Pediatrics Section Congenital Heart Disease is included in the Angio-Interventional Section The following are True/False

questions: 1995, 1992 Regarding croup and epiglottitis: 1. croup characteristically narrows the supraglottic airway (95, 92) 2. croup patients tend to be younger (92) 3. croup is more urgent (92) 4. croup is caused by H. influenza (92) 5. subglottic tracheal narrowing is seen in up to 25% of patients with epiglottitis (95, 92) 6. viral infection is the most common etiology of croup (95) 7. Staphylococcus aureus is the most common cause of membranous croup (95) * Epiglottitis narrows the epiglottis and aryepiglottic folds. Epiglottitis occurs in children older than 3 years, with the peak at 6 years. Epiglottitis is caused by bacteria - usually H. influenze, type B (less likely pneumococcus or Streptococcus Group A). The patient is in mortal danger of suffocation secondary to complete airway closure. Patients often require intubation. Edema can be so severe that it extends to the subglottic area in 25% Croup usually narrows the subglottic airway. Croup thickens the glottis and vocal cords. Croup occurs in children older than 6 months, with the peak at 2-3 years. Croup (acute laryngotracheobronchitis or acute viral spasmodic laryngitis) is usually caused by a virus (parainfluenza, respiratory syncytial virus) and is usually selflimiting. Bacterial croup is possible (called pseudomembranous croup, membranous croup, or bacterial tracheitis) and this can be severe, with the patients often needing tracheostomy. Staphylococcus aureus is usually the causative organism. * Answer: 1. False 2. True 3. False 4. False 5. True 6. Tru 7. True 1995, 1994, 1992 Regarding necrotizing enterocolitis: 8. it results in 60% mortality (95) 9. it most commonly affects the descending colon (95) 10. an air fluid level in the peritoneum indicates perforation (95) 11. air in the portal venous system is always fatal (95) 12. strictures occur in 10-25% of patients (95) 13. term infants have better prognosis than premature infants 14. barium enema can be helpful to exclude other diagnoses 15. finding often confirmed by plain film 16. pneumotosis indicates impending death * Necrotizing enterocolitis is a disease of bowel in infants subjected to hypoxic stress. Splancnic ischemic events lead to indirect mucosal injury, which, in immature gut, allows bacterial overgrowth. Approximately 80% of patients with necrotizing enterocolitis are premature. Term infants or older infants who develop necrotizing enterocolitis usually have severe underlying disease such as congenital heart disease or Hirschsprung disease (do they do worse??) The most common finding in necrotizing enterocolitis is dilated bowel (due to ileus). Pneumatosis is the second most common finding According to Dahnert, barium enema is contraindicated, but “may be used judiciously in selected cases where there is radiologic and clinical doubt.” The Merck manual reports that 2/3 of patients with necrotizing enterocolitis will survive (33% mortality). Gore, Levine, and Laufer reports that the “majority survive.” The terminal ileum is the most common site. An air fluid level in the peritoneum indicates perforation, which is an indication for emergent surgery. However, air in the portal vein is not (necessarily) a preterminal event (unlike adults). In fact, it is not necessarily an indication for emergent surgery. Strictures occur in 6-33% according to Gore, Levine, and Laufer. References: Kirks, pp. 869-876; Dahnert 1993, p. 530 * Answer: 8. False 9. False 10. True 11. False 12. True 13. False? 14. Fals 15. True 16. False 1995 Regarding tracheoesophageal fistula: 17. oligohydramnios 18. greater than 90% present with a fistula 19. ??? *

Answer: 17. Prob False

18. True

19. ???

1993, 1992v Regarding intussusception: 20. the most common site of perforation is the intussusceptom 21. ileocolic is the most common type 22. a lead point is more likely in a recurrent intussusception 23. it most commonly occurs in 18 m/o - 5 y/o. 24. a lead point is found in 50% of recurrent intussusceptions * The most common site of perforation is the place which is most ischemic within the intussusception (intussusceptom centrally or intussuscepiens peripherally?). Perforations have also been reported to occur at the rectum upon reduction no matter where the intussuception resides. Intussusception occurs more in children (94%) than adults (6%). In children, ileocolic is the most common by far (90%) followed by ilioileal (4%) and then colocolic. In adults, ilioileal is most common (40%) followed by ileocolic. In most cases of infantile intussusception, no lead point is identified. A lead point is most likely (5%) in newborns (Meckel’s, polyp, tumor) and in recurrent intussusception. There is an increased incidence in cystic fibrosis patients - the meconium ileus equivalent syndrome. Most intussusceptions occur between the ages of 6 months to 4 yrs with a peak incidence between 18 months and 3 years. References: J. Meyer; Swischuk, 2nd ed., p. 418; Rumack, p. 1210 * Answer: 20. False 21. True 22. True 23. True 24. False 1993, 1991, 1989 Which of the following are causes of gall bladder hydrops in a young child? 25. Kawasaki’s disease 26. sepsis 27. TPN 28. neonatal hepatitis 29. extrahepatic biliary atresia 30. prolonged fasting * Hydrops of the gallbladder is defined as massive distention of the gallbladder without evidence of a congenital malformation, stone, or inflammatory etiology. Most cases are idiopathic. The following are all causes of GB hydrops: - Kawasaki’s - Sjogren’s - Systemic sclerosis - nephrotic syndrome - leukemia - familial Mediterranean fever - TPN - scarlet fever - leptospirosis - ascariasis - typhoid - staph/strep Atresia of the bile duct occurs in about 1 of 15,000 live births. 20% of patients with extrahepatic biliary atresia will have a remnant GB. Types of biliary atresia: I - atresia of CBD II - atresia of CHD III - atresia in the portal right and left ducts Treatment of biliary atresia is with the Kasai procedure in which hilar dissection and adjacent placement of a bowel conduit is performed. Treatment of GB hydrops is conservative with percutaneous drainage if necessary. Avoid surgery. References: Caffey’s, p.452; Walker (Ped GI), p. 1167 * Answer: 25. True 26. True 27. True 28. False 29. False 30. Prob False, should be normal 1993 Which of the following are causes of pancreatitis in a child? 31. choledochal cyst 32. parathyroid adenoma (hyperparathyroidism) 33. cystic fibrosis 34. Henoch-Schonlein purpura 35. L-asparaginase therapy * Reference: Kirks, pp. 785-786, 840

