ABDOMINAL DISTENTIONOR

MASSES

Atan Baas SinuhajiSub Division of Pediatrics Gastroentero-Hepatology

Department of ChildHealth,School of MedicineUniversity of Sumatera Utara/Adam Malik Hospital

Medan

ABDOMINAL

DISTENTION

ABDOMINAL WALL

ABDOMINAL CONTENT

PCM

PRUNE BELLY SYNDR.

OBESITY

GASES

FLUIDS

ABD. MASS

PRUNE BELLY SYNDROME

= EAGLE BARRET SYNDROME

= TRIAD SYNDROME

- DEFICIENT ABDOMINAL MUSCLE

- URINARY TRACT ABNORMALITY

UROPATHY NON OBSTRUCTIVE

- CRYPTORCHIDISM

GASES

OUT PERFORATION

PNEUMOPERITONEUM

IN

OBSTRUCTION

MALABSORPTION

AEROPHAGIA

BOWEL

BOWEL OBSTRUCTION :

1. MECHANICAL/PARALYTIC

2. INCOMPLETE/COMPLETE

3. CONGENITAL/ACQUIRED

OBSTRUCTION

MECHANICAL SIMPLE

STRANGULATION

VASCULAR COMPROMISE

PARALYTIC

= ILEUS

=INTESTINAL PSEUDOOBSTRUCTION

ILEUS

ACUTE

CHRONIC

SPASMOLYTIC

HYPOKALEMIA

PNEUMONIA

MUSCLE & NEURON

(CHRONIC INTESTINAL PSEUDO OBSTRUCTION)

OBSTRUCTION

ACCUMULATION OF BOWEL CONTENTS

OVERGROWTH MICROORG.

GUT CIRCULATION

MUCOSAL DAMAGE

ENTEROCOLITIS

SEPSIS

ABD.MASS

ABD. CAVITY

PELVIC

RETROPERITONEAL-KIDNEYS : -WILMS TUMOR

-NEUROBLASTOMA

-CYSTE

-PANCREAS

PANCREATIC CYST

TRUE PSEUDO

DELINEATED BY EPITHELIAL WALL DELINEATED BY FIBROUS WALL

PANCREATITIS FAIL TO RESOLVE

RESECTIONDRAINAGE

OVARIAL CYST

HEMATOCOLPOS

FETUS

PELVICTUBOOVARIAN ABSCESS

TERATOMA

IN

OUT

ABD. CAV.

WORMS > 100

FECAL IMPACTION

TUMOR

FOREIGN BODY

APP. ABSCESS

TUMOR

- KISTA MESENTERIUM

ORGANOMEGALY

GUT

TUMORS OF THE GUT

1.POLYPS

2.HEMANGIOMA

3.LEIOMYOMA

4.CARCINOMA

5.LIMPHOSARCOMA

6.CARCINOID:

- CHRONIC DIARRHOEA - VASOMOTOR- BRONCHOCONSTRICTION

POLYP

Any mass projecting into lumen of GI Tract

Neoplastic Non neoplastic

=Benigna adenoma=Malignant carcinoma

=Juvenile=Inflammatory=Hyperplastic

POLYPS OF THE GUT

FAMILIAL

ADENOMA

PREMALIGNANT

JUVENILE

HAMARTOMA

INTESTINAL JUVENILE POLYPS

NON SYNDROMIC SYNDROMIC

SOLITARY

AMPUTATED

EXTRAINTESTINAL FEATURES

(-) (+)

JPS = BRRS= CS

INTESTINAL JUVENILE POLYPS

NONSYNDROMIC JUVENILE POLYPOSIS SYNDROME( JPS )

PREMALIGNANTNON MALIGNANT

JUVENILE POLYPOSIS SYNDROME

- 5 JUVENILE POLYPS OF THE COLON OR RECTUM-JUVENILE POLYPS IN OTHER PARTS OF GI TRACT OR-ANY NUMBER OF JUVENILE POLYPS AND A POSITIVE FAMILY HISTORY

BANNAYAN RILEY RUCULCABA SYNDROME( BRRS )

ADDITIONAL FEATURES= MENTAL RETARDASI= MACROCEPHALY= LIPOMATOSIS= HEMANGIOMAS AND= GENITAL PIGMENTATION

COWDEN SYNDROME( CS )

