RD paediatric formatted Flashcards

Question

7) 15 yo male. Generalised bone pain. Cardiomegaly. Broad ribs. Flattened thoracic vertebrae. Most likely: i) Glycogen storage disease ii) Sickle cell iii) Thallasaemia iv) Fibrous dysplasia v) Mets

Answer 1

iii) Thallasaemia T get cardiomegaly, broad posterior ribs, vertebral compression fractures; joint pain (not sure about generalized bone pain) 7) 15yo male. Generalised bone pain. Cardiomegaly. Broad ribs. Flattened thoracic vertebrae. Most likely (SK/JA): i) Glycogen storage disease F- GCS affects liver & muscle ii) Sickle cell F get skeletal pain (osteomyelitis, AVN), cardiomegaly, H-shaped vertebra, rib notching/sclerotic areas (but not listed widening) iii) Thallasaemia T get cardiomegaly, broad posterior ribs, vertebral compression fractures; joint pain (not sure about generalized bone pain) iv) Fibrous dysplasia F v) Mets F

Answer 2

ii) Increased lung volumes T – often hyperinflated, may be asymmetric & patchy 8) Regarding meconium aspiration, which of the following is the most correct? i) Symmetrical involvement F - asymmetric & patchy ii) Increased lung volumes T – often hyperinflated, may be asymmetric & patchy iii) Confluent opacification F - patchy iv) Pneumothorax rare F 20-40% v) Premature infants F rare in pre-term, more common in post-dates babies

Answer 3

i) Not indicated T – according to AAP guidelines 9) Newborn, term infant, normal karyotype, (? sex). Cephalic presentation. No family hx DDH. Normal examination from paediatrician. Intern rings saying Mum worried about DDH and requests US. You advise her: i) Not indicated T – according to AAP guidelines ii) Do hip US within 2 weeks F – ideally wait until 2 weeks, MSK Req says 4-6 weeks iii) Do hip US at 6 weeks F – but if needed is optimal timing iv) Do hip US at 6 months F – do US in infants < 4-6 months before femoral head ossified v) Do Pelvic XRAY at 6 months F – too late, but after 4-6 months do XR not USWA Imaging pathways • Risk factors that predispose to DDH include - o Family historyo Associated congenital orthopaedic conditions o Oligohydramnioso Breech presentationo High birth weight • If risk factors present, and screening is indicated ultrasonography is the initial investigation of choice • Ultrasonography should be performed before 6mnths of age. After this time, plain films of the hip are indicatedWA Imaging pathways • Risk factors that predispose to DDH include - o Family historyo Associated congenital orthopaedic conditions o Oligohydramnioso Breech presentationo High birth weight • If risk factors present, and screening is indicated ultrasonography is the initial investigation of choice • Ultrasonography should be performed before 6mnths of age. After this time, plain films of the hip are indicated

Answer 4

iii) Increased signal on STIR imaging T – T2 FS hyperintense at site of cortical desmoid 11) Which is the most correct regarding a cortical desmoid? i) Posteromedial condyle F – posteromedial distal femoral metaphysis (just lateral to adductor tubercle) – occurs at attachment of medial head gastrocnemius or adductor magnus insertion ii) Lateromedial condyle F iii) Increased signal on STIR imaging T – T2 FS hyperintense at site of cortical desmoid iv) Increased uptake on bone scan in 70% F – Normal or slightly increased uptake in lesion v) Biopsy required for diagnosis F – do not touch lesion

Answer 5

iv) Bilateral Hip US

Answer 6

iii) Ectopic pancreas T

Answer 7

• D = Nasal glioma = (“Sequestered cephalocele”) = Well-circumscribed, solid soft tissue mass off midline at nasal dorsum or within nasal cavity in infant with no CSF connection to brain; T1 mixed-low signal; T2 iso-hyper (?T) **SCS: Agree. AKA: nasal glial heterotopia. Paramedian. Extra nasal 60% (intra- 30, mixed 10). Dysplastic glial cells that have lost their intracranial connections. 15% may be connected by stalk of tissue to brain. No fluid filled tract or Subarach space contiguity. • A = Dacrocystocele = Round-ovoid mass at medial canthus extending along NLD course to inferior meatus; T1 hypo, T2 hyper (T) • B = Nasal dermal sinus = Ovoid mass ± tubular sinus tract; Epidermoid or dermoid seen from nose tip to apex of crista galli; fat intensity (T1 hyper, T2 intermediate) (F) • C = Encephalocele = Direct extension of meninges, subarachnoid space ± brain projecting into cephalocele via defect in cribriform plate or frontal bone; isointense to brain with surrounding CSF (F) • D = Nasal glioma = (“Sequestered cephalocele”) = Well-circumscribed, solid soft tissue mass off midline at nasal dorsum or within nasal cavity in infant with no CSF connection to brain; T1 mixed-low signal; T2 iso-hyper (?T) • E = Narrow posterior nasal cavity (F)

Answer 8

• A = false = not present if complete CC agenesis; should be present in partial agenesis • B = false = abnormal cingulate gyrusPosterior genu and anterior body form first, followed by posterior body, splenium, and finally rostrum.If splenium present & remainder of CC absent → holoprosencephaly (lobar)

Answer 9

c. retinoblastoma • A = Coats = F retinal vascular anomaly = high-density vitreous, but calcification rare • B = persistent hyperplastic vitreous = F hyperdense small globe; S-shaped Clouqet’s canal; does not calcify • C = retinoblastoma = T 98% occur in children < 3 yrs (StatDx says 90-95% Dx by 5 years); punctate/speckled calcifications in posterior globe • D = neuroblastoma = less T typically in children < 2 yrs; ocular involvement in 20% at presentation; calcifications occur

Answer 10

• C = T = may be preceded by viral illnessIntussusception • A = F (assoc/ w/ ↓ success rate, but not contraindication) • B = F (poor success rate if > 24 hrs symptoms) • C = T = may be preceded by viral illness • D = F = 90% idiopathicAir reduction enema • Contraindications o Peritonitis (clinical exam)o Free intraperitoneal air (XR)• Features assoc/ w/ ↓ success rate but not contraindications:o SBOo Symptoms > 24 hours o Poor clinical condition (lethargy)

Answer 11

b. Complications usually include abscess formation and adhesions. F = complications can include abscess, adhesions, obstruction (but not usual) 57. A young girl presents with abdominal pain. Which of the following regarding omental infarction is false? a. The pathology is usually found on the right. T = 90% found on right b. Complications usually include abscess formation and adhesions. F = complications can include abscess, adhesions, obstruction (but not usual) c. The lesion is usually located near the transverse and ascending colon. T = located between anterior abdominal wall and colon d. Omental infarction is a benign and self-limiting disease and usually resolves with conservative measures. T e. US features include a hyperechoic ovoid lesion T Hyperechoic, nonmobile, noncompressible fixed mass

Answer 12

a. hypoinflation Meconium aspiration • A = F Features of meconium aspiration o Hyperinflation (↑ lung volumes) – may be asymmetric & patchy o Consolidation – asymmetric & patchy (usually no air bronchograms) o Rope-like perihilar densities o Subsegmental atelectasis, alternating with areas of hyperinflation/air-trapping o Pleural effusions may be present

Answer 13

Cystic fibrosis • A = T • B = T (usually lobar though according to StatDx; Dahnert says subsegmental/segmental/lobar) • C = F Hyperinflation earliest finding, may be reversible early & then permanent • D = T = recurrent pneumonias, often difficult to detectNote⇨ Most F is C; most T is A

Answer 14

A = infantile haemangioma = ?T JMIRO 2012 – “characterised by a cycle of growth, plateau & involution. The duration of this cycle is variable, yet complete in the majority by 10 years”; high-flow mass; US Clin 2009: the presence of a distinct mass at sonography, the high vessel density, and the low RI with Doppler are the most important diagnostic criteria of infantile hemangioma. Mean peak arterial flow velocity 28 cm/s. Most common tumour of head & neck in infancy (RadClin 2011). A = infantile haemangioma = ?T JMIRO 2012 – “characterised by a cycle of growth, plateau & involution. The duration of this cycle is variable, yet complete in the majority by 10 years”; high-flow mass; US Clin 2009: the presence of a distinct mass at sonography, the high vessel density, and the low RI with Doppler are the most important diagnostic criteria of infantile hemangioma. Mean peak arterial flow velocity 28 cm/s. Most common tumour of head & neck in infancy (RadClin 2011). B = congenital haemangioma = ?F either involute in infancy (‘rapidly involuting congenital haemangioma’) or grow proportional with child (‘non-involuting haemangioma’); MR appearance similar to infantile haemangioma, although may have ill-defined margins. C = AVM = F do not normally demonstrate a soft tissue mass (JMIRO Feb 2012); enlarge with size of child (present at birth, but usually manifest in childhood or later); high-flow vessels; but usually no definable mass; US Clin 2009: characterized by multiple feeding arteries with increased diastolic flow and high-velocity venous return. Typically high-flow, low resistance. D = lymphatic malformation = F = low-flow malformation; cystic spaces at US; grow with child E = venous malformation = F low-flow, do enhance post Gd

Answer 15

d. AP and lateral view of the axial skeleton and AP views of each extremity ?T controversial & location-specific, this is probably the best answer based on Australian practices (WA Imaging Guidelines, Melbourne RDx review)Imaging approach – suspected NAI **LJS - this is closes to what we do in Chch +/- orthogonal views of any suspected fracture • When clinical or imaging findings are suspicious for potential abuse a radiographic skeletal survey is undertaken: (R-ITI + WA Imaging Pathways + Chapman Imaging04)• Skull: AP & lateral skull• Chest: AP +/- oblique rib views – R-ITI says frontal + obliques• Abdomen: AP with AP pelvis & hips• Spine: Lateral +/- AP entire spine (C/T/L) – R-ITI says lateral only• Long bones: AP +/- lateral of both upper & lower limbs – R-ITI says AP only• Hands & feet: DP • Additional: lateral view of any fracture; coned metaphyseal views• Purpose of skeletal survey is to document the presence of findings of abuse for legal reasons so that the child can be removed from exposure to the abuser• Further imaging:o Repeat skeletal survey in 2 weeks (to look for healing injuries not seen on the initial skeletal survey)o NM bone scan (> 2 years of age)o CT/MRI braino Abdominal CT • Ophthalmologic examination is also neededACR–SPR PRACTICE GUIDELINE FOR SKELETAL SURVEYS IN CHILDRENCOMPLETE SKELETAL SURVEY TABLEAPPENDICULAR SKELETON Humeri (AP) Forearms (AP) Hands (PA) Femurs (AP) Lower legs (AP) Feet (AP) AXIAL SKELETONThorax (AP, lateral, right and left obliques), to include ribs, thoracic and upper lumbar spine Pelvis (AP), to include the mid lumbar spine Lumbosacral spine (lateral) Cervical spine (lateral) Skull (frontal and lateral) *ESG - Royal Children's Hospital protocol: AP Chest (for ribs) AP Abdomen including Pelvis AP Femurs AP Tibias and Fibulae AP Feet AP Skull AP Humerii AP Forearms PA Hands Lateral Spine – all regions – cervical, thoracic and lumbarsacral Lateral Sternum Lateral Skull

Answer 16

c. Occur on the posterior cortex of the ?? if posteromedial aspect of distal femoral metaphysis then would be T • Which of the following statements regarding cortical desmoids is MOST correct? a. Occur only on the posterior medial epicondyle of the femur F does NOT occur only at the distal femur, can occur at other sites b. Occur only on the posterior lateral epicondyle of the femur F c. Occur on the posterior cortex of the ?? if posteromedial aspect of distal femoral metaphysis then would be T d. They do not exhibit periosteal new bone F e. Biopsy is the only certain method of diagnosis F do not touch lesion. Distal femoral metaphyseal irregularity (“cortical desmoid”) • MSK Req p243 • Occurs in 11% of boys aged 10-15 years • Related to chronic avulsion at the insertion of the adductor magnus muscle or origin of the medial head of gastrocnemius muscle • May or may not be painfu • XR: o Cortical irregularity along the posteromedial cortex of the distal femoral metaphysis o Best seen on the lateral view o May be associated lucency on AP view o Lamellated periosteal reaction may be present o Can be confused with aggressive tumours – if unsure, perform views of the contralateral knee, because the process is often bilateral (in 25-100%)• CT/MRI: o Characteristic scoop-like defect with an irregular but intact cortex o No associated soft tissue mass o Contralateral lesion • NM: o Can have ↑ uptake on bone scan • A skeletal “don’t touch” lesion (i.e. biopsy not indicated)

Answer 17

c. Valgus refers to distal foot angulation medially F = valgus means lateral angulation • Pediatric foot, which of the following statements is incorrect? a. ‘Talipes’ refers to congenital foot deformity T b. ‘Pes’ refers to acquired foot deformity ??T (pes = foot) c. Valgus refers to distal foot angulation medially F = valgus means lateral angulation d. ‘Equinus’ refers to plantar flexion of the calcaneus T e. ‘Clubfoot’ refers to Talipes equinovarus T

Answer 18

c. The mass often has a number of large air-filled cysts T type I lesions have cysts > 2cm • CCAM, which is the most correct? a. 5% of all congenital lung disease F 25% of all congenital lung disorders, or 95% of cystic lung disease b. The lung mass has a well defined internal bronchial tree F classically communicate with bronchial tree, however lung tissue is dysplastic c. The mass often has a number of large air-filled cysts T type I lesions have cysts > 2cm d. It most commonly appears as a homogeneous lung mass F variable – 10% are microcystic & can appear solid e. CCAM receives its blood supply from the systemic circulation F normal pulmonary vascular supply & drainage1 - > 2cm . 70%2 - < 2 cm. 15-20%3 - < 5mm. 10%4 - unlined cyst0 - lethal. global arrest of lung development

Answer 19

e. Periventricular watershed regions affected in preterm (24-36 week) neonate HIE pattern: Preterm - mild/mod: periventricular - severe : thalamus, brainstem, cerebellum Term - mild/mod: watershed zones - severe : thalamus, brainstem, putamen(posterior), sensorimotor cortex, corticospinal tract, SCS: before 28 weeks hydrancephaly and porencephaly.

