffuse (Infiltrating cells, i.e. linitis plastica) vs. Intestinal Type (glandular)plastica) vs. Intestinal Type (glandular)

Most common in Far East (if Japanese or Most common in Far East (if Japanese or Korean patient with stomach complaint, Korean patient with stomach complaint, think of gastric cancer)think of gastric cancer)

Risk FactorsRisk Factors 11stst degree relative (3x) degree relative (3x) H. pylori – Chronic Atrophic GastritisH. pylori – Chronic Atrophic Gastritis Dietary – NitratesDietary – Nitrates TobaccoTobacco

Gastric Cancer EpidemiologyGastric Cancer Epidemiology

PancreasPancreas

Acute pancreatitisAcute pancreatitis Alcohol or gallstone predominant etiologiesAlcohol or gallstone predominant etiologies Drugs – DDI, diuretics, estrogen, valproic Drugs – DDI, diuretics, estrogen, valproic

acid, 5-ASA, azathioprine, TCN, sulfaacid, 5-ASA, azathioprine, TCN, sulfa Interstitial (85%) vs. necrotizing (15%)Interstitial (85%) vs. necrotizing (15%)

Organ failure in 10% vs. 54%Organ failure in 10% vs. 54% Mortality Rate 3% vs. 17% Mortality Rate 3% vs. 17% 33% of patients with sterile necrosis develop 33% of patients with sterile necrosis develop

infectedinfected necrosis necrosis 47% mortality with MSOF47% mortality with MSOF Interesting fact – Pain radiates to back only Interesting fact – Pain radiates to back only

about 50% of time.about 50% of time.

ComplicationsComplications

SIRS: ARDS, Shock, ARF, GI BleedSIRS: ARDS, Shock, ARF, GI Bleed Necrosis: InfectionNecrosis: Infection Pseudocyst/AbscessPseudocyst/Abscess Pancreatic Ascites, Fistula (pleural Pancreatic Ascites, Fistula (pleural

effusion)effusion) Chronic PancreatitisChronic Pancreatitis Splenic Vein ThrombosisSplenic Vein Thrombosis, ,

PseudoaneurysmPseudoaneurysm

TreatmentTreatment

Mild – NPO, pain control, fluid resuscitationMild – NPO, pain control, fluid resuscitation Severe pancreatitis – Likely ICUSevere pancreatitis – Likely ICU

Adequate pain relief, Adequate pain relief, Adequate IV fluid replacement, especially initially Adequate IV fluid replacement, especially initially

(decrease Hct over first 24 hours to reduce risk of (decrease Hct over first 24 hours to reduce risk of necrosis)necrosis)

ERCP for gallstone panc (cholangitis/jaundice) (suspect ERCP for gallstone panc (cholangitis/jaundice) (suspect if ALT or AST>3x ULN)if ALT or AST>3x ULN)

Nutritional supportNutritional support Enteral feeding better than TPN due to decreased Enteral feeding better than TPN due to decreased

episodes of hyperglycemia and sepsisepisodes of hyperglycemia and sepsis Current teaching to place feeding tube beyond Ligament Current teaching to place feeding tube beyond Ligament

of Treitz (controversial)of Treitz (controversial)

Enteral Nutrition and Severe Enteral Nutrition and Severe PancreatitisPancreatitis

0

2

4

6

8

10

12

14

16

septic complications*P<0.01

any complication**P<0.05

EnteralTPN

# PTS

Kalfarentos et al Br J Surg 1997; 84:1665

TreatmentTreatment

Severe pancreatitisSevere pancreatitis Contrast CT recommended at some point Contrast CT recommended at some point

beyond the first 3 days in severe beyond the first 3 days in severe pancreatitis to rule out necrotizing pancreatitis to rule out necrotizing pancreatitis.pancreatitis.

Otherwise, minimal role for early CT Otherwise, minimal role for early CT No role for prophylactic antibiotics with No role for prophylactic antibiotics with

sterile necrosis (controversial)sterile necrosis (controversial) If concern for infected necrosis (usually If concern for infected necrosis (usually

after 7 days), CT guided aspiration.after 7 days), CT guided aspiration.

Chronic pancreatitisChronic pancreatitis

Chronic PancreatitisChronic Pancreatitis

Chronic epigastric pain/maldigestion Chronic epigastric pain/maldigestion related to fibrotic pancreasrelated to fibrotic pancreas

Diagnosis usually made after disease is well Diagnosis usually made after disease is well established.established.

