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Core > Pediatrics: Gastrointestinal, Solid Organ > Flashcards

Pediatrics: Gastrointestinal, Solid Organ Flashcards

1
Q

What are the major changes that occur to the pancreas in cystic fibrosis?

A
  • Fibrosis (decreased T1 and T2 signal)
  • Fatty replacement (increased T1)
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2
Q

What is the most common imaging finding in an adult with cystic fibrosis?

A

Complete fatty replacement of the pancreas

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3
Q

Enlarged, fatty replaced pancreas in the setting of cystic fibrosis…

A

Lipomatous pseudo hypertrophy of the pancreas

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4
Q

Fibrosing colonopathy

A

Wall thickening of the right colon as a complication of pancreatic enzyme replacement therapy in pts with cystic fibrosis

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5
Q

What is the most common cause of pancreatic insufficiency in kids?

A

Cystic fibrosis (followed by Shwachman-Diamond syndrome)

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6
Q

Kid with diarrhea, short stature, eczema, and pseudo hypertrophy of the pancreas…

A

Scwachman-Diamond syndrome

Note: This is the second most common cause of pancreatic insufficiency in kids.

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7
Q

Dorsal pancreatic agenesis

A

A developmental failure of the pancreatic dorsal bud to form, resulting in an absent pancreatic tail

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8
Q

Dorsal pancreatic agenesis is associated with…

A
  • Diabetes (most of the beta cells are in the pancreatic tail)
  • Polysplenia
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9
Q

What is the most common cause of pancreatitis in children?

A

Trauma (seatbelt)

Note: If no bike/car accident, think non accidental trauma.

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10
Q

What is the most common pediatric solid tumor of the pancreas?

A

Solid and papillary epithelial neoplasm (SPEN)

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11
Q

Pancreatic tumor in a 1 y/o…

A

Think pancreatoblastoma

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12
Q

Pancreatic tumor in a 6 y/o…

A

Think adenocarcinoma

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13
Q

Pancreatic tumor in a 15 y/o…

A

Think SPEN (solid and papillary epithelial neoplasm)

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14
Q

Liver mass in a kid age 0-3 y/o…

A
  • Infantile hepatic hemangioma (heart failure)
  • Hepatoblastoma (calcifications)
  • Mesenchymal hamartoma (predominantly cystic)

Note: Also consider mets from Wills tumor or neuroblastoma in any child.

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15
Q

9 month old with cardiomegaly and a large liver mass…

A

Think infantile hepatic hemangioma (which causes high output heart failure and cardiomegaly)

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16
Q

What lab value is classically elevated in infantile hepatic hemangioma?

A

Endothelial growth factor

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17
Q

Infantile hepatic hemangiomas are associated with…

A

Kasabach-Merritt syndrome (the platelet eater)

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18
Q

Newborn with hepatic tumor and enlarged aorta above the celiac trunk (normal below the celiac trunk)…

A

Think infantile hepatic hemangioma (enlarged aorta above the celiac due to increased blood supply to the hemangioma)

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19
Q

Treatment for infantile hepatic hemangiomas

A

Surgery isn’t needed, these tend to spontaneously involute without therapy over months-years as they progressively calcify

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20
Q

What is the most common primary liver tumor of childhood (<5 y/o)?

A

Hepatoblastoma

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21
Q

Hepatoblastoma is associated with…

A
  • Hemi-hypertrophy
  • Wilms
  • Beckwith-Weidemann
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22
Q

Risk factors for hepatoblastoma

A

Prematurity

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23
Q

Partially calcified right hepatic tumor in a 3 y/o that extends into the portal veins…

A

Think hepatoblastoma

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24
Q

Do hepatoblastomas usually have calcifications?

A

Yes (50% of cases)

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25
Q

What lab abnormality is classic in hepatoblastoma?

A

Elevated AFP

Note: Beta-hCG can also become elevated (may cause precocious puberty).