Answer 20

c. Mycoplasma pneumonia T Mycoplasma pneumonia • Causes 30% of pneumonias in school-aged children • Radiographic findings: variable & can resemble viral LRTI o Reticulonodular opacification, esp. if confined to a lobeo Hazy or ground-glass consolidation (uncommon to have dense consolidation)o Atelectasis • Extra-respiratory manifestations: myocarditis, ADEM & cerebral arteriovenous occlusion

Answer 21

e. Medulloblastoma • Which of the following is LEAST LIKELY to be associated with increased incidence of urinary tract anomaly? (Pocket Radiologist Obstetrics, Dahnert) a. Turners syndrome (renal ectopia and horseshoe kidney) b. Single umbilical artery – 21% of single UA a/w congenital anomalies, of which GU is a common occurrence (hydronephrosis, dysplastic kidney) c. Hemihypertrophy – Wilms, MSK and others d. Inperforate anus (VACTERL) e. Medulloblastoma

Answer 22

*LW: Both D and E are in correct... Hopefully incomplete recall. e. Majority with TAPVR are not cyanosed F A form of cyanotic CHD; some patients with unobstructed TAPVR may not appear to be cyanotic. “The majority of patients with TAPVR have symptoms of cyanosis and congestion within the first year of life.” (Circulation: Cardiovascular Imaging; 2010;3:228-230) . • Congenital cardiac conditions, which is LEAST correct? a. Ebstein’s anomaly and gross cardiomegaly T (box-shaped heart) b. Earliest CXR finding in neonate with failure is pleural fluid ?F enlarged pulmonary veins c. Normal CTR is less than or equal to 0.6 under 3yo ? T (should be < 0.55) d. VSD and left-to-right shunt is greatest in 1st week of life F – worse after 1st 2 weeks of life as pulmonary vascular resistance decreases and left-to-right shunting increases e. Majority with TAPVR are not cyanosed F A form of cyanotic CHD; some patients with unobstructed TAPVR may not appear to be cyanotic. “The majority of patients with TAPVR have symptoms of cyanosis and congestion within the first year of life.” (Circulation: Cardiovascular Imaging; 2010;3:228-230).

Answer 23

⇨ A – F – lungs normal at birth CXR features of CF: • Hyperinflation (early sign) • Atelectasis (early sign): lobar, esp. RUL > segmental & subsegmental • Multiple, small, ill-defined opacities in lung periphery (small airways mucoid impaction) • Increased peribronchial markings • Bronchiectasis – more severe in upper lobes, esp. right side

Answer 24

e. From the splenium of the corpus callosum forwards - T - dorsal to ventral, caudad to cephalad, central to peripheral. Splenium bright on T1 at 4 mths, genu at 6 mths; then low on T2 by 6 mths and 8 mths respectively. [Kieran's 'rules'] 1. Regarding myelination in children, which is of the following is the most correct? (GC) Dahnert 6e p251 good summary a. Myelination causes reduced / low signal on T1 - F - bright on T1 (fatty myelin). b. The anterior internal capsule is myelinated at birth - F - at birth, there is myelination of dorsal WM tracts of brainstem, cerebellar peduncles, dorsomedial tracts of diaencephalon, posterior limb of IC, and WM in the post-central gyrus. The anterior limb and genu of CC myelinate 4 mths later that the posterior limb & splenium. [Clinical MRI 2006] c. White matter and grey matter appear the same on T2 at 24-30 months - F - adult pattern attained by 18 mths. d. Optic nerves myelinated by two months - T - assume this means 2 months after birth... The optic nerves and tracts are myelinated by one month, optic radiations at 3 months. [Osborn] e. From the splenium of the corpus callosum forwards - T - dorsal to ventral, caudad to cephalad, central to peripheral. Splenium bright on T1 at 4 mths, genu at 6 mths; then low on T2 by 6 mths and 8 mths respectively. [Kieran's 'rules'] Changed option 2 from "posterior limb" so only one true answer.

Answer 25

b. Increased lung volumes - T - aspiration of tenacious meconium causes 4. Regarding meconium aspiration, which of the following is the most correct? (GC/SK) a. Symmetrical involvement - F - often asymmetric & patchy. b. Increased lung volumes - T - aspiration of tenacious meconium causes obstruction of small airways - hyperinflation. c. Confluent opacification - F - rope-like perihilar densities, patchy consolidation and atelectasis. d. Pneumothorax rare - F - occurs in 20-40%. e. Premature infants - F - more commonly full term or postmature infants. [Pocket paeds]

Answer 26

a. Posteromedial epicondyle - T - posteromedial aspect of the medial femoral epicondyle along the medial ridge of the linea aspera at the attachment of the adductor magnus (Dahnert p61) 5. Which is the most correct regarding a cortical desmoid? (JS) a. Posteromedial epicondyle - T - posteromedial aspect of the medial femoral epicondyle along the medial ridge of the linea aspera at the attachment of the adductor magnus (Dahnert p61) b. Periosteal change in 5% - F - typically has a lamellated periosteal reaction and can simulate an aggressive lesion radiologically, has a characteristic MRI appearance. c. Painful – F? - no localising signs of symptoms, may or may not be painful (Helms) d. Increased uptake on bone scan in 70% - can have increased uptake on bone scan e. Biopsy required for diagnosis - F - don't touch lesion - biopsy should be avoided in all cases

Answer 27

a. Mycoplasma pneumonia - T - most common in 5-20yo.; dry cough, low fever, malaise; pulmonary infiltrates show significant lag time - focal reticular infiltrate (incl. atelectasis), alveolar infiltrates (patchy inhomogeneous unilateral airspace consolidation in segmental LL in 50%, bilateral in 10-40%); small pleural effusions & hilar LNs in some. 6. 10yo patient presents with 7 day history of dry cough, febrile. Chest x ray shows bilateral patchy lower lobe airspace opacification with associated subsegmental atelectasis. Most likely diagnosis? (GC) a. Mycoplasma pneumonia - T - most common in 5-20yo.; dry cough, low fever, malaise; pulmonary infiltrates show significant lag time - focal reticular infiltrate (incl. atelectasis), alveolar infiltrates (patchy inhomogeneous unilateral airspace consolidation in segmental LL in 50%, bilateral in 10-40%); small pleural effusions & hilar LNs in some. b. Pneumococcus - F - causes lobar or round pneumonia in children. More common in elderly, debilitated, alcoholics, CHF, COPD etc; usually involves one lobe only (predilection for LL, posterior segment of UL) but may have a bronchopneumonia-like pattern. c. TB - F - primary TB is most common form in infants and kids, 90% are asymptomatic; one or more areas of homogeneous dense well-defined airspace consolidation 1-7cm in middle/lower/anterior segment upper lobes. Reactivation of focus acquired in childhood (post primary TB) usually occurs in adolescence/adulthood; 85% occurs in apicoposterior UL, 10% in superior segment LL. d. Staphylococcus aureus - F - in kids causes rapidly developing lobar/multilobar consolidation, pleural effusion/empyema (90%) and pneumatocoele (40-60%). [Dahnert]

Answer 28

*LW: The bronchial tree is dysplastic, so although may connect to the main bronchial tree, I would favour it not being "normally intact". Although type 1 CPAM most common with large cysts, these are commonly associated with occasional small cysts. I would also favour this to be an incomplete recall, where option a) would likely be Macrocyst.... e. intact bronchial tree - T - intralobar mass of disorganised pulmonary tissue communicating with the bronchial tree. [Dahnert] SK – if referring to internal contents, would be false as contains dysplastic tissue. 7. CCAM most likely: (GC/SK) a. at least one microcyst - F - type I single/multiple large cyst(s) >20mm; type II multiple uniform cysts 5-12mm; type III microcystic (3-5mm cysts, appears solid). b. solid homogenous - F - type III may appear as a homogeneously hyperechoic mass on antenatal US but only accounts for 10%. Type I (50%) +type II (40%) are macrocystic with air-fluid or fluid filled cysts on CT/CXR. c. 5% congenital lung disease - F - 25% of congenital lung disorders; 95% of congenital cystic lung lesions. d. supplied by systemic vessel - F - normal vascular supply/drainage (cf. sequestration). e. intact bronchial tree - T - intralobar mass of disorganised pulmonary tissue communicating with the bronchial tree. [Dahnert] SK – if referring to internal contents, would be false as contains dysplastic tissue.

Answer 29

e. normal femoral heads - F - delayed ossification of capital femoral epiphyses predisposes hip to deformation with flattening, lateral extrusion, hinge abduction, and premature osteoarthrits. 8. Spondyloepiphyseal dysplasia. NOT a feature (TW) a. atlanto-occipital - ?F - have C1-C2 neck instability - wonder if they meant "atlanto-axial". Ovoid vertebral bodies and severe platyspondyly. Hypoplasia of odontoid process. b. kyphosis - T - progressive kyphoscoliosis (short trunk) involving thoracic and lumbar spine c. normal hands and feet - ?F - less incorrect cf e. Hands and feet are relatively normal in length, but have multiple accessory epiphyses. d. coxa vara - T - severe coxa vara and genu valgum e. normal femoral heads - F - delayed ossification of capital femoral epiphyses predisposes hip to deformation with flattening, lateral extrusion, hinge abduction, and premature osteoarthrits. AKA spondyloepiphyseal dysplasia: as name indicates: problems with spine, ends of bones, and is present from birth.

Answer 30

d. genu valgus – T? - bowed legs - genu varum. SK: usually genu varus (bow legs) but can be genu valgus (esp. in hypophosphataemic rickets – Wheeless, orthonet, emedicine) 9. Rickets NOT seen (TW) a. flared metaphyses – T (?) - axial widening of growth plate (increase in distance between end of shaft and epiphyseal center due to increased osteoid production). Cupping, splaying and fraying of metaphysis with threadlike shadows into epiphyseal cartilage. b. scoliosis - T – listed in emedicine c. teeth abnormality - T - delayed dentition d. genu valgus – T? - bowed legs - genu varum. SK: usually genu varus (bow legs) but can be genu valgus (esp. in hypophosphataemic rickets – Wheeless, orthonet, emedicine)

Answer 31

a. periventricular watershed T - vascular watershed ("border zone") shifts from periventricular in premature to parasagittal in term infant at about 36wks. Also remember that before 26wks there is no gliosis, vs after 26 wks where develop PVL (vs porencephaly).

Answer 32

e. LLL - T - intralobar sequestration LLL > RLL 60%:40%. Extralobar sequestration almost always LLL. ILS usually presents later than ELS. Patients with ILS usually present in late childhood or adolescence with recurrent pulmonary infections. ELS more apt to present in early life from resp distress / cyanosis / or assoc abnormalities. Both can be an incidental antenatal Dx too. 65% of ELS assoc with other anomalies. ILS not associated with other anomalies.

Answer 33

a. pneumomediastinum

Answer 34

a. pleural effusion is very uncommon. If present suggest TTN or pneumonia

Answer 35

B = SUFE common in this age group, causes hip/groin/knee pain.

Answer 36

pneumoperitoneum in NEC is the only radiologic absolute indication for surgery

Answer 37

A = should be repositioned, not really urgent B = F: AJR April 2003: Air might be introduced inadvertently into the intrahepatic portal venous system at the time of umbilical venous catheter insertion. The air is usually transient and should not be misinterpreted as evidence of necrotizing enterocolitis. C = should be slightly higher D = all UACs do this initially, but if it goes down the iliac artery risk of limb ischaemia UAC – do not position b/w T10-L3 – risk of thrombosis of aorta & branches – ideal is T8-T10 (high line) or below L3 (low line) UVC – should be at RA/IVC junction at level of hemidiaphragm; PV location risks hepatic haematoma or abscess One of the most dreaded complications of the UAC placement is thrombosis leading to an ischemic limb. One of the most dreaded complications of UVCs is perforation of the right atrial or left atrial wall. Rarely the UVC may perforate the portal vein and cause a hematoma within the liver.

Answer 38

a. US obstetrics - echogenic thoracic mass.

Answer 39

*LW: Associated anomalies are relatively common and include: - pulmonary hypoplasia: also a complication - bronchopulmonary sequestration - aneuploidy: can be present in up to 50% of cases trisomy 13 trisomy 18 trisomy 21 - Turner syndrome: monosomy X - Pallister-Killian syndrome: tetrasomy 12p - Fryns syndrome - Cornelia de Lange syndrome - congenital cardiac anomalies - neural tube defects - anencephaly - spina bifida - genito-urinary malformations - Gastro intestinal malformations, including malrotation. Signs suggesting a poor prognosis include: large hernia size early gestational age at diagnosis intra-thoracic liver small contralateral lung the presence of associated abnormalities bilateral CDH unfavourable lung: head ratio a. malrotation: true b. persistent fetal circulation associated with bad prognosis: true. c. renal hypoplasia: likely included within genito-urinary malformations, d. hernia of the gut associated with bad prognosis: likely incorrect answer if referring to bowel vs liver / stomach, however prognosis also affected by size, thus a large hernia containing bowel causing pulmonary hypoplasia will have bad prognosis. **LJS - agree, liver herniation ass/w bad prognosis Previous answers: D = F (herniation of liver or stomach has poorer prognosis) A = T (30-62%) B = T C = T (‘renal anomalies’) D = F (herniation of liver or stomach has poorer prognosis)

Answer 40

Chiari 2 = o Small posterior fossa, with caudal displacement of a dysplastic cerebellum & brainstem into an enlarged foramen magnum & upper cervical canal o Cerebellar tonsils, cerebellar vermis, 4th ventricle & brainstem are herniated thru the foramen magnum o Superior cerebellum towers superiorly thru a widened tentorial incisura, while the rest of the cerebellum may wrap-around the brainstem o The 4th ventricle is small, and its outlets are obstructed, resulting in hydrocephalus. The frontal horns of the lateral ventricles are squared off & the aqueduct is stretched inferiorly o The medulla is elongated & a kink may be present in the cervicomedullary junction o Torcular low o Massa intermedia is abnormally enlarged

Answer 41

B = lateral usually not needed A = best radiologic test B = lateral usually not needed C = lateral decubitus both sides + erect PA if uncooperative child (although a study questions the utility of lat decub films - Pediatr Emerg Care. 2007;23(3):154) → on its own, it may be the least useful D = fluoroscopy useful for uncooperative child E = gold standard

Answer 42

b. xray hip and thighlook for sufe

Answer 43

C - breech -> no convincing evidence Risk factor - diabetes - T21 - hirshprung - umbilical catherisation - probably persistent fetal circulation ( cardiorespiratory difficulties) NEC most common in • Premature (the more premature the higher the risk) • Occcurs at 1-3 weeks (although Paed Req says 3-5 days & Dahnert says 2-3 days) • Enteral feeding increases risk **LJS - variable reports in papers regarding maternal DM as a risk factor for NEC. Nothing convincing for breech - this is least correct

Answer 44

probably C - 38 weeks NEC most common in • Premature (the more premature the higher the risk) • Occcurs at 1-3 weeks (although Paed Req says 3-5 days & Dahnert says 2-3 days) • Enteral feeding increases risk

Answer 45

D = most likely of all these A: born 37 weeks, now 7 days old = unlikely at GA 37/40 (usually need to be < 37/40) B: born 36 weeks, 2 das old but no oral feeds yet = unlikely at 2 days, unlikely at GA 36/40 (StatDx says 1-3 weeks, while Dahnert says 2-3 days, Paed Req says 3-5 days) C = 36 weeks with umbilical catheter, umbilical catheter is risk factor D 28 weeks, now 9 days old = most likely of all these E 34 weeks but no PR blood = possible, PR bleeding is a possible sign of NEC *LW: NEC usually develops 2-3 days following birth, with 90% developing within the first 10 days of life 4. The incidence is inversely proportional to gestational age, with 90% occurring in premature infants. Enteral feeding is a major risk factor and likely a key step in the pathogenesis, so option B - 36 weeks with no oral feeds, makes NEC less likely. UTD: The timing of the onset of symptoms varies and appears to be inversely related to gestational age [7,14]. Overall, 25 percent of cases present later than 30 days after birth. The median age at onset in infants with a gestational age of less than 26 weeks was 23 days, and for those with a gestational age of greater than 31 weeks, the median age at onset was 11 days. 13 percent of cases occur in term infants.

