PAEDIATRICS 2 Flashcards

Question

Types of CPAM/CCAM

Answer 1

* **Congenital Pulmonary airway Malformation or Congenital Cystic adenomatoid malformation** * **Definition**: * multi-cystic masses of segmental lung tissue with abnormal bronchial proliferation. * Part of the spectrum of bronchopulmonary foregut malformations. * Type 0: rare and fatal * Type 1: Most common, large Cysts (2-10cm) unilateral * Type 2: Common, multiple small cysts (0.5-2cm), associated with other congenital abnormalities. * Type 3: Uncommon, large, solid * Type 4: uncommon, thin walled, often multifocal, high association with malignancy, specifically **pleuropulmonary blastoma.** * Type 1 and 4 are difficult to differentiate and carry the risk of malignancy (especially type 4). * **Treatment** is resection.

Answer 2

* Adenopathy * leukaemia, * lymphoma, * tuberculosis * BFM * Vascular malformations

Answer 3

* Hands: * bizarre parosteal osteochondromatous proliferation (BPOP) * arises from the bone cortex and lacks medullary continuity. * Case courtesy of Dr Matt Skalski, Radiopaedia.org, rID: 30785 * Humerus: * supracondylar spur: projects towards the elbow joint. * Malunited fracture.

Answer 4

* Coloboma * Heart defects * choanal Atresia/Cranial nerve defects (facial nerve palsy), Dysphagia. * Retardation of growth and development * Gential +/- urinary abnormalities * Ear abnormalities Dx: * Definate CHARGE: * 4 major characteristics * 3 major + 3 minors. * POSSIBLE CHARGE: * 1 or 2 major characteristics and several minor characteristics * https://radiopaedia.org/articles/charge-syndrome

Answer 5

* 3% of children * Morbidities * poor performance in school * ADD * Excessive daytime sleepiness * FTT. * Secondary to enlargement of the adenoids and palatine tonsils. * Palatine tonsils can be evaluated clinically * imaging is limited to a lateral Xray of the airway to evaluate the adenoids which appear as a soft tissue mass in the posterior nasopharynx. * criteria for enlargement * \>12mm and a convex outward anterior border. * complete obstruction of the posterior nasopharynx.

Answer 6

- Fixed narrowing at the level of the cricoid. - Failure of laryngeal recanalisation in utero

Answer 7

Adenopathy (leukaemia, lymphoma, tuberculosis) BFM Vascular malformations

Answer 8

* left to right shunt 1/400 births * 40% of CHD * more common in neonates * right atrial enlargement * LVH * Plethora * Cant see AP window secondary to PAH * cx * aneurysm to PA * eisenmegners -\> early oligaemia to apices * A/w T21, T18, T13 * Holt oram syndrome * TOF * TA * coarctation * AR * PS

Answer 9

Cortical: "COAG" * Renal cortical necrosis * Oxalosis * Alport syndrome * (chronic) glomerulonephritis Medullary: * deposition of calcium salts in the medulla of the kidney. * Causes "HAM HOP" * **h**yperparathyroidism * renal tubular **A**cidosis * **M**edullary sponge kidney * **H**yper or hypo thyroidism * **O**xalosis * **p**apillary necrosis * Hypervitaminosis D. * Milk alkali syndrome * Sarcoid

Answer 10

* Gastric atresia * single bubble * duodenal atresia * Double bubble * UGIS is performed in those patients with a double bubble and distal gas as these findings will inform the diagnosis. * duodenal stenosis (including annular pancreas, web, jejunal atresia) * Jenunal atresisa * tripple bubble * malrotation with volvulus * The presence of distal gas, alongside a history of bilious vomiting, is very important as it may represent intestinal malrotation with volvulus, which constitutes an emergency, requiring confirmation through an urgent UGIS (or other appropriate diagnostic test). * UGIS is performed in those patients with a double bubble and distal gas as these findings will inform the diagnosis. * there is a “corkscrew” of the duodenum and jejunum (Fig 4), then the diagnosis of **malrotation with volvulus is made**.17 Identification of a “corkscrew” requires notification of the on-call surgeon immediately as this patient requires urgent surgery.17 * If there is no corkscrew, but the duodenojejunal (DJ) flexure is malpositioned (inferior to the first part of the duodenum or to the right of the spine) then **malrotation without volvulus is diagnosed2 (Fig 5).** * An incomplete narrowing of the duodenum with a “windsock” expansion of the duodenum indicates a **duodenal web**18 (Fig 6). * Hypertrophic pylorus (usually slighlty older infants) Questions to ask self when looking at AXR 1. Is there distal gas? no - complete, yes - incomplete (duodenal web/stenosis/recent malrotation with volvulus) 2. single, double or tripple bubble?

