Hepatobiliary Disorders and Pediatric Conditions
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Questions and Answers

What is the most common benign vascular tumor in infants?

  • Choledochal Cysts
  • Hepatoblastoma
  • Infantile Hemangioendothelioma (correct)
  • Biliary Atresia
  • What type of Choledochal Cyst is known as Caroli's disease?

  • Type 3
  • Type 5 (correct)
  • Type 2
  • Type 1
  • What is one of the key functions of the biliary tree?

  • To break down and absorb nutrients (correct)
  • To regulate temperature
  • To regulate blood pressure
  • To produce hormones
  • What sonographic feature is associated with Biliary Atresia?

    <p>Triangular Cord sign (C)</p> Signup and view all the answers

    Which of these is NOT a sign or symptom of Hepatoblastoma?

    <p>Alcoholic stool (C)</p> Signup and view all the answers

    What is the sonographic appearance of Infantile Hemangioendothelioma?

    <p>Hypoechoic or hyperechoic mass/masses (C)</p> Signup and view all the answers

    What is the name of the duct also known as Wirsung's duct?

    <p>Pancreatic Duct (C)</p> Signup and view all the answers

    Which of these conditions is more common in premature infants and infants with low birth weights?

    <p>Hepatoblastoma (A)</p> Signup and view all the answers

    Which of the following signs/symptoms may indicate Adrenal Gland Hemorrhage?

    <p>Painful swelling (C)</p> Signup and view all the answers

    What is the most common cause of hydronephrosis in pediatric patients?

    <p>Ureteropelvic junction obstruction (C)</p> Signup and view all the answers

    A sonographic appearance of multiple noncommunicating cysts within the renal bed is characteristic of:

    <p>Multicystic Dysplastic Kidney (C)</p> Signup and view all the answers

    Which of the following is NOT a risk factor for Adrenal Gland Hemorrhage?

    <p>Hypothyroidism (B)</p> Signup and view all the answers

    What is the characteristic sonographic appearance of a Wilms' Tumor in its early stages?

    <p>Large, spherical mass with heterogeneous echogenicity (D)</p> Signup and view all the answers

    Which of the following is a common sonographic finding in patients with Autosomal Recessive Polycystic Kidney Disease (ARPKD)?

    <p>Bilateral kidney enlargement with echogenic kidneys (C)</p> Signup and view all the answers

    Which of these conditions is primarily related to the development of immature nerve cells?

    <p>Neuroblastoma (C)</p> Signup and view all the answers

    What is the most common sonographic appearance of a Mesoblastic Nephroma?

    <p>Large, solid, homogeneous mass (A)</p> Signup and view all the answers

    Flashcards

    Biliary Atresia

    Life-threatening hepatobiliary disorder in children with persistent jaundice and abnormal GB.

    Signs of Biliary Atresia

    Persistent jaundice, alcoholic stool, dark urine, enlarged girth.

    Types of Choledochal Cysts

    Types 1 to 5 describe different dilations of the common bile duct and intrahepatic ducts.

    Sonographic Features of Choledochal Cysts

    Fusiform dilation of CBD with intrahepatic ductal dilation.

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    Biliary Tree Connections

    Liver, gallbladder, and pancreas connect for digestion and waste removal.

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    Infantile Hemangioendothelioma

    Most common benign vascular tumor in infants, often leading to jaundice and heart failure.

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    Hepatoblastoma

    Rare malignant liver tumor common in premature infants and those with low birth weight.

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    Elevated Serum Alpha Fetoprotein (AFP)

    Indicator of liver tumors like hepatoblastoma and hemangioendothelioma.

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    Adrenal Gland Hemorrhage

    Bleeding in the adrenal gland caused by various risk factors.

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    Neuroblastoma

    A cancer from immature nerve cells, often presenting as an abdominal mass.

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    Hydronephrosis

    Dilation of the renal collecting system, commonly from UPJ obstruction.

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    Multicystic Dysplastic Kidney (MCDK)

    Nonfunctioning kidney replaced by multiple noncommunicating cysts.

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    Autosomal Recessive Polycystic Kidney Disease (ARPKD)

    Genetic disorder with fluid-filled cysts in the kidneys leading to complications.