Answer 46

A = enema contraindicated in presumed acute NEC (StatDx) – Dahnert says Ba enema contraindicated, but “may be used judiciously in selected cases with radiologic & clinical doubt”.

Answer 47

D = ?? MS (mitral stenosis or multiple sclerosis), HPT uncommon in kids A = rhabdo is most common primary malignant orbital tumour in childhood; average age 7 years; 90% occur < 16 yrs B = do occur in vulva/vagina in infants, botyroid (grape-like) C = yes D = ?? MS (mitral stenosis or multiple sclerosis), HPT uncommon in kids

Answer 48

False answer = 7 days old (appears at end of 2nd week of life), cannot be diagnosed until 28 days **LJS - Consensus definition: chronic disease of premature infants born at < 32 weeks with oxygen dependency for > 28 days New BPD (chronic lung disease) Lung appearance may be nearly normal early with progressive diffuse hazy opacification Severe surfactant deficiency disease patients may follow old imaging patterns Similar chronic chest CT findings of old & new BPD Peripheral abnormalities may predominate Cystic/emphysematous change Subpleural cysts & triangular opacities Linear, reticular opacities + parenchymal bands Foci of air-trapping on expiratory images

Answer 49

D = F = extremely rare in premature infant A = True (80% short segment, 15% long segment, 5% total colon) B = False (narrow, small, spasmodic aganglionic segment) (StatDx/Donnelly) – others (like Dahnert) say the aganglionic segment is normal in calibre! UTD says can be normal calibre or narrowed. C = associated with Down syndrome D = F = extremely rare in premature infant E = can present with intermittent constipation & paradoxical diarrhoea (25%), typical is failure to pass meconium; Dahnert. Wji: disagree re b. This is also true. Aganglionic segment is abnormal. ADB: original hard copy had "Aganglionic" , which can also be "grossly normal or contracted"- robins

Answer 50

probably ileal atresia

Answer 51

Down syndrome • 11 ribs (25%) • Flared iliac wings + horizontal acetabular roof • Hypersegmentation of manubrium sterni • Persistent metopic suture (cannot find evidence of early mendosal suture closure – it normally closes at 7 days) • Atlantoaxial subluxation essentially the question is weird

Answer 52

ANS = B = hypothyroidism (features = delayed bone maturation, absent/small epiphyses, SUFE)

Answer 53

C: follow-up US also needed if managed conservatively & to exclude neoplasm.

Answer 54

A = can occur in sinuses; peak incidence in H&N is 2-5 years B = can get fevers, proptosis (orbital mets) & cerebellar ataxia C? = invasive fungal sinusitis typically occurs in adults, but can affect immunocompromised kids.

Answer 55

A = T Optic nerves & tracts myelinated by 1 month. Optic radiations myelinated by 3 months (Osborn). The optic tracts and radiations begin to myelinate early, usually appearing anteriorly within the first 2 to 3 months and extending to involve the calcarine cortex by 4 to 6 months. (Peds Req).

Answer 56

B = F = uncommon A = T (43% in LUL) B = F = uncommon C = T D = T E = T (on initial radiographs, may appear opaque due to retained fetal lung fluid – this resolves and is replaced by progressive hyperlucency)

Answer 57

A = F intralobar drains to pulmonary veins, extralobar has systemic venous drainage B = T

Answer 58

c. PUJ is bilateral in 50% - F – 30% bilateral in black book; 10-40% in UTD. 12. In regards to fetal PUJ obstruction which is false? (TW) a. Bilateral hydroureter leads to a bad outcome – T - not sure how this realtes to PUJ. If bilateral hydroureter - indicates a distal obstruction (eg PUVs), abnormal musculature / development (Prune Belly, congenital megaureter), reflux. **LJS - lower urinary obstruction ass/w 45% mortality if untreated https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3991456/ b. Bilateral agenesis results in oligohydraminos – T – kidneys contribute 800-1200ml/d production near term. c. PUJ is bilateral in 50% - F – 30% bilateral in black book; 10-40% in UTD. d. Hydrops leads to a bad outcome – T – hydrops = excess of total body fluid (2 or more abnormal fetal fluid collections: ascites; pleural effusion; pericardial effusion; skin oedema; polyhydramnios). Immune vs non-immune. 20% cases idiopathic. 40% also have detectable structural anomaly. Prognosis is poor if not treated. 75% survival for immune hydrops if treated with blood transfusion. Near 100% fatal if hydrops + fetal anomaly (excluding tachyarrhythmia).. Incidence of fetal hydronephrosis caused by UPJ obstruction detected in routine antenatal US is nearly 1 in 500.

Answer 59

d. Dislocations associated with fractures - F 13. Which is not typical in NAI? (TW) a. Metaphyseal corner fractures – T – “corner” fracture = “bucket-handle” fracture. Avulsion of an arcuate metaphyseal fragment overlying the lucent epiphyseal cartilage. Fracture extends through the primary spongiosa of the metphysis, the weakest portion. Mechanism is a shearing injury, e.g. holding & shaking the child. b. Diaphyseal fractures – T – spiral long bone fractures in non-ambulatory children are highly suspicious. c. Posterior rib fractures – T – occur near costovertebral joints. Highly specific. Thought to occur from an adult squeezing the infant’s thorax. d. Dislocations associated with fractures - F e. Subdural collections of varying ages – T – acute or chronic subdural haematomas. Infant/young child with multiple hemorrhages, different ages is good diagnostic sign.

Answer 60

b. 60% with calcification will benign.- ?? T - Approx 50% of benign teratomas contain calcification, whereas this feature is seldom observed in malignant tumors (Radiographics 06). 14. In regards to Sacrococcygeal teratomas? (TW) a. MRI is essential for diagnosis - ?? F - can be Dx with US. RG 2006 MR is “the preferred modality for both initial diagnosis & surveillance…”. UTD does not mention US outside of pre-natal screening. b. 60% with calcification will benign.- ?? T - Approx 50% of benign teratomas contain calcification, whereas this feature is seldom observed in malignant tumors (Radiographics 06). c. Anterior sacral meningocele is common - F – not sure how this applies to SCT. Anterior sacral meningocele = congenital abnormality that arises from herniation of the CSF-filled dura mater through a sacral foramen or a defect in sacral bone. ASM is rare; occurs 1 : 40,000. d. Follow post resection is with serial Beta HCG levels - ?F - all patients with SGT should have an initial assay of serum tumor markers, and be monitored with serial tumor markers following resection of apparently benign teratomas. Dahnert says SCT has elevated AFP with mixed malignant teratoma and endodermal sinus tumor. UpToDate says both AFP and BhCG. RG 2006 article only mentions AFP. Sacrococcygeal teratomas are the most common GCT of childhood, accounting for 40% of all GCTs and up to 78% of all extragonadal GCTs. Thought to arise from the primitive knowt or Hensen's node (aggregation of totipotential cells that are the primary organizers of embryonic development).

Answer 61

a. Narrow ribs – F – ribbon ribs with NF. Morquio has bulbous costochondral junctions. MPS – ribs and clavicles are thickened. Ribs are narrower posteromedially, giving then a ‘canoe paddle’ appearance. *LW: confusing wording, as MPS have canoe paddle ribs which are narrow posteromedially, although this is likely relative to thickened anterior ribs....so out of listed options, this is likely the least correct and favoured answer. 15. Features of mucopolysacharisoses on imaging include? (TW) a. Narrow ribs – F – ribbon ribs with NF. Morquio has bulbous costochondral junctions. MPS – ribs and clavicles are thickened. Ribs are narrower posteromedially, giving then a ‘canoe paddle’ appearance. b. Cardiomegaly - ?T (SK) – cardiomyopathy & valvular disease in Hurler syndrome.*LW cardiomegaly can be seen in both Hurler and Hunter syndromes, so is correct. c. J-shaped sella – T – undermining of anterior clinoid process. *LW agrees d. Cysts in the corpus callosum on MRI – T dilated VRS’s by accumulated GAG in corpus callosum & periventricular WM. *LW agrees.

Answer 62

a. Enlarged acetabular angles – F - Decreased acetabular angle secondary to flattened acetabular roof 16. Regarding Downs syndrome which is false? (TW) a. Enlarged acetabular angles – F - Decreased acetabular angle secondary to flattened acetabular roof b. Clinodactyly - T - DS have short tubular bones and a hypoplastic and triangular middle and distal phalanges of 5th finger. 50%; widened space between first 2 digits of hands and feet (sandle gap) c. Short 4th and 5th metacarpal - ? T. see above. DDx, Turners, pseudohypoparathyroidism, McCune Albright. d. Cerebellar hypoplasia – T – Dahnert: “small cerebellum”. Results of pathologic, CT, and MR imaging studies of infants, children, and adults with T21 have shown these patients to have a smaller cerebellum.

Answer 63

c. Intralobar form drains to pulmonary veins - T – ILS drains to pulmonary veins. ELS receives arterial supply from aberrant vessel arising from thoracic aorta, and has anomalous venous drainage to RA, vena cava, or azygous. 17. Regarding Pulmonary sequestration which is true? (TW) a. The intralobar form most frequently presents in neonate - F - ILS usually presents later than ELS. Patients with ILS usually present in late childhood or adolescence with recurrent pulmonary infections. ELS more apt to present in early life from resp distress / cyanosis / or assoc abnormalities. Both can be an incidental antenatal Dx too. b. Intralobar have arterial supply from pulmonary artery - F - systemic arterial supply, usually lower thoracic or upper abdo aorta. Single or multiple arterial trunks. Venous drainage usual normal to pulmonary veins. c. Intralobar form drains to pulmonary veins - T – ILS drains to pulmonary veins. ELS receives arterial supply from aberrant vessel arising from thoracic aorta, and has anomalous venous drainage to RA, vena cava, or azygous. d. Extralobar occur most commonly on the right - F - ELS is almost always LLL (ILS LLL > RLL 60%:40%). e. Extralobar characterized by presence of air bronchograms - F - lacks a bronchial connection to normal proximal airway. May connect to GIT or rarely, to intrpulmonary structures. Infectious complications are uncommon in ELS. ILS 70% : ELS 30%. 65% of ELS assoc with other anomalies. ILS not assoc. with other anomalies.

Answer 64

c. The ureter is atretic – T - Dahnet suggests that ureteric obstruction/atresia is the underlying cause. UTD: malformation is an abnormality of the ureteral bud leading to atresia or absence of the ureter. StatDx: “Probably due to atresia of ureter or ureteropelvic junction during the metanephric stage of intrauterine development “ 18. In multicystic dysplastic kidneys, which is true? (TW) a. There is increased risk of malignancy – F? – low risk of malignancy. Life time risk estimates 1:2000 – 1:3000 based on available evidence. Risk of dying from an associated Wilm’s tumour is about 1:20,000 [Weiss, Comprehensive Urology] Radiopedia-->Controversy exists over the need for prophylactic surgical excision of an MCDK, justified on the grounds of a small risk of malignant transformation, more commonly in adults with a persistent MCDK (i.e. has not spontaneously resolved) b. In the pelvoinfundibular form , the cysts communicate – F – pelvoinfundibular form is more common type (cf hydronephrotic type). Atresia of ureter or renal pelvis and cysts are remnants of dilated calyces. c. The ureter is atretic – T - Dahnet suggests that ureteric obstruction/atresia is the underlying cause. UTD: malformation is an abnormality of the ureteral bud leading to atresia or absence of the ureter. StatDx: “Probably due to atresia of ureter or ureteropelvic junction during the metanephric stage of intrauterine development “ d. Bilateral involvement is common – F – Up to 40% of patients with MCDK have contralateral abnormality (UPJ obstruction and VUR most commonly). Most cases of MCDK are unilateral (UTD): incidence 1 in 3000, with bilateral incidence 1:10000. e. Associated with hepatic cysts – F - ADPKD