Answer 11

* GM is a fetal structure that is a stem source for NEUROBLASTS. * Tupically involutes by term but is still present in prem infants within the Caudothalamic groove. * Highly vascular and subject to hemorrhage with fluctuation in cerebra blood pressure. * Hx most commonly occues in premature infants during the first days after birth. * Complications include * destruction of the precursor cells within the geminal matrix * hydrocephalus * hemorrhagic inffection of the surrounding periventricular tissues

Answer 12

There is an expansile lesion in the proximal humerus at the metadiaphysis. No pathologic fracture is seen. There are no findings of osteoid or chondroid matrix. The most likely diagnosis is an aneurysmal bone cyst, which is typically found in patients younger than 20 years of age in the long bones at the metadiaphysis. As in this case, radiographs will demonstrate a sharply defined expansile lesion with sclerotic margins. This MRI demonstrates a T1-bright, multicystic structure in the proximal humerus with multiple fluid-fluid levels and no enhancing soft-tissue components, confirming the diagnosis of an aneurysmal bone cyst. cHONDROBLASTOM, gIANT CELL TUMOUR, OSTEO BLASTOMA AND FIBROUIS DYSPLASIA can be seen with aneurysmal bone cysts except intraosseous lipomas. Intraosseous lipoma does not have ABC formation

Answer 13

* May be PARTIAL or COMPLETE * May be PRIMARY AGENESIS or SECONDARY DYSGENESIS * Epidemiology * 1:20000 * M:F 2:1. * maternal alcohol consumption during pregnancy has been recognised as a risk factor * Associations * Aneuploidys * T18, T13, T8 * Non-aneuploidy syndromes * Aicardi * Apert * Bickers adams Edwards * Coffin Siris * Fetal alcholo * Fryns * Gorlin * Hydrolethalus * Lowe * Zellweger * Other CNS associations: * Chiari II * Dandy walker * Grey matter heterotopia * Holoprosencephaly * Hydrocephalus * Interhemispheric cysts * Intracranial lipoma * Polymicrogyria * Proencephaly * Schizencephaly. * Inborn errors of metabolism * non-ketotic hyperglycemia * pyruvate metabolism disorders. * Congeitial lactic acidosis * mucopolysaccharidoses * Muolipidoses * Pathology: * insult occuring at approximately 8-12 weeks gestation. The white matter tracts which usually cross the midline, instead are orientated vertically, separating the lateral ventricles widely in a racing car sign configuration. These bundles of white matter PROBST BUNDLES

Answer 14

Nonfunctional pulmonary tissue (nearly always posteromedial segments of the lower lobes). Systemic arterial supply; anomalous arteries from the aorta (or celiac artery). No connection to bronchial tree.

