PEDIATRIC Section 8: SOLID Organ GI

Question 1

Pathology of Cystic Fibrosis in the pancreas

Answer 1

The pancreas is nearly always (90%) with CF patients. Inspissated secretions cause proximal duct obstruction leading to the two main changes in CF:
(1) Fibrosis (decreased T1 and T2 signal) and the more common one
(2) fatty replacement (increased Tl).

Question 2

Which type of patients diagnosed with CF tend to have more pancreatic problem? Why?

Answer 2

Patients with CF diagnosed as adults tend to have more pancreas problems than those diagnosed as children. Those with residual pancreatic exocrine function can have bouts of recurrent acute pancreatitis. Small (l-3mm) pancreatic cysts are common.

Question 3

Most common imaging finding in adult CF?

Answer 3

Complete fatty replacement

Question 4

DIagnosis?

Answer 4

Cystic fibrosis in pancreas

Enlarged with fatty replacement = lipomatous pseudohypertrophy of the pancreas.

Question 5

Where can you find fibrosisng Colonopathy?

Answer 5

Pancreatic Cystic fibrosis

Thick walled right colon as a complication of enzyme replacement therapy.

Question 6

The 2nd most common cause of pancreatic insufficiency in kids (CF #1).

Answer 6

Shwachman-Diamond Syndrome

Question 7

Basically, it’s a kid with diarrhea, short stature, and
eczema.

Answer 7

Shwachman-Diamond Syndrome

Question 8

What happens in Schwachman-Diamond Syndrome?>

Answer 8

Will also cause lipomatous pseudohypertrophy o f the pancreas.

Question 9

You only have a ventral bud (the dorsal bud forgets
to form).

Answer 9

Dorsal Pancreatic Agenesis

Question 10

Dorsal Pancreatic Agenesis disease manifestations

Answer 10

All you need to know is that
(1) this sets you up for diabetes (most ofyour beta cells are in the tail), and
(2) it’s associated with polysplenia.

Question 11

Most common cause of pancreatitis in pediatrics

Answer 11

Trauma (seatbelt)

Question 12

he most common pediatric solid tumor.

Answer 12

Solid and Papillary Epithelial Neoplasm (SPEN)

Question 13

What is the outcome of Solid and Papillary Epithelial Neoplasm (SPEN)?

Answer 13

Very good

Question 14

What are the common pancreatic masses in Peds?

Answer 14

Solid and Papillary Epithelial Neoplasm (SPEN)

Question 15

What do you think first when you see mass in the liver?

Answer 15

AGE!

Question 16

With kids that are newborns you should think about 3 tumors:

Answer 16

1. Infantile Hepatic Hemangioma
2. Hepatoblastoma
3. Mesenchymal Hamartoma

Question 17

Large heart + Liver mass =

Answer 17

Infantile Hepatic Hemangioma

Question 18

What are the associated findings in the Infantile Hepatic Hemangioma?

Answer 18

1. The aorta above the hepatic branches o f the celiac is often enlarged relative to the aorta below the celiac because of differential flow.
2. SKin hemangiomas are present in 50%

Question 19

What is elevated in Intantile Hepatic Hemangioma?

Answer 19

Endothelial growth factor

Question 20

Associated syndrome in Infantile Hepatic Hemangioma

Answer 20

Kasabach-Merritt Syndrome (the platelet eater).

Question 21

How do patients with Infantile Hepatic Hemangioma do?

Answer 21

How do they do? - Actually well. They tend to spontaneously involute without therapy over months-years - as they progressively calcify.

Question 22

Diagnosis? Describe

Answer 22

Hepatic Hemangioma

Question 23

Diagnosis?

Answer 23

1-month-old boy showing sonographic characteristics of infantile hepatic hemangiomas. Transverse sonogram of liver shows multiple well-defined hypoechoic spherical masses.

Question 24

Most common primary liver tumor of childhood (< 5)

Answer 24

Hepatoblastoma

Question 25

What syndromes is hepatoblastoma associated with?

Answer 25

Hemi-hypertrophy Wilms Beckwith-Weidman

Question 26

Risk factor of Hepatoblasoma

Answer 26

Prematurity

Question 27

Diagnosis? Describe

Answer 27

This is usually a well circumscribed solitary right sided mass, that may extend into the portal veins, hepatic veins, and IVC. Calcifications are present 50% of the time.

Question 28

What hepatic mass causes a precocious puberty from making bHCG?