Answer 65

c. Toddlers fracture is typically metaphyseal in location – F – spiral or non-displaced oblique fracture of the mid to distal shaft of the tibia. Occurs when a child begins to walk. Stresses of new activity (walking, jumping) applied to bone previously adapted for that activity. 19. In regards to paediatric imaging, which is least correct? (TW/SK) a. Osteiod osteoma is detected in 100% of bone scans - T - nearly 100% of osteoid osteomas show radioisotope tracer uptake on pyrophosphate bone scan. Sensitivity of bone scan approaches 100%, but lacks specificity. b. SPECT improves the detection of Discitis and TB osteomyelitis on bone scan – T – discitis is the most common pediatric spine problem. SPECT increases the sensitivity of scintigraphy for the Dx of discitis and vertebral osteomyelitis. c. Toddlers fracture is typically metaphyseal in location – F – spiral or non-displaced oblique fracture of the mid to distal shaft of the tibia. Occurs when a child begins to walk. Stresses of new activity (walking, jumping) applied to bone previously adapted for that activity. d. Chondroblastoma is the commonest primary epiphyseal lesion – T – most common primary epiphyseal tumor in children is chondroblastoma (Wheeless’ Ortho). “chicken wire” calcification = pericellular deposition of calcification on histology – virtually pathognomonic. Location of Toddler fractures (StatDx) • Tibiao Toddler's fracture, type 1: Twisting of foot on leg → spiral fracture, usually in distal thirdo Toddler's fracture, type 2: Knee hyperextension → upper tibial metadiaphysis distraction fracture of posterior cortex and compression of anterior cortexo Type 2 may be due to child abuse: Consider possibility if seen in non-ambulatory childo Buckle fracture: Distal metaphysis • Fibulao Distal metadiaphysis • Calcaneuso Near apophysis (vertical) or along base (horizontal) • Taluso Neck and body • Cuboido Near calcaneus head • Metatarsalso Shafts and bases

Answer 66

c. Vertical stripes in bones is suggestive previous Rubella infection – T - “celery stalk” sign – metaphyseal irregular margins and coarsened trabeculae extending longitudinally from epiphysis. (Dahnert) 20. With regards to paediatric bone lesions? (TW) a. Brodies abscess is diaphyseal location – F - Typically occurs in the ends of tubular bones, metaphysis but can be in epiphysis in children and infants. (Dahnert) b. TB typically affects more than one joint – F – joint involvement usually secondary to adjacent osteomyelitis / hematogenous dissemination. Hip, knee >> elbow, wrist, SIJ, glenohumeral, articulart of hand and foot. Monoarticular involvement is typical. (Dahnert) c. Vertical stripes in bones is suggestive previous Rubella infection – T - “celery stalk” sign – metaphyseal irregular margins and coarsened trabeculae extending longitudinally from epiphysis. (Dahnert) d. Human bite wounds tend to be infected more commonly by staphylococcus than streptococcus – F – Strep 84%, Staph 54% Prevotella 36%, Fusobacterium 34%. Talan et al 2003.

Answer 67

**LJS - ?all correct. Depending on wording of triple bubble thing a. Jejunal atresia if 3 gas bubbles on AXR – F – has been reported as a finding (although not specific) on AXR and antenatal US, for proximal jejunal atresia. “triple bubble sign” = intraluminal gas in stomach + duodenal bulb + proximal jejunum. 21. With regards to Duodenal atresia, which is least correct? (TW) a. Jejunal atresia if 3 gas bubbles on AXR – F – has been reported as a finding (although not specific) on AXR and antenatal US, for proximal jejunal atresia. “triple bubble sign” = intraluminal gas in stomach + duodenal bulb + proximal jejunum. b. Duodenal atresia commonest form of neonatal duodenum obstruction – T – DA/DS most common cause of high intestinal obstruction in newborns. 30% of duodenal atresia occur in children with down syndrome. 3-5% of DS have DA. (Donnelly) c. Occurs in 5% of down syndrome babies – T – UTD says 5% of DS have associated GI abnormalities, and 2.5% have DA. Dahnert says <5%. Other texts say 3-5% / 1-5%. d. Typically presents with bile stained vomiting – T – persistent bilious vomiting a few hours after birth / following 1st feeding (75%). Dahnert.

Answer 68

A = T = periosteal reaction seen in diaphyses of humerus, radius & femur in 50% of infants < 6 mo A = T = periosteal reaction seen in diaphyses of humerus, radius & femur in 50% of infants < 6 mo B = F = most favour this represents chronic avulsive stress injury (some say ischaemic) – StatDx calls it a traction apophysitis caused by repetitive microtrauma C = F = lies medial to navicula D = F = broad differential for this

Answer 69

Chiari 1 is not associated with callosal agenesis **SCS: is associated with Chiari II however…

Answer 70

b. Viral gastroeneteritis is a recognized etiological factor - T - most cases in children are thought to be related to lymphoid hypertrophy in the terminal ileum secondary to viral disease (Donnelly) 22. Which is true re: intussusception (JS) a. Plain film is rarely abnormal – F - "Radiographs are rarely completely normal" (Donnelly). Findings include paucity of gas in right abdomen, nonvisualisation of caecum, soft tissue mass, SBO b. Viral gastroeneteritis is a recognized etiological factor - T - most cases in children are thought to be related to lymphoid hypertrophy in the terminal ileum secondary to viral disease (Donnelly) c. Small bowel obstruction is a contraindication to radiological reduction - F - SBO results from intussusception. Contraindications include peritonitis or pneumoperitoneum. Also need adequate fluid resuscitation prior to attempting reduction. SBO is a/w decr success rate. d. Onset >12 hours is a contraindication to radiological reduction - F - Rate of failed reduction is greater if there has been prolonged history (>24h) but this is not a contraindication. <1% mortality if performed <24 hrs. e. Idiopathic in almost half of cases – F - idiopathic in ≈ 90%

Answer 71

a. Echogenic mass with cysts and calcification is compatible with hepatoblastoma – T – 3rd most common abdominal tumor in children. Coarse calcifications / osseous matrix in 12-39%. Location: right lobe liver. Heterogeneous echogenic mass, often with calcifications, occasionally cystic areas (necrosis / extramedullary hematopoiesis). AFP elevated.Typically a solid, heterogenous mass.

Answer 72

a. Echogenic mass with cysts and calcification is compatible with hepatoblastoma – T – 3rd most common abdominal tumor in children. Coarse calcifications / osseous matrix in 12-39%. Location: right lobe liver. Heterogeneous echogenic mass, often with calcifications, occasionally cystic areas (necrosis / extramedullary hematopoiesis). AFP elevated.Typically a solid, heterogenous mass. - Mean age of diagnosis 19 month- normally < 4 yo

Answer 73

**LJS - I think seen earlier in USS, as per other MCQ answers a. US can detect ca2+ earlier than CT – ?F – article 2007 clinical radiology: “US and CT demonstrated equal sensitivity in the detection of intracranial calcification in infants with congenital toxoplasmosis”. Another article says CT has higher sensitivity and specificity cf ultrasound. However, US resolution has increased significantly past years.

Answer 74

d. 15% malignant – T – UTD: dissemination of tumor through the CSF is observed in less than 10% of pts at Dx when ependymoblastomas are excluded. Web: about 85% of Ependymomas are benign myxopapillary Ependymoma. 3-17% CSF dissemination (StatDx),. **SCS: i favour haemorrhage is common. “Radiopaedia : Ependymomas are typically heterogeneous masses with areas of necrosis, calcification, cystic change and haemorrhage frequently seen” - I thought Ependymoma is a malignant tumour… 25. Ependymoma in the pediatric patient (TW) a. most commonly supratentorial location - F - Infratentorial (floor of 4th ventricle) 70% of all intracranial ependymomas. Can occur supratentorial: frontal > parietal > temporoparietal > juxtaventricular. Conus (40-65% of all spinal intramedullary gliomas. In children (intracranial compartment) 70% are infratentorial, 30% supratentorial. Infratentorial Ependymomas most common in kids <3yo. b. homogenous enhancement – F – CT: variable heterogeneous enhancement. MR: moderate, heterogeneous enhancement (Blackbook Neuro). c. hemorrhage is common – F – intratumoral hemorrhage 10% (Dahnert) d. 15% malignant – T – UTD: dissemination of tumor through the CSF is observed in less than 10% of pts at Dx when ependymoblastomas are excluded. Web: about 85% of Ependymomas are benign myxopapillary Ependymoma. 3-17% CSF dissemination (StatDx),. e. arise from lumbar nerve roots in the spine - F - originates from ependymal cells lining the central canal. (--) Most intraspinal ependymomas arise de novo. Although intramedullary and cauda equina tumors may arise from an intramedullary source, these origins are uncommon. Intraspinal ependymomas are believed to arise from the ependymal cells lining the central canal, from the ventriculus terminalis of the conus, from within the filum terminale, or from cerebrospinal fluid (CSF) dissemination (Moser, 1992).

Answer 75

c. Lateral displacement of lateral masses C1/2 by up to 6mm is a normal finding in a 4 year old - T - Total offset (sum of both sides) of more than 6mm of the lateral masses of the atlas with respect to the odontoid is highly suggestive of rupture of the transverse ligament or avulsion of its attachments. (see below).RG = “up to 6mm of displacement of the lateral masses relative to the dens is common in patients ≤ 4 yrs old & may be seen in patients up to 7 yrs” 26. Paediatric C spine which is true: (TW) a. Atlanto axial subluxation is less common than in adults – F - 5x more common in children than adults b. Anterior wedge of C3 is not a normal variant - F - normal variant, however age dependent: eg, anterior wedging in a 3yo at C5 is likely normal variant. However wedged body at C5 in an 8yo is likely pathologically compressed. RG = “physiologic anterior wedging of vertebral bodies” c. Lateral displacement of lateral masses C1/2 by up to 6mm is a normal finding in a 4 year old - T - Total offset (sum of both sides) of more than 6mm of the lateral masses of the atlas with respect to the odontoid is highly suggestive of rupture of the transverse ligament or avulsion of its attachments. (see below).RG = “up to 6mm of displacement of the lateral masses relative to the dens is common in patients ≤ 4 yrs old & may be seen in patients up to 7 yrs” d. Injuries are more commonly lower cervical than upper – F - in patients under 9yo, almost all injures are to the occiput-C2 region (Imaging of Spinal Trauma in Children, Kuhns). RG = usually occur in upper C spine from occiput to C3.Above ans from: Differences between adult and paeds spinal injury (Spinal Trauma in Children, Paeds Radiol 2001) Re: option 3. "Imaging in Pediatric Skeletal Trauma" K Johnson - "The lateral masses of C1 and C2 may be offset bilaterally in young children so that the lateral masses of C1 overhang those of C2 on the AP view, simulating a Jefferson burst fracture. This phenomenon is thought to be secondary to disparity in growth rate between the two vertebra and is most commonly seen at around 4y of age, but often up to 7y of age. Up to 6mm lateral displacement of the lateral masses of C1 relative to the odontoid is within normal limits under these circumstances. Physiological subluxation of C2/3 seen In 25 % children < 8 years. C3/4 15% note less than 3 mm, posterior line retains alignment.

Answer 76

e. Periosteal reaction is seen as a physiological phenomenon - T - physiological periosteal new bone formation can commonly be seen in infants during the first few months of life. Supportive features of physiologic aetiology are: symmetric distribution, benign appearance of the periosteal reaction, appropriate age 27. With regard to pediatric skeletal radiographs, which is true: (TW) a. A spiral tibial fracture in a 6m infant is unlikely to be due to NAI - F - not weight bearing yet, unlike an older infant/child where a Toddlers fracture would be most likley. b. SH1 type fractures have a high risk of long term complications of growth -F c. ABC's are the commonest cause of pathological fractures - F - SBC 40%, NOF 19%, fibrous dysplasia 16%, osteosarcoma 15%, ABC 10%. Oritz et al 2005. d. Radiographically occult fractures are less common than in adults - F e. Periosteal reaction is seen as a physiological phenomenon - T - physiological periosteal new bone formation can commonly be seen in infants during the first few months of life. Supportive features of physiologic aetiology are: symmetric distribution, benign appearance of the periosteal reaction, appropriate age

Answer 77

d. Small bowel atresia – T – although ileal atresia represents 50% of small-intestinal atresias, these do not present for a couple days following birth. e. Meconium ileus – F - assoc with CF. Virtually all infants with MI prove to have28. Most common cause of SBO at birth: (TW, GC, JS) a. Duodenal stenosis – F – duodenal atresia 50%, stenosis 40%, web 10%. most common cause of high intestinal obstruction in newborns. 30% of duodenal atresia occur in children with down syndrome. 3-5% of DS have DA. (Donnelly), but not as common as atresia of ileum (incidence of jejunal and ileal atresia 1 in 1500 to 5000; incidence of DA is i in 20000 to 40000). Intestinal obstruction typically proesents with abdominal distension except in the most proximal lesions. Vomiting usually begins in the 1st 24h to 48h after birth. Presentation may be delayed for days or weeks ininfants with partial obstruction due to stenosis (UTD). b. Malrotation – F - most cases occur during 1st month of life c. Choledochal cyst - F d. Small bowel atresia – T – although ileal atresia represents 50% of small-intestinal atresias, these do not present for a couple days following birth. e. Meconium ileus – F - assoc with CF. Virtually all infants with MI prove to have CF. Failure to pass meconium within 48h. Approximately 95% of intestinal obstructions diagnosed in the first 2 weeks of life are due to atresia and/or stenosis of small intestine (Grosfeld, 1970). Ileal atresia represents 50% of small-intestinal atresias. Meconium Ileus is among of the most common causes of intestinal obstruction in newborns, accounting for 9-33% of neonatal intestinal obstructions. Malrotation frequency is unknown since many asymptomatic patients may never present, although the disease is estimated to occur in 1 in 500 live births. **LJS - technically SB atresia includes duodenal atresia, which is more common than stenosis