Answer 15

1. Toxoplasmosis, 2. Rubella, 3. Cytomegalovirus, 4. Herpes simplex virus

Answer 16

classical metaphyseal lesions

Answer 17

* Foregut Duplication Cysts * Nodes

Answer 18

* Gastric atresia * single bubble * duodenal atresia * Double bubble * UGIS is performed in those patients with a double bubble and distal gas as these findings will inform the diagnosis. * duodenal stenosis (including annular pancreas, web, jejunal atresia) * Jenunal atresisa * tripple bubble * malrotation with volvulus * The presence of distal gas, alongside a history of bilious vomiting, is very important as it may represent intestinal malrotation with volvulus, which constitutes an emergency, requiring confirmation through an urgent UGIS (or other appropriate diagnostic test). * UGIS is performed in those patients with a double bubble and distal gas as these findings will inform the diagnosis. * there is a “corkscrew” of the duodenum and jejunum (Fig 4), then the diagnosis of **malrotation with volvulus is made**.17 Identification of a “corkscrew” requires notification of the on-call surgeon immediately as this patient requires urgent surgery.17 * If there is no corkscrew, but the duodenojejunal (DJ) flexure is malpositioned (inferior to the first part of the duodenum or to the right of the spine) then **malrotation without volvulus is diagnosed2 (Fig 5).** * An incomplete narrowing of the duodenum with a “windsock” expansion of the duodenum indicates a **duodenal web**18 (Fig 6). * Hypertrophic pylorus (usually slighlty older infants) Questions to ask self when looking at AXR 1. Is there distal gas? no - complete, yes - incomplete (duodenal web/stenosis/recent malrotation with volvulus) 2. single, double or tripple bubble?

Answer 19

There is a polypoid mass in the bladder. The grape-like appearance is characteristic of the botryoid type of rhabdomyosarcoma. Rhabdomyosarcomas are the most common malignant bladder masses in the pediatric population. Paragangliomas of the bladder may have a similar appearance with a macrolobulated appearance and T2-hyperintense signal, but they are more often seen in patients ages 30 to 60. Hemangiomas are often seen in the setting of other vascular malformations and are similar in appearance to hemangiomas seen elsewhere. Cystitis glandularis may present as nodules on a background of diffuse bladder wall thickening. Bladder neurofibromas are rare and may demonstrate a target appearance on T2-weighted imaging, with central low signal and peripheral high signal.

Answer 20

* formed by the vertical cortex of the ilium and the triangular labral fibrocartilages (echogenic triangle). * The normal value is less than 77 degrees, but is only useful in assessing immature hips when combined with the alpha angle. * There is a great deal of variability in the beta angle (much more than the alpha angle), and as such, it is not universally used. * In some instances, the beta angle is necessary to determine the subtype of hip dysplasia (e.g. type Ia vs. Ib, or type IIc vs. type D - see the sonographic classification of developmental dysplasia of the hip (DDH)). The cut off beta angle depends on type 3.

Answer 21

* the kidney develops in 3 stages * **pronephros** (3rd week): tubules drain into an excretory duct that terminates in the cloaca * **Mesonephros** (4th week) serves as a precursor for * Male: vas deferens, seminal vesicles, ejaculatory duct * female: vestigial * **Metanephros**: 5th week: * ureteral bud develops from the mesonephric duct, the ureteral bud elongates, undergoes divisions and form real rubes while ascending along the posterior coelomic wall * at 12 weeks there are seven anterior and sever posterior renal lobes separated by a fibrous groove. * At 28 weeks the boundaries between renal lobes become indistinct * persistence of fibrous groove is evident after birth. *

Answer 22

Musculoskeletal System Common Pediatric Bone Tumors * Primary * EG * Ewing sarcoma * OSA * Bone cysts * Unicameral bone cyst (UBC): single cavity, fallen fragment sign * Aneurysmal bone cyst (ABC): eccentric * Secondary * Neuroblastoma metastases * Lymphoma * Leukemia

Answer 23

* INTRO: * Langerhance Cell Histiocytosis is a rare multisyustem disease with a wide and heterogenous clinical specturm and variable extent of involvement. * EPID: * Peak incidence of 1-3 years. * 5/1000000. * Male predilection. * CLINICAL PRESENTATION: * Any part of the body can be effected, therefore clinical presentation varries * can be rapidly progressive, or spontaneously regress. * Three types * Letterer-Siwe Disease * Disseminated multiorgan * Less the 1 year old * fulminant course with poor prognosis. * Hand schuller-christian disease * multiple lesions solitary organ or multi-organ involvement. * Eosinophilic Grnuloma * lesions confined to one organ system/solitary lesion * 70% bone * Best prognosis. * Easier Classification (non Eponymous) * multiple organ systems, multiple sites involved * Single organ system, multiple sites involved * single lesion. * PATHOLOGY * uncontrolled monoclonal proliferation of Langerhans cells (monocyte/macrophage lineage) * Should be considdered a malignancy. But behavious is variable. * Proliferation is accompanied by inflmmationand granuloma formation. * Electron microsopy may reveal Birbeck granules.

