PEDIATRIC Section 8: SOLID Organ GI Flashcards
Pathology of Cystic Fibrosis in the pancreas
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).
Which type of patients diagnosed with CF tend to have more pancreatic problem? Why?
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.
Most common imaging finding in adult CF?
Complete fatty replacement
DIagnosis?
Cystic fibrosis in pancreas
Enlarged with fatty replacement = lipomatous pseudohypertrophy of the pancreas.
Where can you find fibrosisng Colonopathy?
Pancreatic Cystic fibrosis
Thick walled right colon as a complication of enzyme replacement therapy.
The 2nd most common cause of pancreatic insufficiency in kids (CF #1).
Shwachman-Diamond Syndrome
Basically, it’s a kid with diarrhea, short stature, and
eczema.
Shwachman-Diamond Syndrome
What happens in Schwachman-Diamond Syndrome?>
Will also cause lipomatous pseudohypertrophy o f the pancreas.
You only have a ventral bud (the dorsal bud forgets
to form).
Dorsal Pancreatic Agenesis
Dorsal Pancreatic Agenesis disease manifestations
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.
Most common cause of pancreatitis in pediatrics
Trauma (seatbelt)
he most common pediatric solid tumor.
Solid and Papillary Epithelial Neoplasm (SPEN)
What is the outcome of Solid and Papillary Epithelial Neoplasm (SPEN)?
Very good
What are the common pancreatic masses in Peds?
Solid and Papillary Epithelial Neoplasm (SPEN)
What do you think first when you see mass in the liver?
AGE!
With kids that are newborns you should think about 3 tumors:
- Infantile Hepatic Hemangioma
- Hepatoblastoma
- Mesenchymal Hamartoma
Large heart + Liver mass =
Infantile Hepatic Hemangioma
What are the associated findings in the Infantile Hepatic Hemangioma?
- The aorta above the hepatic branches o f the celiac is often enlarged relative to the aorta below the celiac because of differential flow.
- SKin hemangiomas are present in 50%
What is elevated in Intantile Hepatic Hemangioma?
Endothelial growth factor
Associated syndrome in Infantile Hepatic Hemangioma
Kasabach-Merritt Syndrome (the platelet eater).
How do patients with Infantile Hepatic Hemangioma do?
How do they do? - Actually well. They tend to spontaneously involute without therapy over months-years - as they progressively calcify.
Diagnosis? Describe
Hepatic Hemangioma
Diagnosis?
1-month-old boy showing sonographic characteristics of infantile hepatic hemangiomas. Transverse sonogram of liver shows multiple well-defined hypoechoic spherical masses.
Most common primary liver tumor of childhood (< 5)
Hepatoblastoma
What syndromes is hepatoblastoma associated with?
Hemi-hypertrophy
Wilms
Beckwith-Weidman
Risk factor of Hepatoblasoma
Prematurity
Diagnosis?
Describe
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.
What hepatic mass causes a precocious puberty from making bHCG?
Hepatoblasoma
AFP is elevated in this Infantile Liver mass
Hepatoblasoma
WIms + Inc AFP + Precocious puberty =
Hepatoblastoma
Age 0-3 + Cystic mass + (-)AFP + Calcifications are uncommon =
Mesenchymal Hamartoma
Diagnosis?
Age 0-3
What vessel feeds it?
Mesenchymal Hamartoma
Large portal vein branch feeds it
What are the common Liver masses in Ages 5 and up?
- Hepatocellular CA
- FIbrolamellar HCC
- Unfifferentiated Embryonal Sarcoma
This is actually the second most common liver cancer in kids.
HCC
Hepatoblasoma (1st)
> 5y.o. + Liver mass + Inc AFP + Cirrhosis =
HCC
What are the associated conditions in HCC?
Biliar atresia
Fanconi Syndrome
Glycogen Storage Disease
< 35 y.o + NO cirrhosis + Normal AFP + Calcification is common + Central Scar + Gallium avid =
Fibrolamellar HCC
Buzzword for Fibrolamellar HCC
“Central Scar”
Diagnosis?
This scar does NOT enhance, and is T2 dark (the FNH scar is T2 bright).
Tumors with central scars
Fibrolamellar HCC
Focal Nodular Hyperplasia
Difference between Fibrolamellar HCC and FNH?
Centarl scar enhancement
Fibrolamellar HCC - does NOT enhance
Focal Nodular Hyperplasia - (+)enhancement
This is the pissed off cousin of the mesenchymal hamartoma.
Undifferentiated Embryonal Sarcoma:
> 5y.o. + Cystic liver mass + aggressive +
Undifferentiated Embryonal Sarcoma:
Describe the appearance of Undifferentiated Embryonal Sarcoma.
Hypodense + septations + fibrous pseudocapsule
What are the common pediatic Liver masses in any age?