Answer 78

b. Neonatal NEC and maternal diabetes - T (SK) - NEC is seen with prematurity, Hirschsprung disease and bowel obstruction. Maternal diabetes is associated with fetal macrosomia, IUFD, placental insufficiency, polyhydramnios and prematurity.Emedicine “Maternal risk factors that reduce fetal gut blood flow, such as placental insufficiency from acute disease (eg, pregnancy-induced hypertension), chronic disease (eg, diabetes), or maternal cocaine abuse, can increase the baby's risk for developing NEC.” SG – Not so much *LW: agree this is least correct, and although agreeing with above indirect pathophysiology, maternal diabetes is not listed as a common risk factor / association, so would favour this to be the correct option. 29. Which is not an association: (JS) a. Caroli’s and medullary sponge kidney - T - Caroli disease is associated with benign renal tubular ectasia, medullary sponge kidney (80%), infantile polycystic kidney disease, choledochal cyst and congenital hepatic fibrosis. Medullary sponge kidney is associated with Ehlers-Danlos syndrome, parathyroid adenoma and Caroli disease. (Dahnert) b. Neonatal NEC and maternal diabetes - T (SK) - NEC is seen with prematurity, Hirschsprung disease and bowel obstruction. Maternal diabetes is associated with fetal macrosomia, IUFD, placental insufficiency, polyhydramnios and prematurity.Emedicine “Maternal risk factors that reduce fetal gut blood flow, such as placental insufficiency from acute disease (eg, pregnancy-induced hypertension), chronic disease (eg, diabetes), or maternal cocaine abuse, can increase the baby's risk for developing NEC.” SG – Not so much c. Supravalvular aortic stenosis and hypercalcaemia – T – Rare. *LW: Supravalvular AS is associated with hypercalcaemia of infancy (Williams syndrome). Williams syndrome = supravalvular stenosis, peripheral pulmonary artery stenosis, mental retardation, and hypercalcaemia. Mutations in elastin gene result in supra vlavular aortic stenosis (Robbins). d. Typhilitis and haematological malignancy - T - typhilitis is seen in leukaemia, aplastic anaemia, lymphoma, immunosuppressive therapy, neutropenia, MDS and AIDS e. Desmoid tumour and familial polyposis coli – T - Gardners syndrome (a form of familial adenomatous polyposis) = colonic polyposis, osteomas and soft-tissue tumours (including desmoid tumours)

Answer 79

30. Juvenile Angiofibroma, True/False: (GC) a. Displaces bone rather than invades – T – Widens the PPF with anterior bowing of the posterior antral wall. Tend to deossify the adjacent skull base; in these instances the imaging may suggest erosion.StatDx Paed: bone remodeling +/- destruction b. Arises from posterior choanal tissue – T - almost all originate from the posterior choanal tissue. Characteristic origin at sphenopalatine foramen. c. It is predominantly found in females - F - almost exclusively in males, typically 10-18yo. d. There is spontaneous regression by puberty F - treatment of choice is surgery; usually with preoperative embolisation. Unresectable intracranial disease can be irradiated.StatDx H&N: may rarely spontanteously regress. e. There is uniform enhancement on post contrast CT T - but only enhances immediately after contrast bolus (highly vascular). Biospy is contraindicated.

Answer 80

31. TOF, False (TW) a. Oligohydramnios is a recognised association – F – polyhydramnios (but does not develop till after 20wks). In cases with esophageal atresia component (95%), polyhydramnios occurs in approximately 2/3rd of pregnancies. b. AXR commonly shows distended bowel loops – T – if oesophageal atresia and proximal tracheoesophageal fistula: gassless abdomen. If OA and distal TO fistula: abdomen distended by bowel gas in 90%. Dahnert.

Answer 81

B = F – no cingulate gyrus, with radially oriented fissures into high-riding 3rd ventricle (AC= Arnold Chiari)

Answer 82

*LW: Radiopedia states "dilated posterior urethra without urethral obstruction", StatDx states "Dilated posterior urethra; true mechanical obstruction uncommon" thus making option D (Stricture of posterior urethra) false. And would favour A being more correct than D, even tho you can argue defect vs deficiency. A = ?F– anterior abdo wall muscle ‘deficiency’, rather than ‘defect’ ?B = T – bilateral cryptorchidism C = T – pulmonary hypoplasia (lung affected in 55%) D = T = urethral obstruction in 20% E = VUR in > 70% **LJS - dilated posterior urethra but no mechanical obstruction. D most incorrect

Answer 83

a. Insulinomas are usually <1.5cm at diagnosis – T? – Radiographics AFIP 2006 article: Most patients with insulinoma have striking clinical symptoms and present with relatively small tumors, with mean diameter in range 2-2.2cm. Dahnert says < 1.5 cm in 70%, and avg size 1-2cm. 32. Regarding paediatric neuroendocrine tumours, which is most correct: (TW) a. Insulinomas are usually <1.5cm at diagnosis – T? – Radiographics AFIP 2006 article: Most patients with insulinoma have striking clinical symptoms and present with relatively small tumors, with mean diameter in range 2-2.2cm. Dahnert says < 1.5 cm in 70%, and avg size 1-2cm. b. Somatostatinomas are associated with NHDA syndrome – F -? meant to be WHDA (watery diarrhoea + hypokalemia + achlorhydria / AKA Verner-Morrison syndrome) which is also F because associated with VIPomas.Clinical features of somatostatinomas = diabetes, cholelithiasis, steatorrhoea, achlorhydria. c. Insulinomas do not enhance – F – insulinomas and gastrinomas enhance homogeneously and more than normal pancreas on CT. (RG AFIP) d. Insulinomas are frequently ectopic – F – 65% occur in body and tail of pancreas, with the vast majority of insulinomas occurring in the pancreas. Less than 1% in extrapancreatic sites. (RG AFIP) e. Glucagonoma is more common than insulinoma – F – outside of insulinoma and gastrinoma, all other functioning islet cell tumors are much less common in children. Dahnert – insulinoma is the most common syndromic tumour, glucagonoma uncommon.

Answer 84

a. It is usually bilateral F - 10% bilateral (Dahnert) 33. Regarding neonatal adrenal haemorrhage, which is false: (TW) a. It is usually bilateral F - 10% bilateral (Dahnert) b. More common on the right T – R>L 7:3 (D) c. More common with a breech presentation T – difficult labor / delivery / breech (D) d. Occurs in the first 7 days T – first week of life e. Increased with respiratory distress T – assoc with hypoxia /neonatal stress Aetiol : Neonatal stress •Difficult labour – forceps, breech •Hypoxia/asphyxia due to prematurity •Sepsis •Haemorrhagic disorders eg.DIC Increased risk LGA, diabetic mums. (Dahnert 6th pg 918)

Answer 85

b. Embolism is the most common treatment – F – usually require no treatment. Treatment depends on type and sequelae. Embolisation often used pre-resection 34. Peripheral haemangiomas in infants, which is false: (TW) a. More common in premature infants – T – hemangiomas are the most common tumor of childhood occurring in 12% of infants. UTD: the incidence of hemangiomas is increased in preterm infants; the most significant risk factor appears to be low birth weight. b. Embolism is the most common treatment – F – usually require no treatment. Treatment depends on type and sequelae. Embolisation often used pre-resection. c. Increased size after 12 months is atypical - T – characteristically 2-stage process of growth and regression. At birth lesionas are often small and inconspicuous. Shortly after birth, period of rapid proliferation that lasts several months, and typically, involution begins at approximately 10 months of age. 50% lesions completely resolved by 5yo. UTD: the spontaneous involution phase typically begins in the latter part of the 1st year. d. More common in the head and neck – T – have a predilection for head and neck (60%), although they can occur anywhere in the skin, mucous membranes, or internal organs. e. Sequester platelets – T – Kasabach-Merritt syndrome = hemorrhagic diathesis due to platelet sequestration by tumor / disseminated intravascular coagulation; characterised by an associateion of hemangioma or hemangioendothelioma, or angiosarcoma with thrombocytopenia and purpura (secondary to increased systemic fibrinolysis). There is an increased incidence of hemangiomas in females and in premature infants weighing less than 1500 grams Over half of all hemangiomas occur in the cervicofacial region

Answer 86

c. Can present with vague pain – ?? F –35. Regarding fibrous cortical defect, which is false: (TW) a. Longitudinal with bone – T – metaphyseal cortex of long bone. Oval, extending parallel to long axis of host bone. b. Multiloculated – ?? T - for larger lesions – however, specify FCD which is < 2cm c. Can present with vague pain – ?? F –Dahnert: asymptomaticStatDx: may cause pain, pathologic fractureUTD: usually is asymptomatic and discovered incidental to traumaRadCases (Ped): no pain. d. Expansile with thin cortex – T – cortical thinning and expansion may occur StatDx: Outward expansion of overlying cortex e. Most common in age 2-10 – T – peak 7-8yo, range 2-10yo. Mostly before epiphyseal closure.

Answer 87

b. Metacarpal index - F – Metacarpal index is used to diagnose Marfan’s syndrome (average the 4 ratios of length of 2nd to 5th metacarpals divided by their middiaphyseal width; in Marfan’s >8.8 in male or 9.4 in females) (Dahnert)

Answer 88

b. Viral gastroeneteritis is a recognized etiological factor - T - increasing body of evidence suggests that viral triggers may play a role in some cases (ie, seasonal variation of intussusception; has been associated with some forms of rotavirus vaccine; approx 30% of pts experience viral illness before onset) 37. Which is true re intussusception: (TW/SK) a. Plain film is rarely abnormal - F - plain radiographs are less sensitive and less specific than US. A variety of findings can be seen: signs of intestinal obstruction, target sign, soft tissue density projecting into gas of the large bowel (crescent sign). b. Viral gastroeneteritis is a recognized etiological factor - T - increasing body of evidence suggests that viral triggers may play a role in some cases (ie, seasonal variation of intussusception; has been associated with some forms of rotavirus vaccine; approx 30% of pts experience viral illness before onset) c. Small bowel obstruction is a contraindication to radiological reduction - F - it causes bowel obstruction. d. Onset >12 hours is a contraindication to radiological reduction - <1% mortality if performed <24 hrs. Longer duration increases rate of failed reduction and perforation. e. Idiopathic in almost half of cases – in children approximately 90% of cases are considered idiopathic as there is no clear disease trigger or pathological lead point. If over 3 years suspect pathologic lead point. Most common cause of intestinal obstruction in infants between 3 and 36 months of age. It is less common before 3/12 and older than 6y. Presence of air in the cecum or terminal ileum cna help exclude intussusception in pts with a low clinical suspicion of the disease. Pneumatic reduction - perforation risk <1% (perf usually in distal side of intussusception, often transverse colon). Other risk factors of perf = <6mo, long duration of symptoms (>/= 3d), evidence of SBO. Success rate 80-95% of pts with ileocolic intussusception. Recurrence 10%. UTD Contraindications – shock, established perforation with clinical signs of peritonitisSedation – prevents patient using Valsalva (which can increase intraluminal pressure by ~60mmHg), and thus reduces the reduction rate.No use of balloon inflation (works as escape valve) and also balloon is expelled with straining.3 attempts, 3 min intervals, 3 re-attempts before Sx. Up to 120mmHg pressure, or 100cm column of barium (= 150cm column of water / H2O Sol contrast).

Answer 89

e. Gauchers – F – GD: inborn error of metabolisms that affects recycling of cellular glycolipids. Most common lysosomal storage disease. Autosomal recessive. Get accumulation of lipid-laden macrophages in spleen, liver, bone marrow and bone (assoc inflammatory response too). 38. Which is not a recognized assoc with hemihypertrophy (TW) a. Hepatoblastoma – T – hepatoblastoma incidence increased with hemihypertrophy incl. Beckwith Wiedemann syndrome. 3rd most common abdominal tumor in children. Elevated AFP. b. Medullary sponge kidney – T – may be part of other syndromes such as BW syndrome, hemihypertrophy, Caroli, Ehlers-Danlos, Marfan, and pyloric stenosis. c. Glioma – T – glioma associated with NF. Overgrowth syndromes: NF, Klippel-Trenaunay-Weber, Proteus, Beckwith-Wiedemann (BW) syndrome. d. Nephroblastomatosis – T – abnormality of nephrogenesis characterized by incomplete maturation of primitive nephrogenic cells. Precursor to Wilm’s and assoc with Beckwith-Wiedemann and Hemihypertrophy. e. Gauchers – F – GD: inborn error of metabolisms that affects recycling of cellular glycolipids. Most common lysosomal storage disease. Autosomal recessive. Get accumulation of lipid-laden macrophages in spleen, liver, bone marrow and bone (assoc inflammatory response too).

Answer 90

e. Hepatoblastoma is highly echogenic on USS – T – mass typically hypervascular on Doppler sonography. Heterogeneous echogenicity from hemorrhage/necrosis. 39. In the paediatric age range: (TW) a. Adrenal carcinoma rarely manifests with endocrine symptoms – F – approx 60% of adrenocortical carcinomas are secretory. Non functioning ACCs are more common in older adults. Children usually present with virilization (84%) or precocious puberty, while isolated glucocorticoid excess (Cushing’s syndrome) is much less common and occurs in 6% (cf adults where it is common). b. The most frequent site of primary rhabdomyosarcoma is the genitourinary tract – F - The most common sites are the head and neck (28%), extremities (24%), and genitourinary (GU) tract (18%). Other notable sites include the trunk (11%), orbit (7%), and retroperitoneum (6%) (Dahnert). RMS most common is H&N, GU is second most common (Blackbook Paeds). c. Approximately 65% of primary hepatic tumours are benign – F – hepatoblastoma is most common primary liver tumor of childhood (43% of total liver masses). The most common hepatic tumors in children less than 5yo include hepatoblastoma, hemangioendothelioma, mesenchymal hamartoma, and metastatic disease from neuroblastoma or Wilms tumor. In kids older than 5yo the most common tumors are HCC, undifferentiated sarcoma, hepatic adenoma, and mets. d. Teratomas are the commonest retroperitoneal tumour – F – Wilms is most common abdominal neoplasm in children 1-8yo. Neuroblastoma is the most common extracranial solid malignancy in children. e. Hepatoblastoma is highly echogenic on USS – T – mass typically hypervascular on Doppler sonography. Heterogeneous echogenicity from hemorrhage/necrosis.

