Writtens 97 - 02 Peds

PEDS : 

PEDS Radiology Recalls From ’97 to ‘02 PEDS

2002 : 

2002 Multiple T/F: Which of the following organisms are found in increased incidence with cystic fibrosis?a. P. Aeruginosab. S. aureusc. H. influenzaed. P. cepaciae. Klebsiella pneumoniae

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All True.“The organisms most often responsible for pulmonary infection are P. aeruginosa of the mucoid and nonmucoid types, S. aureus, and H. influenzae. The presence of P. cepacia is associated with the terminal stages of the disease.” Fraser 2nd p. 685.Some differences of opinion may be secondary to age group; one article reported:- Pseudomonas spp was cultured from respiratory secretions of 51 (42%)- Staphylococcus spp in 18 (15%)- Klebsiella spp in 8 (7%) - Hemophilus influenzae in 2 (2%) patients http://www.medscape.com/viewarticle/459366 “The most common bacterial pathogens in the sputum of patients with CF are Haemophilus influenzae, Staphylococcus aureus, P aeruginosa, Burkholderia cepacia, Escherichia coli, and Klebsiella pneumoniae.” http://www.emedicine.com/PED/topic535.htm

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Which of the following is true regarding C-spine injuries in kids?a. fractures are more common than soft tissue injuriesb. flexion teardrop fractures are the most commonly missed injuriesc. C1-C2 injuries are more common than C6-C7 injuries d.

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C. C1-C2 injuries are more common than C6-C7 injuries.The occipito-atlanto-axial segment is the most common site of cervical spine injury in children. [Resnick 2nd p. 800] This is because “…The fulcrum of flexion and extension is above C3-C4 in the infant and young child; it is below C3-C4 in the adolescent and adult” [Ped. Req. 2nd p. 297] 72% of spinal injuries under age 8 are cervical. Pediatric Cervical Spine: Normal Anatomy, Variants, and Trauma” Radiographics 2003:23:539-560

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Which of the following is false regarding ultrasound evaluation of hypertrophic pyloric stenosis?a. not useful status post surgical pylorotomyb. muscle is hyperechoic to normal mucosac. muscle thickness better than lengthd. should not empty stomach before imaginge. 3-4 mm muscle thickness considered abnormal

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B. muscle is hyperechoic to normal mucosaChoice B is the False statement (and thus the answer)—on U/S, pyloric stenosis will show a “target sign”, which is a hypoechoic ring of hypertrophied pyloric muscle around an echogenic mucosa centrally on cross-section. Dähnert 5th p. 833Pyloric muscle wall thickness = 3mm is abnormal. [Dähnert] Muscle thickness is better than length because HPS is an abnormality of the circular muscles—longitudinal muscles are unaffected. Gastric contents are used as an US window.(Req 2nd p. 87-88)

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Which of the following is not a risk factor for necrotizing enterocolitis?a. Hypoxiab. C-sectionc. hyperalimentationd. prematurity

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B. C-SectionRisk factors for NEC: < 2500 g, < 37 wk, PROM, preeclampsia, DM, multiparity, early feeding with high osmolar formula, placement of UAC and UVC. (Req 2nd p. 83) Also Hypoxia, stress, CHD (Dähnert 5th p. 851) Prematurity is the major single risk (50-80%); 90% of cases develop within 1st 10 days of life.

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Which of the following is false regarding Hirschsprung’s?a. aganglionic segment is proximal to ganglionic segmentb. dilates proximal to aganglionic segmentc. may present as mucous plugd.

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A. aganglionic segment is proximal to ganglionic segmentThe above statement is false because it is the aganglionic segment that causes a functional obstruction (and proximal dilatation); i.e., the aganglionic segment is distal to the ganglionic [normal] one. In fact, Hirschprungs’ nearly always involves the rectum (skip aganglionosis, though possible, is very rare). Dähnert 5th p. 832.Hirschprung’s disease occurs secondary to an absence of parasympathetic ganglia in the muscle (Meissner plexus) and submucosal layers (Auerbach’s plexus), apparently secondary to a failure of craniocaudal migration. It may present as a Meconium plug syndrome or Meconium ileus. Ped. Req. 2nd p.115.

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Which of the following is best sign of a malrotation?a. high cecumb. jejunum to right of spinec. jejunum-duodenum junction medial and inferior to normal locationd. sigmoid volvulus

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C. jejunum-duodenum junction medial and inferior to normal locationThe most reliable indicator is the position of the ligament of Trietz, normally behind the stomach to the left of the left pedicle and at the level of the duodenal bulb which usually sits around L1/2. Other signs include SMA to left of SMV on US, high obstruction, possibly corkscrewing and abnormal position of bowel loops. Cecal position is not a reliable sign; in fact, malrotation may be associated with a normal cecum in 10-15%. (Req 2nd p. 107)

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Which of the following is not associated with hypovolemic shock in a child?a. dense nephrogramb. flat IVCc. peritoneal fluidd. dilated bowele. decreased enhancement of bowel wall

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E. decreased enhancement of bowel wall.AKA hypoperfusion complex or shock bowel on CT – important to know because kids can mask shock. Findings: intense enhancement of bowel wall, mesentery, adrenals, liver, kidneys, and pancreas. Intense enhancement and decreased caliber of IVC and Aorta. Diffusely dilated fluid filled bowel loops.(Donnelly 131-133) Peritoneal fluid may represent blood in a trauma patient.

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Why is surfactant given to premature infants?a. Further expand open alveolib. Open new alveolic. promote new alveoli growthd.

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A. Further expand open alveoli.Surfactant keeps airways from collapsing and causing atelectasis by diminishing surface tension of expanding alveoli. This promotes air exchange. (Primer 811)

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A child has leg length discrepancy, lower extremity weakness and a sacral dimple with a tuft of hair. Most likely diagnosis:a. chordomab. Myelomeningocelec. tethered cordd. anterior sacral meningiomae. sacral teratoma

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C. tethered cordNote: a meningomyelocele could cause similar symptoms but would present at birth.Tethered cord syndrome - Parasthesias, pain, neurogenic bladder, kyphoscoliosis, incontinence, spasticity. (Primer 570); Dorsal nevus, dermal sinus tract, hair patch in 50%; age of presentation 5-15 years (growth spurt) (Dähnert 5th p. 222). Other sources: Req 2nd p. 293

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Lückenschädel skull is associated with which of the following?a. hydrocephalus b. dandy walker malformation c. Myelomeningocele d.

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C. MyelomeningocelePractically always associated with myelocele or encephalocele with associated meningocele, and with Chiari II; also with spina bifida and cleft palate [Dähnert 5th p. 213]. Note that 85% of Chiari II will have a Lückenschädel skull [Dähnert 5th p. 269] Chiari II always has a myelomeningocele [AFIP syllabus ’02-’03, p. 748].Not caused by increased intracranial pressure and is found in normal sz heads w/o hydrocephalus. Probably secondary to dysplasia of calvaria and its internal periosteum. Usu disappear by 4-5mo. Differ from nl markings which are not visible until 1y.(Caffey 25)

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If a child has bilateral retinoblastomas, where is the next most common location? a. pineal glandb. pituitary gland-anterior lobec. hypothalamusd. pituitary gland-posterior lobe

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A. Pineal glandRetinoblastoma is a rare malignant congenital intraocular tumor arising from primitive photoreceptor cells of the retina. 66% sporadic, 20-25% heritable sporadic, and 5-10% Familial.Bilateral retinoblastoma is characteristic of the familial type (66% of familial will have bilateral tumors). Trilateral retinoblastoma will additionally have a neuroectodermal pineal tumor (pineoblastoma). Quadrilateral has an additional focus in the 4th ventricle. Source: Dähnert 5th p. 345.

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What are the best findings on chest x-ray for situs inversus?a. right stomach bubble and right cardiac apex (dextrocardia)b. right stomach bubble and right aortic knobc. right stomach bubble and left cardiac apexd. stomach on the right side and aortic knob on the lefte. stomach on the left side and cardiac apex on the right side

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A. Right stomach bubble and right cardiac apex (dextrocardia)Key concept—the most general application of the term “situs” is visceroatrial situs [assumed here]. Other uses may refer to the position of the ventricles, great vessels, or abdominal viscera.- position of the atria is almost always concordant with the viscera- discordance (e.g., right stomach bubble, left cardiac apex) ? high risk of CHDSitus inversus (totalis) indicates mirror image development of both the visceral and cardiac anatomy; incidence 0.01%. (Caffey 829); “left atrium and stomach on right and dextrocardia (cardiac apex on right)”—AFIP syllabus ’02-’03 p. 976.- “…the position of the aortic arch (left or right) is NOT a good indicator of situs” AFIP syllabus ’02-’03 p. 977 (bottom).Situs solitus (totalis) - normal; Heterotaxy – ambiguous. Dähnert 5th p. 582.

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Which of the following is not associated with Down’s syndrome?a. Duodenal atresiab. clinodactylyc. scaphocephalyd.

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C. ScaphocephalyScaphocephaly (Dolichocephaly) = premature closure of sagittal suture. Most common type of craniosynostosis (58%); many associations (sickle cell, rickets, hyperthyroidism, etc., etc.) but Down’s syndrome is not one of them. Dähnert 5th p. 174.Clinodactyly – curvature of finger in mediolateral plane (Down’s, normal variant, trauma, etc.) Dähnert 5th p. 21Trisomy 21 anomalies – duodenal atresia, hydrothorax, hydrops, omphalocele, microcephaly, brachycephaly, hypoplastic midface, hypotelorism, short femur, short humerus, epicanthal folds, flat nasal bridge, endocardial cushion defects, small frontal lobes, hypoplastic 5th digit and clinodactaly, sep of 1st and 2nd toes, flat occiput, speckled irides, protruding tongue, prominent ears, flattened acetabular and iliac angles, simian crease (Primer 776, baby Nelson 84)

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A child presents with hypotension and bilateral thalamic hemorrhage. Most likely diagnosis?a. dural sinus / venous thrombosisb. child abusec. hypertensiond. glioblastoma multiforme

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A. dural sinus / venous thrombosisHypotension (dehydration or low-flow state) would be a cause of venous thrombosis in a child. Bilateral subcortical hemorrhages not in an arterial distribution would also be suggestive (depending on the site of thrombosis). Neuro Req. p. 131. Dähnert 5th p. 276.Thalamic hemorrhage due to child abuse may be possible, but more characteristic would be epidural / subdural / subarachnoid hemorrhage(s), with or without diffuse axonal injury Neuro Req p. 164.

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An infant has leukokoria. CT exam shows unilateral microphthalmia with increased attenuation of the globe. Most likely diagnosis?a. Coat’s diseaseb. Retinoblastomac. Retrolental fibroplasiasd. Persistent hyperplastic primary vitreous (PHPV)e. retinopathy of prematurity

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D. Persistent hyperplastic primary vitreous (PHPV)“Leukokoria” refers to an abnormal white / pinkish / yellowish papillary light reflex. The key to this questions is unilateral microphthalmia—associated with PHPV, the 2nd most common cause of leukokoria overall (28%). Note that the microphthalmia in retinopathy of prematurity is bilateral.Otherwise, Retinoblastoma is the most common cause of leukokoria overall (at 58%); Coat’s disease comes in 3rd at 16%. Dahnert 5th p. 335.Leukokoria DDx: retinoblastoma, retinal detatchment, persistent hyperplastic primary vitreous, Toxocariasis, cataract, retrolental fibroplasia, Coat’s disease, trauma, retinopathy of prematuraty (Primer 602, baby Nelson 330)

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Latex allergy in an infant is most commonly associated witha. contrast allergyb. asthmac. myelomeningoceled. PCN allergy

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C. MyelomeningoceleThe following patient groups require latex precautions:known latex allergy, spina bifida including myelomeningocele and sacral or lumbosacral agenesis [* apparently due to multiple surgeries ealy in life], congenital urological anomalies including cloaca and bladder extrophy, and who have been advised to avoid contact. (Vanderbilt University Medical Center Latex Sensitivity Management)

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Which of the following is not seen in a Chiari II malformationa. tectal beakingb. myelomeningocelec. enlargement of massa intermediad. large posterior fossae. hydrocephalus

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D. Large posterior fossaChiari II “almost always” has a small posterior fossa; always has myelomeningocele [AFIP syllabus ’02-’03 p. 748].Other findings in Chiari II--Hydrocephalus, dysgenesis of corpus callosum, towering cerebellum, tectal beaking,lagrge mass intermediaIt is Dandy Walker with a large posterior fossa cyst, hypdrocephalus, cerebellar hypoplasia, agenisis CC Chiari I – tonsillar herniation, syringohydromyelia (Primer 557,560)

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A child has phleboliths within skeletal muscle mass. What is the most likely etiology?a. capillary hemamgiomab. cavernous hemangiomac. venous hemangiomad.

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B. Cavernous hemangiomaA cavernous hemangioma is located in the deeper soft tissues and is frequently intramuscular; Age: childhood. Phleboliths (dys-trophic calcification in organizing thrombus) are seen in nearly 50%.Venous hamangioma: may also occur in muscle and have phleboliths, but age is adulthood.Capillary hemangioma (“strawberry nevus”): typically skin, subcutaneous tissue, vertebral body. 75-90% involute by 7 yrs. Source: Dähnert 5th p. 97

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Which of the following is the most appropriate mA (milliamperes) setting for a pediatric chest CT?a. 20b. 80c. 160d. 300e. 520

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B. 80 (* probably)CT scans comprise only about 10% of diagnostic radiological procedures in large U.S. hospitals, but contribute approximately 65% of the effective radiation dose from all medical x-ray examinations! (think about any night on-call) [www.cancer.gov]There are a number of studies on pediatric CT, some with a reduction of mAs to as low as 40, depending on the weight of the child. The ACR site gives no specific values, just generalities. Therefore, 80 seems to be the best choice, although 160 could be the “correct” answer.