* Answer: 31. True

32. True

33. True

34. True

35. True

1992 Renal agenesis is associated with: 36. typical facies 37. agenesis of the bladder 38. lung hypoplasia 39. oilgohydramnios 40. club foot * There are two main categories of renal aplasia - unilateral and bilateral. Bilateral renal agenesis is also called Potter’s disease and has an incidence of 1:3000-1:10000. It has a typical facies (low set ears, redundant skin, and a parrot beak nose), and is associated with lung hypoplasia, oligohydramnios, and club foot. Unilateral renal agenesis has an incidence of 1:600-1:1000. It has no typical facies and is not associated with agenesis of the bladder, lung hypoplasia, oligohydramnios, or club foot. It does have agenesis of the ipsilateral hemitrigone of the bladder and renal artery, however. * Answer: (unilateral) 36. False 37. False 38. False 39. False 40. False (bilateral) 36. True 37.False 38.True 39. True 40.True 1992 Which of the following organisms commonly affect patients with cystic fibrosis? 41. Klebsiella 42. Staphylococcus 43. Stretococcus viridans 44. Haemophilus 45. Pseudomonas * Answer: 41. True 42. True 43. False 44. True 45. True 1993 Regarding lung development: 46. tracheal development is finished around 20 weeks 47. development of alveoli continues after birth 48. pulmonary arterial muscle extends down to the alveoli level 49. the pulmonary artery and bronchial tree develop in parallel 50. the trachea is a foregut structure * Tracheal development from the foregut is finished at approximately 30-32 days. Alveoli develop after birth * Answer: 46. False 47. True 48. False 49. True 50. True 1994, 1991 Regarding cystic fibrosis: 51. it is an autosomal recessive disease 52. there is atresia of the vas deferens in males 53. there is a higher incidence of gastroesophageal reflux 54. the right upper lobe is most commonly affected 55. meconium plug syndrome is the presenting feature in 10-20% of cases * Cystic fibrosis is an autosomal recessive disease with approximately 5% of Caucasians as carriers. There is atresia of the vas deferens (Harrison’s) with poor development of other Wolffian structures. Although the upper lobes are more commonly affected in the lungs, we could find no reference stating that the right was more common. 5% of patients with cystic fibrosis are born with meconium ileus. Patients can also develop meconium ileus later in life (undigested food and motility problem). This is completely separate from the meconium plug syndrome, which is not related to cystic fibrosis. This is a functional obstruction of the left colon - it is treated with a water soluble enema. It is often seen in infants of diabetic mothers. Other findings in cystic fibrosis: biliary/hepatic cirrhosis (which is rarely clinically significant), rectal prolapse, pancreatitis/pancreatic dysfunction, malabsorption, and gallstones. Interesting fact - if you see gall stones in kids think cystic fibrosis, sickle cell disease, or TPN!

