GASTROENTERO-HEPATOLOGY SYSTEM

Centre of Gastroentero-HepatologyDepartment of Internal Medicine

Wahidin Sudirohusodo General Hospital, Makassar2010

Tingkat Kompetensi Dokter

Kemampuan 1 : mengenali & menjelaskan (gambaran klinik, cara mendapat informasi lanjut penyakit, rujukan yang tepat)Kemampuan 2 : diagnosis & merujuk (buat diagnosis, rujukan utk penanganan lanjut, menindaklanjuti setelah kembali dari rujukan)Kemampuan 3 : diagnosis, penatalaksanaan awal, merujuk (A bukan gawat darurat, B gawat darurat)Kemampuan 4 : diagnosis, penatalaksanaan mandiri & tuntas

ESOPHAGUS

ACHALASIA• Lack of relaxation = failure of the LES to relax• Secondary features : aperistalsis of the body of

esophagus• DEFINITION :

primary motor disorder of esophageal smooth muscle function with 3 diagnostic prerequisite

Relatively uncommon, prevalence 10/10.000; incidence 0.5 cases/y/100.000 p.

Acquired disorders affecting any age group (30-60 y.o, uncommon before 25 yo); M=F

Level of competent : 2

Prerequiste diagnostic1. Complete absence of primary &

secondary peristalsis in the smooth muscle, skeletal muscle function normal.

2. LES doesn’t relax completely with swallow

3. Resting LES pressure usually high

Etiology • Remain a mystery • Associated with viral infection

(herpes), autoimmun disorders

Pathophysiology

Myenteric plexus : loss of ganglion cells (inhibitory

function)

relative sparing of cholinergic (stimulatory function)

persistent LES constriction

Clinical manifestation• Dysphagia solid & liquid (most solid food)• Regurgitation 60-90% with bending or

recumbent position,awekening with previous night’s supper in mouth

• Heartburn : fermentation of food in the aperistaltic esophagus

• Weight loss• Chest pain

• Laboratory study : not helpful• Diagnostic study :

BARRIUM SWALLOW : atonic & dilated esophageal body, narrowing of GE junction “bird’s beak”

Thoracic CT scan on a patient with vigorous achalasia demonstrating marked thickening of the distal esophageal wall. The esophageal lumen is not dilated although it is filled with contrast material.

classical radiological features of a dilated esophagus, retained barium and an intralumenalair fl uid level with smooth tapering of the esophagogastric junction.

MANOMETRY : absent distal peristaltis in body (smooth muscle), incomplete LES relaxation (residual > 8mmHg), high resting LES (>45 mmHg)

The corresponding high-resolution manometry depicts the same phenomena with bizarre morphology of the esophageal contractile activity apparent within the distal esophagus and paucity of activity within the proximal esophagus. An increased intrabolus pressure is evident proximal to the nonrelaxing LES

Low (b) and high (c) power views of a myenteric ganglion showing nitric oxide synthase (NOS)- immunoreactive neurons (*). Numerous lymphocytes infi ltrate this region suggesting ongoing chronic infl ammatory activity of the myenteric plexus. Such inflammation may lead to the eventual destruction of the myenteric neurons that characterizes the pathophysiology of achalasia.

• EsophagoGastroDuodenal Endoscopic (role out pseudoachalasia) : observed retained food, esophageal dilatation, feel a’pop’ at GE junction

Differential diagnosis• Secondary achalasia : associated

with various diseases (cancer, chagas’ disease, amyloidosis, mixed connective tissue

• Pseudoachalasia : infiltrating cancer at GE junction (older age, short duration symptom & rapid weight loss)

•

Pseudo-achalasia

Figure (a) Pseudo- or secondary achalasia. The tapering in thedistal esophagus makes this barium esophagram difficult to distinguish from idiopathic achalasia. Note the dilated esophagus with intralumenal air fl uid level. (b) Abdominal CT scan image in a patient with pseudo- or secondary achalasia. This CT image demonstrates a stellate pulmonary mass originating in the left lung invading the gastroesophageal junction.

Esophageal spasm. The corkscrew appearance results from simultaneousnonpropulsive contractions of the esophagus occurring at multiplelevels.

Treatment • Palliative• Directed removing the functional

obstruction at the LES

PHARMACOLOGIC Nitrates, CCB,

botox

ENDOSCOPICPneumatic ballon

dilatationSURGICAL

Heller myotomy

Complication • Esophageal cancer risk 2-7% : mean

interval 17yr, squamous cell type, 5y survival <5%

Reff• Atlas of Gastroenterology (Wiley,

2009)• Primo Gastro (Lippincot, 2008)

CORROSIVE ESOPHAGUS• Injury of esophagus cause by a number of

caustic agent

Epidemiology• Appproximately 26.000 case/year, suicidal

gestsure most common & most injurious compared to accidental ingestion

• 80% case accidentally in children < 5y.o (consumed household cleaners)