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Kid with SVC syndrome and mediastinal mass.a. Lymphomab. Histoplasmosisc. Thymomad. Castleman’s disease

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A. Lymphoma (* probably)“The etiology [of SVC syndrome] is malignancy in 95% of cases” – [Fraser & Pare 2nd p. 925.] “Malignant Lesion, #1 Bronchogenic CA, #2 Lymphoma” – [Dähnert 5th p. 648.]Given the above references, and the fact that Lymphoma is one of the most common neoplasms of childhood (after leukemia and CNS tumors), lymphoma appears to be the best answer.* This question has come up on multiple prior exams, with different phrasings, e.g., “mediastinal lymphadenopathy” or “calcified mass” or “in an adult”. If it appears again, read carefully!

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NOT associated with asymmetric IUGRa.  Increased HC/AC ratiob.  Decreased flow in umbilical arteryc.  Increased flow in fetal carotid arteryd.  Amniotic Fluid Index > 20

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D. Amniotic Fluid Index > 20“Asymmetric” IUGR is basically a form of malnutrition, and represents 90% of detected IUGR. It is sometimes (though not always) accompanied by oligohydramnios, not polyhydramnios. [US Req p. 182.]Doppler evaluation of the umbilical artery utilizes the systolic to diastolic ration (S/D). A ratio higher than three is considered abnormal, the idea being that there is increased resistance and less diastolic flow. Increased doppler flow velocities in the fetal cartotid arteries, in particular, increased diastolic flow, is felt to reflect “brain-sparing” maneuvers in the setting of hypoxia . This also results in an increased biparietal/abdominal circumference ratio. Incidentally, it is head circumference that is used in these proportionality measurements, not biparietal diameter, which is affected by synostosis disorders such as dolichocephaly. Callen.

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Which organism is most common in membranous croup?a. klebsiellab. strep. pneumoniaec. staph. aureusd. pseudomonas

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C. staph. Aureus [Primer 3rd. p. 803]AKA- exudative tracheitis, bacterial tracheitis, membranous laryngeotracheobronchitis. Uncommon, but potentially life threatening cause of upper airway obstruction in children too old to have standard croup. Purulent infection of trachea with exudative plaques appears as soft tissue defect(membrane) seen in airway on plain radiographs and symmetric or asymmetric subglottic narrowing in child too old to have standard croup. (Donnelly 7)

2001 : 

2001 An afebrile 2 month old child presents with stridor. AP and lateral radiographs demonstrate eccentric narrowing of the trachea. Which of the following is most likely?a. Thyroglossal duct cystb. Papillomac. Subglottic hemangiomad. Rhabdomyosarcoma

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C. Subglottic hemangiomaSubglottic hemangiomas (= Infantile Laryngeal Hemangiomas) are the most common subglottic soft-tissue mass causing upper respiratory tract obstruction and croup-like symptoms in neonates (age < 6 months):- Patients typically have hemangiomas elsewhere- skin, mucosal membranes in 50%, - radiographic findings of eccentric thickening of subglottic portion of trachea (AP view). - these arise from the posterior wall below true cords (on lateral view). Laryngeal papillomatosis is the most common benign tumor of the larynx, and might present with similar symptoms (inspiratory stridor, cough, respiratory distress). But the age of onset is older (1-54 years). Dähnert 5th p. 382.Epiglottis is a supraglottic mass. Laryngomalacia (immaturity of cartilage) is the most common cause of stridor in neonate + young infant, and is the only cause of stridor to get worse at rest. This involves the supraglottic portion of the larynx. Angiofibromas are seen in an older population (teenagers). A thyroglossal duct cyst would more likely present as a neck mass. Source: Dähnert 5th ed. p.381-382

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The best way to differentiate hyaline membrane disease from meconium aspiration is by:a. Lung volumeb. Pleural effusionc. Interstitial patternd. Heart size

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A. Lung volumeMeconium aspiration syndrome is most common cause of neonatal respiratory distress in full term / postmature infants (RDS most common cause in premature infants); produces hyperinflation with areas of emphysema. Diffuse, bilateral opacities are seen. Rapid clearing is noted within 48 hours. Small pleural effusions are sometimes present (20%). Hyaline membrane disease, also known as respiratory distress syndrome of the newborn, is an acute disease with hypoinflation (stiff lungs), immature surfactant production, atelectasis, reticulogranular pattern, and prominent air bronchograms. Source: Dähnert 5th ed. p.504,520; Primer 2nd ed p.753

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A child presents with a chest wall mass. Which of the following is most likely?a. Leukemiab. Lung carcinomac. Neuroblastomad. Ewing sarcoma

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D. Ewing sarcoma Ewing sarcoma of the rib is the most common chest wall tumor in children.- rib involvement is 30% of children < 10 years- predominantly pelvis and lower extremity of an older child (rib 7%)In general, malignant tumors of chest wall in children are more common than benign primary chest wall tumors. Of the remaining choices, rhabdomyosarcoma > metastases (e.g., neuroblastoma, leukemia) > Askin tumor > chondro-/osteosarcoma (rare in children). Source: Dähnert 5th ed. p. 442; Resnick 573-574

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The most common cause of round pneumonia in children is:a. Staph aureusb. Streptococcusc. Psudomonasd. Klebsiella

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B. Streptococcus Streptococcus and Staphlococcus both cause round pneumonia. Streptococcus is more common from age 1-3. S. aureus is more common in infancy. Round pneumonia appears round because of poorly developed collateral pathways (pores of Kohn and channels of Lambert). With time the initially round pneumonia develops into a more typical consolidation. Source: Primer 2nd ed. p.752; Dähnert 5th p. 522.

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Microcolon is NOT associated with which of the following?a. Meconium ileusb. Meconium plugc. Meconium peritonitisd. Hirshprung’s disease

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B. Meconium plug Functional colonic obstruction in the full-term neonate is another name for meconium plug syndrome. A plug of thick meconium lodges in the distal colon, probably caused by poor peristalsis as a result of neuronal underdevelopment. Radiographs may reveal low obstruction with bowel distension. Contrast enemas usually reveal a normal-caliber left colon. Ped. Req. 2nd p. 114.Meconium ileus occurs when meconium obstructs the distal ileum (microcolon always present, 99% associated with CF). Meconium peritonitis occurs when there is antenatal bowel perforation.Differential for microcolon is MI MCA: Meconium ileus/ peritonitis (cystic fibrosis)Ileal/ jejunal atresia Megacystic-microcolon-hypoperistalsis syndrome Colonic atresia Aganglionosis (Hirschsprung dz). Source: Dähnert 5th ed. p.773, 847

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Testicular U/S – 12 y/o boy with symptoms of delayed testicular torsion; which of the following is least likely to be seen?a. Increased extratesticular flowb. Decreased intratesticular flowc. Testicular microlithiasisd. Heterogeneous testicular echogenicitye. Hydrocele

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C. Testicular microlithiasis Testicular microlithiasis is NOT associated with testicular torsion; it does have associations with cryptorchidism and malignancy.Torsion may demonstrate hypoechogenicity in early state. In subacute and chronic phase, testes may be hypoechoic or heterogeneous in echogenicity. There is increased peritesticular flow without parenchymal blood flow. Hydrocele may be seen occasionally in testicular torsion.Chronic testicular torsion is described as follows--- small atrophied homogeneously hypoechoic testis- enlarged echogenic epididymis Source: Dähnert 5th ed. p.966,967

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Regarding MRI in a child, where do you first see red to yellow marrow transformation?a. Distal meta-diaphyseal junctionb. Proximal meta-diaphyseal junctionc. Epiphysisd. Diaphysis

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C. Epiphysis At birth, nearly the entire osseous skeleton is composed of red marrow. When epiphyses and apophyses ossify, they have red marrow within them only transiently, for a matter of a few weeks, before conversion to yellow marrow occurs.Normal conversion of red to yellow marrow occurs in a very predictable and progressive manner and is completed by an individual’s middle 20’s. - Extremities (feet and hands) first, to humeri/femora, to axial skeleton- Individual long bones: epiphyses/apophyses ? diaphysis ? distal metaphysis ? proximal metaphysis. Multiple sources: Kaplan p. 24; Resnick 2nd p. 9; MSK Req. 2nd p. 434.

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Pediatric rings and slings: after double aortic arch, which is the second most common symptomatic vascular ring?a. Rigth arch with isolated left subclavian artery b. Left aortic arch with aberrant right subclavian arteryc. Right aortic arch with mirror image branchingd. Right aortic arch with aberrant left subclavian artery

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D. Right aortic arch with aberrant left subclavian artery The most common type of right aortic arch has an aberrant retroesophageal left subclavian artery. If a left ductus connects this left subclavian artery to the left pulmonary artery, a vascular ring is formed. 5% have symptoms (airway or esophageal compression).A double aortic arch is both the most common and most symptomatic of all vascular rings.- Right arch with mirror image branching: the ductus arteriosus goes from the left subclavian artery to the left pulmonary artery in front of the trachea and does not cause a vascular ring (usually asymptomatic).- Left aortic arch / aberrant right subclavian: most common congenital arch anomaly (1% of persons). Usually asymptomatic because no ring is formed (it’s a sling). Sources: Card. Req. p. 419-421; Primer 3rd. p. 134; Dähnert 5th p. 580.

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Pediatric skull: which would you NOT see wormian bones in?a. Pyknodysostosisb. Ricketsc. Chiari II malformationd. Downs syndrome

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C. Chiari II malformation Chiari II: herniation of the cerebellar tonsils, vermis, 4th ventricle, and brain stem through the foramen magnum; characteristic skull finding is Lückenschädel (not Wormian bones).Wormian bones are intrasutural ossicles in the lambdoid, posterior sagittal, temporosquamosal sutures. They are normal up to 6 months of age.Mnemonic: PORK CHOPS I:Pyknodysostosiss, Osteogenesis Imperfecta, Rickets in healing phase, Kinky hair syndrome, Cleidocranial dysostosis, Hypothyroidism / Hypophosphatasia, Otopalatodigital syndrome, Primary acroosteolysis (Hajdu-Cheney), Pachydermoperiostosis / Progeria, Syndrome of Down, Idiopathic Source: Dähnert 5th ed. p.174

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Pediatric c-spines: which type of cervicocranial fractures is stable?a. Type I odontoidb. Type II odontoidc. Type III odontoidd. Low dens fracturee. Posterior Arch of C1

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E. Posterior arch of C1 Fracture of the C1 arch may occur as an isolated fracture. It is not associated with neurologic deficit or atlantoaxial instability. Harris, Rad. Acute Cerv. Spine Trauma 3rd p. 372.Type I odontoid fracture is a fracture of the upper part of the odontoid (avulsion by alar check ligament). It is a potentially unstable and rare fracture. Source: Primer 3rd ed. p.373[Side note—Harris has finally stated that this fracture really exists; see Rad. Emerg. Med. 4th p. 239-240.]

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In a four day old with pulmonary vascular congestion and a normal heart, which of the following is likely?a. TAPVR type III (infradiaphragmatic)b. Truncus arteriosusc. Transposition of great arteriesd. TAPVR type I (supracardiac)

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A. TAPVR type III (infradiaphragmatic)TAPVR type III represents 15% of the TAPVRs. Majoritiy of the infradiaphragmatic connections are obstructed and show normal heart size with pulmonary edema.Symptomatology depends on presence or absence of obstruction.Source: Primer 2nd ed. p.124; AFIP notes

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In a child, where does the conus end?a. T12b. L2c. L3d. L4

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B. L2 The normal level of the conus medullaris in a child is L2. (Pediatric Requisites, 2nd ed., 293) If the conus is below the L2/L3 disc interspace it should be considered abnormal. Barkovich 3rd p. 647. Dähnert 5th ed. p.196In an adult, the chord ends above the L1/L2 disc level. Kaplan p. 326.

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Two brothers with pneumonia, liver abscesses and other infections. Which of the following do they have?a. DiGeorge syndromeb. Chronic granulomatous diseasec. Agammaglobulinemiad. cystic fibrosis

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B. Chronic granulomatous disease Chronic granulomatous disease of childhood is a recessive X-linked (60%) / autosomal (40%) immunodeficiency disorder resulting in purulent infections and granuloma formation primarily involving lymph nodes, skin and lungs. Onset of symptoms is at childhood with recurrent chronic infections. Hepatic abscess is the most common abdominal process associated with this disease. Caused by abnormal leukocyte function.DiGeorge syndrome consists of congenital absence of thymus and parathyroid glands. It is associated with conotruncal malformation and interrupted aortic arch. DiGeorge syndrome is associated with an immunodeficiency of T-cell response, but would more typically present with PCP pneumonia or dessiminated viral infection. Normally, however, the other anomalies are so severe that patients are diagnosed before presenting with an infection.Agammaglobulinemia is rare disorder that only affects males as it is inherited as an X-linked recessive trait (like hemophilia). - abnormal B-lymphocyte differentiation; decreased humoral immunity- bacterial infections (Haemophilus, pneumococcus, staph.)- URI, Lung, skin infections--hepatic abcess rare Source: Dähnert 5th ed. p.695,568; www.nlm.nih.gov/medlineplus/ency/article/001307.htm

2000 : 

2000 A 2 year old with dehydration and AMS demonstrates bilateral thalamic hemorrhage and associated mass effect. What is the most likely cause?a. Traumab. Non-accidental traumac. Deep cerebral venous thrombosisd. Basilar artery thrombosise. hypertensive hemorrhagef. glioma

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C. Deep cerebral venous thrombosisIn the history 2 year old child with dehydration and AMS can promote venous thrombosis. LESS likely causes of bilateral thalamic hemorrhage would be a basilar artery thrombosis, especially in a child. PNET’s are common in children but without this presentation. Consider child abuse in children found to have suffered from acute/prolonged asphyxia. The neuro deficits develop days following the insult. The asphyxia could result from infection or abuse (induced). The vascular supply to the thalamus is by the PCA via thalamic perforators. (Helms Fundamentals of Radiology pgs102-3;Mov Disord. 1994 Sep 9(5) 493-507;Child Nerv Syst 1993 Jul9(4)243-5;Neuroradiology 1991;33(4)296-9;Arch Fr Pediatr 1989Jan46(a):39-40)

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Which is the most common hereditary hypercoagulable condition?a. Sickle Cellb. Protein S Deficiencyc. Anticardiolipin antibodyd. Disfibrinogeniae. Factor V Leiden

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E. Factor V LeidenThe reference for question no. 49 is a review article on inherited basis of thromosis (NEJM April 19, 2001). The prevalence of this mutation in subjects with idiopathic DVT is ~ 40% Initially, searches for inherited thrombophilias among patients with idiopathic venous thrombosis were disappointing, since only 5 to 20 percent of such patients had inherited thrombophilias. The situation changed remarkably in 1993, after the discovery of resistance to activated protein C. This condition is the most common cause of inherited thrombophilia.In most cases it results from the substitution of adenine for guanine at nucleotide 1691 of the Factor V gene (G1691A), which causes the arginine in residue 506 of the protein to be replaced by glutamine (Arg506Gln). The resulting protein is called factor V Leiden.