Most frequently associated with alcohol Most frequently associated with alcohol abuseabuse

Maldigestion with Maldigestion with steatorrheasteatorrhea/weight loss/weight loss Fat soluble vitamin and B12 deficiencyFat soluble vitamin and B12 deficiency DM common in advanced diseaseDM common in advanced disease

Chronic PancreatitisChronic Pancreatitis

Lipase and amylase normal or only Lipase and amylase normal or only slightly elevatedslightly elevated

May mimic pancreatic cancer or May mimic pancreatic cancer or autoimmune pancreatitis (IgG4, autoimmune pancreatitis (IgG4, ANA) with duodenal or biliary ANA) with duodenal or biliary obstructionobstruction

Complications: pseudocyst, splenic Complications: pseudocyst, splenic vein thrombosis, pancreatic cancer vein thrombosis, pancreatic cancer (4% lifetime risk)(4% lifetime risk)

Diagnosis (difficult)Diagnosis (difficult)

Clinically useful tests for CPClinically useful tests for CP

FunctionFunction StructureStructure

SecretinSecretin stim test stim test ERCP/EUSERCP/EUS

Bentiromide testBentiromide test CT CT scanscan

Serum trypsinogenSerum trypsinogen USUS

Fecal chymotrypsinFecal chymotrypsin KUBKUB

Fecal fatFecal fat

Sensitivity

TreatmentTreatment

Pain reliefPain relief Non-enteric coated pancreatic enzymes Non-enteric coated pancreatic enzymes

(Viokase) with PPI(Viokase) with PPI NarcoticsNarcotics Celiac plexus block (CT vs. EUS)Celiac plexus block (CT vs. EUS) ERCP with stent or stone removalERCP with stent or stone removal Surgical resection or Peustow Surgical resection or Peustow

procedureprocedure Maldigestion (steatorrhea)Maldigestion (steatorrhea)

Coated Pancreas enzyme (Creon)Coated Pancreas enzyme (Creon)

Pancreatic CancerPancreatic Cancer 22ndnd most common GI cancer and 4 most common GI cancer and 4thth most most

common cancer death in UScommon cancer death in US Rare before age 45, M>F, African Rare before age 45, M>F, African

Americans>WhitesAmericans>Whites 28,000 cases per year (27,000 deaths)28,000 cases per year (27,000 deaths) 85-90% originate from pancreatic ductal cells85-90% originate from pancreatic ductal cells

Rarer cancers of acinar cells or neuroendocrine cellsRarer cancers of acinar cells or neuroendocrine cells Painful or painless jaundice, acholic stool, dark Painful or painless jaundice, acholic stool, dark

urine, weight lossurine, weight loss Elevated CA 19-9Elevated CA 19-9 Diabetes frequently diagnosed within past 2 Diabetes frequently diagnosed within past 2

yearsyears

TreatmentTreatment Biggest risk factors – Hereditary Biggest risk factors – Hereditary

pancreatitis, smoking, BRCA-2, chronic pancreatitis, smoking, BRCA-2, chronic pancreatitispancreatitis

Only 20% resectable at diagnosisOnly 20% resectable at diagnosis SurgerySurgery

Head – Whipple procedure Head – Whipple procedure Tail – Distal pancreatectomy/SplenectomyTail – Distal pancreatectomy/Splenectomy Palliation – Intestinal/Biliary bypassPalliation – Intestinal/Biliary bypass

ERCP – Biliary/Duodenal Metal StentERCP – Biliary/Duodenal Metal Stent Chemo - 5FU and GemcitabineChemo - 5FU and Gemcitabine Role of XRT controversialRole of XRT controversial

Pancreatic Neuroendocrine Pancreatic Neuroendocrine TumorsTumors

InsulinomaInsulinoma Usually solitary, 5-10% malignancyUsually solitary, 5-10% malignancy HypoglycemiaHypoglycemia - 48 hour fast - 48 hour fast

GlucagonomaGlucagonoma Necrolytic migratory erythemaNecrolytic migratory erythema, weight loss, diarrhea, weight loss, diarrhea 75% malignancy rate75% malignancy rate

