In addition to lucent metaphyseal bands, other musculoskeletal findings are diffuse osteopenia, focal osteolytic lesions, periostitis (rare) and sutural diastasis. The osteopenia can result in multiple compression fractures of the spine.

b.  F

Lead and other heavy metal intoxication result in dense metaphyseal lines. Additional findings of lead poisoning are widening of the cranial sutures and metaphyseal flaring.

c.  T

Other findings of hypervitaminosis A are premature closure of the physes and widening of the cranial sutures due to hydrocephalus.

2.

E

Hepatic cysts are not associated with Beckwith-Wiedemann Syndrome.

Beckwith-Wiedemann syndrome is a autosomal dominant syndrome resulting in over growth of multiple organs and body parts. Findings include gigantism, hemihypertrophy, enlargement of the abdominal organs and tongue, abdominal wall defects such as omphalocele and bowel malrotation. There is an increased risk of malignancy including Wilms tumor, hepatoblastoma and adrenal carcinoma.

3.

a.  T

Von Gierke, Pompe, Cori, Andersen, McArdle and Hers diseases compose the glycogen storage diseases and are autosomal recessive.

b.  T

Glycogen deposition in the liver results in hepatomegaly and increased echogenicity and attenuation of the liver.

c.  F

Pompe disease is marked by cardiac involvement and children typically die within the first year of life secondary to conduction abnormalities.

d.  T

4.

E

This is a finding associated with Trisomy 18

Down syndrome occurs in 1/600-700 live births. Congenital heart disease is very common with an incidence of 40-70%. Common types are endocardial cushion defect, ASD, VSD and patent ductus. Other findings include 11 ribs, duodenal atresia, tracheoesophageal fistula, hypersegmentation of the sternum, cleft palate, biliary atresia, hypoplasia of the nasal bones, clinodactyly, flared iliac wings and flattened acetabular roofs, annular pancreas, Hirschsprung disease, anorectal malformation and umbilical hernia.

5.

a.  T

Hemangioendothelioma is the most common liver tumor in the pediatric patient with 85% presenting before 6 months. Due to its vascularity, there is AV shunting resulting in CHF. Hemangioendothelioma is seen with cutaneous hemangiomas in 50%. Lesions typically involute over time.

b.  F

Hepatoblastoma often has coarse calcifications sometimes with an osseous matrix. This calcification can help differentiate hepatoblastoma from hemangioendothelioma which typically has fine granular calcification. Hepatoblastoma also secretes alpha fetal protein. Hepatoblastoma is the most common malignant pediatric liver neoplasm with metastases most common to the lungs. There is an association with the Beckwith-Wiedemann syndrome.

c.  T

Fibrolamellar hepatocellular carcinoma is a variant of HCC that occurs in younger patients and, though, malignant has a better prognosis. Unlike HCC, there is usually no underlying cirrhosis and there is no elevation of the alpha fetal protein.

d.  T

Other pediatric conditions associated with increased incidence of hepatocellular carcinoma are galactosemia, tyrosinemia, alpha 1 antitrypsin deficiency, Wilsons disease and biliary atresia.

6.

a.  T

80% of affected fetuses will have their hands in this configuration.

b.  F

Cysts > 1cm are associated with increased risk of trisomy 18.

c.  T

In addition, the face can demonstrate hypotelorism and clefts.

d.  F

This is one of the most common abnormalities in Trisomy 18.

e.  F

Typically affected fetuses demonstrate intrauterine growth retardation.

7.

D

Pericardial defects most commonly occur on the left with less common involvement of the diaphragmatic surface. It is more common in males. Thirty percent are associated with bronchogenic cysts, cardiac malformations, sequestrations and diaphragmatic hernias. Typically there are no symptoms though symptoms can occur with herniation of heart or lung. ECG changes can occur. CXR in a partial defect may  demonstrate a normal heart position with a prominent left atrial appendage. A complete defect will produce displacement of the heart to the left with rotation such that the left heart border is formed by the right side of the heart (RAO view). The abnormality can simulate a pneumothorax or pneumomediastinum when lung enters the defect and lies between the heart and diaphragm or aortic arch.

8.

C

This is a good appearance for a neonate with congenital rubella infection.

Congenital rubella presents with intrauterine growth retardation. Neurologic deficiencies are possible with microcephaly and calcifications in the basal ganglia. The bones may demonstrate the "celery-stalk" sign with coarsened longitudinal trabeculae. Congenital heart disease such as patent ductus arteriosus is common.

9.