Reference: Dahnert * Answer: 51. True 52. True

53. prob False

54. False

55. False

1994 Regarding cystic adenomatoid malformation: 56. it is more common in males than females 57. it has upper lobe predominance 58. it usually presents within the first six months of life 59. it is associated with other congenital abnormalities 60. it is less common in full term babies * Cystic adenomatoid malformation of the lung is a type of hamartoma - disordered but otherwise normal pulmonary tissue - which arises from the terminal portion of the bronchial tree. It occurs with equal frequency in both lungs but has an upper lobe predominance. Two thirds of the cases present in the first week of life due to compression of normal lung/airways. One third remain asymptomatic. 25% have associated anomalies of the kidneys, GI tract, or chromosomes. It occurs with equal frequency in males and females. Major types: 1) macrocystic - best prognosis (Type I) - 50% 2) mixed - multiple small (1-2 cm) cysts. Poor prognosis, secondary to associated anomalies (Type II) - 40% 3) microcystic - (rarest) mass of tiny cysts which may appear solid. Poor prognosis secondary to pulmonary hypoplasia/hydrops. The major complication is pulmonary hypoplasia. (Type III) - 10% Reference: Dahnert 1993, pp. 302-303 * Answer: 56. False 57. False 58. True still within first 6 months 59. True 60. True 1994, 1992 Regarding congenital diaphragmatic hernias: 61. Morgagni hernias are more common 62. intestinal malrotation is a common association 63. the ipsilateral lung eventually becomes normal in size 64. ECMO allows normal development of the pulmonary acini 65. it is more common in the preterm vs. term infant 66. the contralateral lung is normal in size 67. acute bilious vomiting after correction indicates midgut volvulus * Diaphragmatic hernia is the most common intrathoracic fetal anomaly. It occurs in approximately 1 in 400 live-born infants and is twice as common in males. They are typically left-sided. 85-90% of congenital diaphragmatic hernias are the Bochdalek (posterolateral) variety - only 1-2% are Morgagni (anteromedial) hernias (which occur more commonly on the right). Traumatic diaphragmatic hernias are more common on the left. The ipsilateral lung is hypoplastic, probably due to compression during development, and will not develop normally. In addition, the contralateral lung is compressed by the shifted mediastinum and also does not develop normally.(but is not hypoplastic). In a typical case, the critically ill infant survives the operation and appears to do well for several hours (referred to as the honeymoon period). Later, the infant’s condition deteriorates because of pulmonary artery hypertension with right-to-left shunting through the patent ductus arteriosus. GI abnormalities occur in 20% and malrotation is a common association. Normal bowel fixations are absent or loosened in congenital diaphragmatic hernias and the duodenum is poorly fixed; therefore, incomplete rotation and anomalous mesenteric attachment of bowel is present. Intestinal malroatation is common and predisposes to midgut volvulus. While Dahnert makes no mention of prematurity, congenital diaphragmatic hernias are associated with IUGR and stillbirth (30%). They are also associated with CNS abnormalities in 28% (mainly neural tube defects), cardiovascular abnormalities in 13-23%, and GU abnormalities in 15%. In ECMO (extracorporeal membrane oxygenation) cannulas are placed in the neck - blood is dranied from the right atrium through the internal jugular vein, circulated through a membrane oxygenator and heat exchanger, and then returned to the systemic circulation through a cannula in the common carotid artery. The most impressive results have been obtained in infants needing short-term support, such as those with meconium-aspiration syndrome, neonatal respiratory distress syndrome, or sepsis. Results have been less satisfactory in infants with diaphragmatic hernia. Treatment with heparin is required, precluding the use of this technique in infants with intracranial hemorrhage or extreme prematurity. References: Dahnert 1993, pp. 303-305; Swischuk, pp. 100-106, p. 447, NEJM Vol. 319, No. 2 (7/14/88), pp. 87-88 * Answer: 61. False 62. True 63. False 64. False 65. False 66. False 67. True

1991 Findings suspicious for child abuse include: 68. single long bone fracture 69. multiple fractures in different stages of healing 70. metaphyseal cupping 71. metaphyseal chip fracture * A spiral long bone fracture is the most common fracture of child abuse-common but low specificity. Mult fx’s—moderate specificity Metaphyseal cupping is seen in rickets but can also be due to trauma.. Metaphyseal chip fx has high specificity for child abuse.* Answer: 68. False 69. True 70. False 71. True

1993 Regarding hypoperfusion syndrome in a child, which of the following are true? 72. hydrops 73. decreased size of the great vessels 74. delayed nephrogram 75. fluid in the peritoneal cavity * Answer: 72. False 73. True 74. False 75. True 1991 Ectopic parathyroid tissue can be found: 76. anterior to innominate veins 77. posterior to innominate veins 78. in the thymus 79. in the AP window * 10% of the population has ectopic parathyroid tissue. Ectopic parathyroid tissue is most commonly found in the anterior mediastinum (60%), but infrequently (8%) can be found in the posterior mediastinum. * Answer: 76. True 77. True 78. True 79. True 1995 my own Which of the following place a pediatric patient at high risk for latex hypersensitivity? 80. tetralogy of Fallot 81. meningomyelocele 82. unilateral renal agenesis 83. allergy to penicillin 84. bladder exstrophy * Patients with a history of anaphylaxis to latex are obviously at high risk. Patients with unexplained anaphylaxis during surgery are also considered high risk. Patients with meningomyelocele and bladder exstrophy have been proven to have a higher risk of anaphylactic reaction. Patients who have undergone multiple surgical procedures and patients with documented reactions to multiple (3 or more) intravenous drugs may also be at higher risk. Interestingly, no anaphylactic reactions have been reported in children less than 1 year old. * Answer: 80. False 81. True 82. False 83. False 84. True 1994 A one-day old neonate presents with bilious vomiting. An abdominal film demonstrates air within the distended stomach and duodenum with a small amount of air in the distal small bowel. The patient has a normal barium enema (specifically, there is no evidence of microcolon.) Which of the following are likely diagnoses? 85. duodenal atresia 86. preduodenal portal vein 87. midgut volvulus 88. duodenal web 89. pyloric stenosis * Kirks reports that with duodenal atresia, a minimal amount of air can go distally by way of the bile ducts.