VIPoma (aka Verner-Morrison Syndrome or WDHA VIPoma (aka Verner-Morrison Syndrome or WDHA (Watery diarrhea, hypokalemic and achlorhydria)(Watery diarrhea, hypokalemic and achlorhydria) Secretory diarrhea, flushing, achlorhydria, Secretory diarrhea, flushing, achlorhydria,

hypokalemiahypokalemia Elevated fasting VIP LevelElevated fasting VIP Level

Increased risk of neuorendocrine tumors in MEN1, Increased risk of neuorendocrine tumors in MEN1, Von Hippel-Lindau, neurofibromatosis 1, tuberous Von Hippel-Lindau, neurofibromatosis 1, tuberous sclerosis sclerosis

CholedocholithiasisCholedocholithiasis

IOC

EUS us

ERCP

MRCP

Common Bile Duct Common Bile Duct stones/Cholangitisstones/Cholangitis

Obstructive jaundice WITH painObstructive jaundice WITH pain Rising LFTS after Lap Chole – either retained Rising LFTS after Lap Chole – either retained

stone in CBD vs. Bile leakstone in CBD vs. Bile leak Answer is ERCPAnswer is ERCP

CholangitisCholangitis Charcot TriadCharcot Triad: RUQ Pain, Fever, Jaundice: RUQ Pain, Fever, Jaundice Reynolds PentadReynolds Pentad : Shock, MS changes : Shock, MS changes ERCP ERCP stone extraction/biliary stent placementstone extraction/biliary stent placement IR placed percutaneous GB drain IR placed percutaneous GB drain Antibiotics : Floroquinolone or Unasyn/ZosynAntibiotics : Floroquinolone or Unasyn/Zosyn

GallstonesGallstones Presentation: Usually asymptomaticPresentation: Usually asymptomatic

Biliary colic, acute cholecystitis, CBD obstruction (acute Biliary colic, acute cholecystitis, CBD obstruction (acute elevation of ALT, AST, Alk phos and TB with direct elevation of ALT, AST, Alk phos and TB with direct predominance), pancreatitispredominance), pancreatitis

If no symptoms from stones, then no treatmentIf no symptoms from stones, then no treatment Type: 75-80% cholesterol stonesType: 75-80% cholesterol stones Risk factors: 4F’s - fat, fertile, female, fortyRisk factors: 4F’s - fat, fertile, female, forty

Ethnicity, rapid weight lossEthnicity, rapid weight loss 20% pigmented stones20% pigmented stones

Black – Black – hemolysishemolysis and cirrhosis (calcium bilirubinate) and cirrhosis (calcium bilirubinate) Brown – Anaerobic bile duct infections (Rare in US)Brown – Anaerobic bile duct infections (Rare in US)

Diagnosis: Ultrasound best, CT, MRCPDiagnosis: Ultrasound best, CT, MRCP Treatment – If symptomatic, cholecystectomy. If Treatment – If symptomatic, cholecystectomy. If

not surgical candidate, ursodiol.not surgical candidate, ursodiol.

Biliary Tree TumorsBiliary Tree Tumors Carcinoma of GB is Carcinoma of GB is

the leading cause of the leading cause of biliary tree tumors.biliary tree tumors.

Risks include:Risks include: GallstonesGallstones Choledochal cystsCholedochal cysts GB polyps (primarily GB polyps (primarily

single and >1 cm)single and >1 cm) Porcelain GBPorcelain GB

Cholangiocarcinoma-Cholangiocarcinoma-rare but increasing rare but increasing incidence. incidence.

Risks include: Risks include: UCUC PSCPSC ThorotrastThorotrast Choledochal cystsCholedochal cysts Clonorchis and Clonorchis and

OpisthorchisOpisthorchis

Treatment - Surgery in minority vs. Treatment - Surgery in minority vs. Palliative stentPalliative stent

ColonColon

Acute DiarrheaAcute Diarrhea

VirusVirus NorovirusNorovirus RotavirusRotavirus AdenovirusAdenovirus

ProtozoaProtozoa GiardiaGiardia Entamoeba Entamoeba

histolyticahistolytica Cryptosporidium Cryptosporidium

(HIV)(HIV) CyclosporaCyclospora

Toxin mediatedToxin mediated

BacteriaBacteria SalmonellaSalmonella CampylobacterCampylobacter ShigellaShigella E.Coli (0157:H7)E.Coli (0157:H7) C. DifficileC. Difficile YersiniaYersinia VibrioVibrio ListeriaListeria