ADDITIONAL PATHOGNOMONIC FEATURES OF MUCOCUTANEOUSLESION (FACIAL TRICHILEMMOMA,ORAL FIBROMA,ACRAL KERATOSIS)AND ASSOCIATED TUMOR OF THE THYROID, BREAST AND ENDOMETRIUM

DIAGNOSIS OF POLYPS

INVASIVE NONINVASIVE

ENDOSCOPY MATRIX METALLOPROTEINASES IN URINE

MMP(MATRIX METALLOPROTEINASE)

VEGF(VASCULAR ENDOTHELIAL GROWTH FACTOR)

ANGIOGENESIS

PHYSIOLOGICAL PATHOLOGICAL

-DEVELOPMENT-TISSUE REPAIR-REPRODUCTION

-TUMOR GROWTH-METASTASIS

GROWTH

ANGIOGENESIS

MMP(+) IN URINE

Table 1 Lower Gastrointestinal surveillance strategies

Recomendations by Howe et Recommendations by Dunlop

From age 15 or ealier if symptoms:Do full blood examination andendoscopy

If normal,repeat 3 yearlyIf polyps are found,remove

and screen annually untilpolyps free ,then 3 yearly

From age 15-18 or earlier if symptomsInterval 1-2 years

Gene carriers or affected continuesurveillance until age 70

Table 2 Upper gastrointestinal surveillance strategies

Recommendation by Howe et al

Recommendation by Dunlop

Recommendation by Sayed et al

Contemporaneously withcolonoscopy

Biliary and/or pancreatic duct bruishings

recommended if elevated amylase or abnormalliver function test

From age 25

Frequency :1-2yearly contemporaneouslywith colonoscopy

Frequency :SMAD4+ patients :1-3 yearly

Mutation negative orBMPR1A+ patients :5 yearly

HEPATOMEGALY1. INFLAMMATION HEPATITIS

2. CONGESTION : DECOMPENSATION,

CONTRICTIVE PERICARDITIS

3. BLOOD DISORDERS :

HEMOLYSIS : THALASSEMIA

MALIGNANCY : LEUKEMIA

4. TUMORS :CHOLEDOCHAL CYST

HEPATOMA

5. METABOLIC DISORDERS : FATTY LIVER

FATTY LIVER

1. NUTRITIONAL : OBESITY, KWASHIORKOR

2. DRUGS : ESTROGEN, STEROID

3. INTOXICATION : ALCOHOL

4. ALTERATION OF GI ANATOMY : JEJUNOILEAL BY PASS

5. OCCUPATIONAL EXPOSURE : HYDROCARBON

6. METABOLISM : A LIPOPROTEINEMIA

PATHOGENESIS

1.PERIPHERAL MOBILIZ. OF FATTY ACID

2. HEPATIC SYNTHESIS OF FATTY ACID

3. HEPATIC CATABOLISM OF FATTY ACID

4. IMPAIRED SYNTHESIS & EXCRETION VLDL ( VERY LOW DENSITY LIPOPROTEIN) FROM THE LIVER

FATTY LIVER

HEPATIC STEATOSIS

INFLAMATION

ALCOHOLICNON ALCOHOLIC

STEATOHEPATITIS (NASH)

8-20 %

PROGRESIVE FIBROSIS (10-50 % OF NASH)

CIRRHOSIS (10% OF NASH)

FIBROSIS (-)

NO INCREASED MORTALITY

NON INFLAMATION

(BENIGNA STEATOSIS)

NO INCREASED MORTALITY

HEPATIC STEATOSIS

NASH ALC. HEPATITIS

ALT > AST

2 : 1

AST > ALT

2 : 1

ALT = SGPT

ALANINE AMINO TRANSFERASE= SERUM GLUTAMATE PYRUVATE TRANSAMINASSE

AST=SGOT

ASPARTAT AMINO TRANSFERASE = SERUM GLUTAMIC OXALOACETAT TRANSAMINASE

FLUIDS

IN OUT

OBSTRUCTION ASCITES

BOWEL

ASCITES

PERMEABILITY-DHF

-PERITONITIS TBC

-PERITONEAL TUMOR

PORTAL HYPERTENSION

-HEART FAILURE

-CIRRHOSIS

HYDROSTATIC PRESS.

LOSS

- NEPHROTIC SYND.

INTAKE

- PCM SYNTHESIS

- HEPATIC CIRRHOSIS

ONCOTIC PRESS.