A preduodenal portal vein could produce a high grade duodenal obstruction. A patient can have duodenal obstruction secondary to malrotation with midgut volvulus with a normal cecal position. (Kirks) Pyloric stenosis does not appear in 1-day olds - it is most commonly seen at 2-8 weeks of life and is more commonly seen in first born males. In addition, vomiting should not be bilious. Reference: Kirks * Answer: 85. True 86. True 87. True 88. True 89. False 1994 Regarding tuberculosis in children: 90. it presents with enlarged mediastinal lymph nodes * Answer: 90. True AFIP notes 1991 Extrophy of the bladder is associated with: 91. segmental sacral agenesis 92. epispadias 93. UVJ obstruction 94. pelvic symphysis diastasis 95. abdominal muscle deficiency * Extrophy of the bladder is the most common congenital bladder lesion. It is the result of deficiency in development of the lower abdominal wall musculature, so that the bladder is continuous with the skin. There is associated epispadias in which the urethra is open dorsally and urethral mucosa covers the dorsum of a short penis. Skeletal and gastrointestinal anomalies are commonly associated with extrophy. Diastasis of the symphysis pubis correlates well with the severity of the extrophy-epispadias complex. Reference: Dunnick 1991, pp. 30-31 * Answer: 91. True 92. True 93. True 94. True 95. True 1991 Which of the following are found in a neonate with AIDS? 96. LIP 97. periventricular lymphoid infiltrates 98. pneumothorax 99. pneumomediastinum * Answer: 96. True 97. True 98. False 99. False 1991 Regarding prune-belly syndrome: 100. cryptorchidism 101. hydroureteronephrosis 102. diastasis of symphysis pubis 103. patent urachus * Eagle-Barrett, or “prune belly” syndrome is a disorder of uncertain pathogenesis which almost exclusively affects males. It is usually a triad consisting of lax abdominal musculature, undescended testes, and various urinary tract abnormalities. The renal collecting systems are atonic, which produces a nonobstructive hydronephrosis. The resultant dilatation of the ureters, bladder, and posterior urethra can be mistaken for a case of posterior urethral valves. Associated urologic abnormalities include patent urachus and urachal remnants. * Answer: 100. True 101. True 102. False 103. True What of the following are likely causes of diffuse infiltrates in the chest in a child with AIDS? 104. Kaposi sarcoma 105. lymphoma 106. MAI 107. sarcoid * Answer: 104. False 105. True 106. True 107. False

Select the single best answer 1. A 5 d/o male is 2 days S/P cardiac catheterization for coarctation of the aorta. The patient develops abdominal distension and supine examination of the abdomen demonstrates pneumotosis of multiple small bowel segments. Which of the following is the most appropriate next examination? a. cross table lateral examination b. CT c. Doppler ultrasound of the aorta d. ??? * Answer: C probably 1993 2. Which of the following is the most likely cause for bright red blood in the diaper of a 6 month old? a. anal fissure b. juvenile polyp c. intussusception * Anal fissures are common in the neonate-to-1-year-old. They are likely secondary to constipation. They are usually seen dorsally at the midline. They cause bright red blood per rectum and bleeding after defecation. Juvenile polyps are rare in the neonate and present in the 2nd and 3rd years of life as blood in the stool. 80% are solitary. They are usually pedunculated and are usually benign. They are considered to be inflammatory lesions. They are usually seen in the left colon. Intussusception presents in the 3 m/o to 3 y/o age group with colicky abdominal pain. Stool may test heme positive or may have the classic (but infrequent) “currant jelly” appearance. Patients also have vomitting/diarrhea with an abdominal mass in >50%. Rectal bleeding is more frequent in the neonate. 70-90% are ileocolic. Only 310% have an intrinsic bowel abnormality (duplication, Meckel’s, hemangioma, lymphoma, polyp) References: Nelson’s, p. 813; Gore, Levine, Laufer p. 1477, 1480 * Answer: a. anal fissure 1995 3. In a 1 y/o, what is the most common cause of ileocolic intussusception? a. lymphoma b. idiopathic c. Meckel’s diverticulum d. lymphoid hyperplasia e. ??? * Answer: B 1994 4. What is the most common cause of a limp in a child without evidence of a fracture? a. osteomyelitis b. toxic synovitis c. septic arthritis d. Legg-Perthes Disease e. coalition * Kirks states that septic arthritis in children is usually the result of direct extension of osteomyelitis into the joint; therefore osteomyelitis is more likely than septic arthritis. Toxic synovitis (transient synovitis) = Toxic synovitis usually manifests as hip or knee pain which has acute onset. It is usually seen in patients less than 10 y/o with no history of trauma or infection. Males are more commonly affected than females. Joint aspiration shows no viral or bacterial source. Treatment is usually rest, after which the condition usually resolves. A small percentage of cases go on to Legg-Perthes. * Answer: B. Toxic synovitis 1998, 1994, 1992, 1991 5. Regarding tracheoesophageal fistula and esophageal atresia - which of the following is the most common associated anomaly? a. renal agenesis