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    Wilms' Tumor (Nephroblastoma)

    A kidney tumor seen as a palpable abdominal mass with variable appearance.

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    Mesoblastic Nephroma

    A benign kidney tumor typically found in newborns and young infants.

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    Study Notes

    Hepatobiliary Disorders

    • Biliary Atresia: A life-threatening disorder in children, characterized by persistent jaundice, acholic stools, dark urine, and enlarged abdomen.

      • Sonographic finding: Triangular cord sign (echogenic area above portal vein). Abnormal gallbladder in a fasting infant.
    • Choledochal Cysts: Abdominal pain, vomiting, and jaundice are common symptoms.

      • Types: Fusiform dilation of the CBD (type 1, most common), diverticula (type 2), intraduodenal dilation (type 3), combined intra/extrahepatic dilation (type 4), and intrahepatic duct dilation (type 5/Caroli's disease).
      • Sonographic appearance: Fusiform dilation of the CBD often with intrahepatic duct dilation
      • Prevalence: More common in Asian populations.

    Other Pediatric Liver/Kidney Conditions

    • Infantile Hemangioendothelioma: The most common benign vascular tumor in infants, more prevalent in females, causing jaundice, heart failure, and enlarged liver.

      • Sonographic appearance: Well-circumscribed, hypoechoic or hyperechoic mass(es) with visible color flow.
    • Hepatoblastoma: A rare malignant liver tumor more common in premature or low-birth-weight infants. Signs include palpable abdominal mass, elevated AFP, pain, and weight loss.

      • Sonographic appearance: Solid mass with similar echogenicity to the liver, often showing calcifications (55% of cases).
    • Adrenal Gland Hemorrhage: Caused by various factors, including anticoagulation, burns, surgery, and hypotension. Characterized by anemia, painful swelling, potential bluish discoloration, and initial echogenicity transitioning to hypoechogenicity.

      • Causes in healthy infants: Prematurity, prolonged labor, breech position, or large birthweight.
    • Neuroblastoma: Cancer originating from immature nerve cells, presenting as abdominal mass, hypertension, diarrhea, bone pain, and increased catecholamine levels.

      • Sonographic appearance: Small, homogeneous, hyperechoic mass (early); larger, heterogeneous, complex mass (later).

    Kidney Conditions

    • Hydronephrosis: Dilation of the renal collecting system, most commonly caused by ureteropelvic junction obstruction. Symptoms include palpable abdominal mass, oliguria, and anuria.

      • Sonographic appearance: Dilated renal pelvis (mild), dilated pelvis and calyces (moderate), and significant pelvicaliectasis with thinning of parenchyma (severe).
    • Multicystic Dysplastic Kidney (MCDK): Multiple cysts replace functional renal tissue, often non-communicating.

      • Symptoms (bilateral): Respiratory distress; Symptoms (unilateral): Hypertension and risk of Wilms' tumor.
      • Sonographic appearance: Multiple non-communicating cysts within the renal bed.
    • Autosomal Recessive Polycystic Kidney Disease (ARPKD): Genetic disorder causing fluid-filled cysts in the kidneys.

      • Symptoms: Pulmonary hypoplasia due to oligohydramnios.
      • Sonographic appearance: Bilaterally enlarged kidneys, echogenic kidneys, and poor corticomedullary differentiation.
    • Wilms' Tumor (Nephroblastoma): A cancerous kidney tumor appearing with a palpable abdominal mass, pain, and potential rupture/bleeding.

      • Sonographic appearance: Large, spherical, heterogeneous mass with varying appearances (hemorrhage, necrosis, cysts), often intrarenal, with well defined borders.
    • Mesoblastic Nephroma: A benign kidney tumor commonly diagnosed before or within the first three months of life, often presenting with a palpable abdominal mass.

      • Sonographic appearance: Large, solid, homogeneous mass.

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    Description

    This quiz covers key concepts related to hepatobiliary disorders, including biliary atresia and choledochal cysts, along with other pediatric liver and kidney conditions such as infantile hemangioendothelioma. Test your knowledge on symptoms, sonographic findings, and prevalence of these conditions.

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