Slide 76: 

Which is the most specific finding in malrotaion?a. proximal jejunum to the right of the spineb. inferior medial displacement of the duodenal-jejunal junctionc. sigmoid volvulusd. high and medial location of the cecume. jejunum located on the rightf. the cecum in the RUQ

Slide 77: 

B. inferior medial displacement of the duodenal-jejunal junctionThe most reliable indicator is the normal position of the ligament of Trietz (duodenal-jejunal junction) NOT the cecum, NORMAL position being LEFT of the spine at the level of the duodenal bulb. (Pedi Requisites pgs 106-7; Primer pgs 762-3) A normal position of the cecum does NOT exclude malrotation; a normal barium enema will rule out 97% of malrotation

Slide 78: 

6 year old with a pustule on the hand, then develops swollen, painful epi-trochlear and axillary nodes as well as forearm swelling. What is the most likely diagnosis?a. Lyme diseaseb. Cat-scratch diseasec. Mycosis fungoidesd. Lymphomae. RMSFf. Kawasaki dz.g. Eosinophilic fascitis

Slide 79: 

B. Cat-scratch disease60% of cases of cat scratch disease occurs in kids. 3-5 days after a cat scratch, clinical symptoms include a localized papule progressing to a pustule that crusts over. Tender regional adenopathy develops within 1-2 weeks. Scratches are most often sustained over the hands or face producing epitrochlear, axillary, pectoral and cervical adenopathy. Lyme disease is a tick-transmitted spirochetal disease usually presenting as a characteristic skin lesion (erythema migrans). After several days or weeks, the spirochete may spread hematogenously with annular skin lesions, meningitis, neuritis, carditis or migratory MSK pain. Generalized adenopathy is a less common presentation. Rocky Mountain Spotted Fever is caused by Rickettsia ricketsii. The classic clinical triad includes fever, rash and history of tick exposure. Macules first appear on the wrist and ankles and progress to involve the trunk. More severe sequelae include CNS involvement, renal failure, hepatic injury and bleeding. I found no mention of adenopathy.Kawasaki’s disease is an acute febrile multisystem disease of children. It is characterized by nonsuppurative cervical adenitis and changes in the skin and mucous membrane (congested conjunctivae, erythema of the oral cavity, lips and palms), and desquamation of the palms and soles.Mycosis Fungoides (AKA cutaneous T cell lymphoma). The median age of onset is the mid 50s and is more common in males an in blacks. This is an indolent lymphoma and presents suffer with eczematous skin lesions for years before the diagnosis is finally made. In advanced stages, the lymphoma can metastasize to lymph nodes and visceral organs. Eosinophilic fasciitis is a scleroderma-like syndrome of unknown etiology caused by inflammation and then sclerosis of the dermis, subcutis and deep fascia. It is an adult disease occurring after strenuous exercise. Pts have abrupt onset of symmetric tenderness and swelling of the extremities followed by induration of the skin and subcutaneous tissue. Harrison’s Principles of Internal Medicine, 2001.

Slide 80: 

Which of the following place a pediatric pt at high risk for latex hypersensitivity?a. tetrology of Fallotb. Bladder exstrophyc. Unilateral renal agensisd. Allergy to PCN

Slide 81: 

B. Bladder exstrophyA serologic survey of patients with spina bifida revealed that 37% (35/93) had latex-specific IgE by RAST. Of the 35, 8 had histories suggesting clinical latex hypersensitivity, whereas only 1 of the 58 RAST-negative patients reported a similar clinical history.[14] Since exposure appears to increase the probability of latex hypersensitivity, children with other conditions requiring frequent surgery may also be at risk. These conditions include bladder exstrophy, cerebral palsy, myelomeningocele, and spinal cord injury.[15-23] http://www.medscape.com/viewarticle/408718_2

Slide 82: 

A 6 week old afebrile infant present with hyperinflated lung and peribronchial cuffing and peri-hillar infiltrates, what is the most likely cause?a. Chlamydiab. parainfluenzac. RSVd. Ecolie. Kleisbellaf. Strepg. Staphh. Mycoplasma

Slide 83: 

B. Parainfluenza Viral pneumonia (parainfluenza) tends to Present with hyperinflation, atelectasis and perihilar peribronchial cuffingChlamydia pneumonia is caused by a Bacteriumlike intracellular parasite, chlamydia trachomatis which is aquired from the mother by direct contact during vaginal delivery. The average age of onset is 6 weeks.cough,rhinitis, and conjunctivitis(30%) are presenting symptoms. The findings on chest radiographs are far worst than would be predicted on physical exam. The radiographic findings can vary from perihilar / interstitial process to a focal alveolar infiltrate with generalized hyperinflation. Effusions may occur. Consolidation is rare.Bacterial pneumonia tends in the neonate Tends to be caused by streptococcus pneumonia, b and d; enteric bacilli like e.coli---bilateral alveolar infiltrates are seen, and sometimes pleural effusionsMycoplasma causes bronchopneumonia. Ref: emergency pediatric radiology, imaging of newborn chest pg 202-203, ped req p. 36-38.

Slide 84: 

Most commonly seen in fetus of DM mother:A. sacral teratomaB. myelomenigoceleC. lateral meningoceleD. syrngohydromyeliaE. encephaloceleF. filum lipomaG. caudal regression syndrome

Slide 85: 

G. caudal regression syndromeCaudal regression syndrome = sacral agenesis. - Infants of diabetic mothers are predisposed (16-22% of children with sacral agenesis have diabetic mothers). Dähnert 5th ed p. 197Also seen with greater incidence in infants of diabetic mothers:meconium plug syndrome, subvalvular aortic stenosis (hypertrophic obstructive cardiomyopathy), hyaline membrane disease, adrenal hemorrhage, MCDK, renal vein thrombosis Dähnert CD-ROM

Slide 86: 

Membranous croup is caused by:a. Staph. Aureusb. H. fluc. parainfluenzad. strepe. Ecolif. pseudomonasg. kleisbella

Slide 87: 

A. StaphylococcusMembranous croup, AKA exudative tracheitis is caused by a bacterial infection, most commonly Staphylococcus. Donnelly p. 7 see also Primer 3rd. p. 803Noncontagious croup is caused by diphtheria.Viral croup, AKA croup or laryngeotracheobronchitis, is caused by a virus, commonly parainfluenza. Donnelly p. 5Membranous croup is a uncommon bacterial inflammation of the larynx, trachea, and bronchi. Staphylococcus aureus is the most common organism. Generally, patients are older, are sicker than children with viral croup. The cxr showss irregularity fo the tracheal walls due to membranes in the subglottic airway. (Kirks 3e p. 654)

Slide 88: 

Contraindications to intussusception reduction in kids:a. symptoms for 48 hrsb. peritonitisc. heme positive stoold. age < 3moe. age>3yrf. symptoms >24hg. symptoms >12hh. there is an intestinal obstructioni. age <2mo

Slide 89: 

B. peritonitisAbsolute contraindications to BE for intussusception include perforation or peritonitis (Peds Requisites 69).Contraindications for attempting pressure Reduction of an intussuseption include peritonitis on physical exam or pneumoperitoneum on radiography. Ref: fundamental of pediatric radiology pg 116

Slide 90: 

Left arch, dextrocardia, stomach left, liver midline:a. Polyspleniab. Kartagener’s syndromec. Situs Inversus viscerumd. Ebstein’s anomaly

Slide 91: 

A. Polysplenia.“…always consider polysplenia / asplenia when the cardiac apex and situs are discordant”. Primer 3rd p. 137.Polysplenia = bilateral left sidedness- Heart-ASD, VSD, AVC, single ventricle, azygous cont. IVC, dextrocardia, Lung-bilobed, hyparterial bronchi, L pulm. arteries- Abdomen-heterotaxy, absent GB, biliary atresia, malpos.liver and stomach. renal anomalies Ped Req, Dähnert p. 631Kartagener’s-triad of situs inversus, sinusitis, bronchiectasis. (Kirks 3e p. 767) Situs inversus-liver on the left, stomach on the right.Ebstein anomaly: downward displacement of septal and posterior leaflets of dysplastic tricuspid valve with ventricular division into (1) superior atrialized portion and (2) small inferior functional chamber with shortened chordae tendineae. Dähnert 5th p. 627.

Slide 92: 

Davidoff-Dyke-Mason Syndrome is characterized by all of the following except:a. ipsilateral calvarial thickeningb. mastoid air cell hyperpneumatizationc. ipsilateral paranasal sinus hypoplasiad. ipsilateral enlarged lateral ventricle

Slide 93: 

C. mastoid air cell hyperpneumatizationInsult to immature brain resulting in neuronal loss impaired brain growth. Unilateral cerebral atrophy with ipsilateral small skull, seizures, hemiparesis, MR, unilateral skull thickening, unilateral decrease in sz. of cranial fossa, unilateral overdevelopment of sinuses, contraction of a hemisphere/lobe, compensatory enlargement of adjacent ventricle + sulci with midline shift. Dähnert p. 276

Slide 94: 

Atlanto-axial subluxation is seen in all of the following except:a. Down syndromeb. hypothyroidismc. traumad. juvenile RA

Slide 95: 

B. hypothyroidism. Atlantoaxial subluxation represents displacement of the atlas with respect to the axis. Anterior atlantoaxial subluxation is more common than posterior. Measured as the distance between the dens and the anterior arch of C1. (Predental space: >2.5mm and >4.5 in children is abnormal). Causes—pnemonic: “JAP LARD”Juvenile Rheumatoid arthritis, Ankylosing spondylitis, Psoriatic arthritis, Lupus, Accident (trauma), Retropharyngeal abscess / Rheumatoid arthritis, Down’s syndrome. Dähnert 5th p. 180, Primer, 3rd, 471

Slide 96: 

The most common pattern of esophageal atresia is:a. EA with proximal TEFb. EA without TEFc. EA with proximal and distal TEFd. EA with distal TEF

Slide 97: 

D. EA with distal TEFThe most common type is esophageal atresia, with a fistula between the distal esophageal segment and the trachea (85%); the next common is esophageal atresia with no fistula (10%). The other types are an “H” fistula (1%), EA with proximal and distal TEF (1%) and ea with proximal tef (1%). Dähnert p. 807

Slide 98: 

The most common cause of a unilateral cystic kidney in a child is:a. MCDKb. ARPCKDc. ADPCKDd. Wilm's tumor

Slide 99: 

A. MCDK Multicystic dysplatic kidney usually presents unilaterally because bilateral MDK is incompatible with life. The autosomal kidney diseases and medullary cystic kidney disease are bilateral. Genitourinary Requisites 66-69, 108-109, 130-131The causes of renal cysts include:(1) autosomal recessive polycystic kidney disease (ARPCKD)(2) autosomal dominant polycystic kidney disease (ADPCKD)(3) dysplasia related to obstruction (upj, posterior urethral valves, multicystic dysplastic kidney)(4) syndromes: tuberous sclerosis, trisomy 13, 18, 21, Ehlers-Danlos, Beckwith-Weidemann, von Hippel Lindau* Both ARPCKD and ADPCKD effect both kidneys.Bilateral kidneys. Muticystic dysplastic kidney tends to effect one kidney (10-15% of cases have bilateral involvement.) Kirk’s textbook of pediatric radiology gu chapter

Slide 100: 

Oligohydramios and renal cystic disease, best diagnosis ?a. ADPKDb. ARPKDc. Bilateral multicystic dysplasiad. Medullary cystic renal disease

Slide 101: 

C. bilateral MCDKRenal Cystic Disease is commonly detected in utero. Mulicystic dysplastic kidney appears as multiple cysts of varying size. Because affected kidneys do not function, bilateral multicystic dysplastic kidney is associated with oligohydramnios and is not compatible with life. The perinatal ultrasound shows round anechoic regions with little intervening tissue an is diagnostic. The fetus with bilateral multicystic dysplastic kidney ususally dies in the neonatal period because pulmonary hypoloplasia caused by oligohydramnios. (ref Kirks 3e, pp 1050-1051)Massive enlargement of both kidneys associated with oligohydramnios suggests autosomal recessive polycystic disease. The kidneys are predominantly echogenic with a sonolucent rim. Discrete cysts are not evident.Autosomal dominant polycystic kidney disease is occasionally detected in utero. The kidneys are enlarged but lack the sonolucent rim of infantile polycystic kidney disease. Occasional discrete cysts are visualized.Obstructive uropathy such as posterior urethral valves may result in cystic renal dysplasia. Affected kidneys are hydronephrotic, with increased parenchymal echogenicity and cysts of varying size. The kidneys may be dysplastic without cysts being visualized by ultrasound.. Brant & Helms, 2nd Ed, pp902-3.