Answer 24

Thymic hyperplasia Tear to a Malignant lymphoma Cystic hygrometer Thyroid extension Thymomas are extremely rare

Answer 25

* Aniogmyolipomas AMLs are an abnormal collection of vessels smooth muscle and fat cells seen in 55 to 75% if TS patients * Classically AMLs can be distinguished from other enhancing renal leison by macroscopic fat. * Additional renal manifestations include renal cysts renal cell carcinoma, renal oncocytomas and renal failure.

Answer 26

Tumors With Fluid-Fluid Level 1. ABC 2. Telangiectatic OSA 3. Giant cell tumor 4. Single cysts with pathologic fracture

Answer 27

Cortical: "COAG" * Renal cortical necrosis * Oxalosis * Alport syndrome * (chronic) glomerulonephritis Medullary: * deposition of calcium salts in the medulla of the kidney. * Causes "HAM HOP" * **h**yperparathyroidism * renal tubular **A**cidosis * **M**edullary sponge kidney * **H**yper or hypo thyroidism * **O**xalosis * **p**apillary necrosis * Hypervitaminosis D. * Milk alkali syndrome * Sarcoid

Answer 28

Cortical: "COAG" * Renal cortical necrosis * Oxalosis * Alport syndrome * (chronic) glomerulonephritis Medullary: * deposition of calcium salts in the medulla of the kidney. * Causes "HAM HOP" * **h**yperparathyroidism * renal tubular **A**cidosis * **M**edullary sponge kidney * **H**yper or hypo thyroidism * **O**xalosis * **p**apillary necrosis * Hypervitaminosis D. * Milk alkali syndrome * Sarcoid

Answer 29

Widened Joint Space ( Fig. 11.98 ) Widened joint spaces in pediatric patients are most commonly seen in hip joint or shoulders; other joints have strong capsules. Causes of widened joint space include: * Joint effusion * Septic arthritis * Hemarthrosis (intraarticular fracture, hemophiliac) * Transient toxic synovitis (viral) * JRA * Synovial thickening without articular cartilage destruction * JRA * Hemophiliac arthropathy

Answer 30

* Idiopathic (50%) * Obstruction of airway with valve mechanism (50%) * Bronchial cartilage deficiency or immaturity * mucus * web * stenosis * Extrinisic compression * Progressive overdistention of one or more pulmonary lobes but usually not the entire lung. * 10% of patients have CHD (patent ductus arteriosus [PDA] and ventricular septal defect [VSD]).

Answer 31

* Asplenia * Bilat RMB * Midline liver

Answer 32

Multiple cystic pulmonary lesions of variable size. Air fluid levels in cysts. Vasriable thickness of cyst wall.

Answer 33

4Ts * Teratoma * Thymoma * Thyroid * Terrible Lymphoma Thymus -\> The great mimick Lobulated Contour

Answer 34

* * 1st step in workup is an AP and lateral xray to ix wheeze (when asthma thought unlikely). Xray is to exclude cause of upper airway obstruction, evaluate for other processes that can cause wheezing(ie cardiac disease) and to help categorize the abnormality as being more likely to be an intrinsic or an extrinsic airway process. * Look for * evidence of tracheal narrowing, * position of the aortic arch * asymmetric lung aeration * radiopaque foreign body * consolidation * Intrinsic: * Bronchial foreign body * Tracheomalacia * Intrinsic masses * if it is one of these - \> bronchoscopy * Extrinsic: * extrinsic mass * vascular ring * if it is one of these -\> Crossesctional imaging. *

Answer 35

perpendicular to the hilgenreiner line, intersecting the lateral most aspect of the acetabuylar roof. The upper femoral epiphysis should be seen in the inferomedial quadrant.