Level of competent : 3B

Etiology• Severity of damage depends on

corrosive properties & the consentration

• Alkaline cleaning product = most severe injury

class Caustic agent ProductStrong alkalis Ammonia

Lye (sodium & potasium hydroxide)

Cleaning productDisc battery, drain cleaners, nonphosphate detergent, paint removers, washing products

Strong acid Hydrochloric acid

Nitric acidOxalic acidPhosporic acidSulfuric acid

Muriatic acid, soldering fluxes, swimming poool cleaners, toilet bowel cleanersGun barrel cleanersAntirust compoundsToilet bowel cleanersBattery acid, toilet bowel cleaners

Miscellanous Sodium hypochlorite Liquid bleach

Pathopysiology• Alkali Esophagitis : liquefactive necrosis

(complete destruction of entire cell & membranes)

Phase of InjuryAcute (day 1-4) liquefactive necrosis ; sloughing or

ulcer not apparent <24hr, vascular thrombosis, inflammation

Subacute (day 5-14) Sloughing casts; esophageal wall is thinnest, granulation, fibroblast/collagen deposition

Cicatrization (day 15-3mon) Fibroblast proliferate, further collagen deposition, stricture formation, epithelization

• Acid esophagitis : coagulation necrosis with clumping & opaficationof celluler cytoplasm, retained cell boundaries

Clinical Manifestation• Early sign &symptoms not realible

indicator of the severity• A history of chemical ingestion and

physical examination revealing oropharyngeal burns (including white membranes and edema of the soft palate and uvula) usually confirm the diagnosis.

• intense pain in the mouth and anterior chest

• marked salivation • inability to swallow and tachypnea. • Bloody vomitus containing pieces of

esophageal tissue signals severe damage• Signs of esophageal perforation and

mediastinitis, especially crepitation, indicate destruction of the entire esophagus.

• Inability to speak implies laryngeal damage.

Laboratory study : not helpful

Diagnostic study :• Endoscopy (in the first 24 hours after

ingestion) delineates the extent and location of the esophageal injury and assesses the depth of the burn; (a week after ingestion) to assess stricture development.

• Barium swallow (1 week afteringestion and every 3 weeks thereafter) may identify segmental spasm or fistula (no mucosal injury)

Classification of injury by endoscopy

Endoscopic findings

Hospital stay

Risk of stricture

Treatment

No injury None None DischargeGastric only Observe 24-

48 hrsNone Liquid diet

Linear esophageal injury

Observe 24-48 hrs

Low Liquid diet

Circumferential injury

Observe at least 48 hrs

High NPO, NGT

Treatments• ABC, need for intubation?,

obvious sign of mediatinitis/peritonitis requiring injury

• Determine quantitiy & type ofcaustic agent and time ingestion; is the container available?

• Emesis should not be induced (re-exposed esophagus & larynx)

• NG lavage is controversial, potential risk include vomiting, perforation. If NG place, aspirate before cold lavage

• If known acid & within minute injury :large volume water/milk may dilute & neutralize acid; otherwise NPO

• Steroid : reduce potential for stricture to high risk lesions, however no consensus on their use.- should only use in circumferential injury :

prednisone 1.5mg/kg/d & tapered over 2 months- probably use in patients with airway compromise & bronchospasm

• Antibiotics : role has never been established (ampicillin)

• In acute setting, get CXR or CT to role out perforation before endoscopy

• Prophylactic dilatation once acute injury has resolved (3rd week)

• Supportive treatment includes I.V. therapy to replace fluids or total parenteral nutrition while the patient can’t swallow, gradually progressing to clear liquids and a soft diet.

Complication• Strictures

• Esophageal cancer : 1000x fold risk, begin endoscopy survailence 15 years after ingestion (every1-3 years)

HIATUS HERNIALevel of competent : 2

GASTRITISLevel of competent : 4

Variceal GI bleeding

Level of competent : 2

VARICES ESOPHAGUS• Prevalance : 50% of all cirrhotic (80% in

Child C, 20% in Child A)• Incidence : 10% per year in cirrhotic• Variceal bleeding :

- incidence :24% per 2 years w/ moderate to large v.; 50% acute bleeding

- mortality : >20% w/1st bleed; 70% 5 y. mortality; 40-50% will rebleed

Architectural disturbances

Increased hepatic vascular resistence

Garcia-Pagan JC, Grozmann RJ, Bosch J. In Clinical Gastroenterology and Hepatology. 2005; p.709.

Pathophysiology Portal hypertension : an increase in the portal

venous pressure gradient (PVPG) and is a function of portal venous blood flow and hepatic and portocollateral resistence

Hepatic venous wedge pressure gradient (HVWPG) : represent the pressure difference between the wedge hepatic vein (pv pressure) & direct mesurement of abdominal IVC (free hv pressure)≈ 6 mmHg normally, > 10mmHg variceal development, >12mmHg high risk bleeding

MAJOR SITE COLLATERAL

Opening of pre-existingVascular communication

Increases in portal pressureand portal collateral bloodflow by meals, alcohol,physical exercise, increasedintraabdominal pressure

Garcia-Pagan JC, Grozmann RJ, Bosch J. In Clinical Gastroenterology and Hepatology. 2005; p.709.