Answer 28

Hepatoblasoma

Question 29

AFP is elevated in this Infantile Liver mass

Answer 29

Hepatoblasoma

Question 30

WIms + Inc AFP + Precocious puberty =

Answer 30

Hepatoblastoma

Question 31

Age 0-3 + Cystic mass + (-)AFP + Calcifications are uncommon =

Answer 31

Mesenchymal Hamartoma

Question 32

Diagnosis? Age 0-3 What vessel feeds it?

Answer 32

Mesenchymal Hamartoma Large portal vein branch feeds it

Question 33

What are the common Liver masses in Ages 5 and up?

Answer 33

1. Hepatocellular CA 2. FIbrolamellar HCC 3. Unfifferentiated Embryonal Sarcoma

Question 34

This is actually the second most common liver cancer in kids.

Answer 34

HCC Hepatoblasoma (1st)

Question 35

> 5y.o. + Liver mass + Inc AFP + Cirrhosis =

Answer 35

HCC

Question 36

What are the associated conditions in HCC?

Answer 36

Biliar atresia Fanconi Syndrome Glycogen Storage Disease

Question 37

< 35 y.o + NO cirrhosis + Normal AFP + Calcification is common + Central Scar + Gallium avid =

Answer 37

Fibrolamellar HCC

Question 38

Buzzword for Fibrolamellar HCC

Answer 38

"Central Scar"

Question 39

Diagnosis?

Answer 39

This scar does NOT enhance, and is T2 dark (the FNH scar is T2 bright).

Question 40

Tumors with central scars

Answer 40

Fibrolamellar HCC Focal Nodular Hyperplasia

Question 41

Difference between Fibrolamellar HCC and FNH?

Answer 41

Centarl scar enhancement Fibrolamellar HCC - does NOT enhance Focal Nodular Hyperplasia - (+)enhancement

Question 42

This is the pissed off cousin of the mesenchymal hamartoma.

Answer 42

Undifferentiated Embryonal Sarcoma:

Question 43

> 5y.o. + Cystic liver mass + aggressive +

Answer 43

Undifferentiated Embryonal Sarcoma:

Question 44

Describe the appearance of Undifferentiated Embryonal Sarcoma.

Answer 44

Hypodense + septations + fibrous pseudocapsule

Question 45

What are the common pediatic Liver masses in any age?

Answer 45

1. Metastasis - Wilms or Neuroblastoma 2. Hepatic Adenoma 3. HEmangiomas 4. FNH 5. Angiosarcoma

Question 46

Scenario Promiscuous mom + Fetal Hepatomegaly on USD (after 20 weeks)

Answer 46

Think of Congenital Syphilis

Question 47

What is the earliest organ involved and last to resolve in congenital syphillis?

Answer 47

Liver

Question 48

Scenario Advance maternal age + non-promiscuous + Hepatomagaly on 3rd tri US =

Answer 48

Transient Abnormal Myelopoiesis (TAM)

Question 49

This is a preleukemoc syndrome seen only in Downs (Tri 21)

Answer 49

Transient Abnormal Myelopoiesis (TAM) Most of the time (80%) it will get better on its own. The other 20% become myeloid leukemia of Down syndrome - hence the “preleukemic.”

Question 50

Advance maternal age + Fetal hepatomegaly + Downs

Answer 50

Transient Abnormal Myelopoiesis (TAM)

Question 51

congenital dilations of the bile ducts

Answer 51

Choledochal Cysts

Question 52

The most common type of Choledochal cyst? Describe

Answer 52

Type I Focal Dilatation of the CBD

Question 53

Describe The Type II Choledocal cyst

Answer 53

type II: true diverticulum from extrahepatic bile duct

Question 54

Describe The Type III Choledocal cyst

Answer 54

type III: dilatation of extrahepatic bile duct within the duodenal wall (choledochocele)

Question 55

Describe The Type IV Choledocal cyst

Answer 55

type IV: next most common

Question 56

Describe The Type V Choledocal cyst

Answer 56

type V: multiple dilatations/cysts of intrahepatic ducts only (Caroli disease)

Question 57

Describe The Type VI Choledocal cyst

Answer 57

type VI: dilatation of cystic duct Sixtic duct

Question 58

AR disease + Polycystic Kidney didsease + Medullary Sponge + INTRAHEPATIC large saccular Duct dilatation

Answer 58

Caroli's disease

Question 59

Diagnosis? Describe

Answer 59

Caroli's disease Portal Vein surrounded by dilated bile ducts "CENTRAL DOT SIGN"

Question 60

Cysts in the kinds + Fibrosis in the liver =

Answer 60

ARPKD (AR Polycystic Kidney Disease)

Question 61

Describe the relationship of hepatic fibrosis and cystic formation in ARPKD

Answer 61

The degree of fibrosis is actually the opposite of cystic formation in the kidneys bad kidneys ok liver, ok kidneys bad liver

Question 62

Hereditary Hemorriiagic Telangiectasia is a.k.a.?