Slide 102: 

The most common location of bilateral collecting system obstruction in a male infant is:a. UPJb. UVJc. prostatic urethrad. bulbous urethra

Slide 103: 

A. UPJUPJ obstruction is the most common congenital obstruction of the urinary tract. It is often bilateral, but asymmetric in severity. Kirks: textbook of pediatric radiology

Slide 104: 

Work up of UTI in a eight year old female would begin with:a. VCUGb. MRI of the pelvisc. cystogramd. ultrasound

Slide 105: 

D. Ultrasound In children over 3-4 yrs of age who have only signs of lower UTI, VCUG is not recommended if renal U/S is normal. However, if renal U/S is abnormal, then VCUG or radionuclide cytography is recommended. Kirks textbook of pediatric radiology

Slide 106: 

A 2 year old girl develops flank pain, burning micturation, fever and pyuria. What is the next most appropriate study?a. Ultrasound of the kidneys and bladder b. Contrast-enhanced CTc. IVPd. VCUGe. excretory urogram

Slide 107: 

D. VCUG In a child presenting with symptoms of UTI, the main question is “Is there an underlying anatomic abnormality (VUR, UPJ or UVJ obstruction, ureterocele or posterior urethral valve)?” Under age 5, the child should undergo a VCUG. If the VCUG is normal, the next appropriate study is an Ultrasound. If the VCUG is abnormal, an excretory urogram or radionuclide renogram are appropriate. According to Blickman, further workup could be an ultrasound or IVP depending on whether the VCUG is normal. [Hans Blickman. The Requisites: Pediatric Radiology, 1998]

Slide 108: 

A cystic area is seen peripheral to the thalamus in a sonogram of a neonate. The likely cause is:a. infectionb. post hemorrhagec. anoxiad.

Slide 109: 

B. post hemorrhageSubependymal hemorrhage, germinal matrix hemmorhage, and grade I intraventricular hemorrhage, are all terms that refer to bleeding confined to the germinal matrix areas without intraventricular extension.acutely. These bleeds may be imaged as echogenic areas at the caudothalamic groove. Subependymal hemorrhages may resolve entirely over a matter of days.many lesions will liquefy over time and can be imaged as a cystic lesion or lesions in the area of hemorrhage near the thalamus. Textbook of neonatal ultrasound pg 55., Dähnert 5th ed. P. 305-306

Slide 110: 

Child with SVC syndrome, mediastinal mass, and adenopathy:a. TBb. Sarcoidc. Seminomad. Teratomae. Thymomaf. lymphoma

Slide 111: 

F. lymphomaAccording to Kirks, "mediastinal lymphoma, when large and bulky, produces the SVC syndrome and extrinsic airway compression." (Kirks 3e p 784)“The etiology [of SVC syndrome] is malignancy in 95% of cases” – [Fraser & Pare 2nd p. 925.] “Malignant Lesion, #1 Bronchogenic CA, #2 Lymphoma” – [Dähnert 5th p. 648.]Thymoma: 70% 5th/6th decades and no lymphadenopathy on CT. Rosado de Christianson, ML DDX of Mediastinal Masses, 2001 AFIP NotesSeminoma: Caucasian males, third to fourth decade. IbidTeratoma: Children and young adults, Often assymptomatic (symptoms of compression or rupture), notes make no mention of adenopathy. IbidTB: Lymphadenopathy: 95% for children, 43% young adult, 10% elderly. Rosado de Christianson, ML Tuberculosis, 2001 AFIP NotesSarcoid: Young and middle age adults (peak 20-29), bilateral hilar LAD. Galvin JR, An Approach to Diffuse Lung Disease, 2001 AFIP notes.[See Dähnert 5th ed p. 648 for DDx of SVC syndrome. This question has been recalled many different ways...]

Slide 112: 

The most common cause of a pediatric chest mass is:a. lymphomab. thymic hyperplasiac. neuroblastomad.

Slide 113: 

B. Thymic hyperplasia.Thymic hyperplasia is the most common anterior mediastinal mass in pediatric age group through puberty.- Etiology: hyperthyroidism (most common); rebound hyperplasia, myasthenia gravis, acromegaly, Addison’s disease. Dähnert 5th p. 529.

Slide 114: 

Endocardial cushion defect (ECD) is associated with all the following cardiac abnormalities except:a. cleft mitral valve b. gooseneck deformity c. high VSD d. osteum secundum ASD e. notched MVf. Down’s syndrome

Slide 115: 

D. osteum secundum ASD Atrioventricular septal defects (AVSDS) are a complex group of cardiac anomalies that share a deficiency or absence of the AV septum, resulting in an interatrial communication (ostium primum ASD) above the AV valves, a deficiency in the inlet portion of the ventricular septum immediately beneath the AV valves that results in a common AV orifice. Synonymous terms for the condition include AV canal defect, AV defects, AV communis, ostium primum defect, and endocardial cushion defect. Almost always, an interatrial communication exists through the so-called ostium primum defect, which results from the deficiency of the AV septum. In the complete form of AVSD, a moderate to large interventricular communication is present, because the bridging leaflets are floating free, which creates a connection between the ventricles. The communication is also larger in this variety because of the greater deficiency of the septum, when compared with that found with the partial form. The septal defect usually includes the adjacent membranous septum as well as the muscular portion of the inlet septum. (The membranous septum is the high portion of the ventricular septum.) Taveras and Ferrucci Vol 2 CH 80, Acyanotic Congenital cardiac malformations. Taveras & Ferrucci, CD-2000, Atrial Septal Defect—Echocardiography, Vol 2, Chap 84

Slide 116: 

Concerning a thyroglossal duct cyst:a. They are usually suprahyoid. b. Usually superficial to strap muscles c. Most common tumor complication is papillary thyroid carcinomad.

Slide 117: 

C. Most common tumor complication is papillary thyroid carcinoma65% infrahyoid, usually deep to the strap muscles, lined by squamous epithelium and 1% degenerate to CA- the cyst is lined bysquamous epithelium and rarely (1%), can degenerate to "carcinoma" (Dähnert 3e, p295 and AFIP notes Dr. Koeller). Dähnert 5th ed p.394

Slide 118: 

A 3 year-old patient presents with fever, and erythema, and the chest x-ray demonstrates cardiac enlargement. The most likely diagnosis is:a. Henoch-Schönlein purpura b. SLE c. Kawasaki's disease d.

Slide 119: 

C. Kawasaki’s diseaseKawasaki’s disease-acute phase starts with sudden onset high fever, rash, cervical adenitis, erythema of hand and feet. Cardiac enlargement can be seen if myocarditis is severe. (Kirks 3e, p. 579)Henoch-Schönlein purpura - false; purpuric rash in lower extremities, abdominal pain, arthritis.SLE - false; don't usu see cardiac enlargement. Most freq early sx include: fever, arthritis & rash. (Nelson 15e, p. 673)

Slide 120: 

With respect to asymmetric IUGR the following are true except:a. Increased diastolic flow in the carotidsb. Increased HC/AC ratioc. Amniotic fluid index > 20d.

Slide 121: 

C. Amniotic fluid index > 20 In asymmetric IUGR the HC/AC increases because the AC does not grow as fast as the HC to preserve brain development. IUGR is usually associated with oligohydramnios and not polyhydramnios. Doppler studies have shown that the carotid and MCA diastolic flow increases, resulting in a decrease in the S/D ratio. US Requistes, p. 180-189

Slide 122: 

Most likely renal tumor in a 3 month old infant:a. renal cell carcinomab. mesoblastic nephromac. multilocular cystic nephromad.

Slide 123: 

C. mesoblastic nephromaRCC rarely is present during the first two decades of life.MLCN "uncommon nonheriditary cystic neoplasm” is seen in young boys and older women.Mesoblastic nephroma is the most common neonatal renal neoplasm and is almost always discovered during the first few months of life. It is a benign, solid intrarenal mass. Kirks, p. 1120-1121 and AFIP notes

1999 : 

1999 In a patient with a high anal atresia, recurrent UTI’s are due to rectourethral fistula reflux obstruction

Slide 125: 

A. rectourethral fistula High anal atresia occurs above the puborectalis sling. In boys, the rectum almost always terminates in the posterior urethra (less commonly the bladder) and in girls the rectum terminates in the vagina or vestibule. High atresias are associated with GU anomalies (50%). Low anal atresia occurs below the puborectalis sling and usually has a visible perineal orifice and no communications with the GU tract. Recurrent UTIs in a patient with a high anal atresia are thus due to the rectourethral fistula.Part of the specrum of IMPERFORATE ANUS: Prevalence:1:5,000 live birthsLOW ANOMALY (55%) bowel has passed through levator sling fistula to perineum / vulva Rx: readily reparableNTERMEDIATE DEFECT (least common) bowel ends within levator m. as a result of abnormality in posterior migration of rectum fistula opening low in vagina / vestibule Rx: 2- / 3-stage operationHIGH ANOMALY bowel ends above levator sling; M > F fistulous connection to perineum / vagina / posterior urethra (air in bladder in males; air in vagina in females) Cx:associated malformations more common + more severe Kirks, p. 876-878; Primer, p. 767; Dähnert Review Manual

Slide 126: 

What is the first bone to ossify? Femur Humerus Tibia Clavicle Sternum

Slide 127: 

D. clavicleWheeless' Textbook of Orthopaedics http://www.ortho-u.net/orthoo/51.htm

Slide 128: 

Which is the first secondary ossification center about the elbow to ossify? Medial Epicondyle Lateral Epicondyle Capitellum Olecranon Radial Head Trochlea

Slide 129: 

C. capitellumOccurrence of bone centers at elbow: Capitellum (1 yr), Radial head (4 yrs), Internal (medial) humeral epicondyle (7 yrs), Trochlea (10 years), Olecranon (10 yrs), External (lateral) humeral epicondyle (11 yrs). “CRITOE” or “Come Ride My Tree of Love” Dähnert, p. 25

Slide 130: 

What is the most echogenic features on neonatal US cingulate gyrus corpus callosum vermis massa intermedia caudate

Slide 131: 

C. vermisAccording to Rumack, the vermis is the most echogenic of the listed structures. Rumack, Diagnostic UltrasoundThe choroids plexus is probably the most echogneic structure but could not find a reference comparing vermis and choroids so hopefully only one or the other will be a choice

Slide 132: 

A neonate with Coarctation of the Aorta is shown to have pneumatosis on supine abdominal film. The next study to be asked for is CT abdomen US abdomen Cross table lateral UGI

Slide 133: 

C. cross table lateralI could not find the answer to this question in a text or the literature. This is a repeat question and the answers on other remembered questions also had trouble with this one and answers of both US of the abdomen and cross table lateral were given. I did a literature search and found a case report from France about NEC and coarctation, but it did not provide any information on the appropriate workup and listed NEC as a very rare complication. Surgery or pediatric textbooks did not shed any more light. As the pneumatosis was seen on a supine radiograph, a cross table lateral would be help to see if there is free air, thus indicating surgery. If abdominal US was done to evaluate the SMA and possible occlusion, then this study would also be helpful, probably after free air is excluded. Kirks, p. 763-764

Slide 134: 

The presence of linear calcification in a neonate means in utero perforation of the bowel (meconium peritonitis) True false

Slide 135: 

A. trueMeconium peritonitis = sterile chemical peritonitis secondary to perforation of bowel proximal to high-grade / complete obstruction shtat seals in utero due to inflammatory response – can see calcification with 24 hours – on OBUS see multiple linear / clumped foci of calcifications in 84% of cases Dähnert 5th p846

Slide 136: 

Which of the following result from pelvicoinfundibular stenosis multicystic dysplastic kidney megacalycosis megaloureter

Slide 137: 

A. multicystic dysplastic kidneyMulticystic dysplastic kidney (MCDK) results from ureteropelvic atresia or stenosis during embryogenesis. Most commonly this is due to pelvicoinfundibular atresia. In 5% of cases it is due to pelvicoinfundibular stenosis. US imaging shows multiple round, anechoic structures with no definable central pelvis and no or little normal appearing renal tissue. Contralateral UPJ obstruction is seen about 40 % of the time. Kirks, p. 1049-1051

Slide 138: 

What is the best test for nephroblastomatosisIVPCT with contrastMIBG studyUS

Slide 139: 

B. CT with contrastNephroblastomatosis refers to persistent rests of residual nephrogenic blastema. Although it is not itself malignant, these patients have a high incidence of Wilm’s tumor and are usually monitored as such. Nephroblastomatosis may be diffuse, or more commonly, multinodular. The are also generally peripheral or subcapsular. They are best seen on contrast enhanced CT, where they are seen as peripheral, relatively non-enhancing parenchymal lesions. They are also often seen as hypoechoic on ultrasound, but can also variably appear isoechoic or hyperechoic. I saw no reference to nuclear scan appearances. On MR, they are generally hypointense on T1 and T2. Dunnick 137Nephroblastomatosis refers to single or multiple rests of renal blastema. It can be a precursor to Wilms tumor. Pts with Beckwith-Wiedemann, hemihypertrophy, or sporadic aniridia and nephroblastomatosis are screened to look for transformation to Wilms. CT with contrast is the best way to evaluate this condition. To screen for transformation, US is used to decrease radiation exposure. MIBG is used to evaluate tumors of neuroendocrine origin and not nephroblastomatosis. IVP would not be helpful. Kirks, p. 1118-1120 and AFIP notes

Slide 140: 

Schizencephaly is what kind of a disorder? disorder of neuronal migration disorder of neural tube closure disorder of cellular division disorder of neuronal groove

Slide 141: 

A disorder of neuronal migrationSchizencephaly refers to a n abnormality of neuronal migration at the 5th to 7th week of gestation in which a cleft is seen from the ventricular ependyma to the pial surface, due to failure of the germinal matrix to form (Neuro Requisties p253).