Risk factors for rebleeding

- Age > 60 y.o.- Large varices- Severe initial bleed

(Hb <8 g/dl on admission)

- Renal failureOnly 50% of patients

with variceal hemorrhage stop bleeding spontaneously & high risk to recurrent bleeding within 6 weeks

COLLATERAL CIRCULATION(VARICES)

• Clinical manifestation : upper GI bleeding• Laboratory study : Hb/Ht, PT/APTT

(bleeding)• Diagnostic study : screening UGIE

- no varices : repeat EGD 2-3y, no prophylaxis necessary- s. varices : repeat EGD 1-2y, consider β-bloker if Child C- m to L.varices : 1st prevention EBL, β-bloker, etc

Fig. 4. Esophageal varices. a. Large variceal columns b. Varix with cherry-red spot

c. Varix with red wale sign

Fig. Portal Hypertensive Gastropathy (PHG) a. Mild portal hypertensive

gastropathyb. Severe PHG with active

oozing

a b c

a bWu et al. Gastroenterological endoscopy 2002: 541-543

Treatment PHARMACOTHERAPY

• VASOCONSTRICTORS decrease in splanchnic blood flow & leads to decrease in portal venous blood flow and portal pressure (e.g vasopressin, somatostatin, nonselective β-bloker)

• VASODILATORS alter resistence by inducing changes in the intrahepatic perivenular & perisinusoidal myofibroblast as the smooth muscle component of portocollateral vessels (e.g. nitroglycerine, long acting nitrate, prazosine)

Acute Bleeding• Transfuse Hb to 8gm/dl (aggressive transfusion may

precipitate further portal hypertension & bleeding)consider : platelets, FFP, lactulosa, endotracheal intubation

• Pharmacologic (start immediately, do not wait EGD to perform)

- octreotide : 50mcg Iv bolus then 50mcg/hr infusion, continue for 4-7 day

- PPI : IV drip until po can be taken- Antibiotics : all variceal bleeding should get against bacterial gut (3rd generation) during hospitalization for 4-7 days

Once stable, BB (propanolol/nadolol ± nitrate) after EGD

Table 2. Pharmacologic treatment of acute variceal bleeding

Drug route administration DoseVasopressinNitroglycerine

i.Vi.v Continous infusion 0.1-0.4 U/min

40-400 g/minGlypressin i.V Initial bolus

Subsequent bolus2 mg/4 h1-2 mg/4 h

Somatostatin i.V Initial bolusContinous infusion

250-500 g250-500 g/h

Octreotide i.V Initial bolusContinous infusion

25-50 g25-50 g/h

Terlipressin i.v Initial bolusSubsequent bolus

2 mg/4-6h in 2d1mg/4-6h

Treatment should be continued for 5 days

Prophylaxis of variceal bleeding in cirrhosis. Preprimary prophylaxis is aimed at preventing esophagogastric varices (EV) from developing. The goal of primary prophylaxis is to prevent a first variceal bleeding episode once medium or large varices have formed. Secondary prophylaxis is used to prevent recurrent variceal bleeding. Photo by: Lianne Friesen and Nicholas Woolridge

Table 1. Pharmacologic treatment for prevention of variceal bleeding

Drug Initial Dose Therapeutic dose

Propanolol 40 mg bid 40-400 mg Nadolol 40 mg qd 40-160 mg

Timolol 10 mg qd 5 mg qd-40mg

Isosorbide 5-mononitrate

20 mg bid 20 mg tid-20 mg qd

Goal : 20% reduction in HVWPG or 25% heart rate; decreased bleeding risk 50-90% (only 35% patients achieve this goal)

Complication of Variceal bleeding• Syock hypovolemik death• Induce :

– Hepatic encephalopathy– Hepatorenal syndrome– Worsening clotting profile

SA Jenkins, Somatostatin and ocreotide in gastroenterology, Medimedia, 1996

Prognosis• Primary prevention

indicated : moderate-large v. >5mm in D- nonselective BB ±nitrate (50% bleeding)- band ligation (more greater than BB in

bleeding)• Secondary prevention

indicated : patients who has previously bleed; >80% will bleed againin 2 years- band ligation ± nonselective BB with nitrates- if continue to rebleed > TIPS/transplant

PLEASE READ KONSENSUS NASIONAL PERKUMPULAN

GASTROENTEROLOGI INDONESIA (PGI) 2007Panduan penatalaksanaan perdarahn varises pada sirosis hati

• The key factor in the natural history of esophageal varices is increased portal pressure, which in cirrhosis is due to the combination of increased hepatic vascular resistance and increased portal collateral blood flow. The maintenance and aggravation of this situation leads to the progressive dilation of the varices and thinning of the variceal wall, until the tension exerted by the variceal wall exceeds the elastic limit of the vessel, leading to variceal hemorrhage