Answer 62

Osler-Weber-Rendu

Question 63

Autosomal dominant disorder characterized by multiple AVMs in the liver and lungs.

Answer 63

Hereditary Hemorriiagic Telangiectasia (Osler-Weber-Rendu)

Question 64

Hereditary Hemorriiagic Telangiectasia (Osler-Weber-Rendu) can lead to what conditions?

Answer 64

It leads to cirrhosis, and a massively dilated hepatic artery.

Question 65

Diagnosis?

Answer 65

Hereditary Hemorriiagic Telangiectasia (Osler-Weber-Rendu) liver shows heterogeneous perfusion pattern with millimetric hypervascular images disseminated throughout the hepatic parenchyma, referring to telangiectases and arteriovenous shunts that are no longer evident during the venous phase. The early venous drainage with the simultaneous opacification of the dilated hepatic veins and the hepatic artery is evident during arterial phase

Question 66

If you have prolonged newborn jaundice (>2 weeks), think about these things

Answer 66

(1) neonatal hepatitis (2) Bihary Atresia.

Question 67

Diagnosis?SIgn? Describe.

Answer 67

Biliary atresia "Triangular cord sign" represents the fibrous ductal remnant of the extrahepatic bile duct in biliary atresia. The common bile duct is not visualized. At the porta, only two structures are identified, the portal vein and hepatic artery. There is increased echogenicity along anterior wall of portal vein consistent with positive triangular cord sign.

Question 68

Patients with biliary atresia really only have atresia where?

Answer 68

Patients with biliary atresia really only have atresia of the ducts outside the liver (absence of extrahepatic ducts), in fact they have proliferation o f the intrahepatic ducts.

Question 69

Biliary atresia associated conditions

Answer 69

Polysplenia Trisomy 18 (Edwards)

Question 70

Can the gallbladder be absent in Biliary atresia?

Answer 70

Gallbladder may be absent (normal gallbladder —supports neonatal hepatitis

Question 71

Prolonged jaundice + normal gallbladder =

Answer 71

Usually neonatal hepatitis.

Question 72

hereditary cholestasis from paucity of intrahepatic bile ducts, and peripheral pulmonary stenosis.

Answer 72

Alagille Syndrome

Question 73

What is the purpose of liver biopsy in biliary atresia?

Answer 73

Exclude Allagile syndrome

Question 74

How do you fully distinguish Biliary atresia?

Answer 74

Hepatobiliary Scintigraphy with 99m Tc-IDA

Question 75

If you see a peds patient with gallstones think of what?

Answer 75

Sickle Cell

Question 76

What happens to the spleen of kids in Sickle cell disease?

Answer 76

will typically enlarge progressively and then eventually auto-infarct and shrink (during the first decade).

Question 77

Enlargement of the spleen in sickle cell disease can cause what condition?

Answer 77

acute splenic sequestration crisis.

Question 78

This is the second most common cause of death in SC patients younger than 10.

Answer 78

Splenic Sequestration the spleen becomes a greedy little pig and tries to hog all the blood for itself

Question 79

Gamesmanship: Hx of abd pain + VS suggesting low volume (Inc HR, dec BP) + Splenomegaly =

Answer 79

Splenic Sequestration Remember most kids with sickle cell will have smaller spleens (auto infarct) so a big spleen should be your clue.

Question 80

Diagnosis?

Answer 80

Splenic infarct showing "bright bands"

Question 81

Other problems you can run into if your spleen stays big in Sickle cell are?

Answer 81

bscess formation and large infarcts (not same as auto infarcts)

Question 82

Difference of massive infarct vs auto infarcted spleen?

Answer 82

it is typically the combined effort of numerous tiny, unnoticeable, and repetitive micro occlusions leading to progressive atrophy - This doesnt hur (large infarcts do

Question 83

When does Auto infarcted Spleen occur?

Answer 83

Early and is usually complete by age 8

Question 84

Diagnosis?

Answer 84

Auto Infarcted Spleen a tiny (possibly calcified) spleen.

Question 85

''Where is thefucking spleen ?” = Auto Infarct =

Answer 85

Sickle Cell

Question 87

Gamesmanship: If you don't see the spleen but you do see a gallbladder full of stones in a kid less than 15 - you should think -

Answer 87

Sickle cell

Question 88

a rare condition where many organs in the chest and abdomen are formed abnormally, in the wrong position, or are even missing.

Answer 88

Heterotaxia