Answer 36

The SHORT Overview of Chiari Malformations * Chiari I = * downward displacement of cerebellar tonsils below foramen magnum (\>5 mm); * unrelated to Chiari II malformation * Chiari II = * abnormal neurulation leads to a small posterior fossa, * caudal displacement of brainstem and * herniation of tonsils and vermis through the foramen magnum; * myelomeningocele * Chiari III = * encephalocele and * Chiari II findings (rare) * https://www.drawittoknowit.com/course/pathology/neurological-pathologies/developmental-disorders/1418/hindbrain-malformations THE LONG Chiari 1 * Intermittent compression of the brainstem: * Nerve palsies * Atypical facial pain * Respiratory depression * Long tract signs * Associations * Syringohydromyelia, 50%; * weakness of hands, arms, loss of tendon reflexes * Hydrocephalus, 25% * Basilar invagination, 30% * Klippel-Feil anomaly: fusion of 2 or more cervical vertebrae, 10% * Atlantooccipital fusion, 5% * Imaging Features * Tonsillar herniation (ectopia is 3–5 mm, herniation is \>5 mm) is age dependent. * Syringohydromyelia * No brain anomalies CHIARI 2 * Most common in newborns * Associations * Myelomeningocele, 90% * Obstructive hydrocephalus, 90% * Dysgenesis of corpus callosum * Syringohydromyelia, 50% * Abnormal cortical gyration * Chiari II is not associated with Klippel-Feil anomaly or Chiari I. * Imaging Features ( Fig. 6.73 ) * Posterior fossa * Small posterior fossa * Cerebellar vermis herniated through foramen (verminal peg) * Upward herniation of cerebellum through widened incisure (towering cerebellum) * Cerebellum wraps around pons (heart shape). * Low, widened tentorium * Obliterated CPA cistern and cisterna magna * Nonvisualization or very small fourth ventricle * Supratentorium ( Fig. 6.74 ) * Hypoplastic or fenestrated falx causes interdigitation of gyri (gyral interlocking). * Small, crowded gyri (stenogyria), 50% * Hydrocephalus almost always present before shunting. * Bat wing configuration of frontal horns (caused by impressions by caudate nucleus), enlarged atria and occipital horns (colpocephaly) * Small, biconcave third ventricle (hourglass shape because of large massa intermedia) * Beaked tectum * Osseous abnormalities * Lückenschädel skull (present at birth, disappears later) * Scalloped clivus and petrous ridge (pressure effect) * Enlarged foramen magnum * Spinal cord * Myelomeningocele, 90% * Cervicomedullary kink at foramen magnum (pressure effect) * Syringohydromyelia and diastematomyelia

Answer 37

Choanal Atresia * Membranous or bony obstruction of the nasal passage in the newborn. * If bilateral, respiratory distress at birth as newborns are obligate nasal breathers. * Failure to pass small catheter through nasal cavity. CT: findings of enlarged vomer and medial bowing of the lateral walls of the nasal cavity are useful to distinguish bony atresia from membranous. Axial Ct widening of the vomer (long arrow), bowing of the posteromedial maxilla (short arrow), and narrowing of the choana anterior to the pterygoid. https://www.researchgate.net/figure/Axial-Ct-widening-of-the-vomer-long-arrow-bowing-of-the-posteromedial-maxilla-short\_fig7\_51505685

Answer 38

* Most common abdominal malignancy in the newborn; * rare tumor (incidence 1 : 30,000). * Arises in neural crest tissue (adrenal medulla, sympathetic neural system, Zuckerkandl). * Age: 2 years, better prognosis \<1 year * Clinical Findings * Elevated vanillymandelic acid (VMA) and homovanillic acid (HVA) * May cause paraneoplastic syndrome * Radiographic Features * Solid tumor * Hyperechoic by US * Calcification in 85% * Readily extends across midline * Frequently encases vessels * 65% are metastatic at initial presentation; * common extensions include: * Bone * Neural foramina (evaluate) * Lymph nodes * Liver, lung (uncommon) * Case courtesy of Rad\_doc, Radiopaedia.org, rID: 47939

Answer 39

* Results from abnormal budding of the tracheobronchial tree. * Cysts contain respiratory epithelium. * Location * - mediastinum (85% * Posterior\> * middle \> * anterior) * - lung (15%) * Case courtesy of Assoc Prof Frank Gaillard, Radiopaedia.org, rID: 6313

Answer 40

* Anorectal malformations * Hirschprungs * ileal atresia * meconium ileus * meconium plug * colonic atresia

Answer 41

Three types: - diffuse Hypoplasia (30%) - Focal ring like stenosis (50%) - Funnel like stenosis (20%)