Slide 142: 

What is the least likely to cause increased vascularity in a neonate TAPVR type III Tetralogy of Fallot TGA VOG aneurysm

Slide 143: 

B. tetralogy of FallotTetralogy of Fallot (TOF) is the most common cyanotic CHD of childhood. It consists of obstructed RV outflow tract, VSD, overriding aorta and RVH. On CXR there is evidence of decreased pulmonary vascularity. TAPVR and TGA both have increased vascularity and VOG aneurysm results in high output CHF and cardiomegaly. Primer, p. 109, 118-119, 471

Slide 144: 

What is associated with Lückenschädle prematurity myelomenigocele Chiari 1 Von Hipple Lindau Sygringomyelia lambdoid inversion Dandy walker hydrocephalus

Slide 145: 

B myelomenigoceleLückenschädle means “lacunar skull” and it is a mesenchymal dysplasia associated with myelomenigocele, myelocele, and encephalocele. It refers to a defect in ossification and is unrelated to increased ICP. It usually disappears by 6 months of age. Kirks, p. 91

Slide 146: 

Achondroplasia includes All Of The Following Except: Trident Fingers Narrowed Interpedicular Distance Shortening Is Most Significant In Hands And Feet Heterozygotes Display Features Autosomal Dominant Defect of enchondral bone

Slide 147: 

C. shortening is most significant in hands and feetA. T: “trident hand = separation of 2nd + 3rd digit and inability to approximate 3rd and 4th finger.” (Dähnert, p.32)B. T: “The spine typically has narrowing of the interpedicular distances in a caudal direction…” (Brant and Helms, p. 976)C. F: “predominantly rhizomelic shortness of long bones (femur, humerus) (Dähnert, p. 32)D. T: “The classic form is heterozygous, has no associated congenital defects, and is compatible with a normal life span.” (Chew, p. 257)E. T: BUT 80% of heterozygotes are from sporadic cases (Dähnert 5th p40)F. T“Achondroplasia is the result of a generalized defect in encondral bone formation, leading to underdevelopment of the portions of bones that grow by this mechanism.” (Chew, p 257)

Slide 148: 

The least likely to involve the RUL ccam congenital lobar emphysema sequestration TB Tracheal bronchus

Slide 149: 

C. sequestrationSequestration (both extralobar and intralobar) are much more commonly found in the lower lobes. Intralobar sequestration is inside the lungs and involves the lower lobes 98% of the time and is usually associated with recurrent infections and presents in adulthood. It has systemic arterial supply and venous drainage via the pulmonary vein. Extralobar sequestration is outside the lung and usually is located in the left basilar region and is seen in children. It has both systemic arterial and venous supply. Rarely it can occur outside of the chest or in the upper hemithorax. The other listed conditions all frequently involve the RUL. Primer, p. 747-748 Kirks, p. 673-679

Slide 150: 

Where does the trachea originate from Foregut 3rd and 4rth branchial arch 7 aortic branches thoracic somites

Slide 151: 

A. foregutThe trachea buds from the embryonic foregut and then bifurcates into about 24 generations of branches ending with the alveoli. Kirks, p. 659-660

Slide 152: 

What is the cause of a microcolon meconium ileus duodenal atresia infant of a diabetic mother Hirshprung’s

Slide 153: 

A. meconium ileusI imagine this question actually wanted to know the most common cause of microcolon as small left colon syndrome (seen in infants of diabetic mothers) and long segment Hirshprung’s can rarely cause microcolon. The most common cause of microcolon is meconium ileus. Distal ileal atresia can also cause a microcolon. Meconium ileus is the presenting symptom in 10 % of patients with CF. Plain films findings: “soap bubble” appearance representing air mixed with meconium, SBO and calcification due to meconium peritonitis. Kirks, p. 869-875 Primer, p. 763

Slide 154: 

What is the most associated with choroid plexus cysts trisomy 13 trisomy 18 trisomy 21 Alpert’s Turner’s syndrome

Slide 155: 

B. trisomy 18Choroid plexus cysts are fluid filled structures of varying sizes surrounded by normal tissue in the choroids plexus of the lateral cerebral ventricles. A higher frequency of CPC are abserved in fetuses with chromosomal abnormalities, particularly trisomy 18. The mean prevalence of CPC was abut 50% in Trisomy 18. Rumack, Chapter 40 pg 1182.Choroid Plexus Cysts are the most common intraventricular lesions seen in fetal life. 95% of CPC are seen in NORMAL fetuses. Up to 4% of NORMAL fetuses have a CPC, however there is an association between CPC and chromosomal abnormalities, especially Trisomy 18. Over HALF of Trisomy 18 fetuses have CPC. In absence of other abnormalities, <1% of fetuses with a CPC will have Trisomy 18, however if another anomaly is present, especially OVERLAPPING FINGERS (Hallmark of T18), 4% of fetuses with a CPC will have Trisomy 18. While CPC are also weakly associated with Down’s, in pts with chromosomal abnormalities and a CPC, Trisomy 18 outnumbers Trisomy 21 about 4:1. 90% of CPC disappear by 28th week but this does NOT change risk! In general, chromosomal analysis should only be performed if additional US abnormalities are seen. Dähnert p 224, Zwiebel’s Intro to US p 408

Slide 156: 

A baby is put on PGE2 for a prolonged time to keep the PDA open. What is the most likely sequelae osteoscleosis osteomalacia osteoporosis elevation of the periosteum

Slide 157: 

D. elevation of the periosteumProtaglandin therapy results in periosteal new bone formation. Kirks Textbook of Pediatric Imaging Chapter 5 pg. 336.“Periosteal bone formation is characteristic of long term, >40 days, or prostaglandin E1. Affected sites include ribs, tubular bines, and to a lesser extent mandible, clavicle, scapula, and pelvis. Can vary from subtle osseous deposites to widespread alterations. (Resnick: Bone and Joint Imaging 2nd ed. P.904)Radiologic Manifestations [of Prostaglandin-induced hyperostosis]: extensive laminar symmetrical subperiosteal bone reaction…; bone-within-bone appearance; undermineralization (osteomalacia);” (Taybi and Lachman, p. 704)

Slide 158: 

Kawasaki’s syndrome is associated with EXCEPT gallbladder hydrops myocarditiss coronary artery aneuysm renal failure pleural effusions

Slide 159: 

D. renal failureKawasaki is an acute febrile multisystem vasculitis of unknown cause involving large, medium size and small arteries with a predilicion for coronary arteries. It is associated with coronary artery aneuryms, coronary artery stenosis, and coronary artery occlusion, myocarditis (25%), pericarditis, valvulities, polyarthritis, aseptic meningities, hepatitis, and pneumonities. Myocarditis can lead to congestive heart failure, which can lead to pleural effusions. Dähnert pg 528

Slide 160: 

What is the most likely diagnosis in a child with AIDS with bilateral reticular opacities but is asymptomatic PCP LIP UIP TB

Slide 161: 

B. LIPThe pulmonary disease in pediatric AIDS is of two major types: acute pulmonary infections (PCP and CMV pneumonia) and noninfectious interstitial pneumonitis (chronic lymphocytic infiltration). Patients with PCP and CMV pneumonia are clinically ill and the early radiograhic findings are of central infiltrates which may evolve to confluent airspace disease. Noninfectious interstitial pneumonitis (chronic lymphocytic infiltration) in children with AIDS included lymphocytic interstitial pneumonitis (LIP), desquamative interstitial pneumonitis (DIP) and chronic interstitial pneumonitis.LIP usually has an insidious clinical onset with only mild cough and dypnea (or asymptomatic) ---radiographically there are diffuse reticonodular densities. Kirks Textbook of Pediatric Imaging, Chapter 7 pg 756.

Slide 162: 

A 7 year old boy presents with 1 leg longer than the other, a sacral dimple, and scolosis- Cause? tethered cord anterior sacral myelomenigocele lipomyelomengiocele and Chiari II sacral teratom

Slide 163: 

C. lipomyelomengiocele and Chiari IILipomyelomeningoceles are lipomas that are tightly attached to the dorsal suface of a neural placode and extend dorsally through a spinal bifida to be continuous with subcutaneous fat. Terminal lipomas attach to the cord at the conus terminalis which is usually low due to spinal cord tethering and then extend dorsally through a sacral spinal bifida. These lesions constitute about 20% of skin covered lumbar sacral masses and beteen 20-30% of occult spinal dysraphism (sacral dimple). When a lumbar sacral mass is present, patients typically come to medical attention before the age of 6 months. If no mass is present (like this case), the clinical presentation is typically the result of neurological or urological deficits that are noticed between the age of 5-10 yrs (this patient is 7 yrs old) . Patients may present with sensory loss in the sacral dermatomes,bladder dynfuction,lower extremity weakness, scoliosis, orthopedic deformities of the foot or leg pain. Sacrococygeal teratomas are rare congential tumors that develop in the lower sacrum. They may present as external masses protruding from the gluteal cleft or from the perineum or they may intrude into the pelvis causing radicular pain, constipation urinary frequency and incontinence.Anterior sacral meningoceles are anomalies characterized by focal erosions or hypogenesis of the sacral or coccygeal segments with herniation of a CSF-filled meninges sac through the defect into the pelvis. Anterior sacral meningocele are usually diagnosed in the second and third decade of life. Symptoms are produced as the result of pressure on the pelvic vicera, causing constipation, urinary frequency and incontinence dysmenhorrha, dysparunea or pain in the lower back.A tethered cord can be found in anterior sacral meningoceles and lipomyelomeningoceles.Pediatic Neuroimaging, Barckovitch chapter 9, pg 641.

Slide 164: 

F/U Scoliosis In Teen - Obtain Which Of The Following Films PA - Improved Resolution PA - To Reduce Breast Dose AP - To Decrease Vertebral Mag AP - To Reduce Ovarian Dose

Slide 165: 

B. PA to reduce breast doseThe type and frequency of radiological evaluation depends on the age and the likely etiology of the curvature. Initial radiological exam includes a minimum of erect (standing if possible) frontal and lateral radiographs from the external auditory meatus to the iliac crest. To reduce the irradiation of the breast, the frontal film should be PA rather than AP. Kirks Textbook of Pediatric Imaging, Chapter4 pg 318.Lateral curving of the spine with varying degrees of rotation of the vertebral bodies around a vertical axis. Idiopathic (90%), secondary (hemivert, growth asymm due to trauma, infection, XRT, NF, etc.). Pre-op: determine type and degree of spine flexibility, measure scoliotic curve (Cobb method), determine degree of rotation (determines extent of required bone fusion). Post-op: general goal is to achieve 50% correction of the scoliotic curve and to maintain normal lung function since scoliosis causes respiratory compromise. Bony fusion is complete within 9 months post-op, best seen on 60 degree supine/oblique projections. Surgery: anterior approach- Dwyer cable, screws in lat vertebral bodies are fastened with cable; posterior approach- Harrington rod placement to achieve fusion of vertebrae. Evaluate hardware (slippage or fx). (Primer pp. 799-800)In idiopathic scoliosis, genetic component has been implicated and is transmitted as AD trait. F:M 8-9:1. Infantile (<3 yrs age), juvenile (4-9 yrs), adolescent (10 yrs to skeletal maturity). Typically convex right thoracic. May be assoc with spinal dysraphism (20%), GU anomalies (20%), and CHD (20%). Other findings include rib fusion, absence, or hypoplasia, Klippel-Feil syndrome, Sprengel’s deformity. Initial exam includes standing frontal and lateral radiographs. PA is recommended to reduce radiation to developing breasts and gonads. Angles <20 degrees not treated. In 25%, curve may progress. Surgical correction not until angle >40 degrees after skeletal maturity. (Pedi Requisites pp. 230-231)

Slide 166: 

Most Common Type Of Choledochal Cyst I II III IV V

Slide 167: 

A. type IFive types of choledochal cysts have been described. Type I choledochal cysts, accounting for 80%-90% of bile duct cysts are subdivided into IA, IB, and IC types. Type IA is characterized by cystic dilatation of the CBD; Type IB refers to focal segmental dilatation of the distal CBD; Type IC is seen as fusiform dilatation of both the common bile duct and the CBD. Type II cysts, comprising 2% of bile duct cysts, are true diverticuli arising from the CBD. Type III cysts, also called choledochoceles, are the result of cystic dilation of only the intraduodenal portion of the CBD. Type IV cysts account for 10% of bile duct cysts and are subdivided into IVA and IVB. Type IVA is characterized by multiple intrahepatic and extrahepatic cysts and Type IVB has multiple extrahepatic cysts only. Type V cysts show multiple intrahepatic cystic dilatation and is termed Caroli’s disease. Pediatric Body CT, Siegel, Chapter 5, pg168-169

Slide 168: 

Juvenile Tillaux Fx : Metaphysis And Lat Physis Metaphysis And Med Physis Epiphysis And Lat Physis Epiphysis And Med Physis Mid Diaphyseal

Slide 169: 

C. Epiphysis And Lat PhysisIn early adolescence, the medial distal tibia has features of adult bone, while the lateral tibia remain immature. The juvenile Tillaux fracture is due to avulsion of the anterolateral corner of the distal tibial epiphysis by the anterior tibiofibular ligament. The fracture extends saggitally through the epiphysis and transversely through the lateral part of the physis. (Salter Harris 3) Kirks Textbook of Pediatric Imaging Chapter 5 pg 436.

Slide 170: 

The lowest acceptable position of the conus in a child is: T12 L2 L4 S1

Slide 171: 

B. L2The normal level of the conus medullaris in a child is L2. (Pediatric Requisites, 2nd ed., 293) If the conus is below the L2/L3 disc interspace it should be considered abnormal. Barkovich 3rd p. 647. Dähnert 5th ed. p.196In an adult, the chord ends above the L1/L2 disc level. Kaplan p. 326.

Slide 172: 

Diagnosing IUGR on US, use: Head circumference to abdominal circumference Head circumference to biparietal diameter Head circumference to thigh circumference

Slide 173: 

A. Head circumference to abdominal circumferenceIn using intrafetsl proportions en elevated HC:AC ratio for dysmature IUGR is 36% sensitive, 90% specific, and has 67% PPV. Not perfect but the best we have based on the choices. Dähnert 5th p1043

Slide 174: 

A child with an enlarged optic chiasm with increased T2 signal in the cerebellum and BG is seen in: NF1 NF2 Sturge weber TS VHL

Slide 175: 

A. NF1 and to a lesser extent E: VHLA: Optic glioma is one of the diagnostic criteria for von Recklinghausen disease, or neurofibromatosis type I, and 75-90% have CNS hamartomas which are hyperintense on T2 including basal ganglia (usually globus pallidus), cerebellar white matter, pons, and thalamus (Dähnert Radiology Review 4th ed p259). B: Neurofibromatosis type II has most commonly bilateral vestibular schwannomas and occasionally café-au-lait spots or small cutaneous neurofibromas, as well as meningiomas and ependymal gliomas but may not have an optic glioma by definition (Dähnert Radiology Review 4th ed p261). C: Sturge-Webber-Dimitri syndrome consists of leptomeningeal venous angiomas confined to the pia with underlying cortical calcifications and occasional T2 white matter hyperintensity but this is rarely cerebellar, and optical involvement is related to vascular anomalies (Dähnert Radiology Review 4th ed p268-9). D: Bourneville disease, or tuberous sclerosis, is associated with cortical, subcortical, and subependymal hamartomas which may be similar to white matter but may also present as multiple high T2/low T2 nodules reflecting demyelination and gliosis, and may occasionally have optic gliomas and subependymal giant cell astrocytomas which are also high signal on T2 but enhance (Dähnert Radiology Review 4th ed p272-3). E: Von Hipple-Lindau disease, or retinocerebellar angiomatosis, often (40%) has central nervous system hemangioblastomas, commonly (65%) in the cerebellum, which are hyperintense on T2, but optic involvement consists of retinal angiomatosis (Dähnert Radiology Review 4th ed p276).