LYMPH OBSTRUCTION

ABDOMINAL DISTENTIONORMASSES

Atan Baas SinuhajiSub Division of Pediatrics Gastroentero-Hepatology Department of ChildHealth,School of MedicineUniversity of Sumatera Utara/Adam Malik Hospital Medan

1

ABDOMINALDISTENTION

ABDOMINAL WALL

ABDOMINAL CONTENT

PCMPRUNE BELLY SYNDR.OBESITY

GASESFLUIDSABD. MASS

PRUNE BELLY SYNDROME

= EAGLE BARRET SYNDROME = TRIAD SYNDROME

DEFICIENT ABDOMINAL MUSCLE URINARY TRACT ABNORMALITY UROPATHY NON OBSTRUCTIVE CRYPTORCHIDISM

GASES

OUT

PERFORATION

PNEUMOPERITONEUM

IN

OBSTRUCTIONMALABSORPTIONAEROPHAGIA

BOWEL

BOWEL OBSTRUCTION :

1. MECHANICAL/PARALYTIC2. INCOMPLETE/COMPLETE3. CONGENITAL/ACQUIRED

OBSTRUCTION

MECHANICAL

SIMPLE

STRANGULATION

VASCULAR COMPROMISE

PARALYTIC

= ILEUS=INTESTINAL PSEUDOOBSTRUCTION

ILEUS

ACUTE

CHRONIC

SPASMOLYTICHYPOKALEMIAPNEUMONIA

MUSCLE & NEURON

(CHRONIC INTESTINAL PSEUDO OBSTRUCTION)

OBSTRUCTION

ACCUMULATION OF BOWEL CONTENTS

OVERGROWTH MICROORG.

GUT CIRCULATION

MUCOSAL DAMAGE

ENTEROCOLITIS

SEPSIS

ABD.MASS

ABD. CAVITY

PELVIC

RETROPERITONEAL

-KIDNEYS : -WILMS TUMOR -NEUROBLASTOMA -CYSTE-PANCREAS

PANCREATIC CYST

TRUE

PSEUDO

DELINEATED BY EPITHELIAL WALL

DELINEATED BY FIBROUS WALL

PANCREATITIS FAIL TO RESOLVE

RESECTION

DRAINAGE

10

OVARIAL CYST

HEMATOCOLPOS

FETUS

PELVIC

TUBOOVARIAN ABSCESS

TERATOMA

IN

OUT

ABD. CAV.

WORMS > 100FECAL IMPACTIONTUMOR FOREIGN BODYAPP. ABSCESS

TUMOR

- KISTA MESENTERIUM

ORGANOMEGALY

GUT

TUMORS OF THE GUT

1.POLYPS2.HEMANGIOMA3.LEIOMYOMA4.CARCINOMA5.LIMPHOSARCOMA6.CARCINOID:- CHRONIC DIARRHOEA - VASOMOTOR- BRONCHOCONSTRICTION

POLYP

Any mass projecting into lumen of GI Tract

Neoplastic

Non neoplastic

=Benigna adenoma=Malignant carcinoma

=Juvenile=Inflammatory=Hyperplastic

POLYPS OF THE GUT

FAMILIAL

ADENOMA

PREMALIGNANT

JUVENILE

HAMARTOMA

15

INTESTINAL JUVENILE POLYPS

NON SYNDROMIC

SYNDROMIC

SOLITARY

AMPUTATED

EXTRAINTESTINAL FEATURES

(-)

(+)

JPS

= BRRS= CS

INTESTINAL JUVENILE POLYPS

NONSYNDROMIC

JUVENILE POLYPOSIS SYNDROME ( JPS )

PREMALIGNANT

NON MALIGNANT

JUVENILE POLYPOSIS SYNDROME

5 JUVENILE POLYPS OF THE COLON OR RECTUMJUVENILE POLYPS IN OTHER PARTS OF GI TRACT ORANY NUMBER OF JUVENILE POLYPS AND A POSITIVE FAMILY HISTORY

BANNAYAN RILEY RUCULCABA SYNDROME ( BRRS )

ADDITIONAL FEATURES= MENTAL RETARDASI= MACROCEPHALY= LIPOMATOSIS= HEMANGIOMAS AND= GENITAL PIGMENTATION

COWDEN SYNDROME ( CS )