A

 Hypoplastic left heart syndrome describes underdevelopment of the left heart chambers and valves as well as the ascending aorta. It is the most common cause of CHF in the neonate. The right ventricle supplies blood to the systemic circulation via a patent ductus arteriosus. A large ASD is necessary to shunt blood from the left atrium to the right heart. There is typically retrograde flow in the ascending aorta to supply the coronaries. The chest x-ray is marked by pulmonary edema and right heart enlargement. Therapy includes the Norwood procedure (conduit between the pulmonary artery and descending aorta) and heart transplant.

10.

a.  T

The Fontan procedure produces a connection between the right atrium and pulmonary artery.

b.  F

The Norwood procedure is a conduit between the pulmonary artery and descending aorta used in hypoplastic left heart syndrome.

c.  F

A right ventricle to pulmonary artery conduit is often used in Tetralogy of Fallot to bypass the obstructed right ventricular outflow tract.

d.  T

The Waterston-Cooley procedure is an anastomosis between the ascending aorta and right pulmonary artery.

11.

a.  T

Malrotation is most common in the neonate or young infant with a high incidence in the first month of life.

b.  F

Duodenal atresia is associated with Downs syndrome.

c.  F

This would be an ominous sign.

d.  F

Barium enema will demonstrate an abnormally high cecum with a atypical orientation. Although a normal barium enema is 97% accurate in excluding malrotation, upper GI is the test of choice.

12.

D

Kawasaki’s disease is also known as mucocutaneous lymph node syndrome. It is an acute, febrile, multi-system vasculitis with a predilection for the coronary arteries. Patients are usually less than 5 years of age at presentation and have cervical adenopathy in 80 %. There is also mucosal reddening and erythema of the palms. Possible myocardial complications include: coronary artery aneurysms (usually proximal), myocarditis, CHF, myocardial infarction, or rupture of the aneurysms. Hydrops of the gallbladder is an additional well known finding.

13.

A

Lymphoma is the third most common malignancy in children after leukemia and central nervous system tumors.  Pediatric patients with Hodgkin lymphoma most commonly present with asymptomatic cervical adenopathy. Fever and night sweats are common. One third of patients will have mediastinal involvement at the time of presentation (this is less than seen in adults). There is continuous extension of adenopathy often into the mediastinum and occasionally from there into the hila and lungs. Adenopathy in the posterior mediastinum is rare.

14.

C

Meconium peritonitis results from intrauterine gastrointestinal perforation. Although commonly associated with cystic fibrosis, any cause of bowel obstruction can cause the perforation including bowel atresia, malrotation, hernia and intussusception. In utero, the perforation will appear as ascites with dilated loops of bowel. Meconium will calcify within 12-24 hours and may be visible as echogenic masses. The rupture can seal in utero or remain open requiring surgical repair. Meconium can pass through a patent processes vaginalis into the scrotum and calcification within the scrotum is highly suggestive of meconium peritonitis.

15.

A

Coarctation describes narrowing of the aorta. The adult form occurs distal to the ductus arteriosus while the infantile form occurs proximal to the ductus. It appears as narrowing of an extended segment of the arch between the brachiocephalic artery and the ductus. The ductus provides a majority of the systemic blood flow in utero bypassing the coarctation. Therefore, infants are not born with collateral formation and subsequently there is no rib notching. When the ductus closes at approximately 48 hours, congestive heart failure is possible. Coarctation of the aorta has many associations including bicuspid aortic valve in 25-50%, other cardiac abnormalities, Turners syndrome and cerebral aneurysms.

16.

C

Imperforate anus occurs in about 1 of 5000 births. Low anomalies in which the rectum has descended below the levator sling are most common. High imperforate anus is the second most common type and has a high association with multiple other abnormalities including those of the spine and GU tract. In fact the anus typically terminates in the posterior urethra resulting in urinary tract infections. In the postoperative patient a neurogenic bladder and anorectal incontinence are very common. The neurogenic bladder predisposes to urinary tract infection.

17.

a.  F

Alagille syndrome describes abnormalities of the bile ducts in conjunction with other congenital abnormalities.

b.  T

In addition, there is chronic cholestasis, posterior embrotoxon and pulmonary artery hypoplasia.

c.  F

Presentation is in the neonatal period with jaundice.

d.  T

However, Alagille syndrome has the other associated congenital abnormalities.

18.

a.  T

Common pathogens are Haemophilus influenzae, Streptococcus and Pneumococcus.

b.  F

Round pneumonia most commonly appears posteriorly in the lower lobes.

c.  F

Round pneumonia is much more common in children.

d.  F

Round pneumonia occurs because there is poor collateral airflow through the pores of Kohn and canals of Lambert.

19.