b. anal atresia c. syndactyly d. limb anomalies e. polyhydramnios * The most common associated anomaly is cardiac (patent ductus arteriosus, ASD, VSD, right-sided aortic arch) (up to 40%), followed by limb (24%), GI (anorectal and duodenal atresia, 20%), GU (unilateral renal agenesis, 12%). Polyhydramnios is uncommon when there is a tracheoesophageal fistula present (3%) - but it is very common with an isolated tracheal atresia (60%). The most common form of esophageal atresia is a blind-ending proximal portion (mediastinal) with the distal esophagus connecting to the trachea just above the carina. Prematurity is common. This used to be a uniformly fatal condition - now “death in infants with esophageal atresia should be confined to those with irreparable brain or heart defects.” (NEJM, Vol. 319, No. 2, p. 87.(7/14/88)) In esophageal atresia alone (occurs once in every 4000 births), associated anomalies are seen in 17%. In tracheoesophageal fistula alone, anomalies are seen in 23% (mostly cardiovascular). V - vertebral anomalies A - anorectal anomalies (including atresia) C - cardiovascular anomalies (PDA, VSD, ASD, right aortic arch) T - trachea E - esophageal fistula R - renal anomalies (unilateral renal agenesis) A - associated with Trisomy 13, 18, 21 L - limb anomalies (radial ray hypoplasia, polydactyly) Reference: Dahnert, p. 507 * Answer: d. limb anomalies 1998, 1994, 1992 6. Which of the folowing is the most common renal mass in a 2-month old? a. rhabdomyosarcoma b. Wilms tumor c. mesoblastic nephroma d. neuroblastoma e. medullary cystic disease * Differential diagnosis for renal mass in < 1 year old: fluid filled: hydronephrosis > dysplastic > multilocular cystic nephroma > cyst solid: mesoblastic nephroma > nephroblastomatosis > Wilms’ Differential diagnosis for renal mass in 1-10 year old: fluid filled: hydronephrosis > dysplastic > multilocular cystic nephroma > cyst solid: Wilms’ > nephroblastomatosis > adenocarcinoma Sarcomas rank fifth in incidence among malignant solid tumors of children under the age of 15. About 50% are rhabdomyosarcomas, which are aggressive, high-grade tumors that originate in striated muscle and spread by local extension and through the lymphatics and the blood stream. The most frequent primary sites are the head and neck (40%), the genitourinary tract (20%), and the extremities (20%). Three pathological categories of rhabdomyosarcomas: 1. embyonal tumors (70%) - includes sarcoma botryoides 2. alveolar rhabdomyosarcoma (20%) 3. pleomorphic, or undifferentiated, tumors (10%) Although an age under one year is a favorable prognostic factor in patients with Wilms’ tumor and neuroblastoma, it is not a good prognostic factor in those with rhabdomyosarcoma. References: AFIP notes (Davidson, July, 1994, p. GU-75); NEJM, Vol. 319, No. 2 (7/14/88), p. 92 * Answer: c. mesoblastic nephroma 1998, 1994 7. On prenatal ultrasound, a fetus is found to have oligohydramnios and bilateral renal cysts. What is the most likely diagnosis? a. autosomal recessive polycystic kidney disease b. autosomal dominant polycystic kidney disease c. medullary cystic disease

d. multicystic dysplastic kidney e. congenital nephrosis * According to Dahnert, multicystic dysplastic kidney is the most common form of cystic disease in infants. Reference: Dahnert * Answer: d. multicystic dysplastic kidney 1994 8. A child presents with cervical adenopathy, rash, skin changes in his palms, a large heart, and interstitial edema. What is the most likely diagnosis? a. Takayasu’s disease b. Kawasaki’s disease c. Henoch Schonlein purpura d. Goodpasture’s syndrome * Kawasaki’s disease is also called mucocutaneous lymph node syndrome. It is an acute, febrile, multisystem vasculitis with predilection for the coronary arteries. Patients are usually less than 5 y/o at presentation and have cervical adenopathy in 80%. There is also mucosal reddening and erythema of the palms. Possible myocarcial complications include: coronary artery aneurysms, myocarditis, CHF, MIs, or rupture of the aneurysms. Description of Taka. Reference: Dahnert * Answer: b. Kawasaki’s disease 1994 9. Regarding Hodgkin’s disease in a child - the most common presentation would be: a. cervical adenopathy b. subcarinal adenopathy c. pleural effusion d. retroperitoneal adenopathy e. parenchymal disease * Reference: Kirks, p. 666 * Answer: a. cervical adenopathy 1994 10. A child has a history of high imperforate anus. He now has a urinary tract infection. What is the most likely cause? a. vesicoureteral reflux b. rectourethral reflux c. neurogenic bladder d. posterior urethral valves * Imperforate anus occurs in about 1 of 5000 births. 40% of patients with high imperforate anus have genitourinary anomalies including rectourethral fistula (which are usually identified and repaired at the initial surgery), horseshoe kidney, renal agenesis/hypoplasia, ureteral anomalies, vesicoureteral reflux, and anatomic bladder anomalies. If the patient is postoperative, the answer is probably vesicoureteral reflux. Dahnert states the 50% of cases are associated with spinal anomalies - specifically defective sacral segmentation. Posterior urethral valves are the most common cause of urethral obstruction in the male infant. They are remnants of the Wolffian duct, and form obstructing folds at the level of the verumontanum. References: Kirks; Dahnert; NEJM, Vol. 319, No. 2 (7/14/88), p. 90 * Answer: a. vesicoureteral reflux 1994 11. What is the most common other anomaly seen in a child with a history of high imperforate anus? a. renal agenesis b. rectourethral fistula c. malrotation