Slide 176: 

The 4 chamber view of the heart on US can diagnose: DORV Coarctation Aortic stenosis Some type of VSD

Slide 177: 

D. some types of VSDThe four chamber view can evaluate: 1. position of heart within thorax; 2. number of cardiac chambers; 3. ventricular proportion; 4. integrity of atrial and ventricular septa; 5. position + size + excursion of AV valvesTherefore could diagnose some types ov VSD Dähnert 5th p997

Slide 178: 

Salter IV over Salter II fracture more likely to occur at: Medial condyle of the elbow Lateral condyle of the elbow Proximal first metacarpal bone Proximal radius Distal tibia

Slide 179: 

B. lateral condyle of the elbowA: F: Medial epicondyle fracture are due to avulsion of the medial epicondyle and are not included in the SH classification. This is the third most common pediatric elbow frx (5-10%) (behind supracondylar and lateral condylar fractures) usually occurring between ages of 9 and 14 years.B: T: “Lateral condylar fracture are most common between the ages of 4 and 10 years. These are usually Salter type IV injuries sustained during a fall on an oustretched hand.” (Chew, p. 108) A fracture of the elbow lateral condyle is frequent (20 % of pediatric elbow fractures, usually between 6-10 yrs of age) and represents a Salter-Harris type IV injury. The fracture line splits the epiphysis and separates a portion of the adjacent metaphysis and the capitulum. Because the extensors of the forearm are attached to the fragment, it commonly is displaced posteriorly and inferiorly by muscle traction. Because the fracture usually extends through the cartilaginous portion of the growth plate, it may resemble a SH II fracture. These fractures are unstable and prone to non-union. (Harris, The Radiology of Emergency Medicine, 1993) “A fracture of the lateral condyle is frequent and represents s SH type IV” (Resnick: Bone and Joint Imaging 2nd ed. P.749)C: F: “Skeletal injuries of the base of the first metacarpal… may be of Salter-Harris types I or, more commonly, II…” (Harris et al, p. 449)D: F: “Radial neck fractures are not as common in children as in adults because of the greater vulnerability of other structures in the growing elbow… usually a Salter type II separation of the radial head…” (Chew, p. 110)E: F: “The most common fractures [of the ankle] are Salter type II fractures of the distal tibia and Salter type I or II fractures of the lateral malleolus.” (Chew, p. 115)

Slide 180: 

Level of the conus medullaris on a neonate T12 L2 L4 S2

Slide 181: 

B. L2Vertebral bodies grow faster than spinal cord during fetal period. Normal location of tip of conus medullaris: L4/5 at 16 weeks of gestation, L2/3 at birth, L1/2 at 3 months of age. In newborn conus should be at or above L3, if not then investigate fot tethered cord. Dähnert 5th p196

Slide 182: 

Where does the normal cord end in a kid L2 T12 L5 T10 S1

Slide 183: 

A. L2Vertebral bodies grow faster than spinal cord during fetal period. Normal location of tip of conus medullaris: L4/5 at 16 weeks of gestation, L2/3 at birth, L1/2 at 3 months of age. In newborn conus should be at or above L3, if not then investigate fot tethered cord. Dähnert 5th p196

Slide 184: 

12 y/o with index and 3rd digit distal soft tissue and bony enlargement. What does this patient have: Neurofibromatosis Glomus tumor Eosinophilic granuloma macrodystrophia lipomatosa Klippel-Trenaurny-Weber

Slide 185: 

D. macrodystrophia lipomatosaMacrodystrophia lipomatosa is a rare form of localized gigantism characterized by a congenital and progressive overgrowth of all the mesenchymal elements of a digit with a disproportionate increase in the fibroadipose tissue. Findings characteristic in distribution of median and plantar nerves thus 2nd and 3rd digits. Resnick: Bone and Joint Imaging 2nd ed. P.1126; Dähnert Radiology Review Manual 5th ed. P.112

Slide 186: 

Most commonly separates hyaline membrane disease from meconium aspiration on CXR: Pleural effusions atelectasis PTX hypoaearation Ground glass appearance

Slide 187: 

D. hypoaerationMeconium aspiration syndrome is most common cause of neonatal respiratory distress in full term / postmature infants (RDS most common cause in premature infants); produces hyperinflation with areas of emphysema. Diffuse, bilateral opacities are seen. Rapid clearing is noted within 48 hours. Small pleural effusions are sometimes present (20%). Hyaline membrane disease, also known as respiratory distress syndrome of the newborn, is an acute disease with hypoinflation (stiff lungs), immature surfactant production, atelectasis, reticulogranular pattern, and prominent air bronchograms. Source: Dähnert 5th ed. p.504,520; Primer 2nd ed p.753

Slide 188: 

The "other" congenital anomaly which most likely yields stridor besides double aortic arch: left arch with abberant right subclavian right arch with abberant left subclavian pulmonary sling

Slide 189: 

C. right aortic arch with abberant left subclavianRings and Slings: During embryogenesis, 6 pairs of ascending brancial arches on either side of the developing foregut contibute to the formation of the aortic arch and its associated great vessels. The first two arches disappear. The third gives rise to the carotid vessels. The fourth arch gives rise to the aortic arch. The fifth fails to develop. The sixth arches give rise to the pulmonary artery and the ductus arteriosis. (Blickman's Pediatric Requisites 1994 page 73-74)Comcomitant Posterior esophageal and anterior esophageal impressions result from a true vasular ring. The most common entity is a double aortic arch (55%) or a right aortic arch with an aberrant left subclavian artery and a ductus remnant. If the ring is tight and causes tracheal compression (symptoms include stridor, respiratory distress, tachypnea) , surgical treatment may be neccessary. In 75 % of the cases of double aortic arch, the descending aorta is on the left with posterior arch more cephalad and the right arch larger. (Blickman's Pediatric Requisites 1994 page 73-74 and Dähnert page 482). A bulbous configuration of the origin of the aberrant left subclavian artery consistent with remnant of the embryonic left arch is called the diverticulum of Kommerell (Dähnert page 483). A true ring is symptomatic in 80% ( Burton's Essential of Pediatric Radiology 1999 page 253-254). The right arch with the aberrant left subclavian artery is rarely associated with congenital heart disease. Mirror image right arch without aberrant vessels is associated with CHD in >95% ( Burton's Essential of Pediatric Radiology 1999 page 253-254). Anterior tracheal impression 2 cm above the carina is caused by the innominatae artery arising to the left of the tracheal and ascending anteriorly. It is considered normal unless accompanied by respiratory symptoms (Blickman's Pediatric Requisites 1994 page 73-74)( Burton's Essential of Pediatric Radiology 1999 page 253-254). Posterior esophageal impression is the result of an aberrant right subclavian artery with a left aortic arch. The left arch with aberrant right subclavian is the most common arch anomaly ( Burton's Essential of Pediatric Radiology 1999 page 253-254). It is usually aymptomatic. Less commonly, an aberrant left subclavian artery with right aortic arch can be the cause. Can cause dysphagia rarely (Blickman's Pediatric Requisites 1994 page 73-74).Lesion between the esophagus and trachea usually represents an aberrant left pulmonary artery arising from the right pulmonary and slinging to the left. Tracheomalacia is frequently present (Blickman's Pediatric Requisites 1994 page 73-74). According to Primer page 128, Tracheobrochomalacia and/or stenosis occurs in 50%.The pulmonary sling is also associated with 1) "napkin ring trachea--absent pars membranacea (50%) and 2) PDA (most common), ASD, persistent left SVC . Symptoms: Stridor, respiratory infection, feeding problems. Inverted T appearance of the mainstem bronchus is secondary to the lower origin of the right mainstem bronchus. Carrot shaped trachea is from narrowing of the tracheal diameter in caudad direction resulting in functional tracheal stenosis. (Dähnert page 505) .

Slide 190: 

The most common supratentorial tumor in a child Astrocytoma meduloblastoma

Slide 191: 

A. astrocytomaAstrocytomas, a form of glioma, represent 37% of supratentorial pediatric brain tumors (50% of all pediatric CNS tumors), PNET’s only 2.5% (most are medulloblastomas which are infratentorial or ependymoblastomas which are rare), ependymomas 5%, hemangioblastomas 1-2.5% and meningiomas 3% (Dähnert Radiology Review 5th ed p232).

Slide 192: 

A child with a posterior fossa tumor that has an enhancing nodule is most likely: Astrocytoma medulloblastoma hemangioblastoma Ependymoma Germinoma

Slide 193: 

A. astrocytomaBoth juvenile pilocytic astrocytoma and hemangioblastoma are cystic posterior fossa masses with enhancing mural nodules.Juvenile pilocytic astrocytoma is the second most frequent tumor of the posterior fossa in children (20% calcify), after medulloblastoma. The tumor tends to present in children younger than 10 years of age, and in the posterior fossa, most frequently occurs off the midline in one cerebellar hemisphere. The imaging characteristics of pilocytic astrocytomas vary tremendously. This tumor can be completely solid or mostly cystic. However, the characteristic appearance is that of a simple or multiloculated cyst with an enhancing node of solid tumor along the cyst wall known as the "mural nodule."Hemangioblastoma, a benign autosomal dominant tumor of vascular origin associated with von-Hippel-Lindau disease, pheochromocytoma, syringomyelia, manifests radiologically in the paravermian cerebellar hemisphere > spinal cord > cerebral hemisphere/brainstem as a solid (1/3)/ cystic/cystic+mural nodule usually without calcifications. However, this tumor is more common in an older age group than juvenile pilocytic astrocytoma if isolated than if associated with VHL disease; besides, hemangioblastomas are less common than astrocytomas. Medulloblastoma (hyperdense mass, noncalcified) and ependymoma (fourth ventricle, 50% calcify) are solid tumors.Common Posterior Fossa Tumors Astrocytomas: 41% (28% Pilocytic, 10% Astrocytoma [not otherwise specified], 2.4% Anaplastic Astrocytoma, 0.1% Glioblastoma multiforme)Primitive Neuroectodermal Tumor [PNET, Medulloblastoma]; 37.8%Ependymomas: 13.7% (12% Ependymoma, 1.7% Anaplastic Ependymoma)* Based on CBTC Study of 3291 first resection specimensNon-tumor cystic lesions without mural nodules in the posterior fossa include arachnoid cyst, trapped 4th ventricle, megacisterna magna, Dandy-Walker cysts.Dähnert 222, 237, Radiology Volume 3 Chapter 74—Posterior Fossa Neoplasms in Children

Slide 194: 

Child with hydrocephalus symptoms has a hyperdense lesion arising from the cerebellar vermis and extending into the fourth ventricle. Most likely diagnosis. Ependymoma Medulloblastoma Astrocytoma Choroid plexus papiloma Glioma Hemangioblastoma

Slide 195: 

B. Medulloblastoma.Medulloblastoma is the most common neoplasm of the posterior fossa in childhood, followed by cerebellar astrocytoma and arises from the external granular layer of the inferior medullary velum of the roof of the fourth ventricle. 91% are located in the vermis cerebelli and roof of the 4th ventricle (younger age group than those with cerebellar hemispheric tumors). Encroachment on the 4th ventricle and cerebral aqueduct results in hydrocephalus in 85-95% of patients. Classic CT features are noted in 53% and include a slightly hyperdense lesion in 70%, rapid intense homogenous enhancement in 97%. MR signal is mixed/hypo on T1WI and hypo/iso/hyper on T2WI, homogenously enhancing with a hypointense rim. Differential diagnosis of a midline medulloblastoma includes ependymoma and astrocytoma (hypodense). Eccentric medulloblastoma differential includes astrocytoma, meningioma, and acoustic neuroma. Hemangioblastoma and juvenile pilocytic astrocystoma appearances, comprising of a cystic lesion/enhancing mural nodule, are described in the the previous question. Choroid plexus papilloma accounts for 0.5-0.6% of all intracranial tumors and 2-5% of brain tumors in childhood. Abnormally increased rate of CSF production results in signs of increased intracranial pressure. Location is glomus of choroid plexus in trigone of lateral ventricles (children), 4th ventricle and cerebellopontine angle (adults), and 3rd ventricle (unusual). Small foci of calcifications are common, although the tumor is is/mildly hyperdense with homogeneous enhancement on CECT.Dähnert 225, 249.