A

In pulmonary sling (aberrant left pulmonary artery) the left pulmonary artery arises from the right pulmonary artery and travels from right to left between the trachea and the esophagus. In addition to exerting mass effect, pulmonary sling is also associated with hypoplasia and dysplasia of the trachea and major bronchi. Specifically, the tracheal cartilaginous rings can be complete without a posterior membranous portion. This results in an extended length of severe tracheal narrowing termed the napkin ring trachea." The mass effect of the sling most commonly narrows the right main bronchus resulting in hyperexpansion or atelectasis of the right lung. The left hilum typically appears low on radiographs and the carina may be T shaped.

20.

a.  T

The adenopathy may be out of proportion to the pulmonary involvement.  The hilar nodes enlarge first with common involvement of the mediastinal nodes.

b.  T

Primary TB has no lobar predilection though is often subpleural. The lung involvement is often not visible on chest x-ray and almost always less striking than the nodal enlargement.

c.  F

Pleural effusion is a common findings in tuberculosis.

d.  T

Although it is rare for children to get miliary TB, very young children are more susceptible to this form of the disease.

21.

D

The superior parathyroid glands are derived from the 4th pharyngeal pouch and descend with the thyroid gland into the neck. They typically lie in close proximity to the thyroid, often within it. The inferior parathyroid glands are derived from the 3rd pharyngeal pouch and migrate into the neck with the thymus. They can remain within the neck in close proximity to the thyroid or carotid bifurcation. Alternatively they can continue into the upper chest lying in the anterior mediastinum. A location posterior to the innominate vessels in the posterior mediastinum is extremely rare.

22.

C

Typically dense nephrogram

Hypoperfusion syndrome describes profound shock in the pediatric patient. Recognition on CT of the abdomen is important because children are often able to maintain their blood pressure even with significant hypovolemia. Thus clinicians may be unaware of their volume depleted condition. CT findings include fluid filled, slightly dilated loops of bowel, intense contrast enhancement of organs and the bowel walls, decreased diameter of the IVC and aorta and peritoneal fluid.

23.

E

Pyloric stenosis occurs at 3-6 weeks. It is more common in boys. Patients typically present with projectile vomiting. Diagnosis can be made by ultrasound or upper GI. On ultrasound the muscular wall of the pylorus should be less than or equal to 4mm in thickness. The pyloric channel should be less than or equal to 16 mm in length. Upper GI demonstrates a dilated stomach with prominent peristaltic waves termed a caterpillar appearance. The pylorus should appear markedly narrowed resulting in a string sign. The pyloric channel is enlarged indenting both the gastric antrum and duodenal bulb.

24.

B

Bladder exstrophy is exposure of the internal mucosa of the bladder through a defect in the anterior abdominal wall. The bladder mucosa is continuous with the skin. The etiology is incomplete retraction of the cloacal membrane preventing  formation of the normal anterior abdominal wall and musculature. In males exstrophy is accompanied by epispadias and in females there is a bifid clitoris and a cleft along the dorsal urethra. Exstrophy has many associations including omphalocele, inguinal hernias, uterine anomalies, vertebral anomalies and meningomyelocele, undescended testicles and imperforate anus. If untreated, patients will develop UVJ obstruction. There is also increased risk of bladder cancer. Postoperatively the bladder will appear small and demonstrate reflux on VCUG.

25.

C

Prune belly syndrome is also known as the Eagle-Barrett syndrome. It is a congenital abscence of the musculature of the abdominal wall with marked dilatation of the renal collecting systems. It almost always occurs in males and classically there is cryptorchidism. The lack of musculature and urine ascites lead to severe abdominal distention. Severe cases have multiple associations including dysplastic kidneys, patent urachus and prostatic hypoplasia. Prune belly syndrome appears on VCUG as marked dilatation of the bladder with reflux and a patent urachus.

26.

C

This is the most likely diagnosis for a solid renal mass in a child less than three months.

Mesoblastic nephroma is a hamartoma. It is often large upon presentation and primarily solid with cystic areas and possible calcification. The mass is typically resected because of a questionable relationship to sarcomatous degeneration.

27.

a.  T

Meconium plug is also associated with cystic fibrosis in up to 25% of cases. Cystic fibrosis is also associated with meconium ileus. 10-15% of CF infants present with meconium ileus. Almost all patients with meconium ileus have CF. Meconium ileus appears as small bowel obstruction and can be treated with a hyperosmolar barium enema.

b.  T

Meconium plug also has associations with prematurity, preeclampsia and various drugs.

c.  T

Clinically these entities present similarly. Theoretically on barium enema, the rectum should distend normally in the setting of meconium plug syndrome but not with Hirschsprung's disease. However, often patients with barium enemas typical for meconium plug syndrome undergo rectal biopsy.

d.  F

The typical appearance of meconium plug syndrome is narrowing of the descending and sigmoid colon with dilatation of the more proximal colon. The differential for microcolon is diabetic mothers, maternal magnesium sulfate use, ileal atresia, meconium ileus and diffuse Hirschsprung's disease.