Answer 91

d. Chondrolysis is reversible - F - acute cartilage necrosis - occurs 7-10% = rapid loss of >50% of thickness of cartilage. Joint space <3mm 40. Which is false re SUFE: (TW) a. Initial slip is posterior - T - posteromedial displacement of the head (acute slip). b. Bilateral in 20-40% - T - 20-37% bilateral c. AVN in 15% - T - 10-15% AVN of femoral head: risk increases with advanced degree of slip, delayed surgery for acute slip, anterior pin displacement, lots of pins, subcapital osteotomy. d. Chondrolysis is reversible - F - acute cartilage necrosis - occurs 7-10% = rapid loss of >50% of thickness of cartilage. Joint space <3mm SUFE / SCFE - aetiology: growth spurt, renal osteodystrophy, rickets, childhood irradiation, growth hormone therapy, trauma. Overweight 8-17yo boys. M>F 3x. Line of Klein fails to intersect femoral head. CME 03.80 (13) Dahnert 6th ed,

Answer 92

a. Commonly (majority) assoc with other anomalies - F - 25% have associated structural anomalies, and fetal karyotype is indicated in these cases 41. CCAM which is false: (TW) a. Commonly (majority) assoc with other anomalies - F - 25% have associated structural anomalies, and fetal karyotype is indicated in these cases. *AJL - Type 2 (15-20% of cases) are associated with other anomalies eg renal, sequestration, CHD) b. Assoc with mediastinal shift - T - contralateral mediastinal shift in 90% c. Macrocystic form has a better prognosis than microcystic form (Perth MCQ option) – T d. (Perth: is assoc/ w/ normal situs) - T e. Surgery is usually recommended for asymptomatic patients with Dx of CCAM made on prenatal US - T - UpToDate (Jan 2009) - early intervention is recommended to prevent the potential complications of recurrent infections and/or malignancy. In symptomatic patients immediate surgical intervention may be required. ADB--> asymptomatic is controversial f. Can cause fetal hydrops - T - large CCAMs and shift of the mediastinum may lead to obstruction of the IVC and cardiac compression, resulting in development of hydrops from increased central venous pressure. Added options 3 and 4.

Answer 93

d. spinal metastasis occur in the posterior spinal canal more than anterior - T- as normal flow of CSF from the cisterna magna travels first along the posterior margin of the spinal cord before returning to the cistern along the ventral surface of the spinal cord, most mets are found along the posterior maring of the spinal cord. 42. Medulloblastoma, which is true?: (TW) a. resistant to radiotherapy - F - Rx with surgery and radiotherapy (extremely radiosensitive) b. on CT calcification helps to differentiate from pilocytic astrocytoma - F - Ca+ in up to 20% in both MB and pilocytic astrocytomas. *LW: Donnelly says Ca++ rare in pilocytic astrocytoma compared to MB, so is correct but would still favour option D as more correct. c. desmoplastic variant occurs in children younger than 3 years old - F - MB in adult patients are often the desmoplastic histologic type, & prone to recurrence (Radiographics03) d. spinal metastasis occur in the posterior spinal canal more than anterior - T- as normal flow of CSF from the cisterna magna travels first along the posterior margin of the spinal cord before returning to the cistern along the ventral surface of the spinal cord, most mets are found along the posterior maring of the spinal cord. e. most bone metastases are lytic lesions - F - osseous lesions are usually sclerotic (65% cases, lytic in 35% of cases). Bone is the most common extraneural metastatic site (adults and children). Followed by lymphnodes (33%).

Answer 94

b. Anterior wedging of C3 is a normal variant – T - normal variant, however age dependent: eg, anterior wedging in a 3yo at C5 is likely normal variant. However wedged body at C5 in an 8yo this is likely pathologically compressed. RG = “up to 6mm of displacement of the lateral masses relative to the dens is common in patients ≤ 4 yrs old & may be seen in patients up to 7 yrs” 43. Regarding imaging of paediatric cervical spine, which is true?: (TW) a. Atlantoaxial dislocation occurs less in children than adults - F - occurs 5x more common in children than adults. b. Anterior wedging of C3 is a normal variant – T - normal variant, however age dependent: eg, anterior wedging in a 3yo at C5 is likely normal variant. However wedged body at C5 in an 8yo this is likely pathologically compressed. RG = “up to 6mm of displacement of the lateral masses relative to the dens is common in patients ≤ 4 yrs old & may be seen in patients up to 7 yrs” c. Normal CT excludes spinal cord injury - F - SCIWORA - most studies of traumatic myelopathy in children report an incidence of SCIWORA greater than 20% (Medscape). Range 5-65%. d. Displacement of lateral mass of C1/2 of greater than 6 mm is normal - F – >2mm bilateral is always abnormal. >1-2mm or unilateral displacement can be due to head tilt / rotation. Total offset (sum of both sides) of more than 6mm of the lateral masses of the atlas with respect to the odontoid is highly suggestive of rupture of the transverse ligament or avulsion of its attachments. RG = “up to 6mm of displacement of the lateral masses relative to the dens is common in patients ≤ 4 yrs old & may be seen in patients up to 7 yrs” e. Injury to cervical spine is more common in lower than upper spine – F - in patients under 9yo, almost all injures are to the occiput-C2 region (Imaging of Spinal Trauma in Children, Kuhns). Differences between adult and paeds spinal injury (Spinal Trauma in Children, Paeds Radiol 2001) Fulcrum of movement located at C2-3 in the child, C5-6 in adult Relatively large head and weak neck muscles Ligamentous and joint capsule laxity Horizontal orientation of the facet joints in younger children Underdeveloped uncinate processes Mild physiological anterior 'wedging' of vertebral bodies Incomplete ossification of odontoid process

Answer 95

c. Sudden sensorineural hearing loss commonly occurs in vestibular aqueduct syndrome T - often diagnosed following head trauma causing sudden SNHL, although the patient probably had some progressive hearing loss prior to this. UTD: Some patients have normal hearing at birth, followed by progressive or fluctuating hearing loss. Sudden hearling loss may occur spontaneously or after minor head trauma. 44. Regarding sensorineural hearing loss, which is true?: (JS & TW) a. Mondini malformation demonstrates an abnormal basal turn - F - absence of the apical 1 1/2 turns often with preservation of the basilar turn, due to in utero insult at 7 weeks GA; assoc/ w/ Pendred syndrome (Dahnert/Neuro req p405) b. Vestibular aqueduct syndrome is more commonly unilateral - F - bilateral 50-66%. Bilateral VAS is 2x as common as unilateral VAS. (this was given as true answer previously). Harnsberger – bilateral in 90%. c. Sudden sensorineural hearing loss commonly occurs in vestibular aqueduct syndrome T - often diagnosed following head trauma causing sudden SNHL, although the patient probably had some progressive hearing loss prior to this. UTD: Some patients have normal hearing at birth, followed by progressive or fluctuating hearing loss. Sudden hearling loss may occur spontaneously or after minor head trauma.S – Commonly though?? d. Vestibular aqueduct syndrome is best evaluated by coronal CT - F – axial CT shows enlargement of the aqueduct greater than 1.5mm or the adjacent posterior semicircular canal. Alternative is MRI. e. Cochlear abnormality is rarely associated with vestibular aqueduct syndrome - F - commonly associated with other abnormalities such as Mondini malformation. S - Cochlear dysplasia (esp. cystic apex of cochlear with normal basal turn) is part of IP-II (LESA) anomaly (Req p405)

Answer 96

C – F – GFR better measured with DTPA than MAG-3 A – T - In young children, preference must be given to tracers with high extraction rate, such as 123 I-Hippuran or 99mTc-MAG3. MAG3 is actively excreted, while DTPA is undergoes only glomerular filtration B – T – MAG3 is primarily secreted by the tubules, while DTPA is by 100% glomerular filtration *LW: renal excretion refers to global term of urination, and is a sum of filtration, absorption, and secretion. While secretion refers to solute actively or passively passing from tubular cell into urine filtrate. So with regards to EXCRETION, in the global term is true, but it is technically not what the proximal tubules do...rather what the entire nephron does... But I guess I'm splitting hairs here, but I would also doubt they would word a question like this, so likely also an element of a poor recall. C – F – GFR better measured with DTPA than MAG-3 D – T – as above *LW: renal excretion refers to global term of urination, and is a sum of filtration, absorption, and secretion. While secretion refers to solute actively or passively passing from tubular cell into urine filtrate. So with regards to SECRETION, this is TRUE. E – F? – highly protein bound (85-90%)

Answer 97

A renal tubular acidosis – T causes medullary nephrocalcinosis B medullary sponge kidney – T “” C sickle cell disease – F D acute renal vein thrombosis – F usually hypoechoic (↑ cortical echogenicity in subacute phase) E hyperparathyroidism – T medullary nephrocalcinosis

Answer 98

a. Commonly assoc with other anomalies - F - 25% have associated structural anomalies, and fetal karyotype is indicated in these cases 45. CCAM which is false: (TW) a. Commonly assoc with other anomalies - F - 25% have associated structural anomalies, and fetal karyotype is indicated in these cases b. Assoc with mediastinal shift – T - contralateral mediastinal shift in 90%

Answer 99

c. Narrowed ribs – F – Proximally long slender ribs at level of neck, wide distally = spatulate rib confiburation / canoe paddle ribs. (Ribbon ribs in NF). *LW: although the posterior aspect of the ribs are narrowed with thickened anterior ribs forming a canoe paddle morphology, it is likely a relative narrowing, so out of all options, this is likely least correct. 46. Which of the following is not a feature of Hurler's disease (mucopolysaccharidosis 1): a. Hepatosplenomegaly – T – hepatosplenomegaly, umbilical or inguinal hernias, and typical skeletal abnormalities. b. Pointing of the proximal ends of the metacarpals – T – extremities: swelling / enlargement of diaphysis + cortical thinning (due to dilatation of medullary canal) + tapering of either end: distal humerus, radius, ulna, proximal ends of metacarpals (Dahnert). c. Narrowed ribs – F – Proximally long slender ribs at level of neck, wide distally = spatulate rib confiburation / canoe paddle ribs. (Ribbon ribs in NF). d. Localised kyphosis at the thoraco-lumbar junction – T – thoracolumbar kyphosis with lumbar gibbus. Oval centra (vertebra) with normal / increased height + anterior beak at T12/L1/L2. Long slender pedicles. e. Elongation of the sella – T – enlarged, J-shaped sella (undermining of the anterior clinoid process)

Answer 100

b. Asymmetrical involvement - F – usually symmetrical 47. Adrenoleukodystrophy, which is least correct: (TW) a. There is enhancement - T – leading edge (intermediate zone) enhancement. (StatDx). Contrast enhancement strongly linked to progression. CECT can show linear enhancement of intermediate zone. b. Asymmetrical involvement - F – usually symmetrical c. Periatrial and splenium of the corpus callosum involved - T - Classically involves peritrigonal white matter. Pattern: Splenium - peritrigonal WM - corticospinal tracts / fornix / commisural fibers / visual and auditory pathways. d. Affects the white matter, adrenals, and testicles - T – VLCFA accumulate in the i) CNS myelin ii) Adrenal cortex iii) Leydig cells of the testes. Note:- metabolic disorder - no oxidation of the very long chain fatty acidimaging- symmetric- corpus callosum selenium, occipitoparietal lobe, cerebellum - peritrigonal white matter- spares subcortical U fibre3-10yo. X-Linked recessive. Defective peroxisomal fatty acid oxidation due to impaired function of lignoceryl-CoA ligase - get accumulation of saturated very long chain fatty acids in white matter and adrenal cortex and testes.Usually symmetric, confluent & posterior (StatDx

Answer 101

e. Hepatoblastoma is typically echogenic on USS – T – mass typically hypervascular on Doppler sonography. Heterogeneous echogenicity from hemorrhage/necrosis. 1. In the paediatric age range: (TW) a. Adrenal carcinoma rarely presents with endocrine symptoms – F – approx 60% of acrenocortical carcinimoas are secretory. Non functioning ACCs are more common in older adults. Children usually present with virilization (84%) or precocious puberty, while isolated glucocorticoid excess (Cushing’s syndrome) is much less common and occurs in 6% (cf adults where it is common). b. The most frequent site of primary rhabdomyosarcoma is the genitourinary tract – F - The most common sites are the head and neck (28%), extremities (24%), and genitourinary (GU) tract (18%). Other notable sites include the trunk (11%), orbit (7%), and retroperitoneum (6%) (Dahnert). RMS most common is H&N, GU is second most common (Blackbook Paeds). c. Approximately 65% of primary hepatic tumours are benign – F – hepatoblastoma is most common primary liver tumor of childhood (43% of total liver masses). The most common hepatic tumors in children less than 5yo include hepatoblastoma, hemangioendothelioma, mesenchymal hamartoma, and metastatic disease from neuroblastoma or Wilms tumor. In kids older than 5yo the most common tumors are HCC, undifferentiated sarcoma, hepatic adenoma, and mets. d. Teratomas are the commonest retroperitoneal tumour – F – Wilms is most common abdominal neoplasm in children 1-8yo. Neuroblastoma is the most common extracranial solid malignancy in children. e. Hepatoblastoma is typically echogenic on USS – T – mass typically hypervascular on Doppler sonography. Heterogeneous echogenicity from hemorrhage/necrosis.

Answer 102

B = T (cyanotic, egg on string, increased pul vasc) *LW E = T, is also a cyanotic condition with increased pulmonary vasculature, i.e. part of the "4T'S) *AJL - tricuspid atresia only has increased vascularity if there is also a large VSD. 4T'S of cyanotic heart disease with increased pulmonary vascularity: - TGA - Tricuspid atresia - Truncus arteriosus - TAPVR - Singe ventricle.

Answer 103

*AJL - I would favour SUDI to be the most suspicious finding in a case of NAI. The clavicle 1 month after birth may also be suspicious but can be seen following birth trauma. Previous answer: A = F SIDS unusual after 12 months, no longer called SIDS. UTD says SIDS > 6 months should raise suspicion for deliberate asphyxiation. B = F C = T?? Clavicular fractures secondary to birth trauma affect the middle third; heals in 4-6 weeks D = F

Answer 104

A = T B = F (left main bronchus) C =F (posterior)

Answer 105

A = T- low (horizontal) acetabular roof A = T- low (horizontal) acetabular roof B = F – hypersegmentation in T21 C = F – CHD assoc/ w/ T21 assoc/ w/ risk of IE Wji: T21 is associated with a hypersegmented sternum and ddh so increased acetabular angle. I think the relationship with endocarditis is less clear.