NorovirusNorovirus

Nursing homes, hospitals, cruise ships, Nursing homes, hospitals, cruise ships, restaurantsrestaurants

Incubation 24-48 hours (also highest Incubation 24-48 hours (also highest shedding)shedding)

Abd cramps followed by vomiting and Abd cramps followed by vomiting and diarrheadiarrhea Nonbloody watery diarrheaNonbloody watery diarrhea

Myalgias and malaise, low grade feverMyalgias and malaise, low grade fever Lasts 48-72 hoursLasts 48-72 hours Symptomatic treatmentSymptomatic treatment

CampylobacterCampylobacter Food borne disease – 3 day incubationFood borne disease – 3 day incubation Typically with fever, severe abdominal pain Typically with fever, severe abdominal pain

(may mimic appendicitis) and bloody diarrhea(may mimic appendicitis) and bloody diarrhea Lasts on average 7 daysLasts on average 7 days Associated with Associated with Reactive Arthritis, Reactive Arthritis,

Guillain-BarreGuillain-Barre SyndromeSyndrome, and pericarditis, and pericarditis Treatment – Mainly supportive as disease Treatment – Mainly supportive as disease

usually self limitedusually self limited If severe, use Erythromycin (other If severe, use Erythromycin (other

possibilities include Flouroquinolones, possibilities include Flouroquinolones, Macrolides, and Aminoglycosides)Macrolides, and Aminoglycosides) Most resistant to septraMost resistant to septra

Salmonella Non-typhoidSalmonella Non-typhoid

Salmonella typhimuriumSalmonella typhimurium and and Salmonella Salmonella enteritidisenteritidis

Largest number of food borne outbreaks in Largest number of food borne outbreaks in USUS

EggsEggs, poultry, undercooked beef, and pet , poultry, undercooked beef, and pet reptiles and rodentsreptiles and rodents

Nausea, vomiting, diarrhea, fever, abd painNausea, vomiting, diarrhea, fever, abd pain 4-10 days of diarrhea4-10 days of diarrhea

Antibiotics have not been shown to be of Antibiotics have not been shown to be of benefit in routine casesbenefit in routine cases Flouroquinolones or bactrim in severe cases Flouroquinolones or bactrim in severe cases

or comorbidity (HIV)or comorbidity (HIV) May become chronic carriersMay become chronic carriers

EHEC (0157:H7)EHEC (0157:H7) Enterohemorrhagic Escherichia coliEnterohemorrhagic Escherichia coli

Undercooked ground beefUndercooked ground beef, petting zoos, daycare, petting zoos, daycare Differ from other E. coli in the production of Shiga ToxinsDiffer from other E. coli in the production of Shiga Toxins

Enter circulation and target endothelial cells causing Enter circulation and target endothelial cells causing vascular damage and prothrombotic statevascular damage and prothrombotic state

Incubation 3-4 daysIncubation 3-4 days >90% will have bloody diarrhea>90% will have bloody diarrhea

Abdominal PainAbdominal Pain Often lack a feverOften lack a fever

HUS:HUS: 6-9% --- 50% dialysis, 3-5% mortality 6-9% --- 50% dialysis, 3-5% mortality Treatment – SupportiveTreatment – Supportive

Avoid antimotility agents and antibiotics (risk of causing Avoid antimotility agents and antibiotics (risk of causing HUS)HUS)

ShigellaShigella Not susceptible to acid, so few organisms Not susceptible to acid, so few organisms

cause infectioncause infection Fever, abdominal cramps, mucoid or bloody Fever, abdominal cramps, mucoid or bloody

diarrheadiarrhea 3 day incubation, 7 day duration3 day incubation, 7 day duration Rare cause of HUS and reactive arthritisRare cause of HUS and reactive arthritis Associated with seizures and encephalopathy Associated with seizures and encephalopathy

in childrenin children Treatment – antibiotic recommended (FQ)Treatment – antibiotic recommended (FQ)

Reduce shedding and person to person Reduce shedding and person to person transmissiontransmission