Slide 196: 

Hypoplastic left colon is associated with which 2 of the following? meconium ileus meconium plug cystic fibrosis meconium aspiration

Slide 197: 

B. Meconium plug and C. cystic fibrosis.Meconium plug syndrome is local inspissation of meconium leading to low colonic obstruction (small left colon) characteristically presenting in a newborn in 24 hours as abdominal distension, vomiting, failure to pass meconium. Etiologies include cystic fibrosis (25%), Hirshsprung disease, prematurity, maternal magnesium sulfate treatment. The transverse and ascending colon and small bowel are dilated proximal to the obstruction. Occasionally, there is a bubbly appearance to the colon (differential: submucosal air in necrotizing enterocolitis), presacral pseudotumor (no gas in rectum), double-contrast effect (barium between meconium plug and colonic wall). Meconium plug syndrome is the most common cause of colonic obstruction in a patient with cystic fibrosis; in older children/adults, the most common obstructive presentation of CF is meconium ileus equivalent syndrome. Meconium aspiration is the most common cause of neonatal respiratory distress in full term/post mature infants (HMD is most common in premature infants).Microcolon, a result of distal colonic obstruction involves the entire colon, which usually measures less than 1 cm. Etiologies of microcolon include meconium ileus, ileal atresia, and aganglionosis (Hirshsprung’s disease). Duodenal atresia and related entities are not included (too proximal and succus entericus fills out the colon so that it is not “micro”). Dähnert 5th p847

Slide 198: 

Kid with pulmonary edema and pleural effusions could be due to which one of the following: lymphangiectasia TAPVR III Wet lung All of the above

Slide 199: 

D. all of aboveFew conditions mimic the roentgenographic features of HMD. Several congenital heart lesions manifesting pronounced pulmonary venous obstruction (i .e., total anomalous pulmonary venous return type III, pulmonary vein atresia, and hypoplastic left heart syndrome) can produce enough pulmonary edema to masquerade as IRDS, but generally these are uncommon. In addition, some cases of pulmonary lymphangiectasia, transient respiratory distress of the newborn (wet lung syndrome), and neonatal sepsis or pneumonia can be radiographically confused with HMD.Pulmonary lymphangiectasia in the newborn is a rare, severe condition in which proliferation of the lymphatic tissue compromises pulmonary ventilation. The chest radiograph shows severe interstitial edema with reticular areas representing dilated lymphatics and Kerley B lines. This condition is often fatal early in the newborn period. The condition mimics precisely total anomalous pulmonary venous connection below the diaphragm.The relatively mild disorder of transient tachypnea of the newborn may simulate RDS clinically but resolves within a few days and is thought to be due to delayed resorption of fetal lung liquid following birth. “Wet lung"is another term applied to the pulmonary edema-like pattern of the lungs, which consists of perivascular haze,"interlobular septal edema, thickened fissures, and mild cardiomegaly”. A reticulogranular pattern is not apparent, and tachypnea and radiographic abnormalities subside within hours or a few days. In the early stages of the disorder, it is important to exclude other causes of pulmonary edema, particularly congenital heart disease. Radiology Volume 2 Chapter 123—The Premature Infant with Heart Disease

Slide 200: 

What determines the side of the Right atrium IVC Aorta LV Pulm artery

Slide 201: 

A. IVCRight atrium is defined by lack of a pulmonary vein, smooth posterior border, same side as azygous vein, receives the IVC. Smooth posterior border is called the sinus venarum, in contrast to anterior, ridged portion called musculi pentinati. Dähnert 5th p581

Slide 202: 

What is seen in situs inversus Aortic knob and stomach on right Aortic knob on right and stomach on left Aortic knob on left and stomach on right Aortic knob and stomach on left

Slide 203: 

A. Aortic knob and stomach on right.Situs inversus=mirror image. Morphologic left structures on right and right on left. Position of the aortic arch in NOT a good indicator of situs relationships; it is usually on the same side as the morphologic left atrium. Generally, there should be concordance of Ao, LA, LV, and gastric bubble. If the Ao arch is opposite these (discordant), there is increased risk of CHD. For the purposes of answering this question, I assumed there is concordance of the aortic knob with the LA and LV. Dähnert 5th p 582

Slide 204: 

A neonate with increased pulmonary vascularity may include all of the following except: Total anomalous pulmonary venous return Type III (subdiaphragmatic) Tetrology of Fallot Hypoplastic left heart Transposition of the great arteries

Slide 205: 

B. Tetrology of FallotAll of these are cyanotic lesions. Only Tet has decreased PBF. The others are increased.Algorithmic approach to CHD:I. ACYANOTICA. NORMAL PBF If these lesions are severe can affect PBF Pulm Stenosis, Aortic Stenosis, Coarctation (Focal). Diffuse form (Tubular Hypoplasia) can ->CHF B. INCREASED PBF 1. L->R SHUNTS: VSD, ASD, Endocardial Cushion Defect, AP Window Peripheral AVM/ Vein of Galen fistula, Ruptured sinus of Valsalva aneurysm 2. Any cause of failure in heart without shunt: cardiomyopathy, myocarditis, Adriamycin toxicity, sepsis, glycogen storage diseaseII. CYANOTIC = ADMIXTURE lesionsA. INCREASED PBF = “R->L SHUNTS” although more blood -> Rt circ due to lower pressure (mnemonic 4TS+CHD) - TGV, TAPVR, Truncus Arteriosus, Tricuspid Atresia with TGV (25%), Single Ventricle, Common Atrium, HLHS, DORV (the last 3 can result in failure also)B. DECREASED PBF = R->L SHUNTS with Pulm Outflow Obstruction so more blood -> Lt (mnemonic TET P) - Tetralogy of Fallot, Ebstein Anomaly, Tricuspid Atresia without TGV (75%), Pulmonary Atresia (the 1st 3 are most common, the last 3 cause cardiac enlargement)Ref: Gael Lonergan’s excellent handout from AFIP

Slide 206: 

At what age is brain myelination complete? 1 month 3 months 8 months 12 months 24 months

Slide 207: 

E. 24 monthsAt birth, the thalamus, cerebellar peduncles, and median longitudinal fasciculus are myelinated. By the age of 3 months, the cerebellar white matter is myslinated, alsong with the posterior limb of the internal capsule and the paraventricular and paracentral white matter. By 6 months, the optic radioation and splenium are myelinated, and by 1 year the anterior limbs of the internal capsule and genu of the corpus callosum are myelinated. Ceretral lobar myelination progresses during the second year, and an adult pattern is reached by athe age of 24 months. Peds Requisites 2nd p267

1998 : 

1998 Regarding supracondylar fractures:most common elbow fracture in a childis a Salter-Harris II fractureAnterior humeral line intersects the middle third of capitellum indicates abnormality

Slide 209: 

A. most common elbow fracture in a child.Supracondylar fractures are the most common pediatric elboy fracture (60%). Next two most common are lateral epicondylar fracture (15%) and medial epicondylar fracture (10%). It does not involve the growth plates of the elbow. The anterior humeral line normally passes through the middle 1/3 of the capitellum. Primer, 3rd, 845-846

Slide 210: 

Which of the following ossification centers is present in a newborn?proximal femurdistal femurdistal tibia

Slide 211: 

B. distal femur.“The only secondary ossification centers routinely seen in full-term infants are those in the distal ends of the femurs and the proximal ends of the tibiae.” Proximal humerus would have been a better option, but it wasn’t presented.Calcaneus – 23 wks gestationTalus – 28 wksDistal femur – 36 wksProx tibia – 38 wksCuboid – 40 wksProx humerus – 40 wksHyoid – by birthCapitate – 3 months oldCapitellum – 3 monthsProximal femur – 2-6 months (asymmetric)“CRITOE” – capitellum, radial head, medial epicondyle, trochlea, olecranon, lateral epicondylePrimer, 3rd, 845 and Pediatric Requisites, 2nd, 197-198

Slide 212: 

Which of the following is not a cause for cardiomegaly in a newborn?a. viral myocarditisb. TAPVR below diaphragmc. vein of Galen aneurysmd. Hypoglycemiae. Arrhythmia

Slide 213: 

B. TAPVR below the diaphragm.Cardiomegaly caused by valvular disease, cardiomyopathy, CHD, pericardial effusion, and mass lesions. Vein of Galen AVM also associated with cardiomegaly.Massive cardiomegaly in the newborn results from Ebstein’s anomaly (RA enlargement), Uhl’s dz (focal or total absence of RV myocardium), tricuspid atresia, herniation of liver into pericardial sac, massive pericardial effusion.TAPVR with obstruction presents with a normal cardiac silhouette but with severely congested pulmonary vessels and interstitial pulmonary edema. TAPVR without obstruction presents with cardiomegaly (dilated right ventricle and right atrium), an enlarged pulmonary artery segment, and increased pulmonary flow. The classic “snowman” cardiac silhouette occurs in older patients with nonobstructive TAPVR but not in infants and children. The snowman appearance only occurs in supracardiac TAPVR, the most common type, which is infrequently associated with obstruction. Infracardiac (below the heart) TAPVR occur in only 15% of cases and the majority are obstructed. Obstructed TAPVR does not cause an increase in heart size.Primer, 3rd, 131-132, 139, 150, 503-504 and Pediatric Requisites, 2nd, 63

Slide 214: 

12 yo previously healthy male presents with SVC syndrome and calcifications in the mediastinum.a. Teratomab. Lymphomac. Tuberculosisd. Histoplasmosis

Slide 215: 

D. Histoplasmosis.All of these conditions can cause mediastinal lymphadenopathy. Of these, histoplasmosis is the one more likely to calcify. It is the best answer.Middle mediastinal masses can be: adenopathy (benign—sarcoid, TB, fungal) or malignant—mets, lymphoma), Congenital cysts (bronchogenic, pericardial), Aneurysms (aorta or pulmonary artery), Esophagus (HH, neoplasm, diverticula, megaesophagus), Other (hemorrhage, lipomatosis, varices, etc.).In children inflammatory lymph nodes or lymphoma, foregut abnormalities, prominent vessels, or pericardial abnormalities are most common. Primer, 3rd, 101 and Peds requisites, 2nd, 45

Slide 216: 

Which is the most common mediastinal mass in children?Lymphoma neuroblastoma ganglioneuroma teratomabronchogenic cyst

Slide 217: 

B. neuroblastoma.Neurogenic tumors are the most common mediastinal masses of childhood. Neuroblastoma is much more frequent than ganglioneuroma or ganglioneuroblastoma and usually occurs in children less than 2 years of age. In children, posterior:middle:anterior masses are 40%: 30%:30%. The most common pseudomass in the mediastinum of a child is a normal thymus. Anterior and middle mediastinal masses each comprise 30% of mediastinal masses. Posterior mediastinal masses comprise the other 40% and 90% of those are neurogenic tumors.Peds Requisites,2nd, 52

Slide 218: 

Which is not a findings on prenatal ultrasound in Down’s?a. Hyperechoic bowelb. Thickened nuchal foldc. Mild pyelectasisd. Ventriculomegalye. Shortening of the femurs and humeri

Slide 219: 

D. Ventriculomegaly.Down Syndrome usually has sonographically detectable findings (13-50% may not though). Most common anomaly is CHD (40-50%); AV canal defect is usually mentioned with Down’s but ASD and VSD are most common. Other associated anomalies: duodenal atresia, hydrothorax, hydrops, omphalocele, increased nucal thickness, echogenic bowel, short femur length, shorter humeral length, pyelactasis, echogenic intracardiac focus, small frontal lobes, separation of great toe from second toe, hypoplasia of fifth digit and clinodactly (incurving of fifth digit to fourth).Primer, 3rd, 776 and Ultrasound Requisites, 221

Slide 220: 

A 10 mm diverticulum extending from the prostatic urethra and a hypospadias abnormality are present in a patient. What is the embryologic origin of the 10mm lesion?mullerian ductwolffian ducturogenital sinuscloacal septum

Slide 221: 

A. mullerian duct.This is likely a mullerian remnant, a dilated utricle, which is commonly associated with hypospadias and incomplete testicular descent, a complex of findings suggesting intersex. The normal prostatic utricle is 8 to 10 mm in length, narrow at its orifice (2mm ) in the verumontanum, and bulbous at its blind end. Because the dilated utricle communicates with the urethra, it can be imaged during antegrade or retrograde urethrography.Dunnick NR, Snadler CM, Amis ES, Newhouse JH; Textbook of Uroradiology, 2nd ed, Williams & Wilkins, 1997. p. 37

Slide 222: 

Infant with esophageal atresia and TEF most likely also has:a. renal agenesisb. anal atresiac. absent radiusd. polydactyly

Slide 223: 

C. absent radius.Phocomelic abnormalities result from fetal vascular insults and in the past thalidomide embryonopathy. One of the best known of these is the “radial ray” syndrome whereby the radius and first and second digits of the hand are variably underdeveloped. These changes occur in the VATER syndrome. Differential Diagnosis in Pediatric Radiology, 213-214

Slide 224: 

Which of the following is more characteristic of untreated Respiratory Distress Syndrome as compared with TNN (transient tachypnea of the newborn)?low lung volume pleural effusion interstitial edema

Slide 225: 

A. low lung volume.Prominent vascular markings and pleural effusions are features of TTN, whereas pleural effusions are uncommon in RDS. In fact, a normal CXR at 6 hrs excludes RDS / HMD (hyaline membrane disease). RDS will show low lung volumes due to inadequate alveolar expansion due to surfactant deficiency. TTN is delayed resorption of intrauterine pulmonary liquids, similar in appearance to noncardiogenic pulmonary edema. Primer of Diagnostic Imaging, 3nd ed, 1997, pp. 811-814

Slide 226: 

At what level is the obstruction in a male infant with vesico ureteral reflux?a. bladder neckb. prostatic urethrac. membranous urethrad. bulbous urethra

Slide 227: 

B. prostatic urethra.After UPJ obstruction, posterior urethral valves is the next most common cause of hydronephrosis. They occur in the region of the verumontanum of the prostatic urethra—hence, the only occur in males. Peds Requisites, 2nd ed, 160-164

Slide 228: 

Which of the following does not cause atlantoaxial dissociation in children?a. traumab. Morquio’sc. Hypothyroidismd. Down’se. JRA

Slide 229: 

C. hypothyroidism.Mnemonic for atlantoaxial subluxation: JAP LARD” Juvenile rheumatoid arthritis Ankylosing spondylitis Psoriatic arthritis Lupus erythematosus Accident (trauma) Retropharyngeal abscess, Rheumatoid arthritis Down syndrome Also Morquio’s but it’s not in the mnemonic. Primer, 3rd, 471

Slide 230: 

In a three-month old, what is the most likely etiology of a renal mass?multicystic dysplasiaWilms tumorNeuroblastomaMesoblastic nephroma

Slide 231: 