Answer 42

* Asplenia * Bilat RMB * Midline liver

Answer 43

* Germ cell tumours * originate from reproductive cells, cancerous or noncancerous. Gonadal or extragonadal * Seminoma * most common testicular tumours and account forr 45% of all primary testicular tumours. Can occur in the anterior mediastinal * risk factors: * undescended testes 10-40x increased risk * previous tumour in other testes * Fhx * Testicular microlithiasis * HIV, Mumps, orchitis, trauma, organ transplan immunosuppression. * painless testicular mass. * 15% have elevated hcg * non-seminomatous * Tumour of totipotential cells * Embryonal carcinoma * Extra embryonic * Trophoblast * **Choriocarcinoma** * rare and carries worst prognosis * yolk sac * **yold sac carcinoma** * embryonic (**teratoma**) * Contains Ecto, Meso and endoderm * Mature * Immature * Teratoma with malignant transformation * Mixed GCT * is the most common type of NSGCT * 40 of all germ cell tumours * most common combo is teratoma and embryonal cell carcinoma. * Not actually GCT but similar * Epidermoid cyst (ectoderm only) * dermoid cyst (ecto and meso) * Fetus in fetu (fetiform teratoma) * Struma ovarii tumour * subtype of ovairan teratoma * entirly or predominantly of thyroid tissue with variable sized follicles with colloid material.

Answer 44

Thru the umbilicus, travel inferiorly thru the umbilical artery, then int hte anterior division of the internal iliac artery, into the common iliac artery and then into the aorta. Tip position should either be: 1/ high at the T6 - T10 level or in the 2/low position L3/L5. Intermediate positions are generally undersirable due to potential associated throboosies of major aortic branches between T10 to L3.

Answer 45

1. Bronchiolits - normal chest X-ray, overaeration is only diagnostic clue. Commonly caused by RSV 2. Bronchiolitis + Parahilar, peribronchial opacities (most common) dirty perihilar regions caused by peribronchial cuffing. HIlar adenopathy. 3. Bronchiolitis + atelectasis (common). Disordered pattern with atelectasis. Areas of hyperaeration. Parahilar and peribronchial opacities 4. Reticulonodular interstitial (rare). Interstitial pattern. 5. Hazy lungs. Rare. Diffuse increase in density.

Answer 46

* Pneumatosis Soap bubble/linear * PV gas * extra Luminal gas * distended

Answer 47

condroid neoplasm, primarily part of the growth plate which separates and continues growing independantly, without an associated epiphysis, usually away from the nearby joint. The medullary cavity is continuous with the parent bone and hyaline cartilage caps them. Osteochonromas can be congenital or occur as a result of previous tranuma to the growth plate, including previous irratiation. Lower limb 50%.Upper limb 10-20%. spine (posteiror elements of the spine)

Answer 48

* benign fibrous mass of the SCM * AKA: SCM pseudotumour of infancy * focal thickening of the SCM A/w * Increased Vascularity * Iso/hypoechoic ill defined * clinically toritcollosis * R\>L unilater * Rx with Phsyo/sontaneous regression

Answer 49

Post IC CC Pre and post central sulcus Term neonate. Hypoxia at birth

Answer 50

* INTRO: * Langerhance Cell Histiocytosis is a rare multisyustem disease with a wide and heterogenous clinical specturm and variable extent of involvement. * EPID: * Peak incidence of 1-3 years. * 5/1000000. * Male predilection. * CLINICAL PRESENTATION: * Any part of the body can be effected, therefore clinical presentation varries * can be rapidly progressive, or spontaneously regress. * Three types * Letterer-Siwe Disease * Disseminated multiorgan * Less the 1 year old * fulminant course with poor prognosis. * Hand schuller-christian disease * multiple lesions solitary organ or multi-organ involvement. * Eosinophilic Grnuloma * lesions confined to one organ system/solitary lesion * 70% bone * Best prognosis. * Easier Classification (non Eponymous) * multiple organ systems, multiple sites involved * Single organ system, multiple sites involved * single lesion. * PATHOLOGY * uncontrolled monoclonal proliferation of Langerhans cells (monocyte/macrophage lineage) * Should be considdered a malignancy. But behavious is variable. * Proliferation is accompanied by inflmmationand granuloma formation. * Electron microsopy may reveal Birbeck granules.