Decrease fever and diarrhea by 2 daysDecrease fever and diarrhea by 2 days

Clostridium dificileClostridium dificile Associated with antibiotic usage, older age, Associated with antibiotic usage, older age,

and possibly PPIand possibly PPI NAP1/BI/027 strain with larger quantities NAP1/BI/027 strain with larger quantities

of toxins A and B, worse outcomes, and of toxins A and B, worse outcomes, and associated with leukemoid reaction.associated with leukemoid reaction. Think C diff in inpatient on antibiotics with Think C diff in inpatient on antibiotics with

WBC 20K.WBC 20K. Treatment - Fluids, Avoid/Hold antibioticsTreatment - Fluids, Avoid/Hold antibiotics

Metronidazole 500mg TID or 250mg QIDMetronidazole 500mg TID or 250mg QID Oral Vancomycin 125 – 250 mg Oral Vancomycin 125 – 250 mg popo QID QID 20% Relapse rate – retreat with Flagyl or 20% Relapse rate – retreat with Flagyl or

VancomycinVancomycin

Other Infectious CausesOther Infectious Causes Amebiasis (Entamoeba histolytica)Amebiasis (Entamoeba histolytica)

Invade and penetrate colonic mucosaInvade and penetrate colonic mucosa Subacute moderate diarrhea with abdominal pain Subacute moderate diarrhea with abdominal pain

and bloody stools and weight lossand bloody stools and weight loss Treatment – Metronidazole 500-750 mg tid for 7-Treatment – Metronidazole 500-750 mg tid for 7-

10 d10 d Giardia lamblia (Chronic, large volume)Giardia lamblia (Chronic, large volume)

Contaminated water source (stream, well), Contaminated water source (stream, well), or person to person (daycare, MSM)or person to person (daycare, MSM)

Watery diarrhea, malaise, steatorrhea, abd cramps Watery diarrhea, malaise, steatorrhea, abd cramps and bloatingand bloating

Treatment: Metronidazole 250 mg tid for 5 daysTreatment: Metronidazole 250 mg tid for 5 days

Chronic DiarrheaChronic Diarrhea

Chronic Diarrhea Chronic Diarrhea (>4wks)(>4wks)

Diarrhea

Watery Inflammatory Fatty

Osmotic Secretory

Initial Testing: Large Volume (Small Bowel) vs. Small Volume (colon)

1. Stool Osmolar Gap = 290 – 2(Na +K)

<50 is secretory >100 osmotic

2. Fecal Occult Blood Testing and Fecal Leukocytes

3. Fecal Fat - >7g over 24 hours with 100 g fat diet (tends to be higher (i.e. 30g/day) with maldigestion than malabsorption)

Chronic DiarrheaChronic Diarrhea OsmoticOsmotic

Mg, PO4, Carb Mg, PO4, Carb MaldigestMaldigest

FattyFatty Short gut/ResectionShort gut/Resection Bacterial overgrowthBacterial overgrowth Mucosal Disease/CeliacMucosal Disease/Celiac Pancreatic insufficiencyPancreatic insufficiency

InflammatoryInflammatory Inflammatory BowelInflammatory Bowel IschemiaIschemia DiverticulitisDiverticulitis Chronic infectionChronic infection

SecretorySecretory Non-osmotic laxativeNon-osmotic laxative Post-cholecystectomyPost-cholecystectomy Bile acid malabsorptionBile acid malabsorption IBSIBS GastrinomaGastrinoma VIPomaVIPoma MastocytosisMastocytosis Carcinoid syndromeCarcinoid syndrome HyperthyroidHyperthyroid VasculitisVasculitis Microscopic colitisMicroscopic colitis Lymphoma, colon caLymphoma, colon ca

History for Chronic History for Chronic DiarrheaDiarrhea

Osmotic gets better with fasting – others Osmotic gets better with fasting – others don’tdon’t

Bloody BM’s – Inflammatory (UC)Bloody BM’s – Inflammatory (UC) Weight loss – Fatty, InflammatoryWeight loss – Fatty, Inflammatory RLQ pain – think Crohn’sRLQ pain – think Crohn’s Iron Deficiency – think Celiac sprueIron Deficiency – think Celiac sprue Wakes up at night with symptoms – not IBSWakes up at night with symptoms – not IBS Ask about medications or surgeries Ask about medications or surgeries

(cholecystectomy or IC valve resection, (cholecystectomy or IC valve resection, etc)etc)