D. Mesoblastic nephroma.Most likely renal mass in 3 month old is mesoblastic nephroma. This is a non-familial genign fibromatoid mass arising from renal connective tissue. Incidence: most common renal neoplasm in neonate; 3% of all renal neoplasms in children. Age: 3 months mean age at presentation. In 14% it is associated with prematurity, polyhydramnios, GI & GU tract malformations, neuroblastoma. Primer, 3rd, 839-842, 875

Slide 232: 

What is the most common cause of limp in a 3 yr old?a. toxic synovitisb. osteomyelitisc. Legg-Calve Perthes d. septic arthritise. tarsal coalition

Slide 233: 

A. toxic synovitis.“Most common nontraumatic cause of acute limp in a child” Dähnert 5th p. 167* If child were febrile, consider D or B.Septic arthritis is due to secondary joint involvement in osteomyelitis by an infecting organism that crosses the growth plate via vascular channels. It is most often in the shoulder or hips. S. aureus is the most frequent organism. Imaging findings include joint effusion, periarticular osteopenia, and apparent joint dislocation or fracture through the growth plate.Nelson Pediatrics: Febrile child < 3 with acute hip pain, very likely septic arthritis, afebrile child 2-6, most likely idiopathic or toxic synovitis; 5-10, Perthes; obese child > 10, SCFE.Pediatric Requisites, 2nd, 225

Slide 234: 

Matching: 1. Diabetic mother2. Aganglionosis3. Cystic Fibrosis4. Small left colon a. meconium aspirationb. meconium peritonitisc. meconium plugd. meconium ileus

Slide 235: 

1=C 2=B 3=D 4=CAganglionosis is another term for Hirschsprung's disease. Complications include obstruction and perforation which could cause meconium peritonitis. But meconium peritonitis is more commonly discussed with meconium ileus and cystic fibrosis. As the colon becomes obstructed with Hirschsprung’s, meconium plug might be considered as an answer. However, meconium plug is also termed Functional Colonic Obstruction and is due to neuronal underdevelopment not lack of development.Meconium Ileus occurs when meconium inspissates and obstructs the distal ileum in >99% of pt.s with cystic fibrosis. There is always a true microcolon.In meconium plug there is a long thick plug of meconium lodged in the distal colon, probably as a result of neuronal under development...these are full term and have distal obstruction. In premature infants there is often immaturity of the colon, especially in infants of diabetic mothers. In addition there may be functional obstruction caused by inspissated feedings. Contrast enema usually reveals a small descending colon and normal caliber right and transverse colon (aka, neonatal small left colon syndrome). This condition resolves spontaneously after the passage of several stools. Peds Requisites, 2nd, 112-115 and Primer, 3rd, 824-825

Slide 236: 

12 y.o. previously healthy girl presents with soft tissue masses over her shoulders and hips. Plain film reveals multiple calcified soft tissue masses.a. Lesch-Nyhamb. Dermatomyositisc. Polymyalgia rheumaticad. Myositis ossificans progressivae. Tumoral calcinosis

Slide 237: 

E. Tumoral calcinosis.Idiopathic tumoral calcinosis, progressive large nodular juxtaarticular calcified soft tissue masses. Multilocular cystic lesions with a fluid/fluid level occasionally identified. Pararticular in hips>elbows> shoulders, rarely knees. Usually presents in 1st or 2nd decade. Juvenile Gout (Secondary Gout) eccentric juxtaarticular lobulated soft-tissue mass. Joint effusions, calcific deposits. - Location: hands/feet (1st MTP) > ankles > heels > wrists > fingers > elbows- Involvement of hip and spine is rare.Progessive myositis ossificans (Fibrodysplasia Ossificans Progressiva) presents by age 2. Intially painful sub Q masses on neck, shoulder, upper extremities. Ectopic Ossification with ossification of voluntary muscles, complete by age 25 Dähnert 75, 94-95, 170 Resnick 1258Myositis ossificans progressiva is a hereditary disorder that progressively ossifies striated muscles, tendons, and ligaments. The target tissue in this disease is thought to be the interstitial tissues, with muscle involvement secondary to pressure atrophy. The pathologic abnormalities are similar to those of myositis ossificans. The most frequent presenting symptom and location is acute torticollis with a painful mass seen in the sternocleidomastoid muscle. The process then progresses to the shoulder girdle, upper arms, spine, and pelvis. The heterotopic bone often bridges between adjacent bones of the skeleton and eventually causes a severe restriction of motion. It usually occurs in first decade of life, boys > girlsMusculoskeletal Requisites, 2nd, 100-101

Slide 238: 

Which of the following are the most common coalitions:talonavicular and middle subtalar jointtalonavicular and posterior subtalar jointcalcaneonavicular and middle subtalar facetcalcaneonavicular and posterior subtalar facet

Slide 239: 

C. Calcaneonavicular and middle subtalar facet.Tarsal coalition present with painful pes planus. The bridging tissue is fibrous at birth, but usually ossifies in the second decade of life (although fibrous union can persist into adulthood). Kirks says 50% are bilateral; Hadnert says 20%. The two most common forms are calcaneonavicular and talocalcaneal (subtalar – talocalcaneal). Dähnert claims that the calcaneonavicular is the most common, but Kirks says it’s a tie. Within the subtalar joint, the middle facet is most common. Radiologic buzzwords include “dorsal talar beaking” and “ball and socket ankle joint”. Primer, 3rd, 854

Slide 240: 

Growth plate fracture in the distal phalanx of the great toe is most worrisome for:a. growth disturbanceb. osteomyelitisc. subungual exostosis

Slide 241: 

A. growth disturbance.A fracture involving the growth plate is most worrisome for growth disturbance due to compromise of the physis.Primer, 3rd, 845

Slide 242: 

Primary megaloureter is most associated with which one of the following:a. Contralateral renal agenesisb. Delayed ureteral emptyingc. Ipsilateral cryptorchidismd. Hypospadiase. UPJ obstruction

Slide 243: 

E. UPJ obstruction.congenital primary megaureter – intrinsic congenital dilation of lower juxtavesical orthotopic ureter. M:F = 2-5:1. Associated disorders in 40% - contralateral UPJ obstruction, reflux, ureterocele, ureteral duplication renal ectopia, or renal agenesis. Ipsilateral disorders include caliceal diverticulum, megacalicosis, papillary necrosis. L:R = 3:1, bilateral in 15-40%.(Dähnert p. 774)a = Contralateral renal agenesis: 1:1000.b = Delayed ureteral emptying:c = Ipsilateral cryptorchidism:d = Hypospadias:E = UPJ obstruction: Primer, 3rd, 832-833

Slide 244: 

Hypospadias is most commonly associated with:a. Prune - Belly Syndromeb. Cryptorchidismc. Megaloureterd. Malakoplakiae. Leukoplakia

Slide 245: 

B. Cryptorchidism.a = Prune-Belly syndrome: associated with cryptorchidism, VUR.B = Cryptorchidism: 30%.c = Megaloureter: see question 86.d = Malakoplakia: rare, inflammatory condition that affects bladder most commonly. cobblestone appearance.e = Leukoplakia: ureteral, bladder, collecting system.Primer of Diagnostic Imaging. 3rd, 835.

Slide 246: 

Which is NOT associated with Wormian bones?normal variantOsteogenesis imperfectaCleidocranial dysostosisChiari II

Slide 247: 

D. Chiari IIWormian bones are intrasutural bones, most commonly in the lambdoid suture. Differential is“PORKCHOPS”PyknodysostosisOsteogenesis imperfectaRickets in healingKinky hair syndromeCleidocranial dysplasiaHypothyroidismOtopalatodigital syndromePachydermoperiostosis“Syndrome of Down”It may also be a normal variant until 1 year of age. Primer of Diagnostic Imaging, 3rd ed, 864

1997 : 

1997 What is the next appropriate study for a drooling infant with poor feeding and inability to pass a NGT? UGI BE Renal US Head US Chest CT

Slide 249: 

A. UGI.The main concern is for a tracheo-esophageal abnormality.Primer 816

Slide 250: 

6 month old peritoneal findings Inflammatory polyps Intussusception (6 months – 2 years) Meconium equivalent ? Infertile ? Chronic sinusitis

Slide 251: 

B. Intussusception. (assuming age for the presenting symptoms is what is being asked)Intussussception 90% ileocolic and ileoileocolic. Usually first 2 years of life ( 40% from 3-6 months). Pain, vomiting, hematochezia.Meconium equivalent occurs in older patients with CF Primer 821-2

Slide 252: 

Matching: Trisomy 13 Chiari II Coronal suture closure Sagittal suture synostosis Apert's syndrome Dandy Walker BrachycephalyScaphocephalyMicrocephalyMacrocephalyMicrocraniaMacrocraniaDolichocephaly

Slide 253: 

1. C. DX of microcephaly: Intrauterine infection, toxic agents, drugs, hypoxia, radiation, maternal phenylketonuria, premature craniosynostosis, chromosomal abnormalities, Meckel-Gruber. Reference: Dähnert, page 168.2. G. Enlargement of occipital homs and atria due to maldeveloped occipital lobes. Reference: Dähnert, page 199.3. A. Coronal synostosis is the second-most common type and results in plagiocephaly when unilateral ("harlequin eye") and brachycephaly when bilateral. Reference 173-4.4. B. Sagittal suture craniosynostosis is the most common type and results in dolichocephaly = scaphocephaly = elongation of the skull in the AP dimension. Reference 173-45. A. Apert syndrome is AD. Brachycephalic (= abnormally wide) due to coronal craniosynostosis, hypoplasia sinuses, fusion of phalanges, metacarpals, carpals, absence middle phalanges. Reference: Dähnert 456. G. Large skull secondary to hydrocephalus and dolichocephaly (longer than wide) Reference: Dähnert, page 274.

Slide 254: 

Five-day-old full-term baby underwent cardiac angiography for evaluation of VSD and coarctation of the aorta. Two to three days later, an x-ray showed dilated bowel with pneumatosis. Most appropriate action:Repeat the film in 24-48 hoursBarium enema to find perforationContrast-enhanced abdominal CT scanDecubitus film to look for free airAortic ultrasound to detect thrombosisCross-table lateral to rule out free air

Slide 255: 

D. decubitus film to look for free air.The major concern is for ischemic bowel/necrotizing enterocolitis. The only true radiograph indication for surgery is perforation. If a perforation is suspected, a left lateral decubitus film is preferred over the supine cross-table lateral exam. Barium is contraindicated. Remember that gas in the portal system does not imply a bad outcome as is does in the adult. Reference: Blickman, Pediatric Radiology, The Requisites, page 63-65.

Slide 256: 

The most common type of tracheo-esophageal abnormality: Distal fistula with esophageal atresia Proximal and distal fistulas H-type fistula Proximal and distal atresia without fistula Distal atresia and proximal fistula

Slide 257: 

A. Distal fistula with esophageal atresia.Most common is EA and distal fistula (82%), then EA without fistula (9%), no EA with H-type fistula (6%), EA and two fistulas (2%), proximal fistula and distal EA (1%).Reference: Blickman, Pediatric Radiology, The Requisites, page 71.Dähnert 807

Slide 258: 

The lowest level of the tip of the conus in the normal neonate: T10-11 T12 L2 L4 S1

Slide 259: 

C. L2Tethering of the cord id caused by primary shortening of the filum terminale or is secondary to dysraphic changes preventing the normal "ascent" of the conus medullaris above L2-3. In an infant, US may demonstrate low termination of the conus below L2 or L2-3.Reference: Blickman, Pediatric Radiology, The Requisites, page 228.

Slide 260: 

Two-year-old female with first UTI. Next test (Single best answer): VCUG or RNC Renal US DMSA IVP Glucoheptonate

Slide 261: 

B. Renal US.U/S first imaging study of choice to evaluate for structural abnormality. All patients get a U/S after the first UTI. VCUG or scintigraphy to evaluate for vesicoureteral reflux.Primer 837

Slide 262: 

A newborn presents with failure to thrive, difficulty feeding, and intermittent cyanosis. Failure to pass NG tube and normal CXR. What test would you order next? Chest CT Upper GI BE Renal ultrasound Pulmonary function tests

Slide 263: 

B. Upper GI.The main concern is for a tracheo-esophageal abnormality. A carefully performed UGI is the study of choice. Primer 816

Slide 264: 

Match: Hemangioblastoma Venous angioma Port wine stain Telangiectasia Sturge-WeberVon Hippel-LindauOsler-Weber-RenduOther phakomatoses

Slide 265: 

Answers: 1. B. Von Hippel-Lindau is AD (chromosome 3) consisting of retinal, cerebellar and visceral angiomatous lesion including hemangioblastomas 50%.2. A. Sturge-Weber see below.3. A. Sturge-Weber is characterized by facial port wine stain in VI distribution, leptomeningeal venous angiomas, subpial tramlino calcifications.4. C. Also called Hereditary Hemorrhagic Telangiectasia. Autosomal dominant inheritance and manifests as cutaneous and mucous membrane telangiectasias, AVM, and aneurysm. Primer 564,565

Slide 266: 

True/False - Wilms tumor: Associated with hereditary aniridia Associated with Beckwith-Wiedemann syndrome Most common pediatric tumor to invade IVC

Slide 267: 

Answers:1. False. Wilms tumor is associated with sporadic aniridia, hemihypertrophy, Drash syndrome, and GU anomalies.2. True. Beckwith Wiedemann syndrome = EMG syndrome = exophthalmos, macroglossia, gigantism + hepatomegaly, hyperglycemia from islet cell hyperplasia.3. True. Wilms tumor is most common abdominal neoplasm in children from 1-8 y.o. and third-most common malignancy in childhood after leukemia and brain tumors. Four to ten percent invade IVC/RA. Reference: .Primer 840

Slide 268: 

True/False - Celiac sprue is associated with transient intussusception.

Slide 269: 

True.Transient nonobstructive intussusception (20%) without anatomic lead point.Reference: Primer 180

Slide 270: 

True/False - Cystic fibrosis is associated with meconium plug syndrome.

Slide 271: 

False.Meconium plug syndrome is neonatal low colonic obstruction due to colonic inertia, usually in full-term babies. It is associated with diabetic mothers and mothers treated with MgSO4 for preeclampsia. Reference: Primer 821.