Answer 106

d. Calcification is seen on US earlier than CT - T *LW: I think this is incorrect based on prior question reasoning question... 2007 clinical radiology: “US and CT demonstrated equal sensitivity in the detection of intracranial calcification in infants with congenital toxoplasmosis”. Another article says CT has higher sensitivity and specificity cf ultrasound. However, US resolution has increased significantly past years. However, US is the standard of care in this population...which only confuses matters more. 2. PVL: (TW) a. Dilation of ventricle is early Cx – F – with time there is often volume loss of the involved white matter b. U/S differentiates hemorrhage from infarct – F – both can demonstrate increased heterogeneous echogenicity in the periventricular white matter. c. Germinal matrix hemorrhage is a cause of PVL – F – PVL is due to hypoxic-ischaemic encephalopathy or follows infection. PVL is distinct from periventricular hemorrhagic infarction, although the two may coexist in premature infants. PVL is generally symmetric and rarely hemorrhagic. PV hemorrhagic infarction is nearly always unilateral or asymmetric. d. Calcification is seen on US earlier than CT - T PVL refers to injury of cerebral white matter that occurs in a characteristic distribution and consists of periventricular focal necrosis, with subsequent cystic formation, and more diffuse cerebral white matter injury. PVL is the major form of brain WM injury that affects premature infants. PVL can be caused by ischaemia or infection. Because of changes in vascular and cellular factors during development, the brain is more vulnerable to these insults in premature than term infants.

Answer 107

e. Hepatoblastoma is highly echogenic on USS – T – mass typically hypervascular on Doppler sonography. Heterogeneous echogenicity from hemorrhage/necrosis. 3. Regarding the pediatric age range, which of the following are true: (TW) a. Adrenal carcinoma rarely manifests with endocrine symptoms – F – approx 60% of acrenocortical carcinimoas are secretory. Non functioning ACCs are more common in older adults. Children usually present with virilization (84%) while isolated glucocorticoid excess (Cushing’s syndrome) is much less common and occurs in 6% (cf adults where it is common). b. The most frequent site of primary rhabdomyosarcoma is the genitourinary tract – F - The most common sites are the head and neck (28%), extremities (24%), and genitourinary (GU) tract (18%). Other notable sites include the trunk (11%), orbit (7%), and retroperitoneum (6%) (Dahnert). RMS most common is H&N, GU is second most common (Blackbook Paeds). c. 65% of primary hepatic tumours are benign – F – hepatoblastoma is most common primary liver tumor of childhood (43% of total liver masses). The most common hepatic tumors in children less than 5yo include hepatoblastoma, hemangioendothelioma, mesenchymal hamartoma, and metastatic disease from neuroblastoma or Wilms tumor. In kids older than 5yo the most common tumors are HCC, undifferentiated sarcoma, hepatic adenoma, and mets. d. Teratomas are the commonest retroperitoneal tumour – F – Wilms is most common abdominal neoplasm in children 1-8yo. Neuroblastoma is the most common extracranial solid malignancy in children. e. Hepatoblastoma is highly echogenic on USS – T – mass typically hypervascular on Doppler sonography. Heterogeneous echogenicity from hemorrhage/necrosis.

Answer 108

**LJS - pleural effusion is more common in primary than secondary TB, and more common in adults than kids a. Pleural effusions are rare - T - ?rare but best answer? - D says 10% in kids, (Thoracic Imaging [TI]) says patients with primary MTB most often show no radiologic abnormalities. Atelectasis is often encountered in children with pMTB and may be relate dto aireway compression by enlarged lymph nodes. Pleural effusions may occur in patient with pMTB infection . Often when TB is discovered as the cause for pleural effusion, no parenchymal focus of disease is radiographically evident; this pattern is considered characteristic of pMTB pleural infection. Such effusions are usually small and unilateral. 4. Which is true regarding childhood primary pulmonary TB: (TW) a. Pleural effusions are rare - T - ?rare but best answer? - D says 10% in kids, (Thoracic Imaging [TI]) says patients with primary MTB most often show no radiologic abnormalities. Atelectasis is often encountered in children with pMTB and may be relate dto aireway compression by enlarged lymph nodes. Pleural effusions may occur in patient with pMTB infection . Often when TB is discovered as the cause for pleural effusion, no parenchymal focus of disease is radiographically evident; this pattern is considered characteristic of pMTB pleural infection. Such effusions are usually small and unilateral. b. Bilateral hilar lymphadenopathy occurs in the majority - F - massive hilar adenoapthy (60%) / paratracheal (40%) / subcarinal lymphadenopathy, in 80% on the right side (D). Lymphadenopathy commonly occurs in children with pMTB. Usually hilar lymph nodes are involved, and mediastinal lymph nodes, particularly in the right paratracheal region. Unilateral lymphadenopathy is more often seen than bilateral disease (TI). c. Calcification accompanying healing is not typical - F - possible outcomes: containment (delayed hypersensitivity response + granuloma formation - can calcify); progressive primary TB; miliary TB; post primary TB (reactivation TB). d. Presence of atelectasis is associated with bronchiectasis - F e. The lung focus is typically apical - F - if overt infection occurs (as most patients with primary MTB shoe no radiologic abnormalities) it usually involves an entire lobe. Right lung more often than the left, although no definite zonal predominance is seen (TI). Radiographics (TB review 2007) says predominance in lower and middle lobes (esp adults), and kids atelectasis most often anterior segment upper lobe, of medial segment of middle lobe.

Answer 109

d. spinal metastasis occur in the posterior spinal canal more than anterior - T- as normal flow of CSF from the cisterna magna travels first along the posterior margin of the spinal cord before returning to the cistern along the ventral surface of the spinal cord, most mets are found along the posterior maring of the spinal cord. 5. Medulloblastoma: (TW) a. resistant to radiotherapy - F - Rx with surgery and radiotherapy (extremely radiosensitive) b. on CT calcification helps to differentiate from pilocytic astrocytoma - F - Ca+ in up to 20% in both MB and pilocytic astrocytomas. *LW: as above Donnelly states there is a difference in calcificaiton with astrocytoma rare compared to MB, but would favour D as most correct. c. desmoplastic variant occurs in children younger than 3 years old - F - MB in adult patients are often the desmoplastic histologic type, & prone to recurrence (Radiographics03) d. spinal metastasis occur in the posterior spinal canal more than anterior - T- as normal flow of CSF from the cisterna magna travels first along the posterior margin of the spinal cord before returning to the cistern along the ventral surface of the spinal cord, most mets are found along the posterior maring of the spinal cord. e. most bone metastases are lytic lesions - F - osseous lesions are usually sclerotic (65% cases, lytic in 35% of cases). Bone is the most common extraneural metastatic site (adults and children). Followed by lymphnodes (33%).

Answer 110

b. Sensorineural hearing loss - T - this is the most true answer I think??)- commonest 6. Large Aqueduct Syndrome: (JS) a. Following minor sudden loss (? do they mean trauma) - T - although most patients have a progressive SNHL, some are diagnosed after minor trauma when they develop sudden sensorineural hearing loss; this is thought to be due to reflux of endolymph into the cochlea... b. Sensorineural hearing loss - T - this is the most true answer I think??)- commonest cause of congenital deafness c. Sudden loss - F or maybe T - see above d. unilateral < bilateral - F - bilateral in 50-66% (Harnsberger says 90% bilateral!) e. coronal better axial then for imaging - F - best seen on axial imaging with vestibular aqueduct larger than the posterior semicircular canal or > 1.5 mm

Answer 111

a. Necrotizing enterocolitis and stricture formation – T – Intestinal strictures occur in 9-36% of infants treated medically or surgically and are unrelated to the severity of NEC, presence of pneumatosis intestinalis, or gestational age. Majority of strictures occur in the colon, although ileum and jejunum also affected. Multiple strictures are common. 7. The following associations are well recognised: (TW) a. Necrotizing enterocolitis and stricture formation – T – Intestinal strictures occur in 9-36% of infants treated medically or surgically and are unrelated to the severity of NEC, presence of pneumatosis intestinalis, or gestational age. Majority of strictures occur in the colon, although ileum and jejunum also affected. Multiple strictures are common. b. Hypertrophic pyloric stenosis and intussusception - F c. Potters syndrome and polyhydramnios – F – Potter’s syndrome / sequence = features secondary to prolonged Oligohydramnios secondary to bilateral renal agenesis, or anomalies in which the kidneys are present but there is an obstructed urethra, prolonged rupture of membranes, or severe growth retardation. Features or Potter’s syndrome: hypoplasia of lungs; bladder hypoplasia; Potter’s facies (flat nose, parrot-beaked nose, recessed chin, epicanthic folds, wrinkled redundant skin, low set ears, hypertelorism); brachycephaly; limb deformities (bowing of legs, clubbed feet, broad hands); growth retardation.

Answer 112

c. Intralobar form drains to pulmonary veins - T - ILS drains to LV, but can have abnormal drainage to IVC, azygous, or RA. ELS receives arterial supply from aberrant vessel arising from thoracic aorta, and has anomalous venous drainage to RA, vena cava, or azygous. 8. Regarding Pulmonary Sequestration which is true: (TW) a. The intralobar form most frequently presents in neonate - F - ILS usually presents later than ELS. Patients with ILS usually present in late childhood or adolescence with recurrent pulmonary infections. ELS more apt to present in early life from resp distress / cyanosis / or assoc abnormalities. Both can be an incidental antenatal Dx too. b. Intralobar have arterial supply from pulmonary artery - F - systemic arterial supply, usually lower thoracic or upper abdo aorta. Single or multiple arterial trunks. Venous drainage usual normal to left atrium, but can be abnormal and connect to IVC, azygous, or RA. c. Intralobar form drains to pulmonary veins - T - ILS drains to LV, but can have abnormal drainage to IVC, azygous, or RA. ELS receives arterial supply from aberrant vessel arising from thoracic aorta, and has anomalous venous drainage to RA, vena cava, or azygous. d. Extralobar occur mostly on the right - F - ELS is almost always LLL (ILS LLL > RLL 60%:40%). e. Extralobar characterized by presence of air bronchograms - F - lacks a bronchial connection to normal proximal airway. May connect to GIT or rarely, to intrpulmonary structures. Infectious complications are uncommon in ELS. ILS 70% : ELS 30%. 65% of ELS assoc with other anomalies. ILS not associated with other anomalies.

Answer 113

d. Spread across the midline with vascular encasement is a recognised appearance on ultrasound and CT - T 9. Which of the following statements regarding neuroblastoma in children is true: (TW) a. Almost all occur in the abdomen – F – SK – 2/3 occur in the abdomen, of which 2/3 occur in the adrenal/s b. Peak age of incidence is 5 years – F – peak 22mo c. Neuroblastoma in infants has a worse prognosis than those occurring in later childhood – F – stage IVs (if not cortical bone involvement). <1yo: better prognosis, mets to liver and skin. >1yo: worse prognosis, mets to bone. Stage IVs: Age <1yo, metastatic disease confined to skin, liver, bone marrow (near 100% survival). d. Spread across the midline with vascular encasement is a recognised appearance on ultrasound and CT - T e. Bone scintigraphy with 99mTc-Pi1DP is more accurate than 1 131-MIBG for detection of skeletal mestastases – F – MIBG and bone scan for determining distal extent of disease.

Answer 114

c. Association with cystic fibrosis F - NOT related to CF. May see a meconium plug, but this is as a result (and not the cause) of obstruction. ie. meconium plug (SLC) syndrome, cf. meconium ileus in CF babies. 10. With regards to Small left colon syndrome which of the following is false: (GC) a. Bowel dilatation proximal to the splenic flexure T - colonic calibre becomes abruptly diminuitive distal to splenic flexure. b. Association with maternal diabetes T - also maternal substance abuse. c. Association with cystic fibrosis F - NOT related to CF. May see a meconium plug, but this is as a result (and not the cause) of obstruction. ie. meconium plug (SLC) syndrome, cf. meconium ileus in CF babies. d. Immaturity of the myenteric plexus T - results in a transient functional colonic obstruction. [Dahnert] Should be the mesenteric plexus

Answer 115

e. Parenchymal calcification in congenital infection may be seen on ultrasound earlier than on CT scan - T *LW: out of these options is least false, according to above prior questions quoting 2007 clinical radiology article and logic.... Counter argument, is that there is cases of ante natal US documenting fetal Ca++.... so could argue this either way i guess (as ironically if you CT that fetus ante natally, likely see cerebral Ca++ too, but its never done....chicken and egg argument now arises.... 11. Which of the following is true with regard to head ultrasound in the infant: (TW) a. The normal choroid plexus is a homogeneous hypoechoic structure – F - hyperechoic b. Parenchymal haemorrhage and infarction can be readily differentiated – F – both can be echogenic c. The normal cerebellum is less echogenic than the supratentorial cortex – F – relatively hyperechoic d. It is the test of choice for possible extracerebral haemorrhage prior to fontanelle closure – F – cannot visualise all areas due to limitations with fontanella window. *LW: although may screen with US, both CT and MRI have better accuracy of extra axial haemorrhages (multiple studies), so hence US is not the test of choice and is FALSE. e. Parenchymal calcification in congenital infection may be seen on ultrasound earlier than on CT scan - T

Answer 116

b. Complete transposition of the great vessels – T – variable pulmonary flow (cyanosed). Historically d-TGA was categorized as a cardiac lesion associated with increased pulmonary arterial flow, however in areas with well-developed health care systems, pulmonary arterial switch procedures are preformed during the 1st week of life, and increased pulmonary flow, which occurs in older children with transposition is rarely seen now. 12. A cyanosed child presents with hypervascular lung fields. The possible causes include: (TW) a. Fallot's tetralogy – F – TOF = normal or mildly increased heart size (boot-shape) and decreased pulmonary flow (cyanosed). b. Complete transposition of the great vessels – T – variable pulmonary flow (cyanosed). Historically d-TGA was categorized as a cardiac lesion associated with increased pulmonary arterial flow, however in areas with well-developed health care systems, pulmonary arterial switch procedures are preformed during the 1st week of life, and increased pulmonary flow, which occurs in older children with transposition is rarely seen now. c. Ventricular septal defect – F – increased pulmonary arterial flow with L to R shunt (pink baby) d. Collapsed left lower lobe – F – still got rest of lungs working. Shouldn’t be cyanosed e. Tricuspid atresia – T and F – variable flow (cyanosed). Thoracic Imaging: TA can be assoc with TGA, VSD, pulmonary atresia / stenosis. If VSD large and pulmonary stenosis mild or nonexistent, there is pulmonary overcirculation. If there is a pulmonic valvular or subvalvular flor restriction or restrictive VSD then can have restriction of pulmonary blood flow. (Donnelly) **LJS - tricuspid atresia also cyanosed but variable lungs. If small VSD = decr pulmonary vascularity, if large associated VSD = incr pulmonary vascularity

Answer 117

A – SIDS unusual after 12 months, no longer called SIDS B – Clavicular fractures secondary to birth trauma affect the middle third; heals in 4-6 weeks C – birth trauma

Answer 118

a. narrow lumbar canal – T – stenosis of lumbar spine. Wedge-shaped / bullet-shaped vertebra. Ventrodorsal narrowing of spine due to short pedicles. Narrowing of interpedicular distance due to laminar thickening. b. increased acetabular angle – F – decreased acetabular angle. c. increased acetabular angle – F – iliac wings not flared, but squared and flattened. “Tombstone”. “Champagne glass” shaped pelvic inlet. Decreased acetabular angle.