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26
Q

Kid with precocious puberty and a large liver mass…

A

Think hepatoblastoma

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27
Q

2 y/o with a predominantly cystic liver mass containing a large portal vein branch feeding the tumor…

A

Think mesenchymal hamartoma

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28
Q

AFP should be elevated/normal in a pt with a mesenchymal hamartoma

A

Normal (mesenchymal hamartomas are more of a developmental anomaly than cancer)

Note: Hepatic mass in a child with elevated AFP, think hepatoblastoma if less than 3 years old or hepatocellular carcinoma if over 5 years old.

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29
Q

7 y/o with a hepatic tumor and elevated AFP…

A

Think hepatocellular carcinoma and look for signs of cirrhosis

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30
Q

Risk factors for hepatocellular carcinoma in a child

A

Hepatic cirrhosis (e.g. biliary atresia, Falcon syndrome, glycogen storage disease)

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31
Q

Hepatic mass in a child 5 years or older…

A
  • Hepatocellular carcinoma (including fibrolamellar subtype)
  • Undifferentiated embryonal sarcoma

Note: Also consider mets from Wills tumor or neuroblastoma in any child.

32
Q

Partially calcified hepatic mass with a non enhancing T2 dark central scar in a 12 y/o without cirrhosis, and with a normal AFP…

A

Think hepatocellular carcinoma, fibrolamellar subtype

Note: The central scar of FNH should be T2 bright.

33
Q

What age group is fibrolamellar hepatocellular carcinoma most common in?

A

Younger pts (<35 y/o)

34
Q

Is the fibrolamellar subtype of HCC more or less likely to calcify than regular HCC?

A

The fibrolamellar subtype calcifies more often than regular HCC

35
Q

Would a fibrolamellar HCC show up on a gallium scan?

A

Yes, fibrolamellar HCC is gallium-avid

36
Q

Large, hypotenuse cystic liver mass with separations and a fibrous pseudocapsule in a 7 y/o…

A

Think undifferentiated embryonal sarcoma (more aggressive cousin of the mesenchymal hamartoma that is seen in older >5 y/o pts)

Note: Mass rupture is common in undifferentiated embryonal sarcoma.

37
Q

Which primaries should you think of if you suspect liver mets in a child?

A
  • Wilms tumor
  • Neuroblastoma
38
Q

Prenatal ultrasound demonstrated fetal hepatomegaly and a large placenta with a maternal history of sex work…

A

Think congenital syphilis

Note: The liver is the earliest organ involved and the last to resolve after treatment.

39
Q

Prenatal ultrasound for advanced maternal age demonstrated fetal hepatomegaly…

A

Think transient abnormal myelopoiesis (a pre leukemia syndrome seen only in Downs syndrome)

Note: Most of the time this gets better on its own, but in 20% of cases this can progress to myeloid leukemia of Downs syndrome.

40
Q

What is the most common type of choledochal cyst?

A

Type 1 choledochal cyst (focal dilatation of the CBD)

41
Q

What are the types of choledochal cysts?

A
  • 1 (CBD dilatation)
  • 2 (extra hepatic bile duct diverticulum)
  • 3 (choledochocele)
  • 4 (intra and extra hepatic involvement)
  • 5 (intrahepatic only, Caroli’s disease)
42
Q

Large, saccular intrahepatic bile duct dilatation with a “central dot sign”…

A

Carolis disease

Note: Caroli’s is autosomal recessive.

43
Q

Carolis disease is associated with…

A
  • Polycystic kidneys
  • Medullary sponge kidney
44
Q

Hereditary hemorrhagic telangiectasia (AKA Osler-Weber-Rendu)

A

An autosomal dominant disorder characterized by multiple AVMs in the liver and lungs (predisposing to paradoxical emboli/brain abscesses), leading to cirrhosis and massively dilated hepatic arteries

45
Q

Inheritance pattern of Caroli’s disease

A

Autosomal recessive

46
Q

Inheritance pattern of hereditary hemorrhagic telangiectasia (AKA Osler-Weber-Rendu)

A

Autosomal dominant

47
Q

Prolonged newborn jaundice > 2 weeks…

A

Think neonatal hepatitis (if gallbladder present)

OR

Biliary atresia (gallbladder usually absent)

48
Q

Treatment for biliary atresia

A

Surgery within 3 months of life (Kasai procedure)

49
Q

Which bile ducts are absent in biliary atresia?