28.

C

Infantile polycystic kidney is also known as autosomal recessive polycystic kidney disease and results from abnormal dilatation of the collecting tubules. Depending upon the severity of involvement, the disease can present in utero (most common) or later with a juvenile form presenting at 1-5 years of age. There is typically both hepatic and pancreatic involvement with preferential involvement of the kidneys in the infantile type and of the liver in the juvenile type. In fact, these older patients typically die from cirrhosis. Oligohydramnios leads to pulmonary hypoplasia. The kidneys are enlarged with a sunburst/striated nephrogram. The cysts are often too small to be visible by ultrasound though the kidneys may appear hyperechoic.

29.

a.  T

85% of neuroblastomas calcify verses less than 15% for Wilms tumor.

b.  F

Wilms tumor more frequently metastasizes to the lung. Neuroblastoma frequently metastasizes to the bones which is rare for Wilms tumor.

c.  T

Wilms tumor tends to extend into vessels such as the renal vein.

d.  F

Only 75% of neuroblastomas are located in the abdomen. Other common sites include the sympathetic chain and organ of Zuckerkandl.

30.

a.  F

Croup causes narrowing of the subglottic trachea termed the steeple sign on frontal radiographs. The narrowing is secondary to edema of the mucosa and results in partial airway obstruction producing dilatation of the hypopharynnx.

b.  T

Croup involves patients older than 6 months with a peak at 2 years while epiglottitis starts at 3 years with a peak at 6 years.

c.  F

Croup is typically related to viral infection such as RSV or parainfluenza virus.

d.  T

Although rare, membranous croup is related to superimposed bacterial infection typically by staph aureus.

e.  T

Epiglottitis can create sub-glottic narrowing in 25% of cases and, thus, can simulate croup on a frontal radiograph. A lateral view is necessary in the setting of subglottic narrowing to document a normal epiglottis thereby excluding epiglottitis.

31.

a.  F

More than 75% of patients survive NEC. Other statistics include >50% of affected infants are premature and signs of disease typically occur 48-72 hours after birth.

b.  F

The terminal ileum is the most commonly involved location. The initial finding is bowel dilatation with pneumotosis occurring in 80%. This gives the bowel a bubbly appearance.

c.  F

Air in the portal vein is frequently transient and is not indicative of a poor outcome. Perforation on the other hand carries a poor prognosis and requires surgical intervention. It should be noted that perforation can present as ascites with or without free air.

d.  T

Strictures occur in approximately 20% of patients most commonly involving the colon. Thus, patients may benefit from barium enema following recovery.

e.  F

Barium enema is typically contraindicated in the setting of NEC due to increased risk of perforation. At times, however, water soluble enemas may be helpful.

32.

B

Esophageal atresia/ TEF is a common congenital abnormality of the esophagus. It is associated with such disorders as the VACTERL syndrome as well as trisomies 13, 18, and 21. Symptoms depend upon the anatomy of the TEF. Isolated esophageal atresia (10%) presents with inability to handle any liquid at birth and excessive drooling. TEF presents with choking with feeding, If a TEF goes unrecognized (most typically seen with the H type- 1 % of TEF), patients present with recurrent pneumonias. Classically esophageal atresia presents with inability to pass a nasogastric tube and a gasless abdomen. However, most patients will have abdominal gas due to the presence of a fistula in 90%.  The most common type of  TEF is the N-type in which the proximal esophagus is blind-ending and the distal esophagus originates from the trachea. TEF can be detected in utero by polyhydramnios in 33-60%. 
        

33.

A

 Intussusception describes the invagination of a segment of bowel (intussusceptum) into a adjacent segment (intussuscepiens.) In the pediatric age group 95% are idiopathic while 5% have a pathological lead point (Meckel's diverticulum is most common.) Peak incidence is 18 months to 2 years. Findings on KUB include RUQ soft tissue mass and SBO. CT will show multiple rings of bowel in one location giving the appearance of intraluminal fat. Ultrasound findings include the doughnut, target , bulls eye and pseudokidney signs. Barium enema will demonstrate the coiled spring appearance. Intussusceptions can be reduced with barium, gastrograffin (1:4) or air enema with a success rate of greater than 75%. It should be noted that there is a 3-10 % risk of recurrence. A balloon can be employed and is sized to the interdedicular distance at L5. For air reduction pressures should range from 80 to 120 mm HG.