ADDITIONAL PATHOGNOMONIC FEATURES OF MUCOCUTANEOUSLESION (FACIAL TRICHILEMMOMA,ORAL FIBROMA,ACRAL KERATOSIS)AND ASSOCIATED TUMOR OF THE THYROID, BREAST AND ENDOMETRIUM

DIAGNOSIS OF POLYPS

INVASIVE

NONINVASIVE

ENDOSCOPY

MATRIX METALLOPROTEINASES IN URINE

MMP(MATRIX METALLOPROTEINASE)

VEGF (VASCULAR ENDOTHELIAL GROWTH FACTOR)

ANGIOGENESIS

PHYSIOLOGICAL

PATHOLOGICAL

-DEVELOPMENT-TISSUE REPAIR-REPRODUCTION

-TUMOR GROWTH-METASTASIS

GROWTH

ANGIOGENESIS

MMP(+) IN URINE

Table 1 Lower Gastrointestinal surveillance strategies

Recomendations by Howe et

Recommendations by Dunlop

From age 15 or ealier if symptoms:Do full blood examination and endoscopy

If normal,repeat 3 yearlyIf polyps are found,remove and screen annually until polyps free ,then 3 yearly

From age 15-18 or earlier if symptomsInterval 1-2 years

Gene carriers or affected continue surveillance until age 70

Table 2 Upper gastrointestinal surveillance strategies

Recommendation by Howe et al

Recommendation by Dunlop

Recommendation by Sayed et al

Contemporaneously with colonoscopy

Biliary and/or pancreatic duct bruishings recommended if elevated amylase or abnormal liver function test

From age 25

Frequency :1-2yearly contemporaneously with colonoscopy

Frequency :SMAD4+ patients :1-3 yearly

Mutation negative or BMPR1A+ patients : 5 yearly

HEPATOMEGALY

INFLAMMATION HEPATITISCONGESTION : DECOMPENSATION, CONTRICTIVE PERICARDITIS3. BLOOD DISORDERS : HEMOLYSIS: THALASSEMIA MALIGNANCY: LEUKEMIA4. TUMORS :CHOLEDOCHAL CYST HEPATOMA5. METABOLIC DISORDERS : FATTY LIVER

26

FATTY LIVER

NUTRITIONAL : OBESITY, KWASHIORKORDRUGS : ESTROGEN, STEROIDINTOXICATION : ALCOHOLALTERATION OF GI ANATOMY : JEJUNOILEAL BY PASSOCCUPATIONAL EXPOSURE : HYDROCARBONMETABOLISM : A LIPOPROTEINEMIA

PATHOGENESIS

1.PERIPHERAL MOBILIZ. OF FATTY ACID

2. HEPATIC SYNTHESIS OF FATTY ACID

3. HEPATIC CATABOLISM OF FATTY ACID

4. IMPAIRED SYNTHESIS & EXCRETION VLDL ( VERY LOW DENSITY LIPOPROTEIN) FROM THE LIVER

FATTY LIVER

HEPATIC STEATOSIS

INFLAMATION

ALCOHOLIC

NON ALCOHOLIC STEATOHEPATITIS (NASH)8-20 %

PROGRESIVE FIBROSIS (10-50 % OF NASH)

CIRRHOSIS (10% OF NASH)

FIBROSIS (-)

NO INCREASED MORTALITY

NON INFLAMATION(BENIGNA STEATOSIS)

NO INCREASED MORTALITY

HEPATIC STEATOSIS

NASH

ALC. HEPATITIS

ALT > AST2 : 1

AST > ALT2 : 1

ALT = SGPTALANINE AMINO TRANSFERASE= SERUM GLUTAMATE PYRUVATE TRANSAMINASSEAST=SGOTASPARTAT AMINO TRANSFERASE = SERUM GLUTAMIC OXALOACETAT TRANSAMINASE

FLUIDS

IN

OUT

OBSTRUCTION

ASCITES

BOWEL

ASCITES

PERMEABILITYDHFPERITONITIS TBCPERITONEAL TUMOR

PORTAL HYPERTENSION -HEART FAILURE -CIRRHOSIS

HYDROSTATIC PRESS.

LOSS - NEPHROTIC SYND.

INTAKE - PCM

SYNTHESIS- HEPATIC CIRRHOSIS

ONCOTIC PRESS.

LYMPH OBSTRUCTION