d. tracheoesophageal fistula e. vertebral anomalies * Answer: d. 1993 12. Which of the following is likely to be seen in a child within AIDS? a. lymphoid interstitial pneumonia b. immunoblastic lymphadenopathy c. ? * Lymphoid interstitial pneumonia is predominately seen in immunocompromised patients and often in children (M = F). It can be difficult to distinguish it from lymphoma. It has a monoclonal gammopathy (IgM). Immunoblastic lymphadenopathy (angioimmunoblastic lymphadenopathy) is a hyperimmune disorder (likely B cells) which may closely resemble Hodgkins and shows a polyclonal hyperglobulinemia. It is most commonly seen in >50 y/o males. * Answer: a. lymphoid interstitial pneumonia 1991 13. The most common cause of cirrhosis in chilhood is: a. thick bile syndrome b. hepatoblastoma c. hepatic vein thrombosis d. idiopathic portal vein obstruction * Thick bile syndrome secondary to cystic fibrosis can cause cirrhosis. * Answer: a. thick bile syndrome 1992, 1988 14. Which of the following is the most likely cause of an enlarged calcified scrotum in a neonate? a. teratoma b. meconium peritonitis. * Meconium peritonitis results from intrauterine gastrointestinal perforation (from obstruction, volvulus, or intestinal ischemia and necrosis). A few patients can present with swelling or mass in the scrotum secondary to passage of meconium through a patent processes vaginalis. Calcification within the scrotum provides an almost foolproof sign of meconium peritonitis. Testicular teratoma is the second most common testicular tumor is young boys (Leydig most common?) - it accounts for 4-10% of testicular tumors. However, testicular tumors are only 1.5% of all childhood “malignancies.” They are benign in children but may transform into malignancy in adulthood. There is an increase serum alphafetoprotein level. References: Swischuk, pp. 572-573; Dahnert, p. 602 * Answer: b. meconium peritonitis 1998, 1992 15. Complete tracheal ring (napkin ring trachea) is seen with: a. pulmonary sling b. double aortic arch c. right aortic arch d. left aortic arch with aberrant right subclavian artery * 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. It then swings around at the junction of the trachea and the right mainstem bronchus. It can be seen alone or with tracheal stenosis caused by a complete tracheal cartilagenous ring (usually the ring is incomplete posteriorly). This is called the “ring sling” syndrome! With a double aortic arch, a vascular ring encircles the trachea and esophagus. In a right aortic arch, there is no vascular ring unless there is an aberrant left subclavian artery and left ligamentum arteriosum or a PDA is present. In these cases, the vascular ring will encircle both the trachea and the esophagus.

In a left aortic arch with aberrant right subclavian artery patients are usually asymptomatic, probably because there is no right ligamentum arteriosum or right PDA to create a full ring. This is the most common vascular anomaly of all the “vascular rings.” References: Swischuk, pp. 313-322, Dahnert, pp. 364-365 * Answer: a. pulmonary sling 1992 16. A child with biphasic stridor gets upset when his head is flexed. What is the most likely diagnosis? a. pulmonary sling b. double aortic arch c. right aortic arch d. aberrant left subclavian artery e. left aortic arch with aberrant right subclavian artery * Answer: B 1992 17. Bochdalek diaphragmatic hernia is: a. midline b. anteromedial c. anterolateral d. posteromedial e. posterolateral * Also see discussion of question 14. Bochdalek (near the patients “boch”!) = posterolateral Morgagni = anteromedial Reference: Dahnert 1993, pp. 303-304 * Answer: e. posterolateral 1993 18. In a child, regarding a cyst in the thyroid: a. it is more likely to be malignant than in an adult b. is less likely to be malignant than in an adult c. no difference * Answer: C 1993, 1991 19. A two-day-old boy presents with jaundice. On ultrasound there is a cystic mass over the right kidney. What is the most likely diagnosis? a. choledochal cyst b. renal cyst c. adrenal hemorrhage d. choledochocele e. abscess * The most common masses in this area are UPJ obstruction and multicystic dysplastic kidney. GI duplications are usually in the RLQ. Choledochal cysts are the cause of RUQ cystic mass in 5%. Patients with congenital cystic dilatation of the biliary tree usually present with symptoms of biliary obstruction. * Answer: a. choledochal cyst 1993 20. Which of the following is the least common finding in the shaken child syndrome? a. small bilateral subdural hematomas b. white matter shearing injury c. bilateral contusions on the surface of the brain * Answer: b.