Irritable Bowel Irritable Bowel SyndromeSyndrome

Rome III CriteriaRome III Criteria Recurrent abdominal pain or discomfort at Recurrent abdominal pain or discomfort at

least 3 days per month in the last 3 months least 3 days per month in the last 3 months associated with 2 or more of the followingassociated with 2 or more of the following Improvement with defecationImprovement with defecation Onset associated with a change in frequency of Onset associated with a change in frequency of

stoolstool Onset associated with change in form of stoolOnset associated with change in form of stool

Criteria fulfilled for the last 3 months with Criteria fulfilled for the last 3 months with symptom onset at least 6 months prior to symptom onset at least 6 months prior to diagnosisdiagnosis

Irritable Bowel Irritable Bowel SyndromeSyndrome

Pain poorly localized and inconsistent and Pain poorly localized and inconsistent and often in BLQoften in BLQ

Need to have bowel movement soon after Need to have bowel movement soon after meals (gastro-colic reflex)meals (gastro-colic reflex)

Symptoms do Symptoms do NOTNOT wake the person from wake the person from sleepsleep

Symptoms worsened with stressSymptoms worsened with stress Less than age 40 at start of symptomsLess than age 40 at start of symptoms Depression or AnxietyDepression or Anxiety Often overlap with other functional Often overlap with other functional

syndromes (Dyspepsia, FMS, etc)syndromes (Dyspepsia, FMS, etc)

Evaluation for IBSEvaluation for IBS If meet Rome III Criteria then testing has If meet Rome III Criteria then testing has

not been shown to be helpful (not been shown to be helpful (except for except for sprue in diarrhea predominantsprue in diarrhea predominant)) Celiac Sprue serology has been positive in 5%Celiac Sprue serology has been positive in 5% Sigmoidoscopy/Colonoscopy no benefitSigmoidoscopy/Colonoscopy no benefit ESR, FOBT, Stool Culture, O&P etc. no benefitESR, FOBT, Stool Culture, O&P etc. no benefit

If they don’t meet the criteria or fit the If they don’t meet the criteria or fit the usual description then testing is indicated usual description then testing is indicated

For testing purposes, avoid performing For testing purposes, avoid performing diagnostic tests (CT) on patients who diagnostic tests (CT) on patients who meet Rome III criteriameet Rome III criteria

TreatmentTreatment

Treat symptomsTreat symptoms Most medications have unproven Most medications have unproven

benefitbenefit Fiber : Metamucil, Citrucel, BenefiberFiber : Metamucil, Citrucel, Benefiber Antispasmodics: Bentyl, Levsin, DonnatolAntispasmodics: Bentyl, Levsin, Donnatol Tricyclic Antidepressants, SSRI’sTricyclic Antidepressants, SSRI’s Imodium (diarrhea), Miralax (constipation)Imodium (diarrhea), Miralax (constipation) Stress ReductionStress Reduction Avoid fatty foods, Avoid DairyAvoid fatty foods, Avoid Dairy

Celiac SprueCeliac Sprue 40 year old 40 year old IrishIrish immigrant with history of DM 1 immigrant with history of DM 1

and hypothyroidism with steatorrhea, microcytic and hypothyroidism with steatorrhea, microcytic anemia and mild elevation of ALT (80).anemia and mild elevation of ALT (80).

Chronic malabsorption of small intestine Chronic malabsorption of small intestine secondary to exposure to dietary gluten (gliadin secondary to exposure to dietary gluten (gliadin portion)portion)

1:250 in US1:250 in US Symptoms include: Symptoms include:

Diarrhea or steatorrheaDiarrhea or steatorrhea Weight lossWeight loss Transaminase elevationsTransaminase elevations Anemia (iron deficiency)Anemia (iron deficiency) Vitamin deficiencyVitamin deficiency AsymptomaticAsymptomatic

Celiac DiseaseCeliac Disease Associated with Associated with HLA DQ2 and DQ8HLA DQ2 and DQ8 Associated diseases:Associated diseases:

IBSIBS OsteoporosisOsteoporosis IgA nephropathyIgA nephropathy Dermatitis HerpetiformisDermatitis Herpetiformis - Elbows, knees, - Elbows, knees,

buttocksbuttocks Increased risk of lymphoma (NHL) and GI Increased risk of lymphoma (NHL) and GI

malignanciesmalignancies DM 1DM 1 IgA deficiencyIgA deficiency Thyroid diseaseThyroid disease

Celiac SprueCeliac Sprue Diagnose with endomysial Ab or tissue Diagnose with endomysial Ab or tissue

transglutaminase Ab (both IgA). Confirm transglutaminase Ab (both IgA). Confirm diagnosis with small bowel biopsy.diagnosis with small bowel biopsy.