34.

C

Henoch-Schonlein purpura is a pediatric vasculitis typically preceded by infection. An immune response results in the deposition of IgA in blood vessels. Patients present with purpura, abdominal pain, and GI and GU bleeding. Glomerulonephritis results in proteinuria. HSP typically presents on barium studies and abdominal CT as small bowel wall thickening.

Parathyroid adenomas result in hypercalcemia which in turn can precipitate pancreatitis.

L Asparaginase therapy is a well known cause of pancreatitis. Other implicated medications are steroids and acetaminophen.

35.

E

Biliary atresia is classically associated with abscence of the gallbladder. In actuality the gallbladder can be seen in approximately 20% of cases. In this setting the gallbladder is typically small.

Hydrops of the gallbladder is massive dilatation of the gallbladder. There are multiple causes including Kawasaki disease, various infections, sepsis, TPN, nephrotic syndrome and neonatal hepatitis. The gallbladder wall usually appears normal and the hydrops typically resolves with conservative management.

36.

a.  T

Potter facies has low set ears and a receding chin.

b.  F

Potter syndrome is secondary to bilateral renal agenesis and, thus, there is decreased amniotic fluid. Potter sequence describes changes of Potter syndrome secondary to any cause of severe in utero renal failure such as posterior urethral valves, polycystic kidney disease, and bilateral multicystic dysplastic kidney

c.  T

The ratio of male to female is almost 3:1.

d.  T

Oligohydramnios decreases the size of the uterus causing club foot deformity. Other sequelae include pulmonary hypoplasia and bladder agenesis.

37.

B

Klebsiella is not typically associated with cystic fibrosis.

Aspergillus results in allergic bronchopulmonary aspergillosis in patients with cystic fibrosis.

Facts regarding cystic fibrosis:

Autosomal recessive disease with an incidence of 1/2000 caucasian births.

Can be detected in utero as hyperechoic bowel.

Causes elevated levels of chloride and sodium in sweat.

Lung involvement is first seen in the apical and posterior segments of the upper lobes.

Paraseptal bullae can rupture causing pneumothorax.

GI/GU changes include pancreatic insufficiency and pancreatitis, cirrhosis, rectal prolapse, meconium ileus/peritonitis and infertility in males.

Median survival is 28 years.

38.

a.  T

A large majority of effected patients present in the neonatal period with 90% presenting in the first year.

b.  F

There is equal involvement of all lobes. CCAM result from abnormal development of the lung parenchyma though there is normal vascular supply and a connection (often abnormal) to the tracheobronchial tree.

c.  T

25% of cases are associated with other abnormalities. Type 2 is most commonly associated with extra-pulmonary anomalies.

d.  F

The macrocystic variety also known as type 1 has cysts>2 cm and the best prognosis. The microcystic type (III) is less common and may appear as a solid mass. It has the worst prognosis.

39.

a.  T

Cystic fibrosis is associated with meconium ileus with 10-15% of CF infants presenting with meconium ileus. Meconium plug is associated with cystic fibrosis (25%), Hirschsprung disease, prematurity, and maternal magnesium sulfate treatment

b.  F

It is thought that poor contraction by the colon is the primary abnormality. Colonic motility can often be stimulated with a barium enema resulting in resolution of the functional obstruction.

c.  F

Narrowing of the descending colon is the hallmark of meconium plug syndrome (also called neonatal small left colon syndrome). In addition, a long meconium plug is often identified in this location.

d.  T

Hirschsprungs diffusely involves the colon starting distally and thus there will be narrowing of the rectum. However, patients with meconium plug syndrome often undergo rectal biopsy for complete certainty.

40.

a.  T

Other common associations include omphalocele, neural tube defect such as anencephaly, and Trisomies 13, 18 and 21. Therefore, if a hernia is detected in utero amniocentesis is indicated.

b.  F

Bochdalek hernias occur in 90% of cases. These are usually on the left and posterolateral.

c.  F

ECMO is a temporary measure to stabilize the neonate for definitive surgery. Complications of ECMO include intracranial hemorrhage, pneumothorax, pulmonary hemorrhage and pleural effusion.

d.  F

Congenital diaphragmatic hernia has 60% mortality due to the associated pulmonary hypoplasia. This usually effects the ipsilateral side with common contralateral involvement due to mediastinal shift.

41.

B

Metaphyseal cupping though seen in many diseases including trauma is typically associated with rickett's.