- Metastasis - Wilms or Neuroblastoma
- Hepatic Adenoma
- HEmangiomas
- FNH
- Angiosarcoma
Scenario
Promiscuous mom + Fetal Hepatomegaly on USD (after 20 weeks)
Think of Congenital Syphilis
What is the earliest organ involved and last to resolve in congenital syphillis?
Liver
Scenario
Advance maternal age + non-promiscuous + Hepatomagaly on 3rd tri US =
Transient Abnormal Myelopoiesis (TAM)
This is a preleukemoc syndrome seen only in Downs (Tri 21)
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.”
Advance maternal age + Fetal hepatomegaly + Downs
Transient Abnormal Myelopoiesis (TAM)
congenital dilations of the bile ducts
Choledochal Cysts
The most common type of Choledochal cyst?
Describe
Type I
Focal Dilatation of the CBD
Describe The Type II Choledocal cyst
type II: true diverticulum from extrahepatic bile duct
Describe The Type III Choledocal cyst
type III: dilatation of extrahepatic bile duct within the duodenal wall (choledochocele)
Describe The Type IV Choledocal cyst
type IV: next most common
Describe The Type V Choledocal cyst
type V: multiple dilatations/cysts of intrahepatic ducts only (Caroli disease)
Describe The Type VI Choledocal cyst
type VI: dilatation of cystic duct
Sixtic duct
AR disease + Polycystic Kidney didsease + Medullary Sponge + INTRAHEPATIC large saccular Duct dilatation
Caroli’s disease
Diagnosis?
Describe
Caroli’s disease
Portal Vein surrounded by dilated bile ducts
“CENTRAL DOT SIGN”
Cysts in the kinds + Fibrosis in the liver =
ARPKD (AR Polycystic Kidney Disease)
Describe the relationship of hepatic fibrosis and cystic formation in ARPKD
The degree of fibrosis is actually the opposite of cystic formation in the kidneys
bad kidneys ok liver, ok kidneys bad liver
Hereditary Hemorriiagic Telangiectasia is a.k.a.?
Osler-Weber-Rendu
Autosomal dominant disorder characterized by multiple AVMs in the liver and lungs.
Hereditary Hemorriiagic Telangiectasia (Osler-Weber-Rendu)
Hereditary Hemorriiagic Telangiectasia (Osler-Weber-Rendu) can lead to what conditions?
It leads to cirrhosis, and a massively dilated hepatic artery.
Diagnosis?
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
If you have prolonged newborn jaundice (>2 weeks), think about these things
(1) neonatal hepatitis
(2) Bihary Atresia.
Diagnosis?SIgn?
Describe.
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.
Patients with biliary atresia really only have atresia where?
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.
Biliary atresia associated conditions
Polysplenia
Trisomy 18 (Edwards)
Can the gallbladder be absent in Biliary atresia?
Gallbladder may be absent (normal gallbladder —supports neonatal hepatitis
Prolonged jaundice + normal gallbladder =
Usually neonatal hepatitis.
hereditary cholestasis from paucity of intrahepatic bile ducts, and peripheral pulmonary stenosis.
Alagille Syndrome
What is the purpose of liver biopsy in biliary atresia?
Exclude Allagile syndrome
How do you fully distinguish Biliary atresia?
Hepatobiliary Scintigraphy with 99m Tc-IDA
If you see a peds patient with gallstones think of what?
Sickle Cell
What happens to the spleen of kids in Sickle cell disease?
will typically enlarge progressively and then eventually auto-infarct and shrink (during the first decade).
Enlargement of the spleen in sickle cell disease can cause what condition?
acute splenic sequestration crisis.
This is the second most common cause of death in SC patients younger than 10.
Splenic Sequestration
the spleen becomes a greedy little pig and tries to hog all the blood for itself
Gamesmanship:
Hx of abd pain + VS suggesting low volume (Inc HR, dec BP) + Splenomegaly =
Splenic Sequestration
Remember most kids with sickle cell will have smaller spleens (auto infarct) so a big spleen should be your clue.
Diagnosis?
Splenic infarct showing “bright bands”
Other problems you can run into if your spleen stays big in Sickle cell are?
bscess formation and large infarcts (not same as auto infarcts)
Difference of massive infarct vs auto infarcted spleen?
it is typically the combined effort of numerous tiny, unnoticeable, and repetitive micro occlusions leading to progressive atrophy - This doesnt hur (large infarcts do
When does Auto infarcted Spleen occur?
Early and is usually complete by age 8
Diagnosis?
Auto Infarcted Spleen
a tiny (possibly calcified) spleen.
'’Where is thefucking spleen ?” = Auto Infarct =
Sickle Cell
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 -
Sickle cell
a rare condition where many organs in the chest and abdomen are formed abnormally, in the wrong position, or are even missing.
Heterotaxia