Activated T-cells damage villous architecture Activated T-cells damage villous architecture with inflammatory response (blunted villi, with inflammatory response (blunted villi, epithelial lymphocytic infiltrate, crypt epithelial lymphocytic infiltrate, crypt hyperplasia).hyperplasia).

All testing improves with treatmentAll testing improves with treatment Gluten-free dietGluten-free diet

No wheat, rye or barley No wheat, rye or barley Not in oats, though they are often contaminated with Not in oats, though they are often contaminated with

glutengluten Nonresponsive or relapse likely due to dietary Nonresponsive or relapse likely due to dietary

indiscretionindiscretion

Lower GI BleedLower GI Bleed Diverticular (Painless)Diverticular (Painless) Ischemic Colitis Ischemic Colitis

(painful)(painful) Angiodysplasia Angiodysplasia Cancer / Polyps Cancer / Polyps

Ulcerative Colitis / Ulcerative Colitis / Crohn’sCrohn’s

HemorrhoidsHemorrhoids 10% LGIB is from upper 10% LGIB is from upper

sourcesourceDiagnose etiology with colonoscopyDiagnose etiology with colonoscopy

If unable to find source of bleeding or if unable to tolerate If unable to find source of bleeding or if unable to tolerate a colonoscopy then:a colonoscopy then:Tagged RBC ScanTagged RBC Scan (Technetium 99m) - Requires .1-.4ml/min (Technetium 99m) - Requires .1-.4ml/min of active bleedingof active bleedingAngiographyAngiography - Requires .5-1ml/min of active bleeding - Requires .5-1ml/min of active bleeding

Allows directed therapy with gelfoam etc.Allows directed therapy with gelfoam etc.

Inflammatory Bowel Inflammatory Bowel DiseaseDisease

String sign – seen with Crohn’s

Ulcerative ColitisUlcerative Colitis

Recurring episodes of inflammation Recurring episodes of inflammation limited to the mucosal layer of the limited to the mucosal layer of the colon. Invariably involving the rectum colon. Invariably involving the rectum and extends proximally in a and extends proximally in a continuous fashion.continuous fashion.

Bloody diarrheaBloody diarrhea Rectal urgencyRectal urgency Abdominal crampsAbdominal cramps Fever, weight loss, anorexia, N/VFever, weight loss, anorexia, N/V

UC - ComplicationsUC - Complications

Massive hemorrhageMassive hemorrhage Toxic megacolonToxic megacolon Colonic perforation (5%)Colonic perforation (5%) Extraintestinal ManifestationsExtraintestinal Manifestations

Lead pipe – chronic UC

UC - treatmentUC - treatment

Inducing remissionInducing remission Mild = 5-ASA (mesalamine, sulfasalazine)Mild = 5-ASA (mesalamine, sulfasalazine) Moderate = Steroid taperModerate = Steroid taper Severe = IV steroids, Cyclosporine, Severe = IV steroids, Cyclosporine,

colectomy, TNF alpha antagonists (i.e. colectomy, TNF alpha antagonists (i.e. Remicade or Humira)Remicade or Humira)

MaintenanceMaintenance 5-ASA5-ASA Azathioprine/6-MPAzathioprine/6-MP TNF alpha antagonistsTNF alpha antagonists

Crohn’s DiseaseCrohn’s Disease Chronic recurring transmural Chronic recurring transmural

inflammation associated with fibrosis and inflammation associated with fibrosis and sinus tracts that penetrate serosa giving sinus tracts that penetrate serosa giving rise to microperforations and fistulae rise to microperforations and fistulae presenting as skip lesions in any area of presenting as skip lesions in any area of the GI tractthe GI tract MucosalMucosal StricturingStricturing PenetratingPenetrating

Based upon locationBased upon location 50% ileocolitis, 30% ileitis, 5% 50% ileocolitis, 30% ileitis, 5%