1994 21. A child presents with cyanosis, attempted pursing of the lips, choanal atresia, and multiple other anomalies. There is prompt relief of symptoms with placement of an oral airway. Which of the following is the most appropriate examination? a. airway fluoroscopy b. tracheal MR c. nasopharyngeal CT d. chest X-ray e. echocardiography * Neonates with choanal atresia are obligate nose-breathers - any nasal problem has the potential to cause severe respiratory distress. Oral airway relief suggests an upper airway abnormality. (?macroglossia -Pierre Robin?) Therefore, the most appropriate test is probably airway fluoroscopy. * Answer: a, airway fluoroscopy 1994 22. A small child has biphasic inspiratory stridor. She is irritable when the neck is bent forward and she is hoarse. What is the most likely diagnosis? a. double aortic arch b. pulmonary sling c. cervical aortic arch d. aberrant right subclavian artery from left aortic arch e. patent ductus arteriosus * Double aortic arch: represents persistence of both fourth embryonic arches. The trachea and esophagus are trapped by the vascular ring that results. Anterior indentation on the trachea (made by the left arch) is often visible on a lateral chest X-ray. There is also posterior indentation on the esophagus (produced by the right arch). Pulmonary sling: is the most significant sixth-arch anomaly, wherein the left pulmonary artery arises from the right pulmonary artery (instead of the main) and passes between the trachea and the esophagus as it enters the left hemithorax. This can cause tracheomalacia, and consequently respiratory compromise usually develops in infancy. Cervical arch: usually asymptomatic Aberrant right subclavian artery with left aortic arch: does not form a vascular ring and is usually asymptomatic. The subclavian artery passes posterior to the esophagus. Patent ductus arteriosus: will not cause stridor. * Answer: a. double aortic arch (m/c sx ring) 1993E 23. Which one of the following is not a characteristic finding in Alagille syndrome? a. chronic cholestasis b. abnormal facies c. renal agenesis d. butterfly vertebra e. pulmonary artery hypoplasia * Alagille syndrome (also known as arteriohepatic dysplasia) has the following findings: 1) abnormal facies 2) chronic cholestasis 3) posterior embryotoxon - a congential opacity of the periphery of the cornea 4) butterfly vertebra 5) pulmonary artery hypoplasia/stenosis 6) “other congenital heart disease” 7) osteopenia, undertubulation Reference: Caffey’s, p. 4110 * Answer: c. renal agenesis 1994 24. What is the most common cause of pulmonary hypoplasia in a patient with polycystic kidney disease?

a. oligohydramnios b. kidney enlargement c. hormonal * Answer: a. 1991 25. In a neonate with adrenal hemorrhage, where did the hemorrhage originate? a. cortex b. medulla * Reference: Dunnick 1991, pp. 274-275 * Answer: A. occurs centrally within the cortex 1991 26. In rapid decceleration injury to the kidney of a child, which of the following is the most common injury? a. subcapsular hematoma b. parenchymal laceration c. renal artery intimal tear d. renal vein thrombosis e. renal pelvic tear * Answer: ?C 1991 27. Hypertrophic pyloric stenosis is diagnosed by ultrasound if the pyloric muscle is ? mm thick. a. 2 b. 4 c. 6 d. 8 e. 12 * Answer: b. 4 (greater than (or equal to?) 4 mm) 1998, 1991 28. Round pneumonia in a child is most likely due to: a. gram positive organism b. gram negative organism c. anerobic flora d. virus * Answer: A (strep) 1995 29. In a 2 week old infant with a history of shortness of breath, what is the most likely cause of an interstitial pattern on chest radiograph? a. parainfluenza b. chlamydia c. mycoplasma d. pneumococcus * Answer: B The following are matching questions: kde1995 Regarding pediatric liver neoplasms: 1. hemangioendothelioma 2. hepatoblastoma 3. hepatocellular carcinoma 4. fibrolamellar carcinoma 5. metastases from neuroblastoma

a. elevated alpha fetoprotein b. cirrhosis c. glycogen storage disease d. associated with CHF e. central scar f. osteoid and cartilage within lesion Answer: 1. D 2. A ,F 3. B and C or A? 4. E 5. F?