Answer 51

* Divided into cloacal (urorectal) septum into: * a dorsal portion (rectum) * a ventral portion (allantois) * atrophies as umbilical ligament, bladder, urogenital sinus (pelvic and phallic portions) * Wolffian and Mullerian ducts drain into the ventral portion of the cloaca

Answer 52

Well defined round mass, in sub cardinal/parabolas region. Pulmonary cysts commonly located in medial third of lung. Initially no communication with the tracheobronchial tree Cysts are thin walled Can be fluid or air filled.

Answer 53

* Coloboma * Heart defects * choanal Atresia/Cranial nerve defects (facial nerve palsy), Dysphagia. * Retardation of growth and development * Gential +/- urinary abnormalities * Ear abnormalities Dx: * Definate CHARGE: * 4 major characteristics * 3 major + 3 minors. * POSSIBLE CHARGE: * 1 or 2 major characteristics and several minor characteristics * https://radiopaedia.org/articles/charge-syndrome

Answer 54

mechanical effects of the lesion, frature or malignant transformation. Impingement, lump, bursal formation or bursitis.

Answer 55

Can be just generalised WM abnormal signal. More typically periventricular calc.

Answer 56

Max splenic size (cm) * 1/2 Pts age +6

Answer 57

Meconium Plug Sydrome * functional obst 2° retention of mech plugs neonates of m° w diabetes * Enema for dx & rx

Answer 58

- Fixed narrowing at the level of the cricoid. - Failure of laryngeal recanalisation in utero

Answer 59

* Gliomas * astrocytomas * ependymomas * Medulloblastoma * Retinoblastoma * heritable form * Non-heritable (unilateral) * There is a lobulated mass within the right globe that is T1-hyperintense and heterogeneously T2-hypointense relative to vitreous fluid, and which displays restricted diffusion and contrast enhancement. There is retinal detachment with associated hemorrhage, resulting in a fluid/fluid level in the right globe seen on the T2-weighted images. Retinoblastoma is the most common intraocular childhood malignancy, with a median presentation of 12 months. It can be sporadic (approximately 60% of cases), which requires two spontaneous mutations of the retinoblastoma protein tumor suppressor gene (RB), or it can be familial, due to a single spontaneous mutation in combination with an inherited germline mutation. Retinoblastomas appear as mixed calcified and noncalcified soft-tissue intraocular masses. CT and ultrasound are good for depicting intralesional calcifications. On MRI, they will appear as a T1-hyperintense, T2-hypointense mass relative to vitreous fluid. Retinoblastomas will restrict on diffusion imaging given that they have a high cellular content as they fall within the class of small blue round cell tumors. The contralateral globe, pineal region, and suprasellar regions need to be carefully evaluated for the presence of additional sites of disease. Coats disease is characterized by progressive retinal detachment by exudative sub retinal collections and is not typically associated with enhancement, restricted diffusion or calcification. A coloboma is congenital malformation characterize by failure of closure of the choroidal fissure and is seen as a small globe with a posterior defect with vitreous herniation. Astrocytic hamartomas are benign retinal tumors associated with tuberous sclerosis. Retinopathy of prematurity is found in premature infants and seen as vascular proliferation with asymmetrically small globes. * DNET * dysembryoplastic neuroepithelial tumour

Answer 60

## Footnote CT demonstrates extensive periventricular calcifications. Periventricular calcifications are most characteristic of CMV, which is the most common TORCH infection. Polymicrogyria also may occur. Toxoplasmosis calcifications of the basal ganglia with hydrocephalus. Rubella demonstrates ischemia with high T2 signal on MRI. HSV may result in hemorrhagic infarct, with resulting encephalomalacia. HIV also results in atrophy, predominantly in a frontal distribution.

Answer 61

Minimally or undisplaced spiral fracture, usually of the tibia. Does not include spiral femoral fracutes, which should raise suspicion of NAI. Typically occur between 9months and 3 years, result of new stress placed on the bone dure to recent and increasing ambulation.