GastroduodenalGastroduodenal

Crohn’s complicationsCrohn’s complications 74% require surgery74% require surgery Right lower quadrant painRight lower quadrant pain DiarrheaDiarrhea Weight loss/AnorexiaWeight loss/Anorexia Small Bowel ObstructionSmall Bowel Obstruction FistulasFistulas Perirectal Abscess/Intraabdominal Perirectal Abscess/Intraabdominal

abscessesabscesses OsteoporosisOsteoporosis Extraintestinal ManifestationsExtraintestinal Manifestations

Entero-colonic fistula and string sign

Crohn’s - TreatmentCrohn’s - Treatment

Induction of RemissionInduction of Remission ?5-ASA = target to area of disease?5-ASA = target to area of disease Ciprofloxacin / FlagylCiprofloxacin / Flagyl Steroids (Prednisone or Budesonide)Steroids (Prednisone or Budesonide) TNF alpha antagonistsTNF alpha antagonists

MaintenanceMaintenance 5-ASA5-ASA 6-MP/Azathioprine/MTX6-MP/Azathioprine/MTX TNF alpha antagonistsTNF alpha antagonists

Extraintestinal ManifestationsExtraintestinal Manifestations

ArthropathyArthropathy (20%) (20%) Large joint – follows disease activityLarge joint – follows disease activity Small joint – independent of diseaseSmall joint – independent of disease

Ankylosing SpondylitisAnkylosing Spondylitis (10%) (10%) Not associated with disease activityNot associated with disease activity

Erythema NodosumErythema Nodosum (10%) (10%) Associated with diseaseAssociated with disease

Pyoderma GangrenosumPyoderma Gangrenosum (10%) (10%) Not associated with disease activityNot associated with disease activity

Extraintestinal ManifestationsExtraintestinal Manifestations

Episcleritis / UveitisEpiscleritis / Uveitis – 5% – 5% NephrolithiasisNephrolithiasis

Calcium oxalate stones with ileal Crohn'sCalcium oxalate stones with ileal Crohn's Primary Sclerosing CholangitisPrimary Sclerosing Cholangitis

5% of UC, 75% of PSC have UC5% of UC, 75% of PSC have UC 25% cancer at 10 years after PSC diagnosis25% cancer at 10 years after PSC diagnosis

Colon CancerColon Cancer 1% per year after 15 years1% per year after 15 years Start surveillance at 8-10 year after diagnosis Start surveillance at 8-10 year after diagnosis

in pancolitis and after 15 years in left sided in pancolitis and after 15 years in left sided colitiscolitis

IBD = Indication for IBD = Indication for surgerysurgery

UCUC Toxic megacolon/PerforationToxic megacolon/Perforation Failure to control symptomsFailure to control symptoms Dysplasia on surveillanceDysplasia on surveillance

Crohn’sCrohn’s Strictures with obstructionStrictures with obstruction Complicated fistulaComplicated fistula Unresponsive inflammatory massUnresponsive inflammatory mass

Dermatologic and GI Dermatologic and GI AssociationsAssociations

Dermatitis HerpetiformisDermatitis Herpetiformis

Chronic, symmetric, intensely pruritic eruption including vesicles, papules and urticarial wheals

Celiac DiseaseCeliac Disease

Acanthosis NigricansAcanthosis Nigricans

Diffuse, velvety thickening and hyperpigmentation of skin in axilla and other body folds

Gastric CancerGastric Cancer

TricholemmomasTricholemmomas

Cowden’s SyndromeCowden’s Syndrome

Cowden’s syndrome more associated with thyroid and breast cancer as well as GI hamartomas

Necrolytic Migratory Necrolytic Migratory ErythemaErythema

Superficial migratory necrolytic erythema with central blisters or erosions that crust and heal with hyperpigmentation, a beefy red tongue, and angular cheilitis

GlucagonomaGlucagonoma

Peutz Jeghers SyndromePeutz Jeghers Syndrome

Intussusceptions and Intussusceptions and MalignanciesMalignancies

Pancreatic Neuroendocrine Pancreatic Neuroendocrine tumorstumors

NeurofibromatosNeurofibromatosis-1is-1

Tuberous Sclerosis

MEN1, And…

Von Hippel-LindauVon Hippel-LindauHemagioblastomas and retinal angiomasClear cell RCCPheochromocytomaEndolymphatic sac tumors of middle earPancreatic serous cystadenoma or neuroendocrine tumorsPapillary cystadenomas of epididymis and broad ligament

Questions?Questions?

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