1994 5. endocardial cushion defect 6. cystic hygroma 7. duodenal atresia a. Trisomy 21 b. Trisomy 18 c. 45 XO d. 47 XXY e. 47 XYY * 45 XO is Turner’s syndrome. Cystic hygroma is a type of lymphangioma which is most commonly seen in the head, neck, and axilla. It is most commonly seen in hydrops fetalis and Turner’s syndrome. Turner’s syndrome is also characterized by shield-shaped chest, short stature, and short fourth metacarpals. Coarctation of the aorta and aortic stenosis are common. Trisomy 21 is Down’s syndrome. It is associated with a long list of things, some of which are below: 1. duodenal atresia (seen in 30%), duodenal stenosis 2. esophageal atresia 3. congenital heart disease in 40-70% (including endocardial cushion defect, ASD, and VSD) 4. 11 rib pairs 5. hypersegmentation of the manubrium 6. cleft palate 7. biliary atresia 8. hypoplasia of the nasal bones 9. clinodactyly 10. flattened acetabular roofs 11. flared iliac wings 12. annular pancreas 13. Hirschsprung disease 14. anorectal malformation 15. umbilical hernia References: AFIP notes, August, 1994, p. KK5, bone lectures; Dahnert * Answer: 5. a. (Down’s) 6. c. (Turner’s (73%), also seen in Down’s, but less common) 1992 8. Beckwith-Wiedemann 9. von Gierke a. macroglossia b. hepatomegaly c. exophthalmos d. hepatic cysts e. polyuria * Beckwith-Wiedemann Syndrome (GOMPE) G = gigantism (including visceromegaly (liver, pancreas, kidneys), hemihypertrophy, natal/postnatal gigantism (with microcephaly) O = omphalocele M = macroglossia P = pancreatic islet hypertrophy --> hypoglycemia

7. a.

(Nephroblastomatosis also may be present at birth and this may give rise to Wilms tumor later in life. Patients are also at increased risk for hepatoblastoma, adrenal cell carcinomas, and adrenal cystic lesions.) E = exophthalmos von Gierke’s disease is Type I glycogen storage disease. There is hepatomegaly from a defect in glucose6-phosphatase. This leads to excess deposition of glycogen in the liver (also in the kidneys and intestines). There is also increased deposition of fat in the liver. References: Dahnert, p. 441, 629; Swischuk, p. 814, 866 * Answer: 8. a, b, c 9. b 1992 10. leukemia 11. heavy metal intoxication 12. vitamin A a. Wimberger sign b. lucent metaphyseal bands c. dense provisional zone of calcification d. periosteal reaction * Leukemia is associated with: 1. diffuse osteopenia (15-100%) 2. radiolucent (and radiodense) metaphyseal bands (10-85%) 3. osteolytic lesions (30-50%) 4. periostitis (10-35%) 5. sutural diastasis Heavy Metal Intoxication: 1. Lead (lead paint, batteries) a. radiodense metaphyseal lines (lead replaces calcium) b. metaphyseal flaring or widening c. widening of cranial sutures 2. Phosphorous a. radiodense metaphyseal lines (phosphorous replaces calcium) 3. Bismuth (across placenta of woman with syphilis treated with bismuth) a. single/multiple radiodense metaphyseal bands in tubular bones b. less often resembles syphilitic osteochondritis or osteonecrosis 4. Aluminum (antacids, TPN, phosphate binders and dialysis fluids) a. osteopenia b. rickets-like changes c. Looser’s zones (insufficiency fractures) d. periostitis e. fractures 5. Copper (malnutrition, Menky’s kinky hair syndrome) a. osteopenia b. periostitis c. metaphyseal radiodense lines and spurs d. fractures e. physeal disruptions Hypervitaminosis A (in children) a. cortical thinning usually in areas of associated soft tissue nodules, especially ulna, metatarsals, and occipital bones. b. cupping, shortening, and splaying of the metaphysis c. narrowing of the cartilagenous growth plates d. hypertrophy and premature fusion of the epiphyseal ossification centers leading to growth abnormalities. e. sutural widening f. differential diagnosis = infantile cortical hyperostosis (Caffey’s disease) Hypovitaminosis A a. increased intracranial pressure leading to widened sutures (infants)

b. dental developmental abnormalities Reference: Resnick 1989, pp. 924-930, 703-709 * Answer: 10. B (best answer), d 11. c 12. d 1995 ITE 13. colonic pneumatosis 14. colonic stricture 15. colonic shortening a. meconium plug b. Hirschsprung’s disease c. imperforate anus d. meconium ileus e. necrotizing enterocolitis * The characteristic pathology of Hirschsprung’s disease is an absence of parasympathetic ganglia in the submucosal and intermuscular plexuses of the colon. The deficit always involves the lower rectum but can extend proximally, rarely involving the entire colon and small bowel (limited to rectum and sigmoid colon in 74%). The incidence is 1 in 5000 live births. In 20% of cases, no transition zone is seen on barium enema. Full-thickness rectal biopsy shows absence of ganglion cells. Diagnosis based on acetylcholinesterase histochemistry and anorectal manometry is almost 100% accurate. In Hirschsprung’s disease there are abundant nerve fibers in the bowel wall; these are rich in acetylcholinesterase which stains deeply. An endorectal pull-through is the most common treatment - rectal mucosa is removed and normally innervated bowel is pulled through the muscular sleeve. Reference: NEJM, Vol. 319, No. 2 (7/14/88), p. 89 * Answer: 13. e 14. E 15. c 1991 16. dextrocardia 17. male infertility 18. bronchiectasis a. cystic fibrosis b. immotile cilia syndrome c. both d. neither * Reference: Dahnert 1993, p. 303 * Answer: 16. b 17. c 18. c

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