A

Extrahepatic bile ducts (there is actually proliferation of the intrahepatic bile ducts)

Note: The gallbladder is usually absent as well.

50
Q

Biliary atresia is associated with…

A
  • Polysplenia
  • Trisomy 18
51
Q

Triangle cord sign (an echogenic triangular structure by the portal vein)…

A

Biliary atresia

Note: This is thought to represent the remnant of the CBD.

52
Q

Diagnostic imaging test for biliary atresia

A

Hepatobiliary scintigraphy with 99m Tc-IDA

53
Q

The purpose of a liver biopsy in biliary atresia is to exclude…

A

Alagille syndrome (hereditary cholestasis due to a paucity of intrahepatic bile ducts)

54
Q

Pediatric pt with cholelithiasis…

A

Think sickle cell disease

55
Q

How does sickle cell usually affect the spleen?

A

The spleen progressively enlarges and then auto-infarcts and disappears (usually during the first decade of life)

56
Q

Tachycardia and hypotension in a kid with sickle cell disease and a large spleen on imaging…

A

Acute splenic sequestration crisis

57
Q

What problems do kids with sickle cell disease run into if their spleen gets progressively larger, but does not auto-infarct?

A
  • Acute splenic sequestration crisis
  • Splenic abscess
  • Large splenic infarcts (different from auto-infarction)
58
Q

Large area of hypoechoic splenic parenchyma with linear echogenic bands running through it…

A

Think splenic infarction

59
Q

If you can’t identify the spleen in a child…

A

Think auto infarction in sickle cell disease

60
Q

What are the major abnormalities in right-sided heterotaxia syndrome?

A

Left-sided structures are messed up:

  • Two fissures in left lung
  • Asplenia
  • Increased cardiac malformations
  • Aorta is on the right (and IVC is on the left)

Note: This is also called bilateral right-sidedness or right isomerism.

61
Q

What are the major abnormalities in left-sided heterotaxy syndrome?

A

Right-sided structures are messed up:

  • Only one fissure in the right lung
  • Polysplenia
  • Azygos continuation of the IVC

Note: This is also called bilateral left-sidedness or left isomerism.

62
Q

Cardiac malformations are more common in which heterotaxy syndrome?

A

Right-sided

Note: In right isomerism, the left-sided structures (e.g. heart) are messed up.

63
Q

Azygos continuation of the IVC is seen in right/left sided heterodoxy syndrome

A

Left-sided

Note: In left-sided heterodoxy syndrome, the right-sided structures (e.g. IVC) are messed up.

64
Q

Situs solitus

A

Normal positioning of internal organs

65
Q

Situs inversus totalis

A

Total mirror image transposition of the abdominal and thoracic viscera

66
Q

Situs ambiguus

A

AKA heterotaxy, when you have abnormal arrangement of some visceral organs across the left-right axis

67
Q

Situs inversus is associated with…

A

Primary ciliary dyskinesia

68
Q

Heterotaxy syndrome with left isomerism is associated with…

A

Biliary atresia (10%)

Note: In left-sided heterotaxy, the right sided organs (e.g. liver) are messed up.

69
Q

HIDA scintigraphy in an infant demonstrates no radiotracer excretion into the bowel by 24 hours…

A

Biliary atresia

70
Q

On axial slices the SMV is in the 2:00 position relative to the SMA…

A

Malrotation (increased risk for midgut volvulus)

71
Q

What is the V in VACTERL?

A

Vertebral anomalies (37%)

72
Q

What is the A in VACTERL?

A

Anus, imperforate (63%)

73
Q

What is the C in VACTERL?

A

Cardiac anomalies (77%)

Note: This is the most common anomaly in VACTERL associations.

74
Q

What is the T in VACTERL?

A

Tracheoesophageal fistula or esophageal atresia (40%)

75
Q

What is the E in VACTERL?

A

tracheoesophageal fistula or Esophageal atresia (40%)

76
Q

What is the R in VACTERL?

A

Renal anomalies (72%)

77
Q

What is the L in VACTERL?

A

Limb/radial ray anomalies (58%)

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