Answer 119

a. Duodenal Atresia – T – 30% of DA/DS have Down Syndrome, and 3-5% of Down Syndrome have DA/DS

Answer 120

a. Autosomal dominant inheritance – T – AD / sporadic (80%) disease with quantitatively defective endochondral bone formation; epiphyseal maturation and ossification unaffected. e. Reduced acetabular angle – T – squared flattened iliac bones and horizontal acetabula. 18. Achondroplasia. (TW) a. Autosomal dominant inheritance – T – AD / sporadic (80%) disease with quantitatively defective endochondral bone formation; epiphyseal maturation and ossification unaffected. b. Widens the interpedicular distance – F – narrowing of interpedicular space due to laminar thickening. c. Distal limb shortening – F – predominantly rhizomelic (proximal) micromelia of long bones (femur, humerus). d. Premature closure of the growthplate – F – see ans 1. e. Reduced acetabular angle – T – squared flattened iliac bones and horizontal acetabula.

Answer 121

a. Ground-glass opacities. T - symmetric generalized consolidation of variable severity; may see evolving hazy opacities, clearing over several days. CHF, aspiration, pulmonary haemorrhage and infection are other factors that may complicate RDS and produce a GGO pattern. b. Pleural effusion. F – pleural effusion suggests group B strep pneumonia rather than RDS. c. Pleural calcification. F d. Pneumothorax. F - complication of CPAP (up to 25%): alveolar rupture (pseudocyst), air dissects along interlobular septa (PIE, "pseudoclearing" of RDS), interstitial air migrates centripetally to pleural space and/or mediastinum. May also result in subcut emphysema or gas embolism. e. Lobar emphysema. F – but lobar PIE is a complication of RDS.

Answer 122

a. Morgagni hernias have a 25% association with congenital abnormalities elsewhere in the body F – (CME 1998.Q11). Assocns: congenital heart disease, bowel malrotation, chromosomal (T21, Turner's), mental retardation, pericardial deficiency. [Dahnert] *LW: articles and UpToDate state associations > 50%, with some articles stating approx 75%. b. Morgagni hernias show an opaque hemithorax on the presenting film. F - usually small, anterior and central location. CXR: cardiophrenic angle mass, R>L 9:1; gas-filled loops of bowel. Bochdalek hernias are usually large; initial postnatal XR may show hernia as radiodense (prior to air introduced into bowel). 20. Diaphragmatic hernia, which are false: (GC) a. Morgagni hernias have a 25% association with congenital abnormalities elsewhere in the body F – (CME 1998.Q11). Assocns: congenital heart disease, bowel malrotation, chromosomal (T21, Turner's), mental retardation, pericardial deficiency. [Dahnert] b. Morgagni hernias show an opaque hemithorax on the presenting film. F - usually small, anterior and central location. CXR: cardiophrenic angle mass, R>L 9:1; gas-filled loops of bowel. Bochdalek hernias are usually large; initial postnatal XR may show hernia as radiodense (prior to air introduced into bowel). c. Bochdalek's hernias are usually left sided. T - left 80%, right 15%, bilateral 5%. d. If presenting with respiratory distress in the first 24 hours indicates pulmonary hypoplasia. T - assuming CDH known from antenatal scans - respiratory distress in neonatal period; life-threatening deficiency of small airways and alveoli. Degree of hypoplasia depends on when during gestation the herniation occurred - at each end of the spectrum: early herniation assocd with severe bilateral PH (uniformly fatal); whilst postnatal herniation with compression of otherwise normal lung has an excellent prognosis. see Dahnert p490 for Wiseman classifn (I-IV). e. The majority of Morgagni hernias are asymptomatic unless they strangulate T

Answer 123

21. Cystadenomatoid malformation. (TW) a. Is not associated with a long life span – F – ambiguous as depending on the type of CPAM (now there are 5) can be anything from death in utero to excellent prognosis. b. Is associated with non-immune hydrops – T – possible cause: thoracic lesions causing hydrops: CCAM, CDH, sequestration, chylothorax, lymphangiectasis, neoplasms, Bronchogenic cyst. These increase intrathoracic pressure and can obstruct venous return to the heart – peripheral venous congestion, or can obstruct lymphatic duct, resulting in lymphedema. c. Can be diagnosed on antenatal ultrasound – T – cystic and/or echogenic d. Can present as respiratory distress – T – see ans 2. If large can cause compromise of contralateral lung. Respiratory distress and severe cyanosis in 1st week of life in 66%, within first year of life 90% due to compression of normal lung and airways. e. Is associated with tracheo-oesophageal fistula – F – can’t find assoc. Dahnert says 25% of CCAM associated with cardiac malformation, pectus excavatum, renal agenesis, prune-belly syndrome, jejunal atresia, chromosomal anomaly, Bronchopulmonary sequestration.

Answer 124

22. Vesico-ureteric reflux has the following associations. (TW) a. Sibs with VUR – T – increased incidence in siblings of patients with known VUR – 25-40%. b. Nocturnal enuresis – F – causes: maturational delay, genetics, functional small bladder capacity, abnormal diurnal secretion of vasopressin, nocturnal polyuria, detrusor instability, sleep disorders, psychological issues. Most common p/c VUR is UTIs. c. Ipsilateral PUJ – F – PUJ & VUR assoc with MCDK, but don’t think ipsilateral PUJ and VUR assoc. d. Diverticula – T – a ureter entering a bladder diverticulum almost always has associated VUR but rarely obstruction. e. Duplex collecting system – T – upper (moiety) obstructs, lower (moiety) refluxes.

Answer 125

23. The following are usually seen in the prune belly syndrome (TW) a. Megacystis – T – bladder is usually enlarged and nontrabeculated, and has thickened walls (due to collagen deposits) with smooth muscle hypoplasia. b. Cryptorchidism – T – bilateral cryptorchidism in males c. Polyhydramnios – F – Oligohydramnios occurs when the aetiology is urethral obstruction. Other US features may be: bulging abdnomen, lack of abdo muscle wall, dilatation of bladder and ureters, hydronephrosis +/- echodense renal parenchyma, polycystic or dysplastic kidneys, fetal ascites, hypoplastic lungs. d. Ureterectasis – T – ureters are grossly elongated, dilated, and tortuous due to replacement of smooth muscle with fibrous tissue. e. Hypertrophied anterior abdominal wall musculature – F – abdominal muscle deficiency (mesoderm development abnormal) (UTD)

Answer 126

b. Third ventricle visible – F – in alobar HPS there is absence of septum pellucidum, 3rd ventricle, falx cerebri, interhemispheric fissure, CC, fornix, optic tracts, olfactory bulb… etc. (note that in semilobar there is a small 3rd ventricle) 24. The following are features of alobar Holoprosencephaly (TW) a. Fused thalami – T - fused ventricles, thalami/basal ganglia and frontal lobes. b. Third ventricle visible – F – in alobar HPS there is absence of septum pellucidum, 3rd ventricle, falx cerebri, interhemispheric fissure, CC, fornix, optic tracts, olfactory bulb… etc. (note that in semilobar there is a small 3rd ventricle) c. Absence of corpus callosum - T d. Associated with the Neu Laxova syndrome – T – rare, autosomal recessive. Microcephaly. e. Presence of proboscus above the orbits – T – cyclopia (= midline single orbit); may have proboscis (= fleshy supraorbital prominence) and absent nose.

Answer 127

25. Intussusception in children (TW) a. 50% occur less than one year of age – T? - <1yo 50% (Dahnert); StatDx – most common in < 1 yo’s. b. Are most often ileo colic – T – ileocolic 75-95%; ileoileocolic 9%; ileoileal 4%; colocolic. c. May cause a pseudo kidney appearance on ultrasound - T d. 5% occur after surgery – F – can’t see why this would be true. In children over 95% are idiopathic. The remaining 5% are due to a lead point (ie if <3mo: Meckel diverticulum, duplication cyst; if >3yo: lymphoma, Peutz-Jeghers, hemangioma, enterogeneous cyst, ectopic pancreas, suture granuloma, HSP, etc..). e. Usually have normal plain abdominal films – F – 25% no abnormality on plain film. 50-60% have abdominal soft tissue mass, usually in RUQ.

Answer 128

*LW: B = F: Donnelly says Ca++ rare in pilocytic astrocytoma compared to MB, A = T (according to Dahnert), others say equal to PNET-MB, others say 2nd to PNET-MB B = Probably F C = T D = T E = T (heterogenous)

Answer 129

A = F (medical emergency) B = T C = T (microcolon, no meconium in colon) D = T (diagnose & treat) E = F

Answer 130

A = T = normal to increases coarse echoes throughout cortex B = T C = F (usually hyperechoic enlarged kidneys – cysts too small to be resolved by US)

Answer 131

A = T (Dahnert says 10%; StatDx: radiographs in 9%, on CT scan in 15%) B = T (up to 85%) C = T (25-50% D; StatDx 50%) D = F E = F

Answer 132

a. t b. false (delayed ossification) c. false d. t e. t

Answer 133

A =true. affects posteromedial or anterolateral dome of talus b. false (10-15%) c. False d. false (high T2 signal. Not low signal) **SCS: radiopaedia trivia. OCD: -Medial condyle 78%. Classic location lateral surface of medial condyle -Lateral condyle 15% -Talus: predominantly located in the superomedial corner of the talus. -capitellum (rare but remember little leaguer elbow association) Cf: SONK/ subchondral insufficiency fractures: consider in OLDER px. It is almost always unilateral, usually affects the medial femoral condyle (but can occasionally involve the tibial plateau) and is often associated with a meniscal tear.

Answer 134

a. T (5 years) b. F ( 11 years) c. F (centres appear at puberty) d. T ( 2 centres. one at 3 months, the other at 6-8 years) e. F (9-12 years)

Answer 135

a. F (AD) b. F (ascending aorta) c. T d. T e. T (mitral more common) **LJS - Marfans also has tricuspid incompetence (less common than mitral prolapse) https://www.ecrjournal.com/articles/cardiovascular-management-marfan

Answer 136

a. F b. T c. T D. T E. F

Answer 137

a. achondroplasia: FALSE b. 20% of ankylosing spondylitis: TRUE c. retropharyngeal abscess: TRUE d. SLE: TRUE e. 2% of down's; FALSE, 20% of Downs.

Answer 138

a. F b. F (common) c. F (90% functional) d. F (need combination of AXR, U/S normally) e. T

Answer 139

a. T b. T c. T d. T e. F

Answer 140

a intracranial haemorrhage occurs in 40% of patient < 32 weeks in gestation. T (43%) b subependymal haemorrhage may result in subependymal cysts. T c intraentricular haemorrhage is a recognised cause of hydrocephalus. T d the thalamocaudate groove is best seen through the posterior fontanelle. F (posterior fontanelle is only good for assessing posterior fossa and occipital horns) e intracranial haemorrhage in pre-term infants generally occurs at approximately 2 weeks from birth. False (36% occurs within 24 hours, the other 32% within 48 hours. 90% occur by day 6 post birth)

Answer 141

a occurs in only male. F (female more) b may be from AR trait. T c may cause virilisation. . T d may cause hypernatraemia. F (deficiency of aldosterone and low sodium - salt wasting) e the ovaries may be normal. T

Answer 142

a transient tachypnea of the newborn and elective CS.. T b pulmonary hypolasia and renal agenesis. T c neonatal pneumothorax and binary atresia. F d tracheooesophageal fistula and maternal diabetes. F e hyaline membrane disease and maternal diabetes. T

Answer 143

a commonly occurs in adolescent sprinters. T b avulsion os created by pull of abductor muscles. . F (hamstring) c an old healed lesion play present as a mass like expanded lesion of the ischial tuberosity. T d known to occur in cheer leaders. T e the nature of the lesion is obvious by comparison with the normal side. F (other side may be affected)

Answer 144

a. clinical manifestations are similar to acute osteomyelitis. - T (pain swelling and tenderness initially) b aetiologic agent is identified in the majority of cases. F (unknown normally) c the radiographic findings are similar to those of the late healing stage of acute osteomyelitis. . T (dominant finding is bone sclerosis) d antibiotic therapy usually does not alter course of disease. . T (antibiotic is commonly used, but clinical result have been inffective or inconsistent) e it is not uncommon form of osteomyelitis. F (i.e is uncommon)

Answer 145

The following statements are correct in congenital syphilis (t/f) a negative serology in the newborn excludes the diagnosis. f b resolution of periostitis is a reliable of effective treatment. . f (healing of bone lesion occur with or without therapy) c bone within bone appearance is characteristic. f (it is osteopetrosis) d erosion of the medial side of the proximal tibial metaphysis is a feature. t (Wimburger's sign - nearly pathognomonic) e a radiolucent metaphyseal band is a specific features.. f (is a feature but not specific)

Answer 146

the following are recognised association (t/f) a ASD and absent thumbs. T (trisomy 21) - **LJS - disagree. Holt Oram b coarctation of aorta and bronchial carcinoid. F c joint hyper mobility and mitral incompetence. T (marfans) d sarcoidosis and complete heart block. T (restrictive cardiomyopathy) e raymaud's disease and aortic stenosis. F

Answer 147

in medullary cystic disease of the kidney (T/F) a the kidney is always enlarged. F (normal or small) b the inheritance is AD. ?? (adult form is AD, juvenile form is AR) **LJS - most forms AR (Robbins) c 25% of contralateral renal anoamlyl. ?? **LJS - presume always bilateral d it is the commonest cause of a neonatal renal mass. . F e the cysts are diverticula of the distal convoluted tubule. F (separate